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Biomedical subjects

J Jankovic

Publications and source records attributed to J Jankovic.

At least 325 records · Page 18Linked to original sources
PubMed1985

Long-term study of pergolide in Parkinson's disease.

In 18 patients with Parkinson's disease, the effects of pergolide after 28 months of treatment were compared with the response after the initial 10-week therapy. At a mean 3.2-mg daily dose of pergolide, the daily dose of levodopa was still 33% lower than at the onset of pergolide therapy. The mean motor disability score, which decreased by 65% during the first 10 weeks of pergolide, was still decreased by 42% after 28 months. In the 12 patients with on-off effect, the percent time on increased 117% during the first phase of the study and was still increased 63% after more than 2 years of pergolide therapy. Sudden freezing episodes became the most disabling problem in the majority of patients. "Down-regulation" of dopamine receptors may contribute, but it is not the only cause of loss of responsiveness to pergolide.

Adult↗
PubMed1985

Familial tic disorder, parkinsonism, motor neuron disease, and acanthocytosis: a new syndrome.

We report two brothers who were of consanguineous parents and who displayed a unique association of motor and vocal tics, parkinsonism, distal muscular atrophy, and acanthocytosis. In the older brother, leg weakness and muscle wasting started at age 13, and he became wheelchair bound at 40. Electrophysiologic studies and muscle biopsy confirmed diffuse denervation. Involuntary vocalizations and facial tics began at age 36, but within 5 years the tics were replaced by progressive parkinsonism with supranuclear ophthalmoparesis. CSF studies implied impaired central dopamine and serotonin turnover. In the younger brother, orofacial tics started at age 36, vocalizations and fasciculations in the legs began 1 year later, and parkinsonian findings were present at age 40. This is the first report of an association of Tourettism, parkinsonism, motor neuron disease, and acanthocytosis occurring as an autosomal recessive syndrome.

Acanthocytes↗
PubMed1985

Metoclopramide-induced phantom dyskinesia.

A 58-year-old woman was receiving chemotherapy for carcinoma of the breast. She was given 1,700 mg metoclopramide IV for 2 months to prevent nausea and vomiting. Within hours after metoclopramide was given, she had hand tremor, akathisia, and truncal and orofacial dyskinesia. These symptoms resolved, but she was left with persistent shoulder stump chorea, the perception of the phantom left arm involuntarily adducted at the shoulder and flexed at the elbow, and dystonic pronation and extension of the hand away from her body. The motor aspects of the phantom dyskinesia will be emphasized.

Amputation, Surgical↗
PubMed1985

The heterogeneity of Parkinson's disease: clinical and prognostic implications.

In 334 patients with idiopathic Parkinson's disease, deterioration in mental status paralleled severity of bradykinesia, postural instability, and gait difficulty. Tremor was relatively independent of the other cardinal signs and was associated with relative preservation of mental status, earlier age at onset, family history of parkinsonism, and more favorable prognosis. There seem to be at least two Parkinson's subgroups: one with postural instability and gait difficulty and another with tremor as the dominant feature.

Adult↗
PubMed1984

Respiratory morbidity in wollastonite workers.

Medical and environmental surveys were conducted at a wollastonite mine and mill in 1976 and in 1982. Health testing included chest radiography, spirometry, and a questionnaire. Workers at a nearby electronics plant were also examined in 1982 for a comparison of lung function and respiratory symptoms. Both wollastonite and control workers showed significant smoking effects for chronic respiratory symptoms, but differences between the groups were not detected. Pneumoconiosis was found in 3% (3/108) of the wollastonite workers in 1982, but none showed a significant progression from their 1976 radiographs. The lung function tests of the 108 wollastonite workers examined in 1982 showed dust related changes in FEV1, FEV1/FVC ratio, and peak flow rate which were independent of age, height, and smoking habit (p less than 0.01). For non-smokers alone, only the FEV1/FVC ratio declined significantly with dust-years of exposure (p less than 0.01). The comparison of lung function in 1982 between a high dust exposed subgroup of wollastonite workers and the control population showed a significantly lower FEV1/FVC ratio and peak flow rate in the study group (p less than 0.05). Analysis of 1976-82 changes in pulmonary function showed that wollastonite workers with higher dust exposure had a significantly greater decline in peak flow over the period than workers with lower exposures (p less than 0.01). These data suggest that long term cumulative exposure to wollastonite may impair ventilatory capacity as reflected by changes in the FEV1/FVC ratio and peak flow rate.

