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J Jankovic

Publications and source records attributed to J Jankovic.

At least 91 records · Page 5Linked to original sources
PubMed2000

Botulinum toxin in the treatment of tics.

OBJECTIVE: To evaluate the safety and efficacy of botulinum toxin A (BTX) injections in the treatment of tics in patients with Tourette syndrome (TS). BACKGROUND: BTX is an effective treatment for an increasing number of conditions characterized by abnormal muscle contractions. BTX may improve not only the motor component of tics, but also premonitory sensations that precede tics. METHODS: Thirty-five patients (30 male, 5 female) were treated with BTX in the sites of their most problematic tics. Response to BTX was based on a 0 to 4 clinical rating scale (0, no improvement, to 4, marked improvement in both severity and function). Questionnaires were administered to evaluate patients' impressions of overall efficacy and degree of benefit with premonitory sensations. RESULTS: Mean duration of tics prior to initial injection was 15.3 years (range, 1-62 years) and mean duration of follow-up was 21.2 months (range, 1. 5-84 months). The mean peak effect response in 35 patients treated in 115 sessions was 2.8 (range, 0-4); the mean duration of benefit was 14.4 weeks (maximum, 45 weeks); and the mean latency to onset of benefit was 3.8 days (maximum, 10 days). Twenty-one (84%) of 25 patients with premonitory sensations derived marked relief of these symptoms (mean benefit, 70.6%). Total mean dose was 502.1 U (range, 15-3550 U); mean number of visits, 3.3 (range, 1-16); and mean dose per visit, 119.9 U (range, 15-273 U). Sites of injections were as follows: cervical or upper thoracic area (17), upper face (14), lower face (7), vocal cords (4), upper back and/or shoulder (3), scalp (1), forearm (1), leg (1) and rectus abdominis (1). Complications included neck weakness (4), dysphagia (2), ptosis (2), nausea (1), hypophonia (1), fatigue (1), and generalized weakness (1), which were all mild and transient. CONCLUSIONS: Botulinum toxin A injections are an effective and well-tolerated treatment of tics. In addition to improving the motor component of tics, BTX also provides relief of premonitory sensations. Arch Neurol. 2000;57:1190-1193

Adolescent↗
PubMed2000

Gabapentin for essential tremor: a multiple-dose, double-blind, placebo-controlled trial.

Essential tremor (ET) is a common movement disorder that often becomes refractory to conventional pharmacologic management. Open-label studies suggest that gabapentin is efficacious for ET, but the results of controlled trials have been mixed. To determine the efficacy and tolerability of gabapentin in ET, we conducted a double-blind, placebo-controlled, cross-over trial evaluating two doses (1800 mg per day and 3600 mg per day; N = 25). Patients on other ET medications were maintained on their concurrent medications for 3 months prior to study initiation and throughout the study. Twenty patients (mean age, 69.9 +/- 6.1 yrs) completed the study. Overall, patient global assessments (p <0.05), observed tremor scores (p <0.005), water pouring scores (p <0.05), and activities of daily living scores (p <0.005) significantly improved. Accelerometry scores, spirographs, and investigator global impression scores did not improve. The results were similar for high and low doses. Statistical regression models did not demonstrate any significant predictors for response. Gabapentin may be effective in some cases of ET.

Acetates↗
PubMed2000

Niemann-Pick disease type C: two cases and an update.

We describe two patients with juvenile-onset Niemann-Pick disease type C (NPC) to illustrate the variable neurologic features of this condition. One presented with hypersplenism at age 10 and was misdiagnosed with Gaucher disease. He developed complex partial seizures in his teens but remained otherwise neurologically asymptomatic until his mid 30s. At age 45, he had mild dementia and dysarthria, vertical supranuclear ophthalmoplegia, axonal sensorimotor polyneuropathy, and cerebellar ataxia. The second patient presented with rapidly progressive dystonia at age 8, and mild hepatosplenomegaly, vertical supranuclear ophthalmoplegia, severe behavioral disorder, and dementia by age 14. The diagnosis of NPC was based on deficient cholesterol esterification and excessive lysosomal filipin staining in cultured skin fibroblasts. Current notions about diagnosis and pathogenesis of NPC are reviewed.

