vitamin-b-12 and Spinal-Cord-Diseases

Vitamin B12 deficiency can lead to subacute combined degeneration (SCD). Nitrous oxide (N. A comprehensive search of multiple databases was conducted, and information about patient characteristics, symptomatology, clinical work-up, and treatment was extracted from eligible articles. Univariate analyses were performed to identify predictors of poor neurological recovery following SCD.. 32 studies, reporting 37 cases of nitrous oxide-induced SCD, were included through the screening process. These cases included 21 male patients and 16 female patients, with an average age of 50.4 years (SD 17.6). An etiology for subclinical B12 deficiency was determined in 30 reports; of these, 25 were due to vitamin malabsorption secondary to a gastrointestinal disorder. Duration of nitrous oxide exposure was described in 19 reports, and ranged from 30 min to 11 h. Univariate analysis failed to find an association between post-operative recovery and age (p = 0.60), sex (p = 0.46), positive MRI findings (p = 0.47), post-operative serum B12 (p = 1.00), post-operative hemoglobin (p = 0.18), type of surgery (p = 0.58), or post-operative high mean corpuscular volume (p = 0.13).. In patients with postsurgical myelopathy, surgeons should evaluate B12 status and consider the possibility that nitrous oxide could cause a subclinical B12 deficiency to become overt, particularly in patients with malabsorptive GI comorbidities. Treatment with B12 in this population can result in significant improvement of neurological function.

Topics: Adult; Female; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Nitrous Oxide; Spinal Cord; Spinal Cord Diseases; Subacute Combined Degeneration; Vitamin B 12; Vitamin B 12 Deficiency

Topics: Adalimumab; Aged; Antibodies, Monoclonal, Humanized; Arthritis, Rheumatoid; Cervical Vertebrae; Comorbidity; Female; Humans; Magnetic Resonance Imaging; Spinal Cord Diseases; Treatment Outcome; Tumor Necrosis Factor-alpha; Vitamin B 12; Vitamin B 12 Deficiency

Case reports with a comprehensive review of the current literature concerning subacute combined degeneration induced by nitrous oxide inhalation. A differential diagnosis should be considered when young patients present with progressive myelopathy because that the misuse of nitrous oxide has potentially serious outcomes.. Three young patients aged from 18 to 24, one male and two females, were diagnosed with progressive ascending numbness in four limbs or both legs and ataxia. They all had been inhaling nitrous oxide from whipped-cream containers for several months. A cervicothoracic magnetic resonance imaging scan revealed long segmental hyperintensity changes at the posterior column of the spinal cord. Serological examination showed a low level of vitamin B12. Subacute combined degeneration of the spinal cord was diagnosed and the etiology was considered related to nitrous oxide misuse. Their neurological status, neuroimage, and neurophysiologic condition improved after vitamin B12 supplementation and cessation of nitrous oxide inhalation.. Iatrogenic usage of nitrous oxide apparently resulted in subacute combined degeneration in our three patients. Recently, nitrous oxide misuse has increased among young people. Subacute combined degeneration of the spinal cord should be considered as a possible outcome of such abuse.

Topics: Adolescent; Female; Humans; Magnetic Resonance Imaging; Male; Nitrous Oxide; Spinal Cord Diseases; Subacute Combined Degeneration; Vitamin B 12; Young Adult

Subacute combined degeneration is an acquired myelopathy caused by vitamin B12 deficiency. Therapy with B12 leads to improvement in most but to complete recovery in only a few patients. Prognostic indicators in subacute combined degeneration are unknown; therefore, predicting complete recovery of neurologic deficits is challenging.. To identify potential correlates of outcome and to generate hypotheses concerning predictors of complete resolution of neurologic deficits in subacute combined degeneration.. We searched EMBASE (1974 to October 2005), MEDLINE (1968 to October 2005), and references from identified reports. REPORTS SELECTION: Reports of patients with subacute combined degeneration containing results of magnetic resonance imaging (MRI) and description of outcome and 1 patient treated by the authors.. We extracted data from 45 reports and 57 patients (36 males, 21 females; age range: 10 to 81) with a diagnosis of subacute combined degeneration, and estimated the strength of association between clinical, laboratory, and radiological factors and complete resolution of signs and symptoms.. Eight patients (14%) achieved clinical resolution and 49 (86%) improved with B12 therapy. The absence of sensory dermatomal deficit, Romberg, and Babinski signs were associated with a higher complete resolution rate. Patients with MRI lesions in < or = 7 segments and age less than 50 also appear to have higher rates of complete resolution.. B12 therapy is reported to stop progression and improve neurologic deficits in most patients with subacute combined degeneration. However, complete resolution only occurs in a small percentage of patients and appears to be associated with factors suggestive of less severe disease at the time of diagnosis.

Topics: Adolescent; Adult; Aged; Aged, 80 and over; Child; Confidence Intervals; Female; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Radiography; Retrospective Studies; Spinal Cord Diseases; Treatment Outcome; Vitamin B 12; Vitamin B 12 Deficiency

Four patients, three women aged 49, 47 and 74 years, and a man aged 64 years, presented with progressive sensory deficit, pyramidal tract symptoms and postural instability. Tests revealed megaloblastic anaemia and low vitamin B12 levels. Two of the female patients had undergone gynaecological surgery with nitrous oxide anaesthesia, and the male patient had undergone a gastric resection. Subacute combined degeneration of the spinal cord is a neurological disease based on vitamin B12 deficiency. It involves the posterior and lateral columns of the spinal cord, and sometimes the peripheral nerves, the optic nerve or the brain. An MRI scan of the cervical cord revealed abnormalities for three of the four patients. Following parenteral supplementation of vitamin B12, the symptoms and the MRI abnormalities either disappeared or significantly improved. Vitamin B12 deficiency can cause subacute combined degeneration of the cord by interfering with myelin synthesis. As vitamin B12 deficiency is caused by malabsorption in the gastrointestinal tract, oral supplementation is insufficient. It is essential to recognise this treatable disease at an early stage, and not to reject the possible diagnosis if the MRI findings are abnormal. Simple blood tests can lead to the diagnosis and to effective treatment.

Topics: Aged; Anemia, Megaloblastic; Anesthetics; Cervical Vertebrae; Female; Humans; Injections, Intramuscular; Magnetic Resonance Imaging; Malabsorption Syndromes; Male; Middle Aged; Myelin Sheath; Nitrous Oxide; Spinal Cord; Spinal Cord Diseases; Treatment Outcome; Vitamin B 12; Vitamin B 12 Deficiency

Combined medullary sclerosis developed suddenly postoperatively in a patient with unknown Biermer's disease. The neurological lesions were undoubtedly induced by nitrogen protoxide via an inactivation of vitamin B12.

Topics: 5-Methyltetrahydrofolate-Homocysteine S-Methyltransferase; Abscess; Aged; Anemia, Pernicious; Anesthetics, Inhalation; Arthroplasty, Replacement, Hip; Atrophy; Autoantibodies; Autoimmune Diseases; Demyelinating Diseases; Female; Gastric Mucosa; Humans; Intestinal Absorption; Intrinsic Factor; Nitrous Oxide; Oxidation-Reduction; Paresthesia; Postoperative Complications; Proprioception; S-Adenosylmethionine; Sclerosis; Spinal Cord; Spinal Cord Diseases; Surgical Wound Infection; Vitamin B 12

Topics: Humans; Injections, Intramuscular; Peripheral Nervous System Diseases; Prognosis; Spinal Cord Diseases; Vitamin B 12; Vitamin B 12 Deficiency

Certain aspects of the clinical syndrome of dementia, cerebral atrophy, predominantly sensory neuropathy, and vacuolar myelopathy in AIDS resemble those seen in vitamin B12 deficiency. Pathologically, there are similarities not only in the changes in the spinal cord, but also in the brain and peripheral nerves. The pathogenesis of vacuolar myelopathy may be secondary to a combination of immune mediated myelin and oligodendrocyte injury, and simultaneous impairment of repair mechanisms due to a deficiency of S-adenosylmethionine (SAM). Products derived from macrophages may interfere directly with the methyl transfer cycle through the generation of reactive oxygen intermediates and reactions involving nitric oxide and peroxynitrite which may limit the supply of methionine for conversion to SAM, both by direct interaction as well as through inhibition of methionine synthase. Macrophage activation with secretion of cytokines and other biologically reactive substances within the nervous system is sustained in the late stages of HIV infection by the general effects of immune depletion, including loss of T cells (with concomitant reduction of macrophage regulatory molecules) and recurrent opportunistic infections, and may be further augmented by the local presence of the virus itself (or its surface glycoprotein gp120). This would account for the common, but not exclusive, occurrence of vacuolar myelopathy in AIDS. The ability of the virus and its products to stimulate macrophage and microglial activation may also explain the association between severity of vacuolar myelopathy and the presence of HIV encephalitis. A similar mechanism may underlie the pathogenesis of dementia, cerebral atrophy, and peripheral neuropathy. Local factors or differential susceptibility between the central and peripheral nervous system may determine whether myelinotoxic or neurotoxic processes predominate; the prominence of myelin involvement in the spinal cord, and axonal involvement peripherally may reflect both ends of this range, with the brain manifesting a more equal balance of both processes.

Topics: Acquired Immunodeficiency Syndrome; AIDS Dementia Complex; Cytokines; Demyelinating Diseases; Folic Acid; Glutathione; Humans; Macrophage Activation; Oligodendroglia; Peripheral Nervous System Diseases; S-Adenosylmethionine; Spinal Cord Diseases; Vacuoles; Vitamin B 12

Topics: Humans; Prevalence; Spinal Cord Diseases; Vitamin B 12; Vitamin B 12 Deficiency

Vitamin B12 (cyanocobalamin) is an integral component of two biochemical reactions in man: the conversion of L-methylmalonyl coenzyme A into succinyl coenzyme A and the formation of methionine by methylation of homocysteine. The transmethylation reaction is essential to DNA synthesis and to the maintenance of the myelin sheath by the methylation of myelin basic protein. Active vitamin B12 contains cobalt in its reduced form (Co+). Nitrous oxide produces irreversible oxidation to the Co++ and Co forms that renders vitamin B12 inactive. Five cases (four from the literature and one new case) are presented in which patients unsuspected of having vitamin B12 deficiency developed subacute combined degeneration of the spinal cord following nitrous oxide anesthesia. Patients with vitamin B12 deficiency are exceedingly sensitive to neurologic deterioration following nitrous oxide anesthesia. If unrecognized, the neurologic deterioration becomes irreversible and may result in death.

Topics: Anesthesia; Drug Interactions; Gait; Humans; Male; Middle Aged; Nitrous Oxide; Spinal Cord Diseases; Vitamin B 12; Vitamin B 12 Deficiency

In humans, subacute combined degeneration of the spinal cord and brain, a primary demyelinating disease, is caused by cobalamin or methyltetrahydrofolate deficiency. Experimental studies into its pathogenesis suggest that dysfunction of the methyl-transfer pathway may be the cause. Compelling evidence for this comes from the study of inborn errors of cobalamin metabolism where deficiency of methylcobalamin, but not deoxyadenosylcobalamin, is associated with demyelination. Recent studies have focused upon inborn errors of the methyl-transfer pathway. Cerebrospinal fluid concentrations of metabolites of the methyl-transfer pathway have been measured in humans with sequential errors of the pathway and correlated with demyelination demonstrated on magnetic resonance imaging of the brain. This has provided new data suggesting that deficiency of S-adenosylmethionine is critical to the development of demyelination in cobalamin deficiency.

