topiramate and Tremor

Fragile X-associated tremor/ataxia syndrome (FXTAS) is a late onset, X-linked genetic, neurodegenerative disorder caused by a "premutation (PM)" in the fragile X mental retardation 1 (FMR1) gene. Here we report a case of FXTAS from mainland of China who presented with rare orthostatic tremor. A review of tremor of FXTAS in the literature is also included.. A 67-year-old right-handed farmer started with tremor of both legs 8 years ago which was present while standing but absent when sitting or lying and progressed with unsteady gait one and a half years ago. The brain MRI showed high intensity signal in the bilateral middle cerebellar peduncles (MCP) in T2-weighted and fluid-attenuated inversion recovery (FLAIR) images and gene test for premutation for FMR1 was positive with 101 CGG repeats. The patient met the the diagnosis of definite FXTAS. Clonazepam and topiramate were administered to control tremor. We reviewed the literature and identified 64 cases with detailed clinical and genetic information. Orthostatic tremor associated with FXTAS is very rare. We found 85.2% patients reported tremor,42.6% with intention tremor,36.1% with kinetic tremor,32.8% with rest tremor and 29.5% with posture tremor. 37.7% of patients who have tremor showed at least two types of tremor. There were 6 patients with isolated rest tremor. There was 2 patient with voice tremor and 6 with head tremor. We also found that 74.6% FXTAS patients had family history of FMR1 gene associated diseases including Fragile X syndrome (FXS), FXTAS or fragile X-associated primary ovarian insufficiency (FXPOI).. Adding our data to the available literature suggests that orthostatic tremor could be a rare initial manifestation of FXTAS and the review will increasing our understanding the phenotype of tremor in FXTAS. Family history of FMR1 gene associated diseases might be an important clue to the diagnosis.

Topics: Aged; Anticonvulsants; Ataxia; Brain; Clonazepam; Fragile X Syndrome; Humans; Magnetic Resonance Imaging; Male; Topiramate; Tremor

Tremor may be defined as an involuntary movement that is rhythmic (ie, regularly recurrent) and oscillatory (ie, rotating around a central plane) and may manifest in a variety of ways; accordingly, tremor has a rich clinical phenomenology. Consequently, the diagnosis of tremor disorders can be challenging, and misdiagnoses are common. The goal of this article is to provide the reader with straightforward approaches to the diagnosis and treatment of tremors.. Focused ultrasound thalamotomy of the ventral intermediate nucleus of the thalamus is an emerging and promising therapy for the treatment of essential tremor.. The evaluation should start with a detailed tremor history followed by a focused neurologic examination, which should attend to the many subtleties of tremor phenomenology. Among other things, the history and examination are used to establish whether the primary tremor is an action tremor (ie, postural, kinetic, or intention tremor) or a resting tremor. The clinician should then formulate two sets of diagnoses: disorders in which action tremor is the predominant tremor versus those in which resting tremor is the predominant tremor. Among the most common of the former type are essential tremor, enhanced physiologic tremor, drug-induced tremor, dystonic tremor, primary writing tremor, orthostatic tremor, and cerebellar tremor. Parkinson disease is the most common disorder of resting tremor. This article details the clinical features of each of these disorders, as well as those of additional tremor disorders.

Topics: Aged; Diagnosis, Differential; Dystonia; Essential Tremor; Female; Humans; Middle Aged; Topiramate; Tremor; Ventral Thalamic Nuclei

