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J Jankovic

Publications and source records attributed to J Jankovic.

At least 127 records · Page 7Linked to original sources
PubMed1999

Exercise performance in those having Parkinson's disease and healthy normals.

OBJECTIVE: This study assessed and compared the cardiopulmonary function of individuals with Parkinson's disease (PD) with that of healthy normals (HN) in order to provide health professionals with more thorough information about the problems associated with PD. METHODS: 20 men (PD = 13, HN = 7; mean age 64 and 64, respectively) and 23 women (PD = 7, HN = 16; mean age 65 and 66, respectively) were recruited from the Houston metropolitan area. Maximal oxygen consumption (VO2max mL x kg(-1) x min(-1)) and time to maximal exercise in minutes (time(max)) were measured. Exercise was performed on a stationary bicycle using an incremental exercise protocol. Because the assumption of homogeneity of variance was not met for the dependent variable VO2max in women, the nonparametric Wilcoxon-Mann-Whitney-U analysis was used (alpha < or = 0.025). All other group comparisons were analyzed using an independent t-test (alpha < or = 0.025). RESULTS: For men and women, there were no significant differences in VO2max between those having PD and the HN (men: PD = 23.52 vs HN = 25.46 mL x kg(-1) x min(-1), P = 0.50; women: PD = 20.10 vs HN = 16.20 mL x kg(-1) x min(-1), P = 0.35). Likewise, there was no significant differences in time(max) between women (PD = 5.2 vs HN = 5.4 min, P = 0.20). Comparison of time(max) between men did show a significant difference (PD = 9.5 vs HN = 13.10 min, P = 0.02). CONCLUSIONS: Although there were no significant differences in VO2max between the men, the comparison of time(max) indicates those with PD were unable to exercise as long before reaching VO2max, indicating that individuals with PD may be less efficient during exercise and therefore unable to exercise as long before reaching VO2max. Although women with PD had a higher VO2max, comparisons of VO2max and time(max) between those with PD and HN resulted in no significant differences.

Aged↗
PubMed1999

Comparison of mouse bioassay and immunoprecipitation assay for botulinum toxin antibodies.

OBJECTIVE: To compare a recently developed immunoprecipitation assay (IPA) to the mouse protection bioassay (MPB), currently considered the "gold standard", for detecting antibodies against botulinum toxin A (BTX-A) and to correlate these assay results with clinical responses to BTX-A injections. METHODS: MPB and IPA assays were performed on serum samples from 83 patients (38 non-responders, 45 responders) who received BTX-A injections. Six non-responders had serum tested on two separate occasions. Some patients also received a "test" injection into either the right eyebrow (n=29) or right frontalis (n=19). RESULTS: All patients antibody positive (Ab+) by MPB were also Ab+ by IPA, whereas an additional 19 patients (17 with reduced or no clinical response) who were MPB Ab- were Ab+, with low titres, by IPA. Two of these 19 patients (non-responders) were initially MPB Ab- but later became MPB Ab+. Similar to previous studies, the sensitivity for the MPB was low; 50% for clinical, 38% for eyebrow, and 30% for frontalis responses whereas the IPA sensitivity was much higher at 84% for clinical (p<0.001), 77% for eyebrow (p=0.111, NS) and 90% for frontalis responses (p<0.02). The IPA specificity was 89% for clinical, 81% for eyebrow, and 89% for frontalis responses, whereas the MPB specificity was 100% for all three response types, which were all non-significant differences. CONCLUSIONS: Both assays had high specificity although the sensitivity of the IPA was higher than the MPB. In addition, the IPA seems to display positivity earlier than the MPB, and as such, it may prognosticate future non-responsiveness. Eyebrow and frontalis "test" injections correlated well with clinical and immunological results and are useful in the assessment of BTX non-responders.

Adult↗
PubMed1999

Re-emergent tremor of Parkinson's disease.