Topics: Brain Diseases; Demyelinating Diseases; Humans; Metabolism, Inborn Errors; Methylation; Nerve Degeneration; Spinal Cord Diseases; Vitamin B 12; Vitamin B 12 Deficiency

Topics: Anemia, Pernicious; Animal Nutritional Physiological Phenomena; Animals; Ataxia; Behavior, Animal; Central Nervous System Diseases; Chiroptera; Demyelinating Diseases; Disease Models, Animal; Flight, Animal; Humans; Species Specificity; Spinal Cord Diseases; Vitamin B 12; Vitamin B 12 Deficiency

Topics: Aged; Anemia, Pernicious; Animals; Female; Folic Acid; Humans; Male; Middle Aged; Nervous System Diseases; Neurologic Manifestations; Rats; Spinal Cord Diseases; Time Factors; Vitamin B 12; Vitamin B 12 Deficiency

Topics: Alcoholism; Anticonvulsants; Cyanides; Deficiency Diseases; Electroencephalography; Erythrocytes; Folic Acid Deficiency; Humans; Isoniazid; Nervous System Diseases; Neurocognitive Disorders; Pellagra; Polyneuropathies; Schilling Test; Spinal Cord Diseases; Thiamine Deficiency; Transketolase; Tryptophan; Vitamin B 12; Vitamin B 12 Deficiency; Vitamin B 6 Deficiency; Vitamin B Deficiency; Wernicke Encephalopathy

Topics: Humans; Magnetic Resonance Imaging; Spinal Cord; Spinal Cord Diseases; Subacute Combined Degeneration; Vitamin B 12; Vitamin B 12 Deficiency

Topics: Humans; Magnetic Resonance Imaging; Spinal Cord; Spinal Cord Diseases; Subacute Combined Degeneration; Vitamin B 12; Vitamin B 12 Deficiency

Cobalamin C (CblC) deficiency is a rare inborn error in cobalamin (vitamin B12) metabolism which results in impaired intracellular processing of dietary vitamin B12. This leads to a wide range of clinical manifestations including cognitive impairment, psychiatric symptoms, myelopathy, thrombotic events, glomerulonephritis, and pulmonary arterial hypertension. CblC deficiency typically presents in the pediatric population but can also present in adulthood. Diagnosis in adults can be challenging due to the rarity of this condition and its myriad clinical presentations. CblC deficiency is treatable, so early diagnosis is important in preventing permanent neurologic damage. Although CblC deficiency results from a defect in vitamin B12 metabolism, B12 levels remain normal. Diagnosis depends on testing metabolites altered by vitamin B12 dysfunction such as methylmalonic acid (MMA) and homocysteine. We presented a case of a 20-year-old woman who presented with chronic progressive lower extremity weakness and sensory changes. She was eventually diagnosed with subacute combined degeneration because of CblC deficiency and effectively treated. This case highlights the importance of considering inborn errors of metabolism in adult patients and including testing of metabolites such as MMA and homocysteine when suspecting vitamin B12 dysfunction.

Topics: Adult; Amino Acid Metabolism, Inborn Errors; Child; Female; Humans; Methylmalonic Acid; Spinal Cord Diseases; Subacute Combined Degeneration; Vitamin B 12; Vitamin B 12 Deficiency; Young Adult

Nitrous oxide (N

Topics: Humans; Magnetic Resonance Imaging; Nitrous Oxide; Spinal Cord Diseases; Spinal Cord Injuries; Vitamin B 12

We present a case of a 23-year-old woman with a history of celiac disease who presented with a 2-month history of progressive gait unsteadiness and falls. Neurologic examination exhibited preserved motor strength, diffuse areflexia, and ataxic gait. Autoimmune and infectious workups were unremarkable, including vitamin B12. Electrodiagnostic testing showed absent diffuse sensory responses, consistent with sensory ganglionopathy. Total spine magnetic resonance imaging (MRI) revealed a non-enhancing, posterior cord, hyperintense signal from C1-T11. Partial improvement in her sensory ataxia was noted after 6 months of high-dose steroids without dorsal cord signals change on repeat MRI that suggests Wallerian degeneration of sensory axons.

Topics: Adult; Female; Gait Ataxia; Humans; Magnetic Resonance Imaging; Spinal Cord; Spinal Cord Diseases; Vitamin B 12; Young Adult

Topics: Anesthetics, Inhalation; Humans; Magnetic Resonance Imaging; Nitrous Oxide; Spinal Cord Diseases; Subacute Combined Degeneration; Vitamin B 12; Vitamin B 12 Deficiency

Topics: Humans; Nitrous Oxide; Spinal Cord Diseases; Vitamin B 12; Vitamin B 12 Deficiency; Vitamins

Nitrous oxide, also known as 'laughing gas', is one of the most widely used recreational drugs among teenagers in the UK. Copious inhalation of nitrous oxide may increase intra-alveolar pressure, resulting in barotrauma secondary to alveolar rupture. Pneumomediastinum and subcutaneous emphysema are common clinical findings in nitrous oxide-associated barotrauma. Prolonged nitrous oxide misuse may inactivate vitamin B

Topics: Adolescent; Humans; Nitrous Oxide; Spinal Cord Diseases; Subacute Combined Degeneration; Vitamin B 12; Vitamin B 12 Deficiency

Topics: Humans; Mental Disorders; Nitrous Oxide; Peripheral Nervous System Diseases; Research; Spinal Cord Diseases; Vitamin B 12

A 42-year-old man from rural India presented with asymmetric progressive paraparesis mimicking compressive dorsal myelopathy, followed by distal upper limb, truncal and neck-flexor weakness, further complicated by acute urinary retention. His sensory deficits were marked by loss of joint position sense (JPS) and graded loss of vibration sense, along with a definite sensory level. Deep tendon jerks were hypo-to-areflexic, plantar was bilaterally extensor. He had become less attentive and occasionally failed to keep track with conversations. A syndromic diagnosis of myeloradiculoneuropathy with cognitive impairments was made. Further tailored investigations revealed vitamin B

Topics: Adult; Cognitive Dysfunction; Electrodiagnosis; Electromyography; Humans; Injections, Subcutaneous; Magnetic Resonance Imaging; Male; Neural Conduction; Polyradiculoneuropathy; Quadriplegia; Spinal Cord; Spinal Cord Diseases; Subacute Combined Degeneration; Treatment Outcome; Urinary Retention; Vitamin B 12; Vitamin B 12 Deficiency; Vitamin B Complex

Recreational nitrous oxide (N

Topics: Humans; Magnetic Resonance Imaging; Male; Nitrous Oxide; Oxides; Spinal Cord Diseases; Subacute Combined Degeneration; Vitamin B 12; Vitamin B 12 Deficiency; Young Adult

Topics: Adult; Antimetabolites, Antineoplastic; Bone Marrow Transplantation; Female; Humans; Injections, Spinal; Leucovorin; Leukemia, Myeloid, Acute; Magnetic Resonance Imaging; Methotrexate; Peripheral Blood Stem Cell Transplantation; Prednisone; Spinal Cord; Spinal Cord Diseases; Vitamin B 12

Topics: Aged; Humans; Male; Spinal Cord Diseases; Vitamin B 12; Vitamin B 12 Deficiency; Vitamin B Complex

Recreational use of nitrous oxide as a 'legal high' is increasing in the UK. Physicians should be 'street wise' to this increasing prevalence and aware of the potential neurological complications which may result from misuse. We describe a 17-year-old male patient who presented to neurology with a severe myelopathy following prolonged recreational use of nitrous oxide. MRI demonstrated characteristic changes affecting the dorsal columns and blood tests demonstrated a 'functional' B

Topics: Adolescent; Humans; Illicit Drugs; Male; Nitrous Oxide; Spinal Cord Diseases; Treatment Outcome; United Kingdom; Vitamin B 12; Vitamin B 12 Deficiency

Topics: Female; Humans; Spinal Cord Diseases; Vitamin B 12; Vitamin B 12 Deficiency

Whilst the dangers of 'legal highs' have been widely publicised in the media, very few cases of the neurological syndrome associated with the inhalation of nitrous oxide (N. Case series documenting the clinical and investigational features of ten consecutive cases of subacute degeneration of the spinal cord presenting to a hospital with a tertiary neurosciences service in East London.. Sensory disturbance in the lower (± upper) limbs was the commonest presenting feature, along with gait abnormalities and sensory ataxia. MRI imaging of the spine showed the characteristic features of dorsal column hyperintensity on T. A high index of suspicion is required to prompt appropriate investigation, make the diagnosis and commence treatment early. This is the largest reported series of patients with subacute degeneration of the spinal cord induced by recreational use of N

Topics: Adolescent; Adult; Ataxia; Diagnosis, Differential; Female; Humans; Magnetic Resonance Imaging; Male; Neurodegenerative Diseases; Nitrous Oxide; Retrospective Studies; Spinal Cord; Spinal Cord Diseases; Substance-Related Disorders; Vitamin B 12; Young Adult

Nitrous oxide, laughing gas, is used as a party drug to achieve a euphoric effect. It has been gaining popularity in recent years and is considered a relatively innocent substance. Nitrous oxide is known to cause subacute degeneration of the spinal cord by inactivation of active vitamin B12. Vitamin B12 plays an essential role in the synthesis of myelin. Hence, vitamin B12 deficiency can lead to degeneration of the dorsal and lateral columns of the spinal cord. Polyneuropathy is a less known complication. We present a 17-year-old woman and a 19-year-old man with subacute axonal polyneuropathy caused by laughing gas abuse. Abstinence of laughing gas and treatment with intramuscular and oral vitamin B12 suppletion respectively have led to improvement of their symptoms. Our cases demonstrate a less-known but treatable complication of laughing gas.

Topics: Adolescent; Female; Humans; Male; Nitrous Oxide; Polyneuropathies; Spinal Cord Diseases; Vitamin B 12; Vitamin B 12 Deficiency; Young Adult

Severe methylenetetrahydrofolate reductase (MTHFR) deficiency is a rare inborn defect disturbing the remethylation of homocysteine to methionine (<200 reported cases). This retrospective study evaluates clinical, biochemical genetic and in vitro enzymatic data in a cohort of 33 patients.. Clinical, biochemical and treatment data was obtained from physicians by using a questionnaire. MTHFR activity was measured in primary fibroblasts; genomic DNA was extracted from cultured fibroblasts.. Thirty-three patients (mean age at follow-up 11.4 years; four deceased; median age at first presentation 5 weeks; 17 females) were included. Patients with very low (<1.5%) mean control values of enzyme activity (n = 14) presented earlier and with a pattern of feeding problems, encephalopathy, muscular hypotonia, neurocognitive impairment, apnoea, hydrocephalus, microcephaly and epilepsy. Patients with higher (>1.7-34.8%) residual enzyme activity had mainly psychiatric symptoms, mental retardation, myelopathy, ataxia and spasticity. Treatment with various combinations of betaine, methionine, folate and cobalamin improved the biochemical and clinical phenotype. During the disease course, patients with very low enzyme activity showed a progression of feeding problems, neurological symptoms, mental retardation, and psychiatric disease while in patients with higher residual enzyme activity, myelopathy, ataxia and spasticity increased. All other symptoms remained stable or improved in both groups upon treatment as did brain imaging in some cases. No clear genotype-phenotype correlation was obvious.. MTHFR deficiency is a severe disease primarily affecting the central nervous system. Age at presentation and clinical pattern are correlated with residual enzyme activity. Treatment alleviates biochemical abnormalities and clinical symptoms partially.

