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Biomedical subjects

J Jankovic

Publications and source records attributed to J Jankovic.

At least 163 records · Page 9Linked to original sources
PubMed1997

Hemidystonia secondary to carotid artery gunshot injury.

A 9-year-old boy was accidentally shot at close range with a pistol. The bullet entered through the left anterior neck and severed the left common carotid artery. Emergency surgery was performed with an end-to-end anastomosis. He recovered gradually from severe right-sided hemiparesis. CT scans demonstrated left parietal infarction. Within months he developed right hemidystonia, which progressed over the next few years. The movement disorder was refractory to medical therapy. MR scans showed a large demarcated defect in the left parietal lobe extending to the occipital lobe, to the insula and to the posterior ventral putamen. At age 18 the patient underwent a staged left-sided thalamotomy. The hemidystonia improved postoperatively but later partially recurred.

Adolescent↗
PubMed1997

Dystonia and dyskinesia.

Dystonia is defined as a syndrome of sustained muscle contractions, frequently causing twisting and repetitive movements, or abnormal postures. Tardive dyskinesia (TD), estimated to occur in 30% of patients treated with neuroleptics, encompasses a broad spectrum of hyperkinesias associated with exposure to these drugs. Dystonia is one of the most common expressions of tardive dyskinesia. This article aims to provide an overview of classification, phenomenology, epidemiology, genetics, pathophysiology, neuropsychologic aspects, and treatment of these two conditions.

Antipsychotic Agents↗
PubMed1997

Tourette syndrome. Phenomenology and classification of tics.

Motor and phonic tics represent the clinic hallmark of Tourette syndrome. Since there is no diagnostic test for tics, the clinical recognition of phenomenology is essential for proper diagnosis. Some, and possibly most, motor tics are preceded by a premonitory urge or sensation that is relieved by the execution of the tic and thus can be difficult to differentiate from compulsions, hence the term compulsive tic. In the schema of categorization of movements, most tics can be classified as either unvoluntaryin response to an inner sensory stimulus or to an unwanted feeling compulsionor involuntarytics that are usually suppressible. Further studies are needed to elucidate the clinical, anatomical, and physiologic substrates for the different forms of tics. Finally, the relationship between tics and comorbid conditions, such as obsessive-compulsive disorder and attention deficit with hyperactivity needs to be further explored.

Humans↗
PubMed1997

Symptomatic and functional outcome of surgical treatment of cervical dystonia.

OBJECTIVES: Previous studies of surgical treatment for cervical dystonia have reported highly variable rates of postoperative symptomatic benefit and morbidity. Little is known about functional improvement and long term results. This study evaluates the symptomatic and functional outcome of surgical treatment of cervical dystonia in a consecutive series of 46 patients. METHODS: The most affected muscles were selected for denervation after clinical examination and confirmation by four channel EMG studies. Surgical treatment, aiming at selective elimination of pathological activity while preserving normal motor function and avoiding side effects, was achieved by using a broad scope of techniques including intradural denervation, extradural denervation, and myotomy. Rather than carrying out standard operations, the treatment was tailored to the needs of the patient according to the individual pattern of dystonic activity. Long term benefit was assessed with a global outcome score, and a modified Toronto western spasmodic torticollis rating scale (TWSTRS) in those 34 patients who were available for a recent follow up evaluation. RESULTS: The 46 patients underwent a total of 70 procedures with intradural approaches in 33 instances, extradural approaches in 21, and muscle sections (singly or combined) in 22 instances. Transient mild postoperative side effects occurred in 10% of the procedures. The mean duration of long term follow up was 6.5 years. The global outcome was rated as excellent in nine patients (21%), as marked in 12 (27%), as moderate in nine (21%), as mild in nine (21%), and as no improvement in five (11%). A persistent side effect consisting of mild difficulty with balance was noted in one case. There were highly significant changes of the preoperative and postoperative mean values for almost all TWSTRS subscores for severity of cervical dystonia, functional disability, and pain. Patients with excellent outcome underwent a higher number of surgical procedures on average than those patients who achieved no benefit. CONCLUSIONS: Surgical treatment tailored to the specific pattern of dystonic activity in the individual patient is a valuable alternative in the long term management of cervical dystonia.

