vitamin-b-12 and Central-Nervous-System-Diseases

Topics: Central Nervous System Diseases; Dementia; Diagnosis, Differential; Drainage; Humans; Hypercalcemia; Magnetic Resonance Imaging; Peripheral Nervous System Diseases; Prednisolone; Sarcoidosis; Vitamin B 12

Topics: Central Nervous System Diseases; Diagnosis, Differential; Homocystinuria; Humans; Infant, Newborn; Intellectual Disability; Prognosis; Pyridoxine; Vitamin B 12

Indistinguishable hematologic abnormalities are seen in most patients with cobalamin (Cbl, vitamin B12) or folate deficiency. Approximately one third of Cbl-deficient patients develop a wide variety of non-focal neuropsychiatric abnormalities that are not seen in folate deficiency. Serum levels of homocysteine are elevated to a similar degree in Cbl-deficient patients with and without neuropsychiatric abnormalities, and in folate-deficient patients. Serum levels of eight other metabolites including methylmalonic acid also fail to elucidate the biochemical basis for the neuropsychiatric abnormalities. Levels of homocysteine and methylmalonic acid are often only slightly elevated in Cbl-deficient patients who have significant neuropsychiatric defects. Moderate elevations of homocysteine and methylmalonic acid occur in 20%-30% of various elderly populations (mean age 80) and may play a role in the similar neuropsychiatric abnormalities that occur increasingly with aging. Taken together, these studies suggest that an important unknown Cbl-dependent enzyme, metabolic abnormality, environmental factor, or genetic factor may play a major role in the pathophysiology of the neuropsychiatric abnormalities caused by Cbl deficiency.

Topics: Central Nervous System Diseases; Citrates; Demyelinating Diseases; Folic Acid; Homocysteine; Humans; Mental Disorders; Methionine; Methylmalonic Acid; Vitamin B 12

Low serum vitamin B12 levels and vitamin B12 deficiencies are frequently found in the elderly. The full syndrome of a vitamin B12 deficiency is rather simple to diagnose. The large applicability of the vitamin B12 assay also gives rise to many probably incomplete features. Low/low-normal vitamin B12 levels in screening procedures raise uncertainty whether this finding represents deficiency and should be followed by supplementation. In this paper the occurrence of low serum levels of vitamin B12 are discussed. To assess the clinical relevance of low/low-normal outcomes supplementary diagnostic procedures will be needed. Within this scope we illustrate the d.o.s.-test and the MMA-assay. Especially the latter will probably provide more answers to the treatment question. Systematic research is needed to clarify this issue. Meanwhile supplementation of all low and low-normal outcomes of the B12-assay seems the best answer.

Topics: Aged; Anemia, Pernicious; Central Nervous System Diseases; Deoxyuridine; Humans; Methylmalonic Acid; Neurocognitive Disorders; Vitamin B 12; Vitamin B 12 Deficiency

Cobalamin deficiency leads to impaired folate function as demonstrated by markedly impaired single-carbon unit transfer into purine, thymidine and methionine. This occurs in the total absence of 'methylH4folate trapping'. In cobalamin deficiency there is impaired synthesis of formylH4folate and raised levels of endogenous formate in blood and liver. FormylH4folate and methionine reverse the effects of cobalamin deficiency. Methionine provides formate via its metabolism to methylthioribose. Recently it has been suggested that the neuropathy of cobalamin deficiency is due to impaired methylation but this was not confirmed. It is likely that defects demonstrated in marrow and liver are also the explanation for the effects of cobalamin deficiency in the CNS.