Tremor is a hyperkinetic movement disorder characterized by rhythmic oscillations of one or more body parts. It can be disabling and may impair quality of life. Various etiological subtypes of tremor are recognized, with essential tremor (ET) and Parkinsonian tremor being the most common. Here we review the current literature on tremor treatment regarding ET and head and voice tremor, as well as dystonic tremor, orthostatic tremor, tremor due to multiple sclerosis (MS) or lesions in the brainstem or thalamus, neuropathic tremor, and functional (psychogenic) tremor, and summarize main findings. Most studies are available for ET and only few studies specifically focused on other tremor forms. Controlled trials outside ET are rare and hence most of the recommendations are based on a low level of evidence. For ET, propranolol and primidone are considered drugs of first choice with a mean effect size of approximately 50 % tremor reduction. The efficacy of topiramate is also supported by a large double-blind placebo-controlled trial, while other drugs have less supporting evidence. With a mean effect size of about 90 % deep brain stimulation in the nucleus ventralis intermedius or the subthalamic nucleus may be the most potent treatment; however, there are no controlled trials and it is reserved for severely affected patients. Dystonic limb tremor may respond to anticholinergics. Botulinum toxin improves head and voice tremor. Gabapentin and clonazepam are often recommended for orthostatic tremor. MS tremor responds only poorly to drug treatment. For patients with severe MS tremor, thalamic deep brain stimulation has been recommended. Patients with functional tremor may benefit from antidepressants and are best be treated in a multidisciplinary setting. Several tremor syndromes can already be treated with success. But new drugs specifically designed for tremor treatment are needed. ET is most likely covering different entities and their delineation may also improve treatment. Modern study designs and long-term studies are needed.

Topics: Amines; Anticonvulsants; Botulinum Toxins; Cyclohexanecarboxylic Acids; Deep Brain Stimulation; Fructose; Gabapentin; gamma-Aminobutyric Acid; Humans; Topiramate; Tremor

Tremors can be encountered in a variety of disease states but the most common causes are Parkinson disease and essential tremor. This review was undertaken to highlight advances in the field during the last 12 months.. Kinetic tremor may be more prominent in essential tremor than postural tremor. Clinically Parkinson disease and essential tremor may be confused with each other but it may be possible to distinguish between these two nitrites using sophisticated electrophysiology. Monosymptomatic rest tremor has recently been shown to be associated with decreased fluorodopa uptake on the positron emission tomography scan suggesting its relationship to Parkinson disease.. Significant advances have been made in the understanding of the pathophysiology, genetics and therapy of tremor disorders during the last 12 months. This review will consider Parkinson disease, essential tremor and other tremors and highlight advances in the field.

Topics: Acetates; Amines; Anticonvulsants; Antiparkinson Agents; Cyclohexanecarboxylic Acids; Diagnosis, Differential; Electromyography; Essential Tremor; Fructose; Gabapentin; gamma-Aminobutyric Acid; Humans; Levodopa; Motor Cortex; Muscle, Skeletal; Parkinson Disease; Posture; Primidone; Randomized Controlled Trials as Topic; Severity of Illness Index; Somatosensory Cortex; Topiramate; Tremor

To evaluate the safety and potential beneficial effect of topiramate (TPM) as monotherapy or adjunctive therapy to carbamazepine (CBZ) in patients with cerebellar tremor.. Nine patients with cerebellar tremor participated a 4-week, open-label, prospective-controlled trial. TPM was given as monotherapy (n=7 cases), or in combination with CBZ (n=2 cases), at dosages ranging from 25 mg twice daily to 100 mg twice daily. The severity of tremor was assessed clinically on a 0-4 scale, by tremograms, by the Patients Global Impressions Scale, and by a "free writing" task at baseline and after 4 weeks.. TPM was discontinued in four patients due to adverse effects (sedation=2; cognitive impairment=2; increased aggressiveness=2; asthenia=1). During TPM, all patients improved. The mean tremor amplitude, compared with the baseline period, was reduced from 20% to 75%. After TPM, mean clinical scores of postural tremor and kinetic tremor decreased from 2.1+/-0.8 to 0.9+/-0.9 and from 2.1+/-1 to 1.4+/-1 (P<.05), respectively. All patients with head tremor improved. Writing, eating, and drawing were improved with TPM. Four patients chose to keep taking the drug.. Our study indicates that TPM may be useful for the management of cerebellar tremors. A prospective placebo-controlled trial of TPM in this kind of tremor is warranted. TPM dosages should be titrated slowly to avoid the potential side effects of the drug. The range and the frequency of adverse events might limit the clinical usefulness of TPM.