Adolescent↗
PubMed1997

Accuracy of the clinical diagnosis of corticobasal degeneration: a clinicopathologic study.

The accuracy of the clinical diagnosis of corticobasal degeneration (CBD) is unknown. To determine its diagnostic accuracy, we presented 105 cases with known neuropathologic diagnoses, including CBD (n = 10), progressive supranuclear palsy (PSP, n = 24), Parkinson's disease (n = 15), diffuse Lewy body disease (n = 14), multiple system atrophy (n = 16), postencephalitic parkinsonism (n = 7), Pick's disease (n = 7), Creutzfeldt-Jakob disease (n = 4), Alzheimer's disease (n = 4), vascular parkinsonism (n = 3), and Whipple's disease (n = 1), as clinical vignettes to six neurologists unaware of the autopsy findings. Reliability was measured with the kappa statistics. The neurologists' clinical diagnoses were compared with clinicopathologic diagnoses for sensitivity, specificity, and positive predictive values at first and last clinic visits. The group reliability for the diagnosis of CBD significantly improved from moderate for the first visit (mean = 34 months after onset) to substantial for the last (68 months after onset). For the first visit, mean sensitivity for CBD was low (35%), but specificity was near-perfect (99.6%). For the last visit, mean sensitivity minimally increased (48.3%), and specificity remained stable. False-negative misdiagnoses mainly occurred with PSP. False-positive diagnoses were rare. The extremely low sensitivity of the clinical diagnosis of CBD suggests that this disorder is markedly underdiagnosed. Although the validity of the clinical diagnosis might have been improved if neurologists could have examined these patients, more important is that this disorder was misdiagnosed by the primary neurologists. In our data set, the best predictors for the diagnosis of CBD included limb dystonia, ideomotor apraxia, myoclonus, and asymmetric akinetic-rigid syndrome with late onset of gait or balance disturbances.

Basal Ganglia↗
PubMed1997

Long-term effects of tetrabenazine in hyperkinetic movement disorders.

Over the past 15 years we have treated 526 patients with severe hyperkinetic movement disorders with tetrabenazine (TBZ), a monoamine-depleting and a dopamine-receptor-blocking drug. We report here the results in 400 patients with adequate follow-up. The response was rated on a scale of 1 to 5 (1 = marked improvement, 4 = no response, 5 = worsening) and was assessed initially and at the last clinic visit. The average duration of TBZ treatment was 28.9 months (+/- 31.1; range, 0.25 to 180 months). The global response rating of 1 (marked improvement) was recorded in 89.2% of 93 patients with tardive stereotypy, 83.3% of 12 with myoclonus, 82.8% of 29 with Huntington's disease, 80.5% of 82 with tardive dystonia, 79.3% of 29 with other movement disorders, 62.9% of 108 with idiopathic dystonia, and in 57.4% of 47 with Tourette's syndrome. The most common side effects included drowsiness (36.5%), parkinsonism (28.5%), depression (15.0%), insomnia (11.0%), nervousness or anxiety (10.3%), and akathisia (9.5%). The side effects were controlled with reduction in the dosage. TBZ is an effective and safe drug for the treatment of a variety of hyperkinetic movement disorders. In contrast to typical neuroleptics, TBZ has not been demonstrated to cause tardive dyskinesia.

Adult↗
PubMed1997

What are the obstacles for an accurate clinical diagnosis of Pick's disease? A clinicopathologic study.