Topics: Ataxia; Betaine; Child; Female; Folic Acid; Genetic Association Studies; Homocystinuria; Humans; Intellectual Disability; Male; Methionine; Methylenetetrahydrofolate Reductase (NADPH2); Muscle Spasticity; Mutation; Phenotype; Psychotic Disorders; Retrospective Studies; Spinal Cord Diseases; Vitamin B 12

Subacute combined degeneration of the spinal cord (SCD) is often found in vitamin B-12 deficiency and typically shows hyperintensity on T2-weighted images of the lateral and posterior columns. The purpose of the study was to evaluate the use of conventional magnetic resonance examination in diagnosing SCD. Thirty-six patients were clinically confirmed and retrospectively analyzed; conventional spine MRIs were available for all patients and eight of them had contrast enhancement MRIs. 19 out of 36 patients showed abnormal signal intensity on T2 weighted images with a sensitivity of 52.8%, among which 18 in the posterior aspect of the spinal cord and 1 in the anterior horn of the thoracic spinal cord The spinal cord abnormalities were seen at the cervical spine in 12 patients (33.3%) and at the thoracic spine in the other 7 patients (19.4%). Axial T2-weighted images showed symmetric linear T2-hyperintensity as an "inverted V" at the cervical spinal cord in 5 patients, which has been reported as a typical sign of SCD. For patients with thoracic spinal cord abnormalities, the bilateral paired nodular T2-hyperintensity looked like "binoculars" at the thoracic spinal cord. Only one out of the eight patients showed slight enhancement after injection with contrast agent. All the 36 patients reported clinical improvement after appropriate vitamin B-12 treatment. The two follow-up spine MRIs showed a decreased extent of the lesion. Therefore, conventional MRI is useful in the diagnosis and management of SCD caused by vitamin B-12 deficiency.. 脊髓亚急性联合变性（SCD）常发生于维生素B-12 缺乏的患者，T2 加权像上通 常显示为脊髓后索及侧索的异常高信号。本文研究目的在于评估常规磁共振检 查对诊断SCD 的价值。回顾性分析经临床确诊的SCD 患者36 例，所有患者均 行脊柱MRI 扫描，其中8 例行增强MRI 扫描。36 例患者中19 例表现为T2 加 权图像上异常信号，灵敏度为52.8％，其中18 例病变位于脊髓后份，1 例病变 发生于胸髓前份。所有MRI 表现异常的患者中，12 例异常信号位于颈髓 （33.3%），7 例病变位于胸髓（19.4%）。颈髓出现异常信号者5 例，在轴位 T2 加权像上呈对称线性高信号，即“倒V”字征，是SCD 的典型影像学表现。胸 段脊髓异常信号在T2 加权像上呈对称性“望远镜”样高信号。8 例增强MRI 检查 的患者仅有1 例病变表现出轻度强化。所有36 例患者在适当补充维生素B-12 治疗后临床症状得到改善。2 例MRI 随访见异常信号范围较前缩小。因此，传 统的MRI 检查对诊断及评估维生素B-12 缺乏引起的亚急性脊髓联合变性具有 价值。.

Topics: Adult; Aged; Female; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Retrospective Studies; Spinal Cord; Spinal Cord Diseases; Subacute Combined Degeneration; Vitamin B 12; Vitamin B 12 Deficiency

Nitrous oxide is commonly used as an analgesic and anaesthetic agent. Nitrous oxide is also in use in industry as an aerosol propellant and is now recognised as a recreational drug whose use is growing, especially among the young. Nitrous oxide from whipped cream canisters is inhaled to produce a dissociative, intoxicated state. Nitrous oxide is known to inactivate vitamin B12 via oxidation, which can precipitate a demyelinating myelopathy akin to the classical B12 deficiency syndrome, subacute combined degeneration of the spinal cord. This case describes a young woman with chronic pain and a poor nutritional state who took regular nitrous oxide as an opiate-sparing agent. She developed a progressive subacute myelopathy with a sensory level, profoundly impaired joint position sense, extensor plantars and required a wheelchair. Once diagnosed, she responded well to a regime of nitrous oxide withdrawal, high-dose B12 replacement and physiotherapy. The case illustrates the need for clinical teams to be able to dentify a nitrous oxide-precipitated myelopathy as its use as a drug of abuse increases; particularly in the case of malnourished patients who receive nitrous oxide surgically or obstetrically.

Topics: Accidental Falls; Adult; Analgesics, Non-Narcotic; Anesthetics, Inhalation; Chronic Pain; Demyelinating Diseases; Female; Humans; Nitrous Oxide; Pain Management; Spinal Cord Diseases; Vitamin B 12; Vitamin B 12 Deficiency

Topics: Ataxia; Copper; Female; Humans; Male; Spinal Cord Diseases; Vitamin B 12; Zinc

A case of subacute combined degeneration (SCD) of the spinal cord manifesting as severe ataxia and urinary retention in a patient with a history of heavy nitrous oxide abuse and self-supplementation with cyanocobalamin is reported.. A 27-year-old woman was treated in the emergency department for complaints of abdominal pain and inability to urinate for about 12 hours. The patient also complained of worsening lower-extremity weakness for 10 days and a "pins and needles" sensation in the lower extremities for approximately 1 year. She reported nitrous oxide abuse over 3 years (an average of 100-200 "whippit" cartridges daily on 3 or 4 days per week), as well as long-term self-medication with oral and i.m. cyanocobalamin for the purpose of preventing nitrous oxide-induced neurologic symptoms. Results of magnetic resonance imaging (MRI) were highly suggestive of SCD, which is typically seen in primary vitamin B12 deficiency but has been reported in the context of chronic nitrous oxide exposure. Treatment was initiated with cyanocobalamin 1000 μg i.m. daily, to be continued for 5 days and followed by a four-week regimen of 1000 μg i.m. weekly. The patient was discharged after 3 days, despite continued symptoms, with instructions to obtain ongoing care but was lost to follow-up.. A patient who abused nitrous oxide chronically developed ataxia, paresthesia, and urinary retention while self-medicating with cyanocobalamin. A diagnosis of SCD was supported by MRI findings, symptoms, and the known relationship between nitrous oxide exposure and vitamin B12 deficiency.

Topics: Adult; Female; Humans; Magnetic Resonance Imaging; Nitrous Oxide; Self Medication; Spinal Cord Diseases; Subacute Combined Degeneration; Substance-Related Disorders; Vitamin B 12; Vitamin B 12 Deficiency

Herein, we report a case of a 20-year-old (ethnicity not reported) woman with a history of nitrous oxide abuse and clinical symptoms consistent with spinal cord subacute combined degeneration with associated low serum concentrations of vitamin B12, elevated methylmalonic acid levels, and radiologic evidence of demyelination of the dorsal region of the spinal column. The health of the patient improved dramatically with B12 supplementation. In this case, we discuss the interaction of nitrous oxide with the enzymatic pathways involved in the biochemistry of vitamin B12.

Topics: Adult; Demyelinating Diseases; Female; Humans; Methylmalonic Acid; Nitrous Oxide; Paresthesia; Spinal Cord Diseases; Substance-Related Disorders; Vitamin B 12; Vitamin B 12 Deficiency; Young Adult

Vitamin B12 deficiency is a well recognized cause of posterolateral myelopathy. In Indian subcontinent, it may coexist with nutritional copper deficiency producing partial response of patients to B12 supplementation. Hence the study was planned to look for association of hypocupremia and B12 deficiency.. Twenty-three patients with posterolateral myelopathy (Romberg sign positive) were enrolled and investigated for levels of vitamin B12, copper and zinc and followed up for six months.. In three patients, copper deficiency alone was found to be the cause. In another three, both copper and vitamin B12 were deficient. In all these six patients, ceruloplasmin and 24h urinary copper were found to be low suggesting dietary copper deficiency. Hyperzincemia was found in four of these patients. Magnetic resonance imaging of spine was normal in lone Cu deficient patients but showed T2 hyperintensity of posterior column in lone B12 or combined B12 and copper deficiency.. In cases of B12 deficiency myelopathy not responding to supplementation, copper deficiency must be sought at the earliest to avoid and treat persistent neurological disability.

Topics: Action Potentials; Adult; Analysis of Variance; Ataxia; Brain; Copper; Disability Evaluation; Female; Follow-Up Studies; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Spinal Cord; Spinal Cord Diseases; Statistics, Nonparametric; Vitamin B 12; Vitamin B 12 Deficiency; Zinc

Vitamin B12 deficiency has a wide spectrum of clinical presentation with a variety of neurological symptoms and signs. As a result, many patients lack classic features of advanced severe deficiency. Early diagnosis and treatment are crucial in order to prevent the irreversible damage to the nervous system. We describe a 25-year-old man, who presented with predominant sensory symptoms without any signs on clinical evaluation. His serum vitamin B12 levels were low and neuroimaging revealed myelopathy. The patient was treated promptly with cyanocobalamin injections, which lead to a rapid resolution of symptoms and radiological abnormalities.

Topics: Adult; Diagnosis, Differential; Humans; Magnetic Resonance Imaging; Spinal Cord Diseases; Treatment Outcome; Vitamin B 12; Vitamin B 12 Deficiency

A 19-year-old man presented with a 1-month history of progressive 4-limb numbness and gait imbalance. Physical examination revealed mild general muscular weakness, areflexia, and wide-based, ataxic, steppage gait. Sensory tests showed diminished superficial sensation below the level of the cervical-thoracic junction and a glove-and-stocking pattern of sensory loss at the 4 extremities. An initial magnetic resonance imaging examination of the cervical spine revealed an increased bilateral signal from the posterior and anterior columns on T(2)-weighted images. Nerve conduction velocity and electromyographic tests revealed polyneuropathy. On further inquiry, the patient admitted to chronic recreational use of nitrous oxide. The final diagnosis was nitrous oxide-induced neurotoxicity. The patient was treated for 5 days with injections of 1000 μg/day vitamin B(12), followed by an additional 2-month treatment at a dose of 1000 μg/week. The numbness resolved after the first week, but there remained a mild sensory ataxic gait. The patient recovered fully after 2 months of treatment and nitrous oxide abstinence. We recommend an investigation of the patient's history of nitrous oxide exposure in cases where an individual presents to the emergency department or outpatient department with acute numbness characterized by megaloblastic red blood cells and symmetric neurologic deficits.

