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Biomedical subjects

J Jankovic

Publications and source records attributed to J Jankovic.

At least 217 records · Page 12Linked to original sources
PubMed1994

CAG repeat size and clinical presentation in Huntington's disease.

The specific mutation in Huntington's disease (HD) is an expansion of the unstable CAG trinucleotide repeat in the IT15 gene in chromosome 4p. We examined the relationship between the CAG repeat size and clinical presentation in 36 patients with suspected diagnosis of HD. Twelve patients had no relatives with documented HD, and five of them failed to show the expanded (>37) CAG repeats. The remaining 31 patients, including seven patients with atypical clinical features for HD (three without and four with family history of documented HD), were heterozygotes for the CAG repeat expansion. There were large CAG repeats (50 copies) in paternally transmitted HD cases with early onset (age 30 or earlier). The rate of disease progression was faster in paternally transmitted cases regardless of the CAG repeat length or age of onset. We conclude that (1) patients lacking the family history of HD frequently show no expansion of the CAG repeats, and (2) the sex of the affected parent influences both the CAG repeat size and the phenotypic expression of the HD gene in the offspring.

Adult↗
PubMed1993

Deprenyl in attention deficit associated with Tourette's syndrome.

While central nervous system stimulants usually improve attention deficit hyperactivity disorder (ADHD) associated with Tourette's syndrome, they often exacerbate tics and can produce other potentially serious complications. Because deprenyl may have a stimulatory effect and monoamine oxidase inhibitors have been shown to ameliorate hyperactive behavior, we studied this drug in children with the Tourette's syndrome-ADHD combination. Twenty-nine patients, 25 boys and four girls, with a mean age of 11.2 years (range, 6 to 18 years) and duration of symptoms for an average of 6.2 years (range, 1 to 13 years), were enrolled in this open trial after they became refractory to conventional treatments for ADHD. The average duration of treatment with deprenyl was 6.7 months (range, 3 to 15 months) and the average daily dose was 8.1 mg/d (range, 5 to 15 mg/dL). Twenty-six of all patients (90%) reported clinically meaningful improvement in their ADHD (score > or = 2 on a scale of 0 to 4), with the mean global improvement rated at 2.6. There were no serious adverse side effects and only two patients noted exacerbation of their tics. Deprenyl appears to be a safe and effective treatment of ADHD in patients with Tourette's syndrome.

Abdominal Pain↗
PubMed1993

Cocaine-related movement disorders.

We describe four patients, two with Tourette's syndrome, one with the combination of idiopathic dystonia and essential-like tremor, and one with tardive dystonia, who noted marked exacerbation of their movement disorders after exposure to cocaine. These patients provide support for the hypothesis that dopaminergic preponderance plays an important role in the pathogenesis of certain hyperkinetic movement disorders. Cocaine should be regarded as an important cause or precipitant of hyperkinetic movement disorders.

Adult↗
PubMed1993

Hereditary motor-sensory neuropathy and movement disorders.

To explore the relationship between hereditary motor and sensory neuropathy (HMSN) and movement disorders, we examined 7 patients with HMSN referred to our Movement Disorders Clinic and surveyed members of the Charcot-Marie-Tooth association. The following movement disorders were observed in the index patients: postural tremor in 6, rest tremor in 3, and Parkinsonism and dystonia in 2. Tremor, present in 40% of the 201 patients who responded to the survey, was first noted at a mean age of 36 years, and mostly involved the hands. Family history of tremor was more frequent in the tremor group (P < 0.005), which also had a significantly worse writing score than the nontremor group (P < 0.001). The overlap in clinical features between HMSN-associated tremor and essential tremor (ET), the high frequency of family history of tremor, and the lack of a relationship between the severity of tremor and of peripheral neuropathy suggest that the tremor in HMSN is pathogenically related to ET.

Aged↗
PubMed1993

Neurogenic orthostatic hypotension: a double-blind, placebo-controlled study with midodrine.