Several studies have evaluated the reliability and validity of the clinical diagnosis of Alzheimer's disease (AD) using well-defined neuropathologic criteria, but none has attempted to evaluate the diagnostic accuracy of Pick's disease. We determined the accuracy of the clinical diagnosis of Pick's by presenting 105 autopsy-confirmed cases of Pick's (n = 7) and related disorders (non-Pick's, n = 98) as clinical vignettes in randomized order to six neurologists who were unaware of the autopsy findings. The group of raters had moderate to fair agreement for the diagnosis of Pick's as measured by the kappa statistics. The sensitivity for the diagnosis of Pick's for the first visit (mean, 53 months after onset) and last visit (mean, 78 months after onset) was low (range, 0 to 71%), but specificity was near-perfect. Median positive predictive values at both visits were 83 to 85%. False-negative misdiagnoses mainly involved AD. False-positive diagnoses were rare and occurred with corticobasal degeneration (first visit) and with dementia with Lewy bodies (last visit). Pick's was also misdiagnosed by primary neurologists. The best clinical predictors for the early diagnosis of Pick's included "frontal" dementia, early "cortical" dementia with severe frontal lobe disturbances, absence of apraxia, and absence of gait disturbance at onset. However, the first neurologic evaluation in some of the Pick's cases took place in advanced stages of the disease. Our findings suggest that this disorder is underdiagnosed in clinical practice. Although the low sensitivity for the clinical diagnosis of Pick's is disappointing, our data suggest that when clinicians suspect Pick's, their diagnosis is almost always correct. Absence of awareness of the main features of this disorder and of specificity of the frontal lobe syndrome may partially explain the low detection of Pick's disease.

Aged↗
PubMed1997

Botulinum toxin type B: a double-blind, placebo-controlled, safety and efficacy study in cervical dystonia.

We enrolled and treated 122 patients with idiopathic cervical dystonia in a double-blind, placebo-controlled safety and efficacy study of botulinum toxin type B (BotB). Both A-responsive and A-resistant patients were enrolled. Patients received intramuscular injections of either BotB (2,500 U, 5,000 U, or 10,000 U) or placebo. The primary outcome measure of efficacy was the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS)-Total score at 4 weeks following study drug administration. Secondary measures of efficacy were TWSTRS-Severity, -Disability, and -Pain subscale scores, and Analog Pain Assessment, Investigator Global Assessment, Patient Global Assessment, and Sickness Impact Profile scores. Duration of effect was estimated with an intent-to-treat analysis of responders. Safety measures included clinical parameters, laboratory tests, and adverse events. The primary and most of the secondary analyses indicated a statistically significant treatment effect and a dose response. BotB is safe, well tolerated, and efficacious in the treatment of cervical dystonia at the doses tested.

Adult↗
PubMed1997

Microelectrode-guided posteroventral pallidotomy for treatment of Parkinson's disease: postoperative magnetic resonance imaging analysis.

The authors report the postoperative magnetic resonance (MR) imaging findings in 36 patients with advanced Parkinson's disease who underwent unilateral microelectrode-guided posteroventral pallidotomy. The lesions were placed within 1 mm of the ventral border of the globus pallidus internus (GPi) to include pallidothalamic outflow pathways. Sequential MR studies were obtained within 1 to 3 days postoperatively and at 6-month follow-up examination. Thirty-four (94%) of the 36 patients enjoyed sustained moderate or marked improvement of their parkinsonian symptoms 6 months postoperatively. Transient side effects occurred in five patients (14%), but there were no persistent complications. The pallidal radiofrequency lesions were prolate spheroid shaped and were composed of three concentric zones in the early postoperative studies. The mean volume of the middle zone, corresponding to the area of hemorrhagic coagulation necrosis, was 44.4 +/- 17.6 mm3; the mean lesion volume as defined by the outer zone, corresponding to perilesional edema, was 262.2 +/- 111.6 mm3. Additional edema spreading to the internal capsule was noted in 32 of 34 cases and to the optic tract in 11 of 34 cases. In two patients small ischemic infarctions involving the corona radiata were found, and in one a venous infarction was detected. Ischemic infarction resulted in mild transient Broca's aphasia in one patient, but there was no detectable neurological deficit in the other two. The mean volume of late-phase (6 months) lesions was 22 +/- 28.8 mm3. In three patients no lesion was identified despite sustained clinical improvement. The lesion was located in the posteroventral GPi in all cases except in one patient in whom it was confined to the GP externus (GPe). This 49-year-old woman did not experience sustained benefit. The authors found no consistent correlations between lesion size and location and clinical outcome as measured by a global outcome score, the Unified Parkinson's Disease Rating Scale motor, activities of daily living, and bradykinesia "off" scores or rating of dyskinesias. Lesioning of pallidal and subpallidal pathways may contribute to the sustained clinical benefit in this series. Magnetic resonance imaging analysis showed that intraoperative microelectrode recording facilitated accurate placement of the lesion in this critical area.