Topics: Anemia, Megaloblastic; Animals; Central Nervous System Diseases; Coenzymes; Folic Acid; Folic Acid Deficiency; Formates; Humans; Methionine; Models, Biological; Tetrahydrofolates; Thymidine; 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: Acridines; Administration, Oral; Antipsychotic Agents; Butyrophenones; Central Nervous System Diseases; Dopamine; Humans; Molindone; Phenothiazines; Phytotherapy; Piperazines; Plants, Medicinal; Rauwolfia; Schizophrenia; Seizures; Structure-Activity Relationship; Tetrabenazine; Thioxanthenes; Tranquilizing Agents; Vitamin B 12

Aging is associated with a progressive decline in vitamin B-12 status. Overt vitamin B-12 deficiency causes neurologic disturbances in peripheral and central motor and sensory systems, but the public health impact for neurologic disease of moderately low vitamin B-12 status in older people is unclear. Evidence from observational studies is limited by heterogeneity in the definition of vitamin B-12 status and imprecise measures of nerve function.. We aimed to determine whether vitamin B-12 status is associated with electrophysiologic indexes of peripheral or central neurologic function in asymptomatic older people with moderately low vitamin B-12 status.. We used a cross-sectional analysis of baseline data from the Older People and Enhanced Neurological Function study conducted in Southeast England. This trial investigated the effectiveness of vitamin B-12 supplementation on electrophysiologic indexes of neurologic function in asymptomatic older people (mean age: 80 y) with moderately low vitamin B-12 status (serum vitamin B-12 concentrations ≥107 and <210 pmol/L without anemia, n = 201). Vitamin B-12 status was assessed with the use of total vitamin B-12, holotranscobalamin, and a composite indicator of vitamin B-12 status (cB-12). Electrophysiologic measures of sensory and motor components of peripheral and central nerve function were assessed in all participants by a single observer.. In multivariate models, there was no evidence of an association of vitamin B-12, holotranscobalamin, or cB-12 with any nerve conduction outcome. There was also no evidence of an association of vitamin B-12 status with clinical markers of neurologic function.. This secondary analysis of high-quality trial data did not show any association of any measure of vitamin B-12 status with either peripheral or central neurologic function or any clinical markers of neurologic function in older people with moderately low vitamin B-12 status. The results of this study are unlikely to be generalizable to a less healthy older population with more severe vitamin B-12 deficiency. This trial was registered at www.controlled-trials.com as ISRCTN54195799.

Topics: Aged; Aged, 80 and over; Asymptomatic Diseases; Biomarkers; Central Nervous System Diseases; Confounding Factors, Epidemiologic; Cross-Sectional Studies; Dietary Supplements; Double-Blind Method; Elder Nutritional Physiological Phenomena; Electrophysiological Phenomena; England; Female; Geriatric Assessment; Humans; Male; Multivariate Analysis; Neural Conduction; Neurologic Examination; Nutritional Status; Peripheral Nervous System Diseases; Severity of Illness Index; Vitamin B 12; Vitamin B 12 Deficiency

The membrane receptor (TCblR/CD320) for transcobalamin (TC)-bound cobalamin (Cbl) facilitates the cellular uptake of Cbl. A genetically modified mouse model involving ablation of the CD320 gene was generated to study the effects on cobalamin homeostasis. The nonlethal nature of this knockout and the lack of systemic cobalamin deficiency point to other mechanisms for cellular Cbl uptake in the mouse. However, severe cobalamin depletion in the central nervous system (CNS) after birth (P<0.01) indicates that TCblR is the only receptor responsible for Cbl uptake in the CNS. Metabolic Cbl deficiency in the brain was evident from the increased methylmalonic acid (P<0.01-0.04), homocysteine (P<0.01), cystathionine (P<0.01), and the decreased S-adenosylmethionine/S-adenosyl homocysteine ratio (P<0.01). The CNS pathology of Cbl deficiency seen in humans may not manifest in this mouse model; however, it does provide a model with which to evaluate metabolic pathways and genes affected.