Adult↗
PubMed1984

Hemiballism and metastatic brain tumor.

We describe a 78-year-old man with right hemiballism as the presenting symptom of a metastatic occult adenocarcinoma of the lung. CT demonstrated two contrast-enhancing lesions: one in the left subthalamic region and the other in the left parietal region. Metastatic tumor was confirmed at autopsy.

Adenocarcinoma↗
PubMed1984

Effect of tetrabenazine on tics and sleep of Gilles de la Tourette's syndrome.

Supersensitivity of dopaminergic receptors may be responsible for the tics of Tourette's syndrome. Symptoms improve after treatment with dopamine blockers, but side effects limit use of these drugs. We evaluated tetrabenazine (which has both presynaptic monoamine-depleting effects and postsynaptic blocking action) in nine patients. Marked and lasting (more than 6 months) improvement occurred in four patients (ages 10 to 14 years), mild or transient (less than 6 months) improvement occurred in three patients (ages 11 to 20 years), and two patients (age 48 years) had minimal or no response. Side effects included drowsiness in six, "nervousness" in two, depression in two, parkinsonism in one, and oculogyric crises in one, but all undesirable effects cleared with maintenance or reduction of the dosage.

Adolescent↗
PubMed1984

Blepharospasm: demographic and clinical survey of 250 patients.

The pathophysiologic mechanisms of blepharospasm are poorly understood and the condition is often misdiagnosed. In an attempt to improve our understanding of blepharospasm, 250 patients with blepharospasm were surveyed. This heretofore largest reported series of blepharospasm patients reveals findings of clinical and demographic interest. For example, there is a 3 to 1 female preponderance, the age of onset is in the fifth or sixth decade of life in two-thirds of patients, the onset is usually gradual with progressive deterioration and involuntary spasms of other portions of face and body. Although specific ocular abnormalities may predispose a patient to blepharospasm, in most cases the blepharospasm is a form of focal dystonia often associated with other involuntary movements and neurologic abnormalities. Increased awareness of blepharospasm among primary care physicians as well as ophthalmologists, neurologists, psychiatrists, and other specialists should lead to early recognition and management of this disabling condition.

Adult↗
PubMed1984

Progressive supranuclear palsy. Clinical and pharmacologic update.

A distinct clinicopathologic entity, PSP is differentiated from Parkinson's disease by the presence of supranuclear ophthalmoparesis. Downward gaze palsy that can be overcome by oculocephalic maneuver is the most characteristic clinical finding. The other distinguishing clinical features of PSP include axial distribution of rigidity and broad-based gait with early postural instability. Furthermore, pseudobulbar palsy is an early feature, while profound dementia usually occurs late in the course of the disease. Because of the variable clinical presentation and occasional lag in the onset of ophthalmoparesis and other distinguishing signs, the diagnosis of PSP is often delayed for many years. However, the constellation of axial rigidity, pseudobulbar signs, and parkinsonism without tremor when combined with ophthalmoparesis should suggest the correct diagnosis. Pathologic examination of the PSP brain reveals neuronal cell loss, gliosis, granuolvacuolar degeneration, and unique neurofibrillary tangles in the pontomesencephalic tegmentum, tectum, basal ganglia, vestibular nuclei, periaqueductal gray matter, and dentate nuclei. The etiology of this neurodegenerative disorder is unknown and the neurodiagnostic studies usually are not helpful in proving the diagnosis. The treatment of PSP is unsatisfactory, but the anti-parkinson drugs, particularly dopamine agonists, may be useful in the early stages of the disease.

Aged↗
PubMed1984

Practical management of dystonia.

The diagnosis of dystonic movements is based on the phenomenologic characteristics of the movements themselves. Therefore to evaluate and manage the patient, it is important to be cognizant of the varied clinical appearances; to classify the type of dystonia according to age at onset and distribution of the movements; to determine its etiology; to treat with specific therapy if available; and to reduce the symptoms, if therapy is not available. These steps are the focus of this article.