OBJECTIVE: To characterise postural tremors in patients with Parkinson's disease. Rest tremor is a well recognised cardinal symptom of Parkinson's disease, but postural tremors associated with the disease may cause more disability than the more typical rest tremor. Postural tremor of Parkinson's disease has been attributed to enhanced physiological tremor, clonus, or coexistent essential tremor. It is postulated that one type of postural tremor in Parkinson's disease represents a rest tremor that re-emerges after a variable delay while maintaining posture, hence "re-emergent tremor". METHODS: Accelerometry, peak frequency, peak frequency amplitude, root mean square (RMS) amplitude, and latency were determined in 18 patients (mean age: 63.2 (SD 9.8) years) with Parkinson's disease who had clinically evident postural tremor, 20 (mean age: 66.9 (SD 5. 8) years) with typical essential tremor, and seven (mean age: 68.7 (SD 15.3) years) with the combination of pre-existing essential tremor and subsequent Parkinson's disease (essential tremor/Parkinson's disease). Latency, the time interval starting with the assumption of an outstretched posture and ending with the onset of postural tremor, was measured by marking the start time by a pulse produced from interrupting a beam to a photocell when the arm reached a horizontal position. RESULTS: The latency for the re-emergent tremor (9.37 (SD 10.66) s), present in 12 of 18 patients with Parkinson's disease, was significantly (p<0.0005) longer than the latency for postural tremor of essential tremor (1.29 s in one patient, absent in 19 others); five of seven essential tremor/Parkinson's disease patients had an observed latency (6.57 (SD 8.23 s) which was also significantly (p<0.005) longer than that for essential tremor. There was no difference in the mean tremor frequency ( approximately 5.5 Hz) between the re-emergent tremor and the more typical Parkinson's disease rest tremor. The amplitudes were generally higher for the postural tremor associated with Parkinson's disease compared with those of essential tremor. CONCLUSION: These studies suggest that the re-emergent tremor of Parkinson's disease can be differentiated from the postural tremor of essential tremor and that it may share pathophysiological mechanisms with the more typical rest tremor.

Aged↗
PubMed1999

Tetrabenazine treatment for tardive dyskinesia: assessment by randomized videotape protocol.

OBJECTIVE: Tetrabenazine, a monoamine depleter and dopamine receptor blocker, is used to treat several hyperkinetic movement disorders. The authors studied the use of tetrabenazine for tardive dyskinesia. METHOD: Twenty patients with tardive dyskinesia (mean duration = 43.7 months) were videotaped before and after tetrabenazine treatment. Randomized videotapes were scored with the motor subset of the modified Abnormal Involuntary Movement Scale (AIMS) by raters blind to pre- or posttreatment status. RESULTS: One patient did not tolerate tetrabenazine owing to sedation. The remaining 19 were rated after a mean of 20.3 weeks at a mean tetrabenazine dose of 57.9 mg/day. There were significant improvements in mean scores on both the patient AIMS self-rating and the AIMS motor subset evaluated by the blind videotape raters. All 19 patients continued to take tetrabenazine after the study. CONCLUSIONS: Tetrabenazine was well tolerated and resulted in significant improvements in AIMS scores for patients with refractory tardive dyskinesia.

Adult↗
PubMed1999

Attitudes toward neurosurgical procedures for Parkinson's disease and obsessive-compulsive disorder.

Similar neurosurgical procedures exist for Parkinson's disease (PD) and obsessive-compulsive disorder (OCD). Because PD is seen as a brain disease and OCD as a disease of the mind, neurologists and psychiatrists may be more aware of and more optimistic toward neurosurgery for PD than for OCD. A questionnaire was sent to randomized American Psychiatric Association and American Academy of Neurology members, and 569 of 1,188 eligible members (47.9%) responded. Some 82.8% of the psychiatrists and 27.4% of the neurologists were aware of neurosurgical procedures for OCD, whereas 84.7% of psychiatrists and 99.4% of neurologists were aware of neurosurgery for PD (P < 0.001). Of psychiatrists, 74.1% would refer appropriate patients for OCD neurosurgery, 67.4% for PD neurosurgery (P = 0.15); of neurologists, 25.6% would refer for OCD, 94.3% for PD (P < 0.001). Specialty affected willingness to refer for OCD neurosurgery. Specialty and degree of contact with neurosurgeons affected willingness to refer for PD neurosurgery. There is poor physician awareness of neurosurgical options for OCD compared with PD, as well as a risk-benefit bias against OCD surgery by the neurologists surveyed.

Adult↗
PubMed1999

Reduced Nurr1 expression increases the vulnerability of mesencephalic dopamine neurons to MPTP-induced injury.

Mutation in the Nurr1 gene, a member of the nuclear receptor superfamily, causes selective agenesis of dopaminergic neurons in the midbrain of null mice. Homozygous Nurr1 knockout mice (Nurr1-/-) die 1 day after birth, but heterozygous mice (Nurr1 +/-) survive postnatally without obvious locomotor deficits. Although adult Nurr1 +/- mice show significantly reduced Nurr1 protein levels in the substantia nigra (SN), they display a normal range of tyrosine hydroxylase-positive neuron numbers in the SN and normal levels of dopamine in the striatum. The reduction in Nurr1 expression in Nurr1 +/- mice, however, confers increased vulnerability to the selective dopaminergic neurotoxin 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) compared with wild-type (Nurr1 +/+) mice. This study suggests that Nurr1 may play an important role in maintaining mature mesencephalic dopaminergic neuron function and that a defect in Nurr1 may increase susceptibility to SN injury.

