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Biomedical subjects

J Jankovic

Publications and source records attributed to J Jankovic.

At least 271 records · Page 15Linked to original sources
PubMed1989

Comparison of Sinemet CR4 and standard Sinemet: double blind and long-term open trial in parkinsonian patients with fluctuations.

"Wearing-off" effect, the most common form of levodopa-induced fluctuations, seems to be related to the short plasma half-life of the drug. More sustained plasma levodopa levels may be achieved with a new controlled-release formulation of carbidopa/levodopa, Sinemet CR4. We studied 20 patients, 12 men and 8 women, with Parkinson's disease complicated by "wearing-off" phenomenon. Mean age was 61.1 +/- 8.1 years, duration of symptoms 8.3 +/- 2.4 years, and the Hoehn-Yahr stage 3.0 +/- 0.9. In a 12-week double-blind study, the average number of tablets administered per day decreased from 5.7 +/- 1.2 to 3.8 +/- 0.7 when Sinemet CR4 (50/200) was substituted for the standard Sinemet (25/100) (p less than 0.001). However, this was at the expense of reducing the "on" time (without dyskinesia) from 9.3 +/- 4.6 to 7.5 +/- 4.3 (p less than 0.05), although the total "on" time did not significantly change. In a long-term follow-up of 18 patients, the "on" time with dyskinesia and morning dystonia significantly increased (p less than 0.05). There was no significant change in the total daily dosage of levodopa, but the daily number of doses and tablets significantly decreased (p less than 0.001). Despite increased dyskinesia, most patients preferred taking fewer tablets and have elected to continue taking Sinemet CR4 instead of standard Sinemet. Sinemet CR4 seems to offer a new and effective strategy for the management of levodopa-related fluctuations.

Aged↗
PubMed1989

Tardive oculogyric crises.

Involuntary ocular deviations, or oculogyric crises (OGC), commonly occur in acute dystonic reactions to dopamine receptor blocking drugs (neuroleptics). We describe 4 patients with tardive OGC due to prolonged exposure to neuroleptics. In addition to the OGC, the patients had other tardive movement disorders. All patients improved with tetrabenazine. We conclude that tardive OGC are often not recognized and represent part of the spectrum of tardive dystonia.

Aged↗
PubMed1989

Clinical, biochemical, and neuropathologic findings following transplantation of adrenal medulla to the caudate nucleus for treatment of Parkinson's disease.

We transplanted autologous adrenal medullary tissue into the caudate nucleus of 3 patients with advanced Parkinson's disease. The 1st patient, a 59-year-old man with parkinsonian symptoms for 15 years, had mild improvement in his motor functioning after the operation. However, his postoperative course was characterized by prolonged drowsiness and complex visual hallucinations. The patient died suddenly 8 months after the transplant, and an autopsy revealed coronary atherosclerosis. Examination of the graft site showed necrotic adrenal medullary tissue surrounded by inflammatory cells. The 2nd patient, a 50-year-old man with a 21-year history of parkinsonian symptoms, improved the most after the procedure. The 3rd patient, a 43-year-old man with 12 years of parkinsonian symptoms, had mild improvement in his motor functioning. CSF homovanillic acid increased postoperatively in the 3 patients, but then returned to preoperative levels in all except the 2nd patient. The anatomic, neurochemical, and physiologic basis for the modest clinical improvement shown in these patients is not yet understood.

Activities of Daily Living↗
PubMed1988

Lesch-Nyhan syndrome: a study of motor behavior and cerebrospinal fluid neurotransmitters.

We studied 5 boys, 2 to 10 years old, with marked or complete deficiency of hypoxanthine-guanine phosphoribosyltransferase and Lesch-Nyhan syndrome with varying degrees of mental retardation, dysarthria, chorea, dystonia, spasticity, and ataxia. Four patients had marked reduction of homovanillic acid in the cerebrospinal fluid (CSF) and all showed low CSF 3-methoxy-4-hydroxy phenylethylene glycol, indicating reduced dopamine and norepinephrine turnover. Three patients showed high CSF 5-hydroxyindoleacetic acid, suggesting increased serotonin turnover. Some patients improved with carbidopa-levodopa, but others benefited from tetrabenazine, a monoamine-depleting agent. This study provides support for the theory of abnormal central monoamine metabolism in Lesch-Nyhan syndrome.