Topics: Adult; Aged; Cerebellar Diseases; Female; Fructose; Humans; Male; Middle Aged; Prospective Studies; Statistics, Nonparametric; Topiramate; Tremor

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis, associated with immunoglobulin G (IgG) autoantibodies against the GluN1 subunit of the NMDAR, is one of the most common types of autoimmune encephalitis. In patients with anti-NMDAR encephalitis, movement disorders (MDs) are often frequent, mainly presenting as facial dyskinesias and stereotyped movements. The alternating clinical manifestation of limb tremor with unilateral ptosis is rare. Here, we report an interesting case of a 22-year-old woman with rapid weight loss presenting with staged dyskinesia. Interestingly, she typically showed persistent tremor of the right upper limb, which would stop when her left upper eyelid drooped uncontrollably, a phenomenon that lasted for a few seconds, followed by automatic upper eyelid lift and continued persistent tremor of the upper limb. Moreover, it was fortunate to find anti-NMDAR antibodies in her cerebrospinal fluid (CSF), which indicated the patient had anti-NMDAR encephalitis. And abnormal apparent diffusion coefficient (ADC) hyperintense signals on the left midbrain interpeduncular fossa explained this manifestation of focal neurological deficit. After the systematic administration of immunotherapy (intravenous immunoglobulin, IVIG), steroid pulse therapy, and symptomatic treatment, the initial symptoms were significantly relieved except for limb tremor. The MDs were becoming less visible for the next six months under topiramate prescriptions. Noteworthy, there are no specific MD phenotypes in anti-NMDAR encephalitis. We describe the young women with unique MDs and rapid weight loss to help us get a more comprehensive understanding of anti-NMDAR encephalitis.

Topics: Anti-N-Methyl-D-Aspartate Receptor Encephalitis; Autoantibodies; Dyskinesias; Female; Humans; Immunoglobulin G; Immunoglobulins, Intravenous; Movement Disorders; Steroids; Topiramate; Tremor; Weight Loss

Holmes tremor is a rare symptomatic movement disorder, characterized by a combination of resting, postural, and intention tremor. It is usually caused by lesions in the brainstem, thalamus, and cerebellum. Despite pharmacological advances, its treatment remains a challenge; many medications have been used with various degrees of effectiveness. Stereotactic thalamotomy and deep brain stimulation in the ventralis intermedius nucleus have been effective surgical procedures in cases refractory to medical treatment.. Here we report a young woman with topiramate-responsive Holmes tremor secondary to a brainstem cavernoma.. Herein we report a Holmes tremor responsive to Topiramate.

Topics: Adult; Anticonvulsants; Brain; Deep Brain Stimulation; Female; Fructose; Humans; Magnetic Resonance Imaging; Thalamus; Topiramate; Tremor

Historically, adrenocorticotropic hormone was used as a first-line treatment for infantile spasms; however, there has been increasing use of topiramate as initial therapy. Here, we report a retrospective study of adrenocorticotropic hormone (ACTH) and topiramate as initial treatment for infantile spasms. The neurology patient database at the Children's Hospital of Philadelphia was searched using the International Classification of Diseases, Ninth Revision code for infantile spasms, and 50 patients were randomly chosen for chart review. We identified 31 patients receiving either adrenocorticotropic hormone or topiramate monotherapy (adrenocorticotropic hormone n = 12, topiramate n = 19) as a first-line treatment for infantile spasms. A total of 26 patients were symptomatic and 5 cryptogenic. Six patients treated with adrenocorticotropic hormone had resolution of clinical spasms and hypsarrhythmia within a month, but 3 relapsed. Of the 19 patients treated with topiramate, 4 patients eventually, though over a period of 0, 1, 8, or 69 months, had resolution of spasms and hypsarrhythmia.

Topics: Adrenocorticotropic Hormone; Age of Onset; Anorexia; Anticonvulsants; Brain; Dose-Response Relationship, Drug; Female; Fructose; Hormones; Humans; Infant; Lethargy; Male; Retrospective Studies; Selection Bias; Spasm; Spasms, Infantile; Topiramate; Treatment Outcome; Tremor

Topics: Adult; Anticonvulsants; Astrocytoma; Cerebellar Diseases; Cerebellar Neoplasms; Electromyography; Fructose; Humans; Male; Postoperative Complications; Posture; Tomography, X-Ray Computed; Topiramate; Tremor

Topics: Aged; Anticonvulsants; Fructose; Humans; Male; Suicide, Attempted; Topiramate; Tremor

Topics: Anticonvulsants; Brachial Plexus; Cervical Plexus; Chickenpox; Dystonia; Fructose; Humans; Male; Middle Aged; Neurologic Examination; Thorax; Topiramate; Tremor