1-Methyl-4-phenyl-1,2,3,6-tetrahydropyridine↗
PubMed1998

The G209A mutation in the alpha-synuclein gene is not detected in familial cases of Parkinson disease in non-Greek and/or Italian populations.

OBJECTIVE: To determine whether the G-to-A substitution at nucleotide 209 (G209A) mutation in the alpha-synuclein gene is responsible for familial Parkinson disease (PD) in the US population. DESIGN: Polymerase chain reaction-based DNA analysis of consecutive patients with PD and family history of PD. SETTING: A university-affiliated movement disorder clinic and a Veterans Affairs clinical research laboratory. PATIENTS: Forty-four patients with PD and family history of PD and 29 patients with sporadic PD, all with no known Greek and/or Italian background. RESULTS: None of the DNA samples showed the G209A mutation. CONCLUSION: The G209A mutation is rare in US patients with familial PD.

Adult↗
PubMed1998

Accuracy of the clinical diagnoses of Lewy body disease, Parkinson disease, and dementia with Lewy bodies: a clinicopathologic study.

BACKGROUND: Whether Parkinson disease (PD) and dementia with Lewy bodies (DLB) represent 2 distinct nosologic entities or are diverse phenotypes of Lewy body disease is subject to debate. OBJECTIVES: To determine the accuracy of the diagnoses of Lewy body disease, PD, and DLB by validating the clinical diagnoses of 6 neurologists with the neuropathologic findings and to identify early predictors of the diagnoses. METHODS: Six raters who were unaware of the neuropathologic diagnoses analyzed 105 clinical vignettes corresponding to 29 cases of Lewy body disease (post hoc analysis of 15 patients with PD and 14 with DLB) and 76 patients without PD or DLB whose cases were confirmed through autopsy findings. MAIN OUTCOME MEASURES: Sensitivity and positive predictive value (PPV) were chosen as validity measures and the K statistic as a reliability measure. RESULTS: Interrater reliability for the diagnoses of Lewy body disease and PD was moderate for the first visit and substantial for the last, whereas agreement for diagnosis of DLB was fair for the first visit and slight for the last. Median sensitivity for diagnosis of Lewy body disease was 56.9% for the first visit and 67.2% for the last; median PPV was 60.0% and 77.4%, respectively. Median sensitivity for the diagnosis of PD was 73.3% for the first visit and 80.0% for the last; median PPV was 45.9% and 64.1%, respectively. Median sensitivity for the diagnosis of DLB was 17.8% for the first visit and 28.6% for the last; median PPV was 75.0% for the first visit and 55.8% for the last. The raters' results were similar to those of the primary neurologists. Several features differentiated PD from DLB, predicted each disorder, and could be used as clinical pointers. CONCLUSIONS: The low PPV with relatively high sensitivity for the diagnosis of PD suggests overdiagnosis. Conversely, the extremely low sensitivity for the diagnosis of DLB suggests underdiagnosis. Although the case mix included in the study may not reflect the frequency of these disorders in practice, limiting the clinical applicability of the validity measures, the raters' results were similar to those of the primary neurologists who were not exposed to such limitations. Overall, our study confirms features suggested to predict these disorders, except for the early presence of postural imbalance, which is not indicative of either disorder.

Adult↗
PubMed1998

Hemifacial spasm: clinical findings and treatment.

Hemifacial spasm (HFS) is a peripherally induced movement disorder characterized by involuntary, unilateral, intermittent, irregular, tonic or clonic contractions of muscles innervated by the ipsilateral facial nerve. We reviewed the clinical features and response to different treatments in 158 patients (61% women) with HFS evaluated at our Movement Disorders Clinic. The mean age at onset was 48.5+/-14.1 years (range: 15-87) and the mean duration of symptoms was 11.4+/-8.5 (range: 0.5-53) years. The left side was affected in 56% instances; 5 patients had bilateral HFS. The lower lid was the most common site of the initial involvement followed by cheek and perioral region. Involuntary eye closure which interfered with vision and social embarrassment were the most common complaints. HFS was associated with trigeminal neuralgia in 5.1% of the cases and 5.7% had prior history of Bell's palsy. Although vascular abnormalities, facial nerve injury, and intracranial tumor were responsible for symptoms in some patients, most patients had no apparent etiology. Botulinum toxin type A (BTX-A) injections, used in 110 patients, provided marked to moderate improvement in 95% of patients. Seven of the 25 (28%) patients who had microvascular decompression reported permanent complications and the HFS recurred in 5 (20%). Although occasionally troublesome, HFS is generally a benign disorder that can be treated effectively with either BTX-A or microvascular decompression.