PURPOSE: To investigate the efficacy and safety of midodrine for treatment of patients with orthostatic hypotension due to autonomic failure. PATIENTS: Ninety-seven patients with orthostatic hypotension were randomized in a 4-week, double-blinded, placebo-controlled study with a 1-week placebo run-in period. Patients ranged in age from 22 to 86 years (mean: 61 years). METHODS: After a 1-week run-in phase, either placebo or midodrine at a dose of 2.5 mg, 5 mg, or 10 mg was administered three times a day for 4 weeks. Both the placebo group and the 2.5-mg midodrine group received constant doses throughout the double-blind phase. The patients receiving 5 mg or 10 mg of midodrine were given doses that were increased at weekly intervals by 2.5-mg increments until the designated dose was reached. Efficacy evaluations were based on an improvement at 1-hour postdose in standing systolic blood pressure and in symptoms of orthostatic hypotension (syncope, dizziness/lightheadedness, weakness/fatigue, and low energy level). RESULTS: Midodrine (10 mg) increased standing systolic blood pressure by 22 mm Hg (28%, p < 0.001 versus placebo). Midodrine improved (p < 0.05) the following symptoms of orthostatic hypotension compared to placebo: dizziness/lightheadedness, weakness/fatigue, syncope, low energy level, impaired ability to stand, and feelings of depression. The overall side effects were mainly mild to moderate. One or more side effects were reported by 22% of the placebo group compared with 27% of the midodrine-treated group. Scalp pruritus/tingling, which was reported by 10 of 74 (13.5%) of the midodrine-treated patients, was most frequent. Other reported side effects included supine hypertension (8%) and feelings of urinary urgency (4%). CONCLUSION: We conclude that midodrine is an effective and well-tolerated treatment for moderate-to-severe orthostatic hypotension associated with autonomic failure.

Adult↗
PubMed1993

Use of botulinum toxin in the treatment of hand dystonia.

Forty-six patients with hand dystonia, considered disabling despite optimal pharmacologic therapy, were injected in the forearm musculature with botulinum A toxin. Thirty of these patients were followed long enough to provide adequate data for analysis of 86 treatment sessions. There was a 63% female preponderance, with an average age at initial evaluation of 46 years and symptom duration of 7.9 years. Average baseline severity of dystonia was rated as 3.5 on a severity rating scale (0-4 rating; 4 = maximum severity). The average peak effect response for all injections (79 into wrist flexors and 29 into wrist extensors) was 2.2 for dystonia and 3.0 for pain (0-4 rating; 0 = no response, 4 = maximum benefit). The latency from injection to onset of effect averaged 5.6 days. Total response duration averaged 9.3 weeks and maximum improvement was 7.5 weeks. Only local complications occurred and consisted primarily of hand weakness (25 patients, 44 sessions). The results show that botulinum toxin injections effectively control hand dystonia in instances where other forms of therapy have failed.

Botulinum Toxins↗
PubMed1993

Current approaches in the treatment of Parkinson's disease.

Treatment options for patients with Parkinson's disease have rapidly expanded in the last five years. They now include pharmacologic therapies designed not only to improve parkinsonian symptoms, but also to prevent disease progression. In addition, neurosurgical options and other novel treatment strategies are being developed and may become clinically useful in the next decade. This chapter addresses current and evolving Parkinson's disease medications, future surgical interventions, and specific strategies for maximizing patient motor abilities.

Antiparkinson Agents↗
PubMed1993

Clozapine use in diffuse Lewy body disease.

Diffuse Lewy body disease, a severely disabling neuropsychiatric disease, presents with progressive dementia, psychotic symptoms, depression, and parkinsonian symptoms. The authors report a case illustrating that clozapine, a novel neuroleptic drug, has special efficacy in treating psychotic symptoms in these patients.

Clozapine↗
PubMed1993

Psychogenic myoclonus.