Adolescent↗
PubMed2000

Antioxidant property of pramipexole independent of dopamine receptor activation in neuroprotection.

Pramipexole and several other dopamine agonists have been recently demonstrated to have neuroprotective effects in vitro and in vivo. We report here that pramipexole can protect DAergic cell line MES 23.5 against dopamine (DA), 6-hydroxydopamine (6-OHDA), and hydrogen peroxide (H2O2)-induced cytotoxicity in vitro possibly through antioxidant effects, and such neuroprotection could not be blocked by selective D2 or D3 antagonists. Incubation with pramipexole (5-20 microM) in MES 23.5 cell cultures increased cellular levels of glutathione (GSH), and elevated glutathione peroxidase (GSH-Px) and catalase activities, but only marginally enhanced SOD activity. Pretreatment with D2 or D3 antagonists did not block the stimulating effects of pramipexole on the cellular levels of GSH, and on the activities of GSH-Px and catalase in MES 23.5 cells. These results indicate that pramipexole's neuroprotective effects are likely associated with its antioxidant property independent of D2/D3 receptor activation.

Animals↗
PubMed2000

Weight gain following unilateral pallidotomy in Parkinson's disease.

OBJECTIVE: To determine the clinical correlates and infer pathogenesis of weight gain following pallidotomy in patients with Parkinson's disease (PD). BACKGROUND: Surgical ablation of the globus pallidus internus (GPi) improves levodopa induced dyskinesias, moderately improves most other "cardinal" manifestations of PD, and has been noted to result in increased weight. METHODS: We incorporated Unified Parkinson's Disease Rating Scales (UPDRS) subscales, the Beck depression inventory and feeding questionnaire data into a linear regression model in order to determine which post-surgical change(s) may lead to weight gain over the first year following pallidotomy, n = 60. RESULTS: The mean weight gain 1 year after pallidotomy was 4.0 +/- 4.1 kg. Improvement in "off" motor scores (P < 0.005), especially gait subscores (P<0.0001), and to a lesser extent improvement in "on" motor scores (P<0.05) predicted weight gain. Changes in dyskinesia ratings, mood, food intake, dysphagia, levodopa dose, weight loss in the year prior to pallidotomy, age, and duration of PD did not correlate with subsequent weight gain. CONCLUSION: The high correlation between post-pallidotomy weight gain and "off" motor scores, suggests that this phenomenon is related to some change in underlying homeostasis associated with changes in the cardinal manifestations of PD itself, rather than secondary changes resultant from the surgery.

Dyskinesia, Drug-Induced↗
PubMed2000

Postural control in Parkinson's disease after unilateral posteroventral pallidotomy.

Postural control changes were studied in 27 patients with Parkinson's disease after unilateral posteroventral pallidotomy (PVP). Patients were evaluated before PVP and at 3, 6 and 12 months post-PVP, both 'off' and 'on' parkinsonian medications, with selected evaluation tools representing functional performance, functional balance and posturographic components of balance. The majority of variables in the 'off' state were significantly improved at 3 months post-PVP. Improvement was maintained at 6 months but had declined for some variables by the 12 month follow-up. Standing up from a chair (P = 0.009), the balance and gait sections of the Performance-Oriented Assessment (P < or = 0.0004), and the limits of stability (LOS) posturography variables (P < 0.0005) of the average time to reach a target, the number of targets missed and the initial excursion distance to the target (P = 0.029) retained significant improvement at the 12 month follow-up. When the patients were in the 'on' state, LOS posturography variables of average time to target, average path length deviation, and the number of targets missed were the only variables significantly improved at 3 months post-PVP (P = -0.013) and this improvement was maintained at 12 months post-PVP (P = 0.012-0.041). Unilateral PVP improves axial symptoms of Parkinson's disease involved in functional performance such as gait disturbance as well as improving postural stability in the 'off' state. Generally, the maximum improvement is seen at 3 months post-PVP with many variables remaining significantly improved at 12 months post-PVP. Axial dyskinesias in the 'on' state are also significantly reduced with the improvement maintained at 12 months post-PVP. These findings suggest that unilateral pallidotomy is not only effective in abolishing levodopa-induced dyskinesias, but that it also improves the axial signs of Parkinson's disease.

