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Biomedical subjects

J Jankovic

Publications and source records attributed to J Jankovic.

At least 307 records · Page 17Linked to original sources
PubMed1986

Familial spastic paraparesis and deafness. A new x-linked neurodegenerative disorder.

We studied a large kindred with a chronic neurodegenerative disorder, affecting at least six male members in three generations. Spastic paraparesis, beginning at about 10 years of age, and hearing deficits were present in all affected members. Additionally, tremor ophthalmologic abnormalities, sensory deficits, short stature, hypogonadism, elevated cerebrospinal fluid protein, and absent or prolonged somatosensory evoked potentials were seen in some relatives. Although clinically similar to adrenomyeloneuropathy, the plasma and fibroblast levels of saturated very long-chain fatty acids were normal. This syndrome probably represents a new type of familial spastic paraparesis.

Adolescent↗
PubMed1986

Segmental myoclonus. Clinical and pharmacologic study.

We observed 37 patients (mean age at onset, 48.5 years; range, 13 to 84 years) with segmental myoclonus (18 branchial, 19 spinal). Etiologies for branchial myoclonus included brain-stem demyelination, cerebrovascular disease, Meige's syndrome, cerebral arteritis secondary to bacterial meningitis, central nervous system Whipple's disease, acute cervicomedullary trauma, and cerebellar degeneration. Spinal myoclonus was associated with laminectomy, remote effect of cancer, spinal cord injury, post-operative pseudomeningocele, laparotomy, thoracic sympathectomy, poliomyelitis, herpes myelitis, lumbosacral radiculopathy, spinal extradural block, and myelopathy due to demyelination, electrical injury, acquired immunodeficiency syndrome, and cervical spondylosis. The latency between the predisposing condition and the onset of myoclonus ranged from immediate to 33 years (mean, 2.9 years). In six patients, the myoclonus was the presenting symptom of a serious underlying disease. Treatment with clonazepam, tetrabenazine hydrochloride, or other medications provides a satisfactory control in most patients.

Adolescent↗
PubMed1986

Parkinson's disease in monozygotic twins.

Recent studies of twins have demonstrated an unexpectedly low concordance of Parkinson's disease in monozygotic twins. Only two monozygotic twin pairs concordant for it have been reported. However, both pairs were atypical because of an early age at onset and other unusual features. We studied a monozygotic twin pair concordant for typical Parkinson's disease. The brothers have lived apart for forty years. The onset of tremor occurred three months apart, at age 71. The progression of the symptoms has been identical. Although one of the twins is more severely affected, both have typical manifestations of Parkinson's disease that respond well to dopaminergic medication. The occurrence of Parkinson's disease in these monozygotic twins suggests that genetic susceptibility is important in the tremor-dominant variety of Parkinson's disease.

Aged↗
PubMed1986

Parkinsonism and acquired hydrocephalus.

Left hemiparkinsonism was the first symptom of increased intracranial pressure in a 14-year-old girl. The parkinsonism resolved when the increased intracranial pressure was relieved by a ventriculo-peritoneal shunt. This is the first case of parkinsonism associated with hydrocephalus caused by non-neoplastic aqueductal stenosis.

Adolescent↗
PubMed1986

Arteriovenous malformation presenting as hemidystonia.

Two men, aged 25 and 33 years, had progressive hemidystonia and an arteriovenous malformation (AVM) in the contralateral cerebral hemisphere. One patient with an AVM in the posterior basal ganglia of the right hemisphere had an older brother with severe generalized dystonia. The second patient had an AVM in the left cortical and subcortical parietal area with no obvious lesion in the basal ganglia. Unlike generalized dystonia, a focal lesion is commonly found in patients with unilateral dystonia. The association of AVM-induced hemidystonia and family history of dystonia suggests that genetic predisposition may be important in some patients with hemidystonia.

Adult↗
PubMed1986

Hyperkinetic mutism: bilateral ballism and basal ganglia calcification.

We studied a 70-year-old woman with a unique combination of hyperkinesia and mutism. These findings differed from akinetic mutism because there was continuous bilateral ballism and dystonia--hence the term "hyperkinetic mutism." CT demonstrated bilateral calcifications in the basal ganglia, and MRI indicated bilateral watershed infarcts. Different dopaminergic mechanisms may underlie the hyperkinesia and mutism.

Aged↗
PubMed1985

Association between essential tremor and Parkinson's disease.

