Movement disorders: resources in Rhode Island.
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Biomedical subjects
Publications and source records attributed to J H Friedman.
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"Rubral" tremor is a rare movement disorder that occurs typically with midbrain damage. It is defined by its presence at rest, with sustained posture, and with movement. Whether it is a single-tremor disorder or a combination of two distinct tremors is debated. This report chronicles a severe neuroleptic induced "rubral" tremor in a patient who had had a stable posttraumatic ataxia. The dramatic response to benztropine and bromocriptine is illustrated in the videotape.
Myoclonic dystonia is a rare disorder that occurs in an hereditary and a sporadic form. The autosomal-dominantly inherited form is responsive to alcohol but not to other drugs. The sporadic form has been relatively resistant to drug treatment. We report a young man with myoclonic dystonia who displayed only little response to alcohol but improved significantly with a combination of sodium valproate for myoclonus and trihexiphenidyl hydrochloride for dystonia. His rehabilitation, however, was confounded by public authorities who thought the patient's appearance was indicative of drug use.
Rosenthal fibers are present in several neurologic disorders. Their occurrence in widespread subependymal, subpial, and perivascular locations is thought to be diagnostic of Alexander's disease, but they have also been reported in rare adult cases. Some of these patients were neurologically intact with severe systemic illnesses. In symptomatic cases, the neurologic disorder has simulated multiple sclerosis. We report an elderly woman with progressive parkinsonism, dementia, and hallucinations whose brain showed severe Rosenthal fiber proliferation.
BACKGROUND: Because fluoxetine may be associated with an induction or exacerbation of parkinsonism, caution has been suggested when considering fluoxetine as an antidepressant for patients with Parkinson's disease. METHOD: We retrospectively reviewed the medical records of 23 outpatients with Parkinson's disease who were receiving or had received fluoxetine. One author evaluated all patients using the Northwestern University Disability Scale for scoring parkinsonism. Rather than employing a formal depression scale, we assessed depression globally. Concurrent medications were permitted. RESULTS: Twenty of the 23 patients experienced no worsening of parkinsonism while being treated with up to 40 mg of fluoxetine per day. The other 3 patients' parkinsonism worsened to a mild degree: a 74-year-old man experienced an increase in akinesia, tremor, and rigidity; a 77-year-old man experienced a slight worsening in tremor and rigidity; and a 56-year-old man experienced a decline in gait and akinesia. It was unclear if these declines, which were neither acute nor severe, were due to fluoxetine treatment or the progression of the disease. Signs of parkinsonism in 2 patients appeared to improve during fluoxetine treatment. CONCLUSION: Fluoxetine, in doses up to 40 mg/day, does not appear to be associated with exacerbations of parkinsonian signs and symptoms in outpatients with Parkinson's disease. Further investigation of fluoxetine for the treatment of depression in patients with Parkinson's disease is warranted.
Parkinson's disease (PD) is frequently associated with psychiatric problems. Depression generally responds to antidepressant medications or to electroconvulsive therapy (ECT). The nondepressive psychoses generally require a reduction in parkinsonian medications, or possibly a "drug holiday." In patients whose psychosis fails to respond to a reduction in medication or who cannot tolerate the worsening parkinsonism, an antipsychotic drug should be added. Clozapine is probably the drug of choice, with low potency neuroleptics being second-line options.
Clozapine is an atypical neuroleptic medication that is free of parkinsonian side effects and that has been reported to ameliorate the tremor of Parkinson's disease. We report (with videotape illustration) the dramatic improvement of severe yet classic rest tremors in one elderly nonpsychotic patient with Parkinson's disease and significant improvement in four others. We believe that clozapine, in low doses, may be a useful medication for the treatment of rest tremor in Parkinson's disease, even in cases without mental abnormalities.
Senile chorea, a late-onset, slowly progressive disorder without mental changes, is a poorly characterized entity. It is often thought to represent late-onset Huntington's disease in which the chorea precedes the mental changes and the family history is absent due to parental death before symptoms. The following case differs pathologically from Huntington's disease and from previous reports of senile chorea.
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Nineteen patients with mild-to-moderate Parkinson's disease completed a 1-year open label study of a controlled release preparation of carbidopa/L-DOPA (Sinemet CR). Twelve patients were thought to have improved compared with baseline, and only one was worse. The total daily dose of L-DOPA was not significantly changed, but dosing frequency was almost halved. Patients with complex drug schedules on standard Sinemet liked the drug most. "Wearing off" patients also benefitted from the change, whereas "on/off" patients did not do significantly better on Sinemet CR.
Parkinsonism associated with boxing has attracted recent media attention, yet little has been written about it in the medical literature. This report presents a typical case with a review of the literature.
Twenty patients with asymmetric Parkinson's disease were studied in a reaction time (RT) experiment in which the performance of the more affected ("bad") hand was compared with performance of the less affected ("good") hand. Simple RT and choice RT were tested in separate blocks, and the benefit afforded by advance information in the simple RT condition (choice RT minus simple RT) served as a measure of motor preparation. RT was longer in the "bad" hand in both the simple RT and choice RT conditions. There was no difference in the effect of advance information between the two hands. It is concluded that slowness in RT movement initiation in Parkinson's disease is not due to a deficiency in motor preparation, and that intact basal ganglia function is not required for this stage of motor programming.
We discuss siblings with galactose-1-phosphate uridyl transferase deficiency who developed neurologic complications after the age of 30. One has partial complex seizures and the other has generalized seizures, progressive ataxia, and apraxia. As more galactosemic children survive into adulthood, more neurologic complications may become more prevalent.
Clozapine is an antipsychotic medication that is virtually free of extrapyramidal side effects. We report our successful treatment of 6 patients with idiopathic Parkinson's disease and various psychoses using clozapine on a chronic basis along with carbidopa/L-dopa.
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