Adult↗
PubMed1997

[Medial posteroventral pallidotomy for the treatment of Parkinson's disease].

Stereotactic medial posteroventral pallidotomy for treatment of Parkinson's disease attracts increasing attention. We report on the preliminary results of 12 patients at 1 year after microelectrode-guided unilateral pallidotomy. The primary indications were severe bradykinesia and levodopa-induced dyskinesias. After radiofrequency lesioning all patients had immediate improvement of contralateral parkinsonian signs. Postoperative magnetic resonance imaging confirmed the localization of the lesions. At the 1-year follow-up, all patients had sustained benefit. The global improvement was rated as moderate in six cases, and as marked in six other cases. The mean values of various subscores of the Unified Parkinson's Disease Rating Scale (UPDRS) showed highly significant changes in the "off" state (pre/postoperatively): UPDRS Motor score (60.3/31). UPDRS Activities of Daily Living (ADL) score (33.2/18.3), gait/postural stability score (13.8/7.0), and subscores for contralateral rigidity (4.9/2.1), tremor (7.1/1.4) and bradykinesia (11.6/5.3). There was also significant improvement of ipsilateral bradykinesia and rigidity. Furthermore, we found significant changes of the mean values of the UPDRS ADL and motor "on" scores, an increase of the percentage of "on" time with reduced on-off fluctuations, and a decrease of the percentage of levodopa-induced dyskinesias, with marked improvement or complete abolition of contralateral dyskinesias in particular. The preoperative levodopa regimen was maintained, in general, or only slightly modified, if necessary. Two patients had transient complications: one patient suffered postoperative pneumonia and altered mental status; another patient displayed mild Broca's aphasia secondary to a small stroke involving the dorsal thalamus and the adjacent white matter. There were no persistent side effects at the 1-year follow-up. Contemporary unilateral pallidotomy is an effective and promising therapeutical option for surgical treatment of advanced Parkinson's disease.

Adult↗
PubMed1997

Botulinum toxin: historical perspective and potential new indications.

Botulinum toxin (BTX), a purified form of the neurotoxin responsible for botulism, is used worldwide for the treatment of abnormal muscle contractions. The ability of BTX to block acetylcholine release in a long-lasting but reversible fashion with few side effects has made it an important tool in a wide variety of neuromuscular disorders, including the dystonias, tremor, tics, and spasticity. There are seven antigenically distinct toxins. BTX-A has been in clinical use for almost two decades, with an outstanding efficacy and safety profile. Clinical effects are often seen within 1 week of injection, and benefits typically last from 3-6 months. Patient selection and the proper choice of dose and administration site are the most important determinants of a favorable response to BTX treatment.

Anti-Dyskinesia Agents↗
PubMed1996

Molecular detection of new mutations, resolution of ambiguous results and complex genetic counseling issues in Huntington disease.

Huntington disease (HD) is an autosomal dominant neurodegenerative disorder caused by expansion of a variable length (CAG)n repeat in the 5' coding region of a novel gene on chromosome 4p16.3. We provide comprehensive molecular analysis of a sporadic case of HD in which a paternally derived normal length allele expanded to an affected length allele. Linkage analysis and paternity testing confirm the paternal origin of the expansion and demonstrate that unequal crossing over during meiosis is an unlikely mechanism for de novo expansion in HD. This case identifies a complex genetic counseling issue for the families of sporadic cases since calculations of recurrence risk are not possible at this time. In addition, we describe utilization of a combination of polymerase chain reaction (PCR) based assays for examination of both the CAG repeat and an adjacent variable length CCG repeat in the huntingtin gene. The combination of these assays can increase the accuracy of molecular diagnosis for HD and may clarify any ambiguous results obtained during molecular testing of HD families.