Topics: Animals; Biological Transport, Active; Brain; Central Nervous System Diseases; Disease Models, Animal; Female; Humans; Mice; Mice, Inbred C57BL; Mice, Knockout; Pregnancy; Receptors, Cell Surface; Vitamin B 12; Vitamin B 12 Deficiency

A case of a 44-years-old patient with unusual clinical presentation of encephalomyelopolyneuropathy in vitamin B12 deficiency is presented. The disease manifested itself with gastrointestinal bleeding, which necessitated emergency hospitalisation in surgical clinic. Clinical examinations revealed atrophic gastritis, pernicious anemia, neurological and mental complications. The diagnosis was made according to the following criteria: physical examination--smooth tongue, atrophic gastritis, mild hepatosplenomegaly; laboratory findings--pernicious anemia, low vitamin B12 serum levels; neurological examination--syndrome of combined damage of the posterior and lateral columns of the spinal cord; magnetic resonance imaging--typical hyperintense areas on T2-weighted images in the posterior columns in the cervical regions of the spinal cord; transcranial magnetic stimulation--prolonged central motor conduction time of the motor evoked potentials bilaterally; psychological examination--cognitive decline. After treatment with vitamin B12 an improvement of the hematological findings, neurological deficit and cognitive impairments was found.. Neurological complications could be an early manifestation of vitamin B12 deficiency. In diagnostic aspect similar complaints require examination of the serum levels of vitamin B12. The delay in diagnosis and inadequate therapy bear the risk of incomplete recovery of the neurological deficit. The current problem of "cognitive decline" necessitates routine examination of the serum levels of vitamin Bl2 in all patients with initial cognitive impairments and their prompt and approapriate treatment.

Topics: Adult; Central Nervous System Diseases; Cognition Disorders; Humans; Male; Polyneuropathies; Vitamin B 12; Vitamin B 12 Deficiency

Thirty-four symptomatic cases of inherited transcobalamin II (TCII) deficiency were analysed in order to determine the frequency and nature of neurologic manifestations. In no instance was there definite evidence of a neurologic disorder at the time of presentation as a young infant. One child of 2 1/2 years transiently lost deep tendon reflexes at a time of suboptimal treatment. A syndrome of mental retardation and other neurologic manifestations was observed in three cases, all with the following in common: (1) an extended duration of illness of 2-17 years; (2) inadequate or not treatment with Cbl; (3) treatment with folic of folinic acid. TCII deficiency rarely if ever presents with neurologic manifestations. However, neurologic disorders can be produced subsequently by improper treatment.

Topics: Central Nervous System Diseases; Folic Acid; Humans; Infant; Infant, Newborn; Intellectual Disability; Leucovorin; Nervous System Diseases; Time Factors; Transcobalamins; Vitamin B 12

Vitamin B12 and folate concentrations were measured in serum and cerebrospinal fluid (CSF) in 293 neurological patients. Serum and CSF vitamin B12 concentrations showed a positive correlation. In individual patients CSF B12 concentrations varied considerably for a given serum concentration. The median serum vitamin B12 concentration of the Alzheimer's type dementia group was significantly lower compared with that of a control group. Lower median CSF vitamin B12 concentrations were found in groups of patients with multiple sclerosis and Alzheimer's type dementia. Five patients with heterogeneous clinical pictures had unexplained low serum and CSF B12 concentrations without macrocytosis. Two patients had very high serum B12 and low-normal CSF concentrations which could be explained by a blood-brain barrier transport defect. Serum and CSF folate concentrations did not show significant differences between the various groups.

Topics: Adolescent; Adult; Aged; Aged, 80 and over; Blood-Brain Barrier; Central Nervous System Diseases; Child; Female; Folic Acid; Humans; Male; Middle Aged; Multiple Sclerosis; Neurologic Examination; Reference Values; Retrospective Studies; Vitamin B 12

Topics: Adult; Antineoplastic Agents; Central Nervous System Diseases; Cytophotometry; DNA, Neoplasm; Female; Humans; Lymphoma, Non-Hodgkin; Male; Methotrexate; Middle Aged; Protein Binding; Vitamin B 12

In a total of 55 samples of cerebrospinal fluid (CSF) and an equal number of serum samples obtained from 45 patients with neurological disorders and 10 controls, folic acid and vitamin B12 were measured. A radioisotopic assay method was used. A significant decrease of CSF folic acid was noted in the group with cerebral tumors.