Carbidopa↗
PubMed1988

Tardive dystonia in patients with Tourette's syndrome.

We describe two patients with Gilles de la Tourette's syndrome (TS) who developed tardive dystonia after treatment with neuroleptics. The dystonic movements persisted after the offending drugs were stopped and improved with tetrabenazine. Tardive dystonia in TS has not been documented previously.

Adult↗
PubMed1988

Dystonia and tremor induced by peripheral trauma: predisposing factors.

Movement disorders are usually of central origin, but sometimes involuntary movements occur after peripheral trauma. Twenty eight patients, 13 women and 15 men, mean age 37 years (range 15-78), were studied with dystonia or tremor in whom the onset of abnormal movements was related, in time and in distribution, to injury of a body part. Among 23 patients with latency of less than one year after injury, focal dystonia of the involved body part was found in 18, nine of whom had associated reflex sympathetic dystrophy (RSD). One of five patients with peripherally induced tremor had RSD. Abnormal electromyography or nerve conduction velocities were found in the affected limb in four patients, but other electrophysiologic techniques provided evidence for disturbed central function. In 15 patients (65%) possible predisposing factors may have contributed to the pathogenesis of the trauma induced abnormal involuntary movements.

Adolescent↗
PubMed1988

Rett syndrome: qualitative and quantitative differentiation from autism.

Patients with Rett syndrome appear to fulfill the Rendle-Short criteria for the diagnosis of autism, but the pattern of their behavior is qualitatively different from children with autism. Until a biologic marker is identified, diagnosis is based on clinical assessment. In order to standardize this clinical assessment and to provide objective criteria for the evaluation of potential therapeutic modalities, motor and behavioral characteristics of 15 Rett patients were analyzed. The patients with Rett syndrome differed from autistic children in having ataxia, breath-holding, hyperventilation, bruxism, simplicity of stereotypies, and hand apposition. The children with autism demonstrated complex stereotypies and verbal but not motor regression. The more typical features of autism, namely, poor eye contact, lack of sustained interest, speech disturbance, and repetitive truncal rocking motions were poor discriminators between the two groups.

Adolescent↗
PubMed1988

Tetrabenazine therapy of dystonia, chorea, tics, and other dyskinesias.

Dopamine receptor blocking drugs, commonly used in the treatment of involuntary movements, may cause potentially serious adverse effects, including tardive dyskinesia. Tardive dyskinesia has not been reported with tetrabenazine, a dopamine-depleting drug. We report a follow-up in 217 patients treated with tetrabenazine for about 18 months (range, 1 to 80). The response was rated on a scale of 0 to 5 (1 = marked improvement, 4 = no response, 5 = worsening). The mean effect from tetrabenazine was rated as follows: 2.3 in 44 patients with tardive dyskinesia, 2.6 in 15 with tardive dystonia, 2.6 in 10 with Huntington's disease, 2.7 in 17 with Gilles de la Tourette's syndrome, 2.8 in 19 with generalized dystonia, 2.8 in 57 with Meige's syndrome, and 3.4 in 25 with other focal dystonias. Twenty-two patients with a variety of unusual movement disorders had a mean effect of 2.9. Parkinsonism occurred as a side effect in 53 patients, sedation in 28, depression in 23, anxiety in 16, insomnia in 11, and akathisia in 10. The choreatic movement disorders are most amenable to tetrabenazine therapy, but tardive and idiopathic dystonia may also be responsive. Tetrabenazine is an effective and relatively safe drug for a variety of hyperkinetic movement disorders.

Chorea↗
PubMed1988

Focal task-specific tremor and dystonia: categorization of occupational movement disorders.

We studied 28 patients with focal task-specific disorders. Three groups were identified, according to the occurrence of focal tremor alone (group I, n = 10), dystonia alone (group II, n = 8), and tremor-dystonia combination (group III, n = 10). Four of six patients in groups I and III showed improvement with beta blocking agents alone, and another patient improved with primidone and metoprolol. Four of seven patients in groups II and III improved at least moderately with only anticholinergic medication. The coexistence of focal tremor and focal dystonia in the same limb in 10 patients (group III) suggests a link between these focal disorders. We conclude that focal tremor and focal dystonia are related to generalized essential tremor and generalized dystonia. A scheme which conceptualizes these interrelationships is proposed.

Adolescent↗