Topics: Emergency Service, Hospital; Humans; Inhalant Abuse; Male; Nitrous Oxide; Polyneuropathies; Spinal Cord Diseases; Vitamin B 12; Vitamin B Complex; Young Adult

Topics: Adult; Diet, Vegetarian; Evoked Potentials, Somatosensory; Female; Gait Disorders, Neurologic; Humans; Magnetic Resonance Imaging; Motor Neurons; Neurologic Examination; Sensory Receptor Cells; Spinal Cord; Spinal Cord Diseases; Subacute Combined Degeneration; Vitamin B 12; Vitamin B 12 Deficiency; Vitamins

We describe a 35 year-old man presenting with a four-week history of non-painful limb paraesthesias and unsteady gait causing falls. On examination he had an ataxic gait associated with dorsal column sensory loss. He had a medical history of a partial gastrectomy six years prior and anaemia. He had received monthly intramuscular hydroxycobalamin injections since the gastrectomy. Laboratory tests revealed normal vitamin B12 and holotranscobalamin levels, a reduced serum caeruloplasmin of 0.05 g/L (normal: 0.22-0.58 g/L), a copper-to-caeruloplasmin ratio of 1.9 μmol/L (11.0-22.0 μmol/L) and a reduced 24-hour urinary copper concentration of <0.30 μmol/L (0-0.3 μmol/L). Cerebrospinal fluid analysis, nerve conduction studies, electromyography and visual-evoked responses were unremarkable. MRI revealed abnormal hyperintense signal in the cervical dorsal columns. Hypocupric myelopathy was diagnosed and he was treated with daily oral elemental copper. Three months later, his walking and balance had improved although there was no change noted on MRI.

Topics: Adult; Gait Ataxia; Humans; Longitudinal Studies; Magnetic Resonance Imaging; Male; Sensation Disorders; Spinal Cord; Spinal Cord Diseases; Subacute Combined Degeneration; Vitamin B 12

Topics: Adult; Cervical Vertebrae; Diagnosis, Differential; Female; Follow-Up Studies; Humans; Magnetic Resonance Imaging; Spinal Cord Diseases; Vitamin B 12; Vitamin B 12 Deficiency; Vitamin B Complex

Vitamin B12 deficiency causes haematological, gastrointestinal and neurological diseases. Subacute combined degeneration (SCD) of the spinal cord is characterised by degeneration of the posterior and lateral columns. We report a case of SCD associated with nitrous oxide anaesthesia.

Topics: Afferent Pathways; Anesthetics, Inhalation; Causality; Disease Progression; Gait Ataxia; Humans; Iatrogenic Disease; Magnetic Resonance Imaging; Male; Middle Aged; Myelin Sheath; Nitrous Oxide; Paresthesia; Spinal Cord; Spinal Cord Diseases; Subacute Combined Degeneration; Vitamin B 12; Vitamin B 12 Deficiency; Wallerian Degeneration

Vitamin B12 deficiency is an important nutritional disorder causing neurological manifestations of myelopathy, neuropathy and dementia. Sub-acute combined degeneration (SCD) with involvement of the posterior columns in the cervical and thoracic cord is a common presentation of this disorder. In this case report, we describe a 43 year old woman with pernicious anemia and myelopathy with atypical clinical features. The patient presented with motor symptoms, a sensory level and bladder dysfunction. She had severe autonomic disturbances including an episode of unexplained bronchospasm, which has not been previously reported as a manifestation of vitamin B12 deficiency. We review the literature regarding these rarely reported features of vitamin B12 deficiency, and discuss aspects of management of this reversible condition. We emphasize the importance of awareness of autonomic disturbances in B12 deficient individuals.

Topics: Adult; Anemia, Pernicious; Autonomic Nervous System Diseases; Bronchi; Bronchial Spasm; Female; Humans; Leg; Magnetic Resonance Imaging; Muscle, Skeletal; Neural Pathways; Paraparesis; Sensation Disorders; Spinal Cord; Spinal Cord Diseases; Subacute Combined Degeneration; Sympathetic Nervous System; Treatment Outcome; Urinary Bladder, Neurogenic; Vitamin B 12; Vitamin B 12 Deficiency

A previously healthy 27-year-old woman developed a subacute myeloneuropathy after receiving nitrous oxide anesthesia for dental procedures. Neurologic evaluation revealed that she was vitamin B(12) deficient due to underlying pernicious anemia. Discontinuation of nitrous oxide and supplementation with vitamin B(12) resulted in dramatic clinical improvement, with near-complete normalization of her neurologic examination. This case and published reports reviewed here emphasize that favorable outcomes are possible following prompt recognition and treatment of vitamin B(12) deficiency.

Topics: Adult; Anemia, Pernicious; Anesthetics, Inhalation; Female; Humans; Leg; Magnetic Resonance Imaging; Nerve Fibers, Myelinated; Nitrous Oxide; Peripheral Nervous System Diseases; Spinal Cord; Spinal Cord Diseases; Treatment Outcome; Vitamin B 12; Vitamin B 12 Deficiency

Vacuolar myelopathy (VM) in leukemia is rare. We report a boy with leukemia who developed isolated central nervous system (CNS) relapse during reinduction therapy. 5 months after cranial radiotherapy, he gradually developed quadriparesis. Magnetic resonance imaging revealed an intramedullary lesion which extended through the cervical spine. Serum vitamin B12, folic acid, cerebrospinal fluid methyl malonic acid were normal. Viral screening by ELISA was negative. He had lymphopenia, and reduced immunoglobulins, from a cardiac arrest. Biopsy revealed VM. He responded to weekly vitamin B12 treatment but on the 6th week of the therapy he died after developing periventricular, gliotic, hyperintense lesions in the brain.

Topics: Central Nervous System Neoplasms; Child; Humans; Magnetic Resonance Imaging; Male; Precursor Cell Lymphoblastic Leukemia-Lymphoma; Recurrence; Spinal Cord Diseases; Vitamin B 12

A 26-year-old patient developed ascending weakness and paresthesias. Megaloblastic anemia and mildly reduced serum vitamin B12 (B12) concentration were noted. Myoclonus-like muscular contractions appeared over four extremities and in the trunk. She admitted inhaling nitrous oxide (N2O) as a euphoriant repeatedly at party. Following parenteral B12 administration, her neurological deficit promptly resolved. This case demonstrated the abuse of N2OI is an important cause of subacute combined degeneration (SCD) of the spinal cord. To our knowledge, this is the first report of involuntary movements in a patient with N2O intoxication. Although the mechanism remains unknown, involuntary movements similar to myoclonus should be considered as one of the extraordinary neurological manifestations of N2O intoxication.

Topics: Adult; Female; Humans; Magnetic Resonance Imaging; Myoclonus; Nitrous Oxide; Spinal Cord Diseases; Subacute Combined Degeneration; Vitamin B 12

We present a case of a patient who received nitrous oxide on two occasions within a period of 8 weeks and who subsequently developed a diffuse myelopathy, characterized by upper extremity paresis, lower extremity paraplegia and neurogenic bladder. Laboratory testing revealed hyperhomocysteinaemia and low levels of vitamin B(12). Because of this uncommon clinical presentation, we analysed the patient's DNA, and found a polymorphism in the MTHFR gene that is associated with the thermolabile isoform of the 5,10-methylenetetrahydrofolate reductase enzyme, which explained the myelopathy experienced by the patient after being exposed to nitrous oxide. Soon after initiating supplementary therapy with folic acid and vitamin B(12), the neurological symptoms subsided.

Topics: Anesthetics, Inhalation; Folic Acid; Genetic Predisposition to Disease; Humans; Hyperhomocysteinemia; Male; Methylenetetrahydrofolate Reductase (NADPH2); Middle Aged; Nitrous Oxide; Paralysis; Polymorphism, Genetic; Postoperative Complications; Spinal Cord Diseases; Vitamin B 12

Subacute combined degeneration (SCD) of the spinal cord is a characteristic complication of vitamin B12 deficiency, but it has never been neuropathologically demonstrated in a B12-inborn error of metabolism. In this report SCD is documented in a 15-year-old boy with early-onset cobalamin C (cblC) disorder. The neuropathologic findings included multifocal demyelination and vacuolation with predilection for the dorsal and lateral columns at the mid-thoracic level of the spinal cord, confirming the similarity of SCD in cblC disorder to the classic adult SCD due to vitamin B12 deficiency. SCD developed in this boy despite treatment for cblC disorder that began at 3 months of age. There is clinical and experimental evidence to suggest that a deficiency in remethylation with concomitant reduction in brain methionine may be the cause of SCD. In this patient plasma methionine levels were low without betaine and/or l-methionine supplementation and in the normal range for only a 2-year period during compliance with therapy. In cblC disorder, a consistent increase in blood methionine to high normal or above normal levels by the use of betaine and l-methionine supplementation may be helpful in preventing SCD. This is especially important now that the presymptomatic detection of cblC disorder is possible through the expansion of newborn screening.

Topics: Adolescent; Brain; Child; Child, Preschool; Fatal Outcome; Humans; Hydroxocobalamin; Infant; Infant, Newborn; Male; Median Nerve; Metabolism, Inborn Errors; Muscle, Skeletal; Neurodegenerative Diseases; Spinal Cord; Spinal Cord Diseases; Vitamin B 12

Topics: Diagnosis, Differential; Female; Humans; Male; Middle Aged; Sjogren's Syndrome; Spinal Cord Diseases; Vitamin B 12; Vitamin B 12 Deficiency

Vitamin B12 deficiency due to malnutrition or malabsorption may lead to pernicious anemia and neurological disorders. Although randomized prospective studies have shown that pernicious anemia can be safely treated with oral vitamin B12 even in the absence of intrinsic factor, it is still common practice to treat patients with neurological symptoms with intramuscular cyancobalamin injections. We report the successful oral treatment of subacute combined degeneration of the spinal cord in a 24-year-old woman closely monitored clinically with MRI and plasma levels of vitamin B12, homocysteine, and methylmalonic acid. We suggest monitored oral substitution therapy as first-line therapy for neurological disorders related to vitamin B12 deficiency.

Topics: Administration, Oral; Adult; Anemia, Pernicious; Autoantibodies; Dose-Response Relationship, Drug; Female; Folic Acid; Homocysteine; Humans; Intrinsic Factor; Magnetic Resonance Imaging; Methylmalonic Acid; Neurologic Examination; Spinal Cord; Spinal Cord Diseases; Vitamin B 12; Vitamin B 12 Deficiency

We report a rare case of subacute combined degeneration of the spinal cord concomitant with gastric cancer. A 67-year-old man was admitted because of posterior column symptoms, pyramidal tract sign and peripheral neuropathy with severe hyperchromic anemia. He was treated with mecobalamin 1 mg IM, after which his anemia and neurological signs recovered. He was diagnosed as having subacute combined degeneration with pernicious anemia. Subsequent stomach biopsy revealed gastric cancer, and the patient underwent gastrectomy. It is a well known association that chronic atrophic gastritis is associated with gastric cancer or subacute combined degeneration. Our findings suggest that in this case subacute combined degeneration and gastric cancer are independent of each other; rather, both resulted from chronic atrophic gastritis.