Topics: Brain Neoplasms; Central Nervous System Diseases; Demyelinating Diseases; Folic Acid; Humans; Meningitis; 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

The vitamin B12 levels of cerebrospinal fluid were assayed microbiologically (Lactobacillus leichmannii method) using samples from 44 patients with various neurological disorders, 4 patients with megaloblastic anemia and 34 controls. Twenty-seven controls that did not receive vitamin B12 showed a mean cerebrospinal fluid vitamin B12 level of 21.5 pg/ml (range: 0-60). No decrease in cerebrospinal fluid vitamin B12 level was seen in patients with subacute myelo-optico-neuropathy (SMON). High levels of cerebrospinal fluid vitamin B12 were observed only in the patients receiving long term administration of the vitamin. Intrathecal administration of vitamin B12 caused only a slight increase in serum vitamin B12 level after four hours. The existence of blood brain barrier for vitamin B12 was suggested.

Topics: Anemia, Megaloblastic; Central Nervous System Diseases; Humans; Myelitis; Optic Neuritis; Syndrome; Vitamin B 12

EEGs were recorded in 54 patients with pernicious anemia. Six patients without nervous system involvement had normal EEGs, 10 patients with spinal cord or peripheral nervous system involvement had normal or minimally abnormal EEGs, 17 of 19 patients with evidence of mental dysfunction had abnormal EEGs with the most consistent finding being an excess of theta slowing, and 19 patients with pernicious anemia and other neurologic diseases showed EEG findings reflecting the complicating disease process. In general, the EEG abnormalities in patients with pernicious anemia correlated well with the presence of cerebral dysfunction but not with the degree of anemia. The EEG was also a good indicator for detecting and confirming other intracranial disease processes unrelated to pernicious anemia.

Topics: Adult; Aged; Anemia, Pernicious; Beta Rhythm; Central Nervous System Diseases; Cerebrovascular Disorders; Electroencephalography; Female; Hemoglobinometry; Humans; Male; Mental Disorders; Middle Aged; Theta Rhythm; Vitamin B 12

Topics: Central Nervous System; Central Nervous System Diseases; Folic Acid; Humans; Vitamin B 12

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: Anemia, Pernicious; Central Nervous System Diseases; Diagnosis; Humans; Intrinsic Factor; Muscle Spasticity; Paralysis; Spinal Cord; Spinal Diseases; Vitamin B 12

Topics: Biomedical Research; Blood Chemical Analysis; Central Nervous System Diseases; Cerebrospinal Fluid; Humans; Multiple Sclerosis; Sclerosis; Vitamin B 12

Topics: Anemia; Anemia, Pernicious; Central Nervous System Diseases; Clinical Laboratory Techniques; Cobalt Isotopes; Humans; Neurologic Manifestations; Postgastrectomy Syndromes; Schilling Test; Spinal Cord; Urine; Vitamin B 12

Topics: Central Nervous System Diseases; Cobalt Isotopes; Corrinoids; Diagnosis; Fluids and Secretions; Humans; Schilling Test; Spinal Cord; Spinal Diseases; Urine; Vitamin B 12

Topics: Anemia; Anemia, Pernicious; Central Nervous System Diseases; Corrinoids; Diagnosis, Differential; Folic Acid; Geriatrics; Humans; Intracranial Embolism; Intracranial Embolism and Thrombosis; Metabolism; RNA; Toxicology; Vitamin B 12; Vitamin B Complex; Vitamins

Topics: Blood; Central Nervous System Diseases; Cerebrospinal Fluid; Cysticercosis; Epilepsy; Humans; Hydrocephalus; Meningitis; Neurosyphilis; Peripheral Nervous System Diseases; Polyradiculopathy; Spinal Cord; Sulfonamides; Vitamin B 12