Female↗
PubMed1996

A randomized, double-blind, placebo-controlled study to evaluate botulinum toxin type A in essential hand tremor.

Twenty-five patients with hand tremor of 2+ (moderate) to 4+ (severe) on the tremor severity rating scale were randomized to receive either 50 U of botulinum toxin (BTX) type A (Allergan, Irvine, CA) or placebo injections into the wrist flexors and extensors of the dominant limb. If patients failed to respond to the initial injection, they were eligible to receive another injection of 100 U 4 weeks later. Rest, postural, and kinetic tremors were evaluated at 2- to 4-week intervals over a 16-week study period using tremor severity rating scales, accelerometry, and assessments of improvement and disability. A significant improvement (p < 0.05) was observed on the tremor severity rating scale 4 weeks after injection in patients treated with BTX as compared with placebo, and this effect was maintained for the duration of the study. Four weeks after injection, 75% of BTX-treated patients vs. 27% of placebo-treated patients (p < 0.05) reported mild to moderate improvement (peak effect rating > or = 2). There were no significant improvements in functional rating scales, although trends were observed for some items. Postural accelerometry measurements showed a > or = 30% reduction in amplitude in nine of 12 BTX-treated subjects and in one of nine placebo-treated subjects (p < 0.05). Although all patients treated with BTX reported some degree of finger weakness, no severe, irreversible, or unexpected adverse events occurred. Chemodenervation with BTX may significantly ameliorate essential hand tremor in patients who fail to improve with conventional pharmacologic therapy.

Aged↗
PubMed1996

Botulinum toxin injection into vocal cord in the treatment of malignant coprolalia associated with Tourette's syndrome.

We describe a 13-year-old boy with Tourette's syndrome (TS) manifested chiefly by severe coprolalia, obsessive compulsive disorder, and attention deficit hyperactivity disorder. He was treated with unilateral injections of botulinum toxin to the vocal cord and experienced marked improvement in coprolalia as well as marked reduction in the premonitory urges associated with the vocal tics and coprolalia. As a result of the improvement, the patient was able to attend school and church and was able to socialize. Botulinum toxin may interfere with a sensory reflex arc required to perpetuate tics and coprolalia in TS, possibly by reducing local build-up of tension or muscle contraction in affected areas.

Adolescent↗
PubMed1996

Severe motor tics causing cervical myelopathy in Tourette's syndrome.

We describe two patients with Tourette's syndrome in whom severe motor tics involving the neck were complicated by cervical myelopathy. The first patient, a 21-year-old man, had complex tics consisting of violent twisting and extending movements of the neck preceded by an irresistible urge to produce the abnormal postures. Two years after onset of these tics, paraesthesias, sensory deficits up to the level of C4, and a gait disturbance developed. Neuroimaging studies confirmed compressive myelopathy, and the symptoms gradually improved with botulinum toxin injections in the posterior cervical muscles. The second patient, a 42-year-old man, had violent clonic tics consisting of thrusting head jerks. The repetitive flexion-extension neck movements had been present since the age 10 years. At age 23, he developed progressive weakness of all four extremities and bladder and sexual dysfunction. Myelography demonstrated cervical spinal canal stenosis with retrospondylosis from C3 through C5 and a Klippel-Feil malformation. After cervical decompression by C3-C5 laminectomies, his spinal cord symptoms improved temporarily. The tics, however, continued, and the neurologic deficits of cervical myelopathy progressed again after age 34. He did not benefit from a second operation. This report draws attention to the possibility that some tics can produce disabling compressive myelopathy. Early diagnosis and treatment of the movement disorder is essential in such cases.

Adult↗