Topics: Aged; Anemia, Hypochromic; Humans; Male; Nerve Degeneration; Peripheral Nervous System Diseases; Spinal Cord; Spinal Cord Diseases; Stomach Neoplasms; Vitamin B 12

Topics: Cervical Vertebrae; Demyelinating Diseases; Female; Humans; Magnetic Resonance Imaging; Middle Aged; Nerve Fibers, Myelinated; Neural Pathways; Neurodegenerative Diseases; Spinal Cord; Spinal Cord Diseases; Treatment Outcome; Vitamin B 12; Vitamin B 12 Deficiency

Topics: Diagnosis, Differential; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Spinal Cord Diseases; Vitamin B 12; Vitamin B 12 Deficiency

Topics: Demyelinating Diseases; Diagnosis, Differential; Diet, Vegetarian; Female; Humans; Magnetic Resonance Imaging; Middle Aged; Neurologic Examination; Peripheral Nervous System Diseases; Spinal Cord; Spinal Cord Diseases; Vitamin B 12; Vitamin B 12 Deficiency

A 55 year old male presented 2 years after a jejuno-iliectomy with weakness of all limbs, paraesthesiae, and difficulty in walking. Clinical examination revealed loss of posterior column sensations. Investigations were suggestive of a deficiency of vitamin B12 and folate. MRI showed a band of hyperintensity on T2 image, in the dorsal portion of the spinal cord.

Topics: Humans; Magnetic Resonance Imaging; Male; Middle Aged; Nerve Degeneration; Sensation Disorders; Spinal Cord; Spinal Cord Diseases; Vitamin B 12; Vitamin B 12 Deficiency

Topics: Aged; Demyelinating Diseases; Female; Humans; Magnetic Resonance Imaging; Nerve Degeneration; Spinal Cord Diseases; Vitamin B 12; Vitamin B 12 Deficiency

Topics: Administration, Inhalation; Adult; Analgesics, Non-Narcotic; Humans; Male; Nitrous Oxide; Spinal Cord Diseases; Vitamin B 12

Topics: Cervical Vertebrae; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Nerve Degeneration; Spinal Cord; Spinal Cord Diseases; Thoracic Vertebrae; Vitamin B 12

Vitamin B12 deficiency is common, with most patients lacking classic features of advanced severe deficiency. Early diagnosis and treatment prevent severe anemia and irreversible damage to the nervous system. We describe a 34-year-old man with pernicious anemia who presented with clinical and radiologic features of early myelopathy and borderline low serum levels of vitamin B12. Prompt diagnosis based on the measurement of serum methylmalonic acid and treatment with cyanocobalamin injections led to rapid resolution of clinical manifestations and magnetic resonance imaging abnormalities. We review the literature of magnetic resonance imaging in vitamin B12 deficiency myelopathy and discuss the issues relating to diagnosis and early treatment of this potentially reversible condition.

Topics: Adult; Diagnosis, Differential; Humans; Magnetic Resonance Imaging; Male; Methylmalonic Acid; Paresthesia; Spinal Cord Diseases; Time Factors; Treatment Outcome; Vitamin B 12; Vitamin B 12 Deficiency

White matter vacuolization of the spinal cord is common in patients with AIDS and may lead to clinical manifestations of myelopathy. The pathogenesis of AIDS-associated myelopathy (AM) is unknown and may be related to metabolic abnormalities rather than to direct HIV infection. The striking pathologic similarity between AM and the vacuolar myelopathy associated with vitamin B(12) deficiency suggests that abnormal metabolism of the B(12)-dependent transmethylation pathway may be important in the pathogenesis of AM.. The authors compared S-adenosyl-methionine (SAM), methionine, homocysteine, and glutathione in serum and CSF of 15 patients with AM vs. 13 HIV-infected controls without myelopathy (HWM). They also compared the results with a non-HIV--infected reference population (NC). All patients had normal B(12), folate, and methylmalonic acid levels.. There was a decrease in CSF SAM in the AM group compared with the HWM group (p < 0.0001) and the NC group (p < 0.0001). CSF SAM in the HWM group was also lower than that in the NC group (p = 0.015). Serum methionine was also reduced in serum of the myelopathic group compared with the NC group (p = 0.006).. AM is associated with an abnormality of the vitamin B(12)-dependent transmethylation pathway.

Topics: Adult; Female; Glutathione; HIV Infections; Homocysteine; Humans; Male; Methionine; Methylation; Middle Aged; S-Adenosylmethionine; Spinal Cord Diseases; Vitamin B 12

Cobalamin deficiency is common in the elderly. However, most of the patients are asymptomatic or present with few symptoms. A subacute combined degeneration of the spinal cord accounts for only 10% of the neurological complications. Revealing forms of this myelopathy are exceptional, and were rarely documented by magnetic resonance imaging (MRI) study.. We report a case of subacute combined degeneration of spinal cord in an 81-year-old woman evaluated by a spinal cord MRI. We discuss the role of the spinal cord radiographic study in the diagnosis, the etiopathogeny of cobalamin deficiency and the benefit of vitamin supplementation even at a late stage of the disease in geriatrics patients.. The spinal cord MRI is useful in the diagnosis of late-form combined spinal cord degeneration. The high frequency of atrophic gastritis in elderly prevents the identification of a deficiency of the cobalamin etiology. The treatment relies on vitamin therapy even in the late stages. The severe disability of cobalamin deficiency neurological complications must encourage an earlier diagnosis in elderly patients.

Topics: Age Factors; Aged; Aged, 80 and over; Female; Follow-Up Studies; Gastritis, Atrophic; Humans; Injections, Intramuscular; Magnetic Resonance Imaging; Sclerosis; Spinal Cord; Spinal Cord Diseases; Spinal Puncture; Time Factors; Vitamin B 12; Vitamin B 12 Deficiency

Topics: Aged; Anemia, Pernicious; Humans; Magnetic Resonance Imaging; Male; Spinal Cord Diseases; Vitamin B 12

Topics: Adolescent; Diet, Vegetarian; Follow-Up Studies; Humans; Male; Nerve Degeneration; Risk Assessment; Severity of Illness Index; Spinal Cord Diseases; Treatment Outcome; Vitamin B 12; Vitamin B 12 Deficiency

Pernicious anaemia is the most common cause of cobalamin deficiency. Nervous disorders associated with cobalamin deficiency are neuropathy, optic atrophy, dementia and myelopathy (subacute combined degeneration). In this case, symptoms are those of posterior and lateral column dysfunction of the spinal cord, with diminished vibratory sensation, ataxia, weakness of limbs, hyperreflexia, extensor plantar response and spasticity. Macrocytosis and anaemia are often lacking. There is an inverse correlation between the degree of anaemia and the extent of nervous impairment. The most sensitive tool for the diagnosis of cobalamin deficiency is the serum cobalamin level. But a normal cobalamin assay does not fully exclude cobalamin deficiency. Levels of serum methylmalonic acid and total homocysteine are useful as ancillary tests in the diagnosis. Treatment is based on intramuscular injections of vitamin B12.

Topics: Anemia, Pernicious; Ataxia; Humans; Injections, Intramuscular; Muscle Weakness; Spinal Cord Diseases; Vitamin B 12; Vitamin B 12 Deficiency

Topics: Anemia, Megaloblastic; Anesthetics; Humans; Malabsorption Syndromes; Mass Screening; Nitrous Oxide; Preoperative Care; Spinal Cord Diseases; Vitamin B 12; Vitamin B 12 Deficiency

Topics: Administration, Oral; Anemia, Pernicious; Drug Costs; Humans; Injections, Intramuscular; Netherlands; Spinal Cord Diseases; Vitamin B 12; Vitamin B 12 Deficiency

To observe the presentations and in the patients with subacute combined degeneration (SCD) changes of electrophysiology (EP) and magnetic resonance image (MRI) before and after treatment and to analyze their values in diagnosis.. 10 patients received a series of examinations, including clinical neurological functions, serum vitamin B12 and folic acid concentrations, spinal cord MRI, peripheral nerve conduction velocity, sensory evoked potential (SEP) and visual evoked potential(VEP). They were followed up for 3 to 36 months. The changes of spinal cord MRI and SEP before and after the treatment were studied.. After Vitamin B12 therapy, all the patients improved,but there were still some abnormalities of SEP left. The lesions could be shown sensitively by EP. The changes of SEP and clinical symptoms correlated with each other. The spinal cord MRIs of most patients showed the lesions objectively, demonstrating primarily long-tape abnormal signals in posterior and lateral columns, iso-intense on T1 and hyper-intense on T2 without contrast. Most lesions were seen at the thoracic cord. The lesions constricted or disappeared when the clinical conditions improved.. Spinal cord MRI and EP could objectively show the lesions of SCD and the change before and after the treatment. These examinations and the follow-up of disease could be helpful in the diagnosis of SCD.

Topics: Adult; Aged; Evoked Potentials, Somatosensory; Female; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Nerve Degeneration; Spinal Cord; Spinal Cord Diseases; Vitamin B 12; Vitamin B 12 Deficiency

We present a case of vitamin B12 deficiency and subacute combined degeneration in a patient with a gastrectomy. MRI showed high-signal lesions on T2-weighted images in both the posterior and anterior columns, associated with minor thoracic spinal cord expansion. The patient was treated with B12 supplements and clinical improvement was associated with reduction of the size of the lesions on MRI.

Topics: Gastrectomy; Humans; Image Processing, Computer-Assisted; Magnetic Resonance Imaging; Male; Middle Aged; Neurodegenerative Diseases; Spinal Cord; Spinal Cord Diseases; Vitamin B 12; Vitamin B 12 Deficiency

A 23 year old man presented with a severe posterior column myelopathy related to prolonged nitrous oxide abuse obtained from whipped cream bulbs. The site of pathology was identified by magnetic resonance imaging (MRI) and somatosensory evoked potentials. The mechanism of toxicity involves inactivation of vitamin B12 dependent enzymes. Appropriate treatment with methionine and vitamin B12 was instituted quickly with good neurological outcome. There are major concerns regarding the availability of nitrous oxide in supermarkets.

Topics: Adult; Cervical Vertebrae; Humans; Male; Methionine; Nitrous Oxide; Spinal Cord Diseases; Substance-Related Disorders; Vitamin B 12

Deficiency of vitamin B12 may produce neuropsychiatric disturbances. In the CNS the disease affects mainly myelin sheaths, and the spongy degeneration and diffuse demyelination of the posterior and lateral columns of the spinal cord are the classical pathological changes in patients with subacute combined degeneration. Similar changes also occur in cerebral hemispheres and MRI abnormalities in brain of such patients could be expected, but have received little attention. We report a case of pernicious anemia with neurological manifestations and brain MRI abnormalities. We discuss the neuropathological aspects and we suggest that pernicious anemia is a differential diagnosis to consider in central demyelinating lesions at MRI.

Topics: Acute Disease; Anemia, Pernicious; Brain Diseases; Demyelinating Diseases; Female; Follow-Up Studies; Humans; Magnetic Resonance Imaging; Middle Aged; Spinal Cord Diseases; Vitamin B 12

Topics: Administration, Inhalation; Folic Acid; Homocysteine; Humans; Infant, Newborn; Lung Diseases, Obstructive; Nitric Oxide; Spinal Cord Diseases; Vitamin B 12

Little is known about vitamin B12 deficiency myelopathy's magnetic resonance imaging (MRI) manifestations and their relationship to the onset, evolution, and resolution of neurologic signs and symptoms.. We present a case and review eleven additional reported cases of subacute combined degeneration of the spinal cord detected by MRI.. Our patient had increased T2-weighted signal and gadolinium contrast enhancement of the posterior columns in the cervical and thoracic regions and enhancement of the lateral columns in the high cervical region. This is a case with imaging evidence for lateral column lesions. Two prior reports have shown posterior column enhancement. T1-weighted images may show decreased signal in the posterior columns and sometimes demonstrate reversible spinal cord swelling. MRI abnormalities typically improve after vitamin replacement therapy. However, clinical signs may persist despite resolution of imaging abnormalities, and these abnormalities do not always resolve completely. In addition, symptoms may precede the imaging abnormality.. Vitamin B12 deficiency may produce an increased T2-weighted signal, decreased T1-weighted signal, and contrast enhancement of the posterior and lateral columns of the spinal cord, mainly of the cervical and upper thoracic segments. Because the symptoms may precede any imaging abnormality, it is clear that spinal cord MRI may not be a highly sensitive, early test for subacute combined degeneration.