Aged↗
PubMed2000

Tardive and idiopathic oromandibular dystonia: a clinical comparison.

OBJECTIVE: Most patients with tardive dystonia have a focal onset involving the cranial-cervical region. Because of its resemblance to idiopathic cranial dystonia, a common form of dystonia, it often poses a diagnostic problem. To compare clinical features and response to botulinum toxin (BTX) injections between patients with tardive and idiopathic oromandibular dystonia (OMD). METHODS: Patients seen in a movement disorder clinic who satisfied the inclusion criteria for tardive or idiopathic OMD were studied. The clinical variables and responses to BTX between the two groups of patients were compared. In the tardive group, we also compared the clinical variables between those with oro-facial-lingual stereotypies, and those without. RESULTS: Twenty four patients with tardive OMD and 92 with idiopathic OMD were studied. There were no differences in the demographic characteristics. Most were women, with duration of symptoms longer than 8 years. The mean duration of neuroleptic exposure was 7.1 (SD 7.9) years. Jaw closure was the most frequent subtype of OMD (tardive=41.7%, idiopathic=51.1%). Idiopathic patients were more likely to have coexistent cervical dystonia (p<0.05), whereas isolated OMD was significantly higher in tardive patients (p<0.05). Limb stereotypies, akathisia, and respiratory dyskinesia were seen only in the tardive OMD. Frequency of oro-facial-lingual stereotypy was significantly higher in the tardive than the idiopathic group (75.0% v 31.5%, p<0.0001). The peak effect of BTX was similar in both groups. CONCLUSIONS: Oro-facial-lingual stereotypies were significantly more frequent in the tardive than the idiopathic group. Presence of stereotypic movements in the limbs, akathisia, and respiratory dyskinesias in patients with OMD strongly suggests prior neuroleptic exposure. Dystonia in tardive OMD is more likely to be restricted to the oromandibular region, whereas in patients with idiopathic OMD, there is often coexistent cervical dystonia. BTX is equally effective in both groups of patients.

Adult↗
PubMed2000

Neuropsychological outcome after unilateral pallidotomy for the treatment of Parkinson's disease.

OBJECTIVE: To assess the long term cognitive outcome of unilateral posteroventral pallidotomy (PVP) and the overall efficacy of the surgery. METHODS: Forty two (29 left and 13 right PVP) patients with Parkinson's disease underwent neurological and neuropsychological testing before PVP and at 3 and 12 months after PVP. The neuropsychological testing battery emphasised measures of verbal learning and memory, visuospatial abilities, speed of information processing, executive functioning, and affective functioning. RESULTS: All patients demonstrated motor improvements after surgery during their off state, and 86% of patients also showed improvements in motor functioning in their on state. Repeated measures ANOVA showed significant improvements in confrontational naming, visuospatial organisation, and affective functioning 3 months and 12 months after surgery, with inconsistent improvements in executive functioning 12 months post-PVP. Patients demonstrated a transient impairment in verbal memory, with verbal learning performance returning to baseline 12 months post-PVP after a significant decline 3 months after PVP. When three patients with lesions extending outside of the PVP were excluded from the analysis, a decline in verbal fluency performance after PVP was not found to be significant. Differences due to side of lesion placement were not found on any of the cognitive measures. CONCLUSIONS: In the largest long term follow up study reported to date, the cognitive changes found up to a year after PVP are minimal compared with the robust improvements in motor function. The findings highlight the need to investigate the relation between the specific fibre tracts affected by the lesions and cognitive outcome.

Activities of Daily Living↗
PubMed2000

Occurrence of tics in Asperger's syndrome and autistic disorder.

Asperger's syndrome is a condition in the autistic spectrum in which language development is normal. Patients with Asperger's syndrome frequently exhibit repetitive movements (stereotypies), and can have motor and phonic tics in addition to other behavioral abnormalities. We present 12 patients with autistic spectrum disorders who were referred to our Movement Disorders Clinic for evaluation of tics. Eight of the 12 had normal language development and therefore met criteria for Asperger's syndrome. All patients exhibited stereotypic movements; in addition, seven had tics and six of these met diagnostic criteria for Tourette syndrome. Of the six patients with clinical features of both Asperger's syndrome and Tourette syndrome, three had severe congenital sensory deficits. The autistic patients in our series were clinically heterogeneous and though tics were clearly present, other aberrant movements demonstrated by them were harder to classify. Our series confirms the wide range of clinical manifestations in Asperger's syndrome and autism, including tics and other features of Tourette syndrome. Furthermore, it suggests that sensory deprivation contributes to the development of adventitious movements in this population.