Age Factors↗
PubMed1983

Progressive supranuclear palsy: clinical features and response to treatment in 16 patients.

Among 415 patients with parkinsonism, 16 (3.9%) had findings of progressive supranuclear palsy (PSP). This report reviews the clinical features and response to drug therapy in those 16 patients. Anticholinergic drugs failed to benefit any of the 5 patients treated, while presynaptic dopaminergic drugs (Sinemet or amantadine) were beneficial in only 5 of 22 patient trials. Alternatively, dopamine agonists (bromocriptine and pergolide) caused improvement in 9 of 14 patient trials despite the fact that all but 1 of these patients had previously failed to respond to presynaptic dopaminergic drugs. Dopamine agonists such as bromocriptine and pergolide may be useful in some patients with PSP.

Aged↗
PubMed1983

Blepharospasm and orofacial-cervical dystonia: clinical and pharmacological findings in 100 patients.

We evaluated prospectively 100 patients, the largest reported series, with blepharospasm and orofacial-cervical dystonia, or Meige syndrome. The mean age at onset was 51.7 years, and 81% presented between the ages of 40 and 70. Women outnumbered men three to two. Blepharospasm was the initial symptom in 58 patients, but only 23 had involuntary movements localized to the orbicularis oculi. Sixty-one patients had the complete syndrome, blepharospasm and oromandibular dystonia, and 60 had neck or generalized dystonia in addition to the orofacial movements. Twenty-one patients with spasmodic dysphonia were included; in 12 of these patients, spasmodic dysphonia was part of the complete (Meige) syndrome, and 16 of these patients had neck or generalized dystonia or essential tremor. An organic cause of Meige syndrome is supported by a high correlation with essential tremor and other movement disorders and by positive family history in some patients. Response to medication was inconsistent, but 69% of patient trials resulted in some improvement; in 22% the benefit was marked and persistent. Tetrabenazine, lithium, and trihexyphenidyl were most useful for the treatment of oromandibular dystonia, and clonazepam was useful in some patients with blepharospasm.

Adult↗
PubMed1983

Controlled trial of pergolide mesylate in Parkinson's disease and progressive supranuclear palsy.

We evaluated pergolide in 22 patients with Parkinson's disease and 3 with progressive supranuclear palsy (PSP). After achieving an optimal dose of pergolide and Sinemet, a matching placebo was substituted in double-blind manner. The mean dose of levodopa (in Sinemet) was reduced by 68%; in eight patients, pergolide completely replaced levodopa. In parkinsonian patients, the mean Hoehn-Yahr stage decreased from 3.2 to 1.6, and the mean total disability score decreased from 48.3 to 17.8. In 10 patients with on-off phenomenon, the time on increased 174% with pergolide. There was little effect in PSP. Postural light-headedness and reversible mental changes were seen.

Adult↗
PubMed1983

Sleep in Gilles de la Tourette's syndrome: disorder of arousal.

Overnight polygraphic sleep studies, which included accelerometry and video monitoring, were performed on 14 Tourette patients before therapy and on 11 age-matched controls. Tourette patients less than 23 years old had a significantly increased percentage of stage 3/4 sleep, had an increased number of awakenings, had a decreased percentage of REM sleep, experienced paroxysmal events during stage 4 sleep, and had motor tics during all stages of sleep. Three patients were treated with tetrabenazine and subsequently showed significant decreases in percentage of total sleep, number of awakenings, and number of tics during sleep. These findings suggest a disorder of arousal in Tourette patients.

Adolescent↗
PubMed1983

Blepharospasm associated with brainstem lesions.

We studied six patients with clinical and radiographic evidence of rostral brainstem lesion and bilateral blepharospasm. Two patients also had other facial dystonic movements. Four patients suffered rostral brainstem strokes, and two had multiple sclerosis. None had been treated with antipsychotic drugs prior to the onset of blepharospasm. Medical treatment was helpful in two patients, and bilateral selective facial nerve section was used in another patient. Possible pathogenic mechanisms are discussed.

Adult↗