Topics: Adult; Anemia, Pernicious; Humans; Magnetic Resonance Imaging; Male; Spinal Cord Diseases; Vitamin B 12; Vitamin B 12 Deficiency

We describe a case of myeloneuropathy resulting from nitrous oxide abuse. MR imaging of the spine revealed symmetric abnormal signal in the posterior columns of the cervical cord. Myeloneuropathy is caused by inactivation of vitamin B12 by nitrous oxide. This syndrome can also be seen in patients with borderline vitamin B12 deficiency who have recently been anesthetized with nitrous oxide.

Topics: Adult; Humans; Magnetic Resonance Imaging; Male; Neck; Nitrous Oxide; Spinal Cord; Spinal Cord Diseases; Substance-Related Disorders; Vitamin B 12

We report a 35-year-old patient with megaloblastic anemia who presented with features of subacute combined degeneration of the cord. Electrophysiological studies showed features of axonal neuropathy. In addition, there was evidence of prominent focal proximal conduction block in several nerves. After treatment with cyanocobalamin the neuropathy improved, and the peripheral nerve conduction block detected earlier disappeared. Reversible nerve conduction block as a feature of vitamin B12 deficiency in man, to our knowledge, has not been reported in literature, so far.

Topics: Adult; Axons; Demyelinating Diseases; Humans; Male; Neural Conduction; Peripheral Nerves; Spinal Cord Diseases; Vitamin B 12; Vitamin B 12 Deficiency

Vitamin B12 deficiency is a systemic disease that often affects the nervous system. One of the most prevalent manifestations is subacute combined degeneration (SCD) of the spinal cord. To access the clinical, electrophysiological, and structural abnormalities associated with SCD, a study was conducted in nine patients.. Clinical, electrophysiological (electroneurography, somatosensory and motor evoked potentials), and MRI evaluations were performed in patients before and after treatment.. The most prominent clinical and electrophysiological findings in all patients were dysfunctions of the posterior column. Corresponding hyperintense lesions in the posterior column of the spinal cord were found in two patients by T2 weighted MRI. Damage to the central motor pathway was identified in four patients. Demyelinating neuropathy was present in one patient and axonal neuropathy in four. All patients showed improvement of their symptoms after treatment with cobalamin. Abnormalities of the spinal cord on MRI disappeared early in recovery. Motor evoked potentials and median somatosensory evoked potentials typically normalised after treatment, whereas tibial somatosensory evoked potentials remained abnormal in most patients.. Clinical, electrophysiological, and MRI findings associated with SCD in vitamin B12 deficiency are diverse. Thus vitamin B12 deficiency should be considered in the differential diagnosis of all spinal cord, peripheral nerve, and neuropsychiatric disorders.

Topics: Acute Disease; Aged; Aged, 80 and over; Axons; Diagnosis, Differential; Evoked Potentials, Motor; Evoked Potentials, Somatosensory; Female; Humans; Magnetic Resonance Imaging; Male; Nerve Degeneration; Retrospective Studies; Spinal Cord Diseases; Tibial Nerve; Vitamin B 12; Vitamin B 12 Deficiency

The totally gastrectomized (TGX) rat is a new experimental model for studying the pathogenesis of cobalamin (Cbl)-deficient myelopathy, i.e., subacute combined degeneration, total gastrectomy (TG) serving as a surgical paradigm of human pernicious anemia. We determined the serum levels of some biochemical indicators of Cbl deficiency in TGX rats at 2 to 10 months after TG. Methylmalonic acid (MMA) rose within 2 months and progressively increased thereafter until the end of the investigation period. 2-Methylcitric acid (MCA) rose significantly by 6 months and showed a further increment 4 months later. Homocysteine was only clearly elevated much later than the serum MMA, i.e., 10 months after the operation. The concentrations of MMA, MCA, and cystathionine were increased in kidney, liver, and spinal cord (SC) of TGX rats at 10 months. Chronic treatment of TGX rats with Cbl greatly decreased the serum levels of all the metabolic indicators of Cbl deficiency. Chronic peroral administration of the antibiotic lincomycin to TGX rats in an attempt to suppress the enteric flora markedly decreased serum MMA levels. Only Cbl, however, given either for the first 2 months after TG or for the third and fourth postoperative months (i.e., after SC abnormalities had already appeared) significantly decreased the severity of spongy vacuolation in SC white matter, although not completely preventing or repairing the neuropathological damage. Therefore, neither the early impairment in TGX rats of the Cbl-dependent methylmalonyl-coenzyme A mutase reaction nor the more delayed impairment of the Cbl-dependent methionine synthase step, as reflected by changes in serum metabolite levels, seems to be causally related to the TG-induced spongy vacuolation in SC white matter.

Topics: 5-Methyltetrahydrofolate-Homocysteine S-Methyltransferase; Anemia, Pernicious; Animals; Bacteria, Anaerobic; Citrates; Cystathionine; Disease Models, Animal; Erythromycin; Gastrectomy; Homocysteine; Humans; Intestines; Lincomycin; Male; Methylmalonic Acid; Methylmalonyl-CoA Mutase; Postgastrectomy Syndromes; Rats; Rats, Sprague-Dawley; Spinal Cord; Spinal Cord Diseases; Vacuoles; Vitamin B 12; Vitamin B 12 Deficiency

Topics: Adult; Follow-Up Studies; Humans; Magnetic Resonance Imaging; Male; Spinal Cord Diseases; Vitamin B 12; Vitamin B 12 Deficiency

Topics: Acute Disease; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Nerve Degeneration; Spinal Cord Diseases; Spinal Nerves; Vitamin B 12

We report on two siblings, a girl of 7 years and a boy of 2 years, who presented in infancy with hypotonia, athetoid movements, myopathy and severe developmental delay. The progressive clinical course was characterized by ophthalmoplegia, pyramidal tract signs, loss of visual contact and failure to thrive. The older sister died at the age of 7 years. The younger brother followed an almost identical clinical course. MRI of the brain revealed bilateral hypodensities and atrophy of the putamen. Neurophysiological investigations were consistent with peripheral neuropathy. Investigations for neurometabolic disorders in urine, plasma and CSF of both patients revealed a consistent increase of methylmalonic acid in urine, plasma and CSF as well as borderline low free GABA in CSF. Except for an inconstant elevation of lactate in the boy, metabolic acidosis, hypoglycaemia, episodic ketoacidosis, or hyperammonaemia, the usual concomitants of organoacidopathies, were absent in both children. Homocystinuria was excluded. Methylmalonic aciduria did not respond to antibiotic treatment, vitamin B12 therapy nor dietary protein restriction. Incorporation of [14C]propionate into protein in cultured fibroblasts was pathologically but inconsistently decreased. Both patients' cell lines showed only minimal response to hydroxocobalamin and normal methylmalonyl-CoA mutase activity.. Even though the definitive underlying enzymatic defect in this sibship remains obscure our results suggest a new genetic disorder. This report illustrates that hitherto undescribed metabolic disorders remain to be elucidated even in long investigated areas of intermediary metabolism such as methylmalonic aciduria.

Topics: Amino Acid Metabolism, Inborn Errors; Brain Diseases; Child; Child, Preschool; Disease Progression; Fatal Outcome; Female; Fibroblasts; Humans; Male; Methylmalonic Acid; Nuclear Family; Spinal Cord Diseases; Vitamin B 12

We observed a case of pernicious anaemia in which MRI of the spine demonstrated both intrinsic lesions of the spinal cord and abnormal signal in the bone marrow. The latter resolved with replacement therapy. Only partial recovery of the cord lesions was observed.

Topics: Aged; Anemia, Pernicious; Biopsy, Needle; Bone Marrow; Bone Marrow Diseases; Female; Humans; Injections, Intramuscular; Magnetic Resonance Imaging; Spinal Cord; Spinal Cord Diseases; Vitamin B 12; Vitamin B 12 Deficiency

We reviewed 153 episodes of cobalamin deficiency involving the nervous system that occurred in 143 patients seen over a recent 17-year period at 2 New York City hospitals. Pernicious anemia was the most common underlying cause of the deficiency. Neurologic complaints, most commonly paresthesias or ataxia, were the first symptoms of Cbl deficiency in most episodes. The median duration of symptoms before diagnosis and treatment with vitamin B12 was 4 months, although long delays in diagnosis occurred in some patients. Diminished vibratory sensation and proprioception in the lower extremities were the most common objective findings. A wide variety of neurologic symptoms and signs were encountered, however, including ataxia, loss of cutaneous sensation, muscle weakness, diminished or hyperactive reflexes, spasticity, urinary or fecal incontinence, orthostatic hypotension, loss of vision, dementia, psychoses, and disturbances of mood. Multiple neurologic syndromes were often seen in a single patient. In 42 (27.4%) of the 153 episodes, the hematocrit was normal, and in 31 (23.0%), the mean corpuscular volume was normal. Neutropenia and thrombocytopenia were unusual even in anemic patients. In nonanemic patients in whom diagnosis was delayed, neurologic progression frequently occurred although the hematocrit remained normal. In 27 episodes, the serum cobalamin concentration was only moderately decreased (in the range of 100-200 pg/ml) and in 2 the serum level was normal. Neurologic impairment, as assessed by a quantitative severity score, was judged to be mild in 99 episodes, moderate in 39 and severe in 15. Severity of neurologic dysfunction before treatment was clearly related to the duration of symptoms prior to diagnosis. In addition, the hematocrit correlated significantly with severity, independent of the longer duration of symptoms in nonanemic patients. Four patients experienced transient neurologic exacerbations soon after beginning treatment with cyanocobalamin, with subsequent recovery. Followup evaluation was adequate to assess the neurologic response to vitamin B12 therapy in 121 episodes. All patients responded, and in 57 (47.1%), recovery was complete, with no remaining symptoms or findings on examination. The severity score was reduced by 50% or greater after treatment in 91% of the episodes. Residual long-term moderate or severe neurologic disability was noted following only 7 (6.3%) episodes. The extent of neurologic involvement after treatment

Topics: Adolescent; Adult; Aged; Aged, 80 and over; Anemia, Pernicious; Ataxia; Evoked Potentials, Somatosensory; Humans; Memory Disorders; Mental Disorders; Middle Aged; Nervous System Diseases; Paresthesia; Peripheral Nervous System Diseases; Reflex, Abnormal; Regression Analysis; Sensation; Spinal Cord Diseases; Time Factors; Vitamin B 12; Vitamin B 12 Deficiency

An increased prevalence of vitamin B12 deficiency has been reported in patients infected by the human immunodeficiency virus (HIV). We report an unexpectedly high prevalence (20%) of such abnormal vitamin B12 metabolism in a population of HIV-infected patients referred for neurological evaluation. This abnormality was associated with both peripheral neuropathy and myelopathy. A majority of those treated with cyanocobalamin had a therapeutic response. Selected neuropathological results suggest a relationship between vitamin B12 deficiency and vacuolar myelopathy. Vitamin B12 deficiency may be a frequent and treatable cause of neurological dysfunction in patients with HIV infection.