We report 18 patients (13 women, 5 men; age range, 22 to 75 years; mean, 42.5), whom we ultimately diagnosed as having "psychogenic myoclonus." The myoclonus was present for an average of 36 months (range, 1 to 110), and it was segmental in 10, generalized in seven, and focal in one. Stress precipitated or exacerbated the myoclonic movements in 15 patients; 14 had a definite increase in myoclonic activity during periods of anxiety. A combination of the following findings helped to establish the psychogenic nature of the myoclonus: (1) clinical features incongruous with "organic" myoclonus, (2) evidence of underlying psychopathology, (3) an improvement with distraction in 14 and with placebo in nine, and (4) the presence of incongruous sensory loss or false weakness in five. Over half of all patients with adequate follow-up improved after gaining insight into the psychogenic mechanisms of their movement disorder. This study attempts to characterize psychogenic myoclonus, the most common psychogenic movement disorder in our movement disorders clinic, and provides a guide to its diagnosis and treatment.

Adult↗
PubMed1993

Longitudinal experience with botulinum toxin injections for treatment of blepharospasm and cervical dystonia.

We performed a longitudinal analysis of the effects of repeat botulinum toxin (BTX) injections in 42 patients with blepharospasm (BL) and 115 patients with cervical dystonia (CD) who received BTX injections between 1984 and 1992 in at least five separate treatment sessions. Although the BL patients were significantly older (59.8 years) than the CD patients (50.6 years; p < 0.001), there were no other demographic differences between the two groups. While the total dose per treatment session and the peak effect rating remained the same, the duration of benefit increased and the number of complications decreased during the course of treatment. We concluded that chronic treatment with BTX in patients with BL and CD is not associated with any decline in benefit, and that efficacy may improve slightly with repeat treatments.

Adolescent↗
PubMed1993

Tardive stereotypy and other movement disorders in tardive dyskinesias.

We reviewed the medical records and videotapes of 100 patients with tardive dyskinesia (TD) referred to our movement disorders clinic to characterize the spectrum of hyperkinetic movement disorders caused by dopamine receptor blocking drugs (DRBD). Tardive stereotypy, present in 78 patients, was the most common type of TD, followed by tardive dystonia, akathisia, tremor, chorea, and myoclonus. Sixty-four had a combination of these hyperkinesias. In a second study, a "blind" review of videotapes of patients with a variety of movement disorders found that DRBD were the cause of stereotypic movements in 89.3% of patients, and 96.1% of patients with TD had stereotypy. We conclude that stereotypy can be readily differentiated from other hyperkinetic movement disorders and that its presence in an adult is highly suggestive of prior exposure to DRBD.

Adult↗
PubMed1993

Movement disorders.

This article focuses on the current knowledge about movement disorders associated with alcohol and drug abuse. Chronic alcohol use can produce a wide spectrum of movement disorders including tremor, withdrawal parkinsonism and dyskinesias, cerebellar ataxia, and asterixis. MPTP, a neurotoxin first reported to cause parkinsonism in a group of drug abusers, has provided important insights into the pathogenesis of Parkinson's disease. There is a growing body of literature providing evidence that dyskinesias such as tics and dystonia may be precipitated or exacerbated by cocaine. Amphetamines have been implicated in the production of stereotypies and exacerbation of tics.

1-Methyl-4-phenyl-1,2,3,6-tetrahydropyridine↗
PubMed1992

Tardive tremor.

A variety of hyperkinetic movement disorders has been associated with the use of neuroleptics (dopamine receptor blocking drugs), but tardive tremor has not been previously documented. We describe five patients in whom tremor occurred after chronic treatment with neuroleptics, was aggravated by and persisted after neuroleptic withdrawal, and improved after treatment with the dopamine depleting drug tetrabenazine. This involuntary oscillatory movement, with a frequency range of 3-5 Hz, was most prominent during maintenance of a posture, but was also present at rest and during a goal-directed movement. The tremor was accompanied by other tardive movement disorders, including akathisia, chorea, dystonia, myoclonus, and stereotypy. There was no family history or other explanation for tremor in these patients. We suggest that this hitherto unreported movement disorder is best termed "tardive tremor."

Aged↗
PubMed1992

Persistent dystonia possibly induced by flecainide.

A 55-year-old man developed clicking of his teeth, involuntary biting, chewing and grimacing within three days of initiation of flecainide. We suggest flecainide should be added to the list of benzamide derivatives associated with drug-induced movement disorders.

Bruxism↗