To examine a possible relationship of essential tremor to Parkinson's disease (PD), 130 patients with essential tremor were studied. Twenty-five patients had both essential tremor and PD. These 25 patients were matched for age and duration of PD symptoms with 25 patients who had idiopathic PD. Bradykinesia, postural instability, and gait difficulty were more severe in the patients with idiopathic PD. Degree of tremor, rigidity, and functional disability did not differ in the two groups. The prevalence of PD in the population with essential tremor was 24 times greater than expected. This study suggests that some patients with essential tremor have a genetically increased risk for PD.

Adolescent↗
PubMed1985

Low cancer rates among patients with Parkinson's disease.

Among 406 patients with Parkinson's disease, the cancer rate (all sites combined) was bout one-third that for the general population. The risk of cancer increased during the treatment period but remained significantly low. Malignant and benign thyroid neoplasms were significantly more frequent than expected among patients with Parkinson's disease. We suggest that high levels of total body potassium in patients with Parkinson's disease is the protective factor against cancer.

Age Factors↗
PubMed1985

Herpes zoster ophthalmicus followed by contralateral hemiparesis: report of two cases and review of literature.

Two patients with herpes zoster ophthalmicus and contralateral hemiparesis are described, and their findings compared with 49 patients previously reported. These patients presented with delayed contralateral hemiparesis approximately seven weeks after the onset of herpes zoster ophthalmicus. Most patients had evidence of infarction of the ipsilateral middle cerebral artery by angiography or by CT scan. Cerebrospinal fluid pleocytosis and elevated protein commonly were found. Twenty per cent of the reported patients died, but they were older than the patients who survived and predisposed to have diffuse CNS lesions. The pathogenesis of this syndrome is thought to be due to direct viral invasion of the blood vessel wall with resulting angiitis. Further studies need to be performed to clarify the role of specific antiviral therapy or anti-inflammatory agents in treating this complication of herpes zoster.

Adult↗
PubMed1985

Hemidystonia: a report of 22 patients and a review of the literature.

Hemidystonia defined as involuntary, sustained posturing of the unilateral arm, leg, and face was studied in 12 male and 10 female patients. Hemidystonia was caused by cerebrovascular disease in eight patients, perinatal trauma or childhood injury in four, head trauma and its sequelae in three, neuronal storage disorders in two, neurodegenerative disease in two, lesions after thalamotomy in two, and presumed encephalitis in one. Sixteen patients (73%) had CT evidence of contralateral basal ganglia damage, history of hemiparesis, or both. Brain damage before 7 years of age produced contralateral hemidystonia with a mean delay of 9-7 years. In older patients hemidystonia appeared within 6 months after injury. Hemidystonia may result from a disconnection between the striatum and the thalamus with relative preservation of the corticospinal pathways.

Adolescent↗
PubMed1985

Placebo-controlled study of mesulergine in Parkinson's disease.

We used a new D2 dopamine agonist, mesulergine (8-alpha-amino-ergoline, CU 32-085), to treat 20 patients (12 men and 8 women), mean age 62.6 (SEM = 1.7) and mean duration of illness 5.9 (SEM = 1.0) years. Wearing-off effect was the principal indication for new therapy in 15 patients, and the others had inadequate response to levodopa. All continued on levodopa therapy, and 10 patients were studied in a double-blind controlled test. The mean motor disability decreased from 2.8 (SEM = 0.12) to 1.6 (SEM = 0.18) with mesulergine (p less than 0.0001) and increased to 1.9 (SEM = 0.20) with placebo (p less than 0.001). Tremor improved most, followed by rigidity, bradykinesia, gait, and postural instability. Side effects included dyskinesia, light-headedness, hallucinations, nausea, vomiting, drowsiness, and ankle edema, but, in general, mesulergine was tolerated well.

Aged↗
PubMed1985

Late-onset Hallervorden-Spatz disease presenting as familial parkinsonism.

We studied a 68-year-old man who died after 13 years of progressive dementia, rigidity, bradykinesia, mild tremor, stooped posture, slow and shuffling gait, dystonia, blepharospasm, apraxia of eyelid opening, anarthria, aphonia, and incontinence. At autopsy, he had generalized brain atrophy with large deposits of iron pigment in the globus pallidus, caudate, and substantia nigra. Axonal spheroids were found in the globus pallidus, substantia nigra, medulla, and spinal cord. The neurochemical analysis of the brain revealed marked loss of dopamine in the nigral-striatal areas, with relative preservation of dopamine in the limbic areas. This is the oldest case of familial Hallervorden-Spatz disease reported and the first with neurochemical analysis of the brain.

Aged↗