Topics: HIV Infections; Humans; Nervous System Diseases; Neurologic Examination; Parenteral Nutrition; Peripheral Nervous System Diseases; Spinal Cord; Spinal Cord Diseases; Vitamin B 12

Detailed electrophysiological studies were performed in 4 patients with myeloneuropathy induced by abuse of nitrous oxide for 1 to 4 years. All presented with paresthesias, weakness, and Lhermitte's phenomena, and exhibited signs of sensorimotor polyneuropathy, ataxia, and arreflexia. Two had subnormal serum vitamin B12 levels. Baseline electrophysiologic testing revealed reduced motor unit potentials, prolonged F wave latencies, absent H reflexes, denervation potentials, and delays in motor and sensory conduction. Three had peripheral and nuchal delay after median nerve stimulation. All were reevaluated after 3 to 12 months' abstinence and treatment with vitamin B12, and all showed substantial clinical improvement. Parallel improvement in electrophysiologic findings occurred, but residual minor conduction delays, loss of H reflexes, electromyographic evidence of denervation, or abnormalities of posterior tibial SEP were noted. These findings confirm the reversibility of myeloneuropathy of nitrous oxide abuse and describe the profile of electrophysiologic recovery in subjects who abstain from further neurotoxic exposure.

Topics: Adult; Ataxia; Electromyography; Electrophysiology; Female; Humans; Male; Neural Conduction; Nitrous Oxide; Paresthesia; Spinal Cord Diseases; Substance-Related Disorders; Vitamin B 12

Central and peripheral nerve conduction was studied in two patients with subacute combined degeneration by using the short-latency somatosensory evoked potentials and the peripheral nerve conduction study during treatment with cyanocobalamin. Before the treatment, somatosensory evoked potentials with median nerve stimulation were normal, but those with peroneal nerve stimulation revealed prolonged central conduction indicating dysfunction within the posterior column. Peripheral sensory and motor nerve action potentials were reduced with normal or slightly reduced conduction velocity. After treatment, marked shortening of the central conduction time (by 24% and 31%, respectively) was observed with mild or no recovery of peripheral nerve action potentials. These physiologic findings suggest that the main pathologic changes in the central nervous system may be demyelination in the posterior column in addition to axonal degeneration in the peripheral nerve. The former was responsive to treatment but the latter was poorly responsive to treatment. Sensory symptom in subacute combined degeneration appears to be, at least partially, attributed to the spinal cord lesion.

Topics: Adolescent; Adult; Aged; Central Nervous System; Evoked Potentials, Somatosensory; Female; Humans; Median Nerve; Middle Aged; Neural Conduction; Peripheral Nerves; Peroneal Nerve; Spinal Cord Diseases; Vitamin B 12

Topics: Adult; Consciousness Disorders; Diagnosis, Differential; Humans; Male; Spinal Cord Diseases; Vitamin B 12; Vitamin B 12 Deficiency

Topics: Adolescent; Child; Female; Homocystinuria; Humans; Hydroxocobalamin; Infant; Male; Malonates; Metabolism, Inborn Errors; Methylmalonic Acid; Mutation; Neurocognitive Disorders; Spinal Cord Diseases; Vitamin B 12

Pernicious anaemia (PA) has previously not been reported from Bangladesh. A case is described which had the typical clinical features of PA with subacute combined degeneration of the cord and polyneuropathy. The patient had typical macroovalocytosis, megaloblastic bone marrow and Schilling test result in the range of PA. Vitamin B12 level in the serum was markedly reduced. There was rapid clinical and haematological response to vitamin B12 therapy.

Topics: Anemia, Pernicious; Bangladesh; Elliptocytosis, Hereditary; Humans; Male; Middle Aged; Spinal Cord; Spinal Cord Diseases; Vitamin B 12

The authors describe two patients with organic psychosis who had vitamin B12 deficiency and no hematologic or spinal cord abnormalities. They review the literature that supports a causal relationship between B12 deficiency and cerebral dysfunction, as measured by the EEG, and consequent organic mental changes. The authors cite evidence that these EEG and organic mental changes are reversible with B12 replacement. They emphasize that psychiatric manifestations may be the first symptoms of vitamin B12 deficiency and thus antedate anemia and spinal cord disease. They recommend consideration of B12 deficiency and serum B12 determinations in all patients with organic mental symptoms.

Topics: Anemia, Pernicious; Electroencephalography; Female; Humans; Middle Aged; Neurocognitive Disorders; Spinal Cord Diseases; Vitamin B 12; Vitamin B 12 Deficiency

Topics: Adult; Anemia, Pernicious; Blood Transfusion; Female; Humans; Middle Aged; Spinal Cord Diseases; Vitamin B 12

Neurologic complications associated with vitamin B12 deficiency include spinal cord degeneration, peripheral neuropathy and alteration of mental status. The possibility of vitamin B12 deficiency must be considered in all patients with these nervous system disorders, even in those who do not have anemia. If treatment is started early, most of the neurologic deficits will resolve. Delayed therapy usually halts the progression of the disease, but permanent sequelae may occur.

Topics: Adult; Aged; Anemia, Pernicious; Central Nervous System Diseases; Confusion; Female; Humans; Male; Memory Disorders; Mental Disorders; Schilling Test; Spinal Cord Diseases; Vitamin B 12; Vitamin B 12 Deficiency

Four pairs of monkeys were maintained in an atmosphere of nitrous oxide under conditions which had previously been shown to produce subacute combined degeneration (SCD) of the spinal cord. The diet of one of each pair was supplemented with methionine. In every case the monkey with the unsupplemented diet became ataxic at around 10 weeks and the disorder progressed over a period of 2-3 weeks until the animal was moribund. During this period there was no detectable clinical change in the monkeys receiving methionine supplementation. Microscopical examination of the spinal cord and peripheral nerves of the unsupplemented monkeys showed the classical changes of SCD. The histological changes correlated with the clinical observations. Sections form the methionine-supplemented monkeys showed no change or only slight changes. These results suggest that, in these animals, inability to resynthesise methionine from homocysteine leads to SCD. It seems probable that the primary lesion producing SCD in human beings with pernicious anaemia is also inability to maintain methionine biosynthesis.

Topics: Animals; Demyelinating Diseases; Disease Models, Animal; Humans; Macaca fascicularis; Methionine; Nitrous Oxide; Spinal Cord; Spinal Cord Diseases; Tetrahydrofolates; Vitamin B 12

Topics: Anemia, Pernicious; Child; Humans; Male; Spinal Cord Diseases; Vitamin B 12

In 28 patients suffering from subacute combined degeneration of the spinal cord, vitamin B12 metabolism was investigated. Two postulates, proving vitamin B12 deficiency and excluding another cause for the clinical symptoms, have to be fulfilled. Two patients had no disturbance in their vitamin B12 metabolism. Seven patients had a distinct vitamin B12 deficiency. In the remaining 19 patients we found a mild vitamin B12 deficiency. Of these patients, 5 had had a subtotal gastrectomy, one had had a low absorption of vitamin B12, and 13 patients we could not find a distinct cause for the vitamin B12 deficiency. It is not impossible that nutritional habits can be hold responsible for this deficiency. The question whether these 13 patients should be treated with vitamin B12 for the rest of their lives is difficult to answer. It is a conditio sine qua non that in the patients with S.C.D. the vitamin B12 metabolism is examined circumstantially. By so doing, it may be possible to detect, in cases with minor clinical signs and symptoms of S.C.D., the cause of their illness.

Topics: Adult; Aged; Diagnosis, Differential; Female; Folic Acid; Folic Acid Deficiency; Follow-Up Studies; Humans; Hydroxocobalamin; Intestinal Absorption; Male; Middle Aged; Nerve Degeneration; Recurrence; Spinal Cord; Spinal Cord Diseases; Vitamin B 12; Vitamin B 12 Deficiency

Topics: Blood-Brain Barrier; Humans; Injections, Intramuscular; Radioligand Assay; Spinal Cord Diseases; Vitamin B 12

In 42 gastrectomized patients with low serum B12 the vibration perception threshold (VPT) was significantly elevated as compared with a control group. Forty patients were followed up after 6-12 months of intensive vitamin B12 therapy. Within an adequately treated group (25 patients) remission of symptoms and signs of peripheral neuropathy was observed, including a statistically significant reduction of the VPT measured on the medial malleolus and big toe. Such a reduction was not observed in the adequately treated group of patients with myclopathy. Findings in the inadequately treated group were less definite, both as regards remission of clinical findings and VPT. In four untreated patients the neurological symptoms and signs progressed during the follow-up period. On the basis of these findings intensive and long-lasting treatment with vitamin B12 is recommended for gastrectomized patients showing signs of neuropathy.

Topics: Adult; Aged; Electric Stimulation; Female; Follow-Up Studies; Gastrectomy; Humans; Male; Middle Aged; Nervous System Diseases; Neurologic Manifestations; Perception; Peripheral Nervous System Diseases; Reflex; Spinal Cord Diseases; Vibration; Vitamin B 12; Vitamin B 12 Deficiency

One case each of pernicious anemia and folic acid deficiency with chronic malabsorption with disease of the cord and histologically demonstrated concomitant disease of the peripheral nerve system in the sense of a polyneuropathy are described. The histological findings of nerve obtained by biopsy show, in both cases, the loss of individual nerve fibers as an expression of a chronic axonal degeneration. The pathogenetic basis to be considered in these cases is presented.

Topics: Aged; Anemia, Pernicious; Axons; Female; Folic Acid Deficiency; Humans; Leg; Malabsorption Syndromes; Male; Middle Aged; Nerve Degeneration; Neurologic Manifestations; Peripheral Nerves; Peripheral Nervous System Diseases; Spinal Cord Diseases; Vitamin B 12; Vitamin B 12 Deficiency

This paper describes the clinical history of an adult male who was found to be suffering from subacute combined degeneration of the spinal cord. It is believed that this is the first report of this condition in Papua New Guinea.

Topics: Acute Disease; Humans; Male; Middle Aged; Nerve Degeneration; Neurologic Examination; Spinal Cord Diseases; Vitamin B 12

Vitamin B12 turnover studies were carried out in 2 patients with pernicious anemia and subacute combined degeneration of the spinal cord. The retention of a tracer dose (0.5 mug) of intravenously administered 58Co-labeled vitamin B12 in these 2 patients was measured by whole-body monitoring over periods of 94 and 134 days. Vitamin B12 turnover (per cent per day) was calculated and the results compared with normal subjects and with pernicious anemia patients having no neurologic dysfunction. The loss of radioactive-B12 from the body was described by a single exponential model, confirming that the loss of vitamin B12 takes place as though from a single pool. There was no difference in vitamin B12 turnover between the patients with subacute combined degeneration of the spinal cord and normal subjects or other pernicious anemia patients without neurologic involvement. These findings contradict the suggestion that the development of neuropathy in vitamin B12 deficiency might be related to an increased requirement for vitamin B12.