Adolescent↗
PubMed2000

Treating severe bruxism with botulinum toxin.

BACKGROUND: Locally administered botulinum toxin, or BTX, is an effective treatment for various movement disorders. Its usefulness in treating bruxism, however, has not been systematically evaluated. SUBJECTS AND METHODS: The authors studied 18 subjects with severe bruxism and whose mean duration of symptoms was 14.8 +/- 10.0 years (range three-40 years). These subjects audibly ground their teeth and experienced tooth wear and difficulty speaking, swallowing or chewing. Medical or dental procedures had failed to alleviate their symptoms. The authors administered a total of 241 injections of BTX type A, or BTX A, in the subjects' masseter muscles during 123 treatment visits. The mean dose of the BTX A was 61.7 +/- 11.1 mouse units, or MU (range 25-100 MU), per side for the masseter muscles. RESULTS: The mean total duration of response was 19.1 +/- 17.0 weeks (range six-78 weeks), and the mean peak effect on a scale of 0 to 4, in which 4 is equal to total abolishment of grinding, was 3.4 +/- 0.9. Only one subject (5.6 percent) reported having experienced dysphagia with BTX A. CONCLUSION: The results of this study suggest that BTX administered by skilled practitioners is a safe and effective treatment for people with severe bruxism, particularly those with associated movement disorders. It should be considered only for those patients refractory to conventional therapy. Future placebo-controlled studies may be useful in further evaluating the potential of BTX in the treatment of bruxism.

Adolescent↗
PubMed2000

Dystonia in corticobasal degeneration.

Although a referral bias may have resulted in a higher proportion of atypical cases and consequently an overestimation of dystonia, asymmetric limb dystonia particularly affecting one arm initially was observed in 92% of all our CBD cases. Predominant leg dystonia is uncommon, and head, neck, or axial dystonia is rare. Dystonia is often associated with myoclonus, rigidity, apraxia, alien hand phenomenon, and sensory cortical signs in the affected limb, and there are no significant differences between the occurrence of these or other features, between patients with or without dystonia. There is no effective treatment for this relentless disorder except for temporary relief of dystonia and pain with local botulinum toxin injections. Further clinicopathologic studies are needed to elucidate the anatomical and physiologic substrates of dystonia in this disorder.

Basal Ganglia Diseases↗
PubMed2000

Essential tremor: clinical characteristics.

Essential tremor (ET) is the most common movement disorder. However, only a small percentage of people affected by this genetically transmitted neurologic disorder seek medical attention. Lack of consensus on the diagnostic criteria for ET is an impediment to accurate diagnosis and leads to difficulty in accessing accurate prevalence data. Although a positive family history, alcohol sensitivity, and propranolol responsiveness are characteristic of ET, these factors should not be considered necessary for the diagnosis of ET. ET can produce substantial physical and psychosocial disabilities. The occasional coexistence of ET and Parkinson's disease (PD) in the same individual may present a diagnostic challenge.

Adult↗
PubMed2000

Complications and limitations of drug therapy for Parkinson's disease.

Levodopa is the most effective drug treatment for Parkinson's disease (PD). However, its chronic use is associated with dyskinesia, motor fluctuations, and hallucinations. There are various approaches to the management of levodopa complications. Motor fluctuations can be treated with a long-acting levodopa preparation, liquid levodopa, or the addition of dopamine agonists or catechol-O-methyl transferase (COMT) inhibitors. Drugs that may improve levodopa-induced psychiatric complications are olanzapine, clozapine, and ondansetron. A large proportion of patients with long-standing PD cannot be controlled adequately with the available pharmacologic tools and are candidates for surgery. Pallidotomy and deep brain stimulation (DBS) of the globus pallidum pars interna and the subthalamic nucleus are currently the preferred surgical approaches. Appropriate patient selection becomes a critical issue for achieving the best surgical results.

Humans↗