Topics: Adult; Anemia, Pernicious; Cobalt Radioisotopes; Humans; Male; Middle Aged; Spinal Cord Diseases; Syndrome; Vitamin B 12

A syndrome of ataxic myelopathy with optic atrophy and perceptive deafness has been described. This is essentially similar to the Nigerian ataxic myelopathy which has been ascribed to chronic cyanide intoxication through dietary means. The results of biochemical investigations and bone marrow examination indicate that neither vitamin B12 deficiency nor cyanide intoxication are likely factors in the detiology of our cases. It is possible that the disease may be due to an unrecognized toxic for factor which may or may not be of dietary origin.

Topics: Adult; Age Factors; Ataxia; Cyanides; Diet; Female; Foodborne Diseases; Humans; India; Male; Nigeria; Spinal Cord Diseases; Thiocyanates; Vitamin B 12; Vitamin B 12 Deficiency

Topics: Animal Nutritional Physiological Phenomena; Animals; Body Weight; Bone Marrow Cells; Demyelinating Diseases; Disease Models, Animal; Erythrocyte Count; Erythrocytes; Folic Acid; Haplorhini; Hematocrit; Hemoglobins; Leukocyte Count; Liver; Macaca; Male; Malonates; Nervous System; Spinal Cord Diseases; Vitamin B 12; Vitamin B 12 Deficiency

Topics: Diagnosis, Differential; Female; History, 19th Century; History, 20th Century; Humans; Intestinal Absorption; Male; Middle Aged; Myelitis; Neurologic Manifestations; Retrospective Studies; Spinal Cord Diseases; Syndrome; Vitamin B 12; Vitamin B 12 Deficiency

Topics: Animals; Axons; Carboxylic Acids; Cyclopentanes; Methyltransferases; Mice; Myelin Sheath; Nerve Degeneration; Spinal Cord Diseases; Vitamin B 12

Topics: Adult; Crohn Disease; Folic Acid; Humans; Ileum; Malabsorption Syndromes; Male; Nerve Degeneration; Postoperative Complications; Spinal Cord Diseases; Vitamin B 12; Vitamin B 12 Deficiency

Topics: Adolescent; Age Factors; Bone Marrow; Bone Marrow Cells; Cerebrospinal Fluid Proteins; Female; Histiocytes; Humans; Lipid Metabolism; Lipidoses; Liver; Myelography; Pigments, Biological; Spinal Cord Diseases; Vitamin A; Vitamin B 12; Vitamin E; Vitamin E Deficiency

Lhermitte's sign is a common early symptom of subacute combined degeneration of the cord occurring in 11 out of 44 patients admitted to the National Hospitals for Nervous Diseases during the decade 1962-71 with this diagnosis. Two patients, in both of whom it was the presenting complaint, are described in detail. It is concluded that, in these cases, Lhermitte's sign is due to stretching of demyelinated fibres in the posterior columns in the cervical cord, produced by neck flexion. The symptoms disappear after treatment with vitamin B(12). The clinical importance of this symptom is emphasized.

Topics: Adult; Female; Humans; Male; Middle Aged; Nerve Degeneration; Sensation; Spinal Cord Diseases; Vitamin B 12

A 68-year-old man presenting with chronic intermittent diarrhea and progressive ataxia was found to have idiopathic hypoparathyroidism. Intrinsic factor-resistant vitamin B(12) malabsorption was demonstrated. Both the diarrhea and vitamin malabsorption were reversed by correction of hypocalcemia.His neurological profile was a combination of peripheral nerve, posterior column and cerebellar deficits. He had calcifications in the dentate nuclei of the cerebellum. Possible etiological factors such as vitamin B(12) deficiency, folic acid deficiency and steatorrhea have been excluded. Posterior column and cerebellar abnormalities improved with treatment. It is postulated that hypocalcemia causes functional, reversible spinal cord and cerebellar dysfunction.

Topics: Aged; Calcinosis; Calcium; Cerebellar Diseases; Diarrhea; Humans; Hypocalcemia; Hypoparathyroidism; Male; Peripheral Nervous System Diseases; Phosphorus; Spinal Cord Diseases; Vitamin B 12; Vitamin D

Topics: Anemia, Macrocytic; Humans; Nervous System Diseases; Neurocognitive Disorders; Neurologic Manifestations; Spinal Cord Diseases; Vitamin B 12; Vitamin B 12 Deficiency

Topics: Aged; Diverticulitis; Diverticulum; Escherichia coli; Folic Acid; Humans; Intestines; Jejunum; Male; Neomycin; Neuritis; Radiography; Spinal Cord Diseases; Tetracycline; Vitamin B 12; Vitamin B 12 Deficiency

Topics: Diet, Vegetarian; Female; Humans; Middle Aged; Nerve Degeneration; Neurologic Manifestations; Spinal Cord Diseases; Vitamin B 12

Topics: Adult; Anemia, Pernicious; Humans; Male; Spinal Cord Diseases; Vitamin B 12

Topics: Bone Marrow Diseases; Cobalt Isotopes; Diagnosis, Differential; Humans; Intestinal Absorption; Liver; Male; Middle Aged; Spinal Cord Diseases; Vitamin B 12; Vitamin B 12 Deficiency

Topics: Atrophy; Brain Diseases; Humans; Multiple Sclerosis; Spinal Cord Diseases; Vitamin B 12

Clinical and laboratory signs of neurological disease were found in 14 patients many years after partial gastrectomy. Vitamin B(12) deficiency occurred in most patients, but only those three who had signs of subacute combined degeneration of the cord and no osteomalacia responded well to treatment.

Topics: Adult; Aged; Escherichia coli; Female; Gastrectomy; Humans; Jejunum; Male; Middle Aged; Neurologic Manifestations; Osteomalacia; Peripheral Nervous System Diseases; Spinal Cord Diseases; Vitamin B 12; Vitamin B 12 Deficiency

Topics: Anemia, Macrocytic; Celiac Disease; Diverticulum; Duodenum; Humans; Intestinal Diseases; Jejunum; Male; Middle Aged; Spinal Cord Diseases; Tetracycline; Vitamin B 12

Topics: Anemia; Female; Humans; Malonates; Middle Aged; Spinal Cord Diseases; Vitamin B 12; Vitamin B 12 Deficiency

Topics: Anemia, Pernicious; History, 20th Century; Humans; Hydroxocobalamin; Spinal Cord Diseases; Vitamin B 12; Vitamin B 12 Deficiency

Topics: Gastric Acidity Determination; Humans; Medical History Taking; Methods; Neurologic Manifestations; Paresthesia; Psychotic Disorders; Spinal Cord Diseases; Vitamin B 12; Vitamin B 12 Deficiency

Topics: Central Nervous System Diseases; Folic Acid; Folic Acid Deficiency; Humans; Peripheral Nervous System Diseases; Psychopathology; Spinal Cord Diseases; Vision Disorders; Vitamin B 12; Vitamin B 12 Deficiency

Topics: Adult; Aged; Carbohydrate Metabolism; Celiac Disease; Cobalt Isotopes; Friedreich Ataxia; Humans; Intestinal Absorption; Iron; Lipid Metabolism; Malabsorption Syndromes; Male; Middle Aged; Neuritis; Peripheral Nervous System Diseases; Schilling Test; Spinal Cord Diseases; Spinal Nerves; Vitamin B 12; Vitamin B 12 Deficiency

Topics: Amblyopia; Anemia, Pernicious; Animals; Ceruloplasmin; Chromatography, Ion Exchange; Chromatography, Thin Layer; Dextrans; Folic Acid; Horses; Humans; Immunodiffusion; Immunoelectrophoresis; Malonates; Peripheral Nervous System Diseases; Rabbits; Spinal Cord Diseases; Ultracentrifugation; Vitamin B 12; Vitamin B 12 Deficiency

Topics: Aged; Anemia, Pernicious; Diverticulum; Female; Humans; Malabsorption Syndromes; Spinal Cord Diseases; Vitamin B 12

Topics: Anemia, Macrocytic; Diagnosis, Differential; Female; Humans; Infusions, Parenteral; Male; Middle Aged; Psychotic Disorders; Spinal Cord Diseases; Vitamin B 12; Vitamin B 12 Deficiency

Topics: Anemia, Pernicious; Child; Child Behavior Disorders; Child, Preschool; Cobalt Isotopes; Humans; Infant; Male; Schilling Test; Spinal Cord Diseases; Vitamin B 12

Topics: Ataxia; Gastrectomy; Humans; Male; Middle Aged; Postgastrectomy Syndromes; Postoperative Complications; Spinal Cord Diseases; Stomach Neoplasms; Stomach Ulcer; Vitamin B 12; Vitamin B 12 Deficiency

Topics: Adult; Amebiasis; Anemia, Pernicious; Gastrointestinal Hemorrhage; Humans; Iron-Dextran Complex; Malabsorption Syndromes; Male; Spinal Cord Diseases; Vitamin B 12

Topics: Adrenocorticotropic Hormone; Demyelinating Diseases; Humans; Spinal Cord Diseases; Vitamin B 12

Topics: Humans; Spinal Cord Diseases; Vitamin B 12

Topics: Anemia, Macrocytic; Anemia, Pernicious; Humans; Hydroxocobalamin; Meningoencephalitis; Neuritis; Pellagra; Postgastrectomy Syndromes; Spinal Cord; Spinal Cord Diseases; Spinal Cord Neoplasms; Vitamin B 12; Vitamin B Complex

Topics: Hematinics; Humans; Spinal Cord Diseases; Subacute Combined Degeneration; Vitamin B 12

Topics: Humans; Spinal Cord Diseases; Vitamin B 12

Topics: Bone Marrow; Bone Marrow Diseases; Humans; Spinal Cord Diseases; Vitamin B 12

Topics: Corrinoids; Humans; Spinal Cord Diseases; Subacute Combined Degeneration; Vitamin B 12

Topics: Humans; Nervous System Diseases; Spinal Cord Diseases; Vitamin B 12; Vitamin B Complex

Topics: Humans; Spinal Cord Diseases; Vitamin B 12

Topics: Blood; Hematinics; Humans; Spinal Cord Diseases; Subacute Combined Degeneration; Vitamin B 12

Topics: Anemia; Anemia, Pernicious; Black People; Hematinics; Humans; Spinal Cord Diseases; Vitamin B 12

Topics: Anemia; Anemia, Pernicious; Folic Acid; Hematinics; Humans; Mental Disorders; Psychotic Disorders; Spinal Cord; Spinal Cord Diseases; Vitamin B 12

Topics: Anemia; Anemia, Pernicious; Hematinics; Humans; Liver Extracts; Spinal Cord Diseases; Subacute Combined Degeneration; Urine; Vitamin B 12

Topics: Hematinics; Humans; Pyruvates; Pyruvic Acid; Spinal Cord Diseases; Subacute Combined Degeneration; Vitamin B 12

Topics: Corrinoids; Hematinics; Humans; Spinal Cord Diseases; Vitamin B 12

Topics: Corrinoids; Humans; Spinal Cord; Spinal Cord Diseases; Subacute Combined Degeneration; Vitamin B 12; Vitamins