Adult↗
PubMed1998

Variability of the immunologic and clinical response in dystonic patients immunoresistant to botulinum toxin injections.

Immunoresistance (Ab+) to botulinum toxin type A (BTX-A) has been a serious concern since the introduction of BTX-A in the treatment of dystonia and other disorders associated with abnormal muscle contractions. We studied seven patients who developed Ab+ and later reverted to antibody-negative (Ab-) status. These seven patients, six women (mean age, 56 years; range, 41-80 years), with an average duration of dystonia for all patients of 197 months (range, 84-360 months), received a total mean cumulative dose of 1659 units (U) (range, 810-1975 U), with an average dose of 207 U per visit. All of these patients became unresponsive to BTX-A treatment and became Ab+ as determined by mouse bioassay. Their response to BTX-A after they reverted to Ab- was analyzed. The average latency between the initial BTX-A treatment and development of Ab+ was 27 months (range, 1543 months). The average duration between the detection of Ab+ status and subsequent reversal to Ab- status was 30 months (range, 10-78 months). Six of these Ab- patients were reinjected with BTX-A, and all six benefited from repeat injections comparable with their earlier response. Three patients lost their clinical response to subsequent injections and were found to be again Ab+. Two of the five patients who became immunoresistant to BTX-A received botulinum toxin type F (BTX-F) injections and one patient received a single session of BTX-B with improvement in their symptoms. In conclusion, this unique group of patients who were Ab+ and became Ab- responded favorably to repeat BTX-A injections, but some lost the benefit with subsequent injections. These observations suggest that the anamnestic immunologic response to BTX-A can wane, but can be reactivated by repeat BTX-A treatments. The presence of antibodies did not interfere with the response to BTX-F or BTX-B injections, thus confirming the antigenic specificity of various BTX serotypes.

Aged↗
PubMed1998

Pallidotomy for generalized dystonia.

We systematically evaluated the efficacy of both unilateral and bilateral stereotactic pallidotomy in eight patients with generalized dystonia. Six patients had a marked improvement in dystonic movements and motor function; the other two patients also benefited from the procedure but to a lesser extent. The severity of dystonia was assessed before and after surgery by the Burke-Marsden-Fahn Dystonia Scale (BMFDS), the Unified Dystonia Rating Scale (UDRS), and the Activity of Daily Living Scale (ADL). The BMFDS scores decreased by 59.0%, UDRS by 62.5%, and ADL by 47.8% postoperatively. Adverse events were limited to a transient weakness in one patient. We conclude that pallidotomy is a safe and effective treatment in medically refractory cases of generalized dystonia.

Activities of Daily Living↗
PubMed1998

Evidence that a gene for essential tremor maps to chromosome 2p in four families.

In this Journal, we previously reported genetic linkage between loci on chromosome (chr)2p(ETM) and dominantly inherited essential tremor (ET) in a large American kindred of Czech ancestry. Other investigators reported another ET susceptibility locus on chr 3q (FET1) which accounted for over half of the Icelandic families that were studied. We now report evidence for linkage to the ETM locus in three additional, unrelated American families with ET and exclude the FET1 locus in these families. Fine mapping results, using an "affecteds-only" model in all four American families, demonstrate positive combined pairwise lod scores (Z) at the ETM locus with aZ(max) = 5.94 at a recombination fraction (theta) = 0.00 for locus D2S220. Haplotype reconstruction places the ETM gene in a 9.10 cM interval between the D2S224 and D2S405 loci. Multipoint linkage analysis suggests that the ETM gene is in the 2.18 cM interval between loci D2S2150 and D2S220 with a Z(max) = 8.12. These findings may facilitate the search for a gene that causes ET and may further our understanding of other disorders that are associated with tremor [corrected].

Adult↗
PubMed1998

Retrospective application of a set of clinical diagnostic criteria for the diagnosis of multiple system atrophy.

We estimated the accuracy of a modified commonly used set of clinical diagnostic criteria for the diagnosis of multiple system atrophy (MSA) by retrospectively applying the criteria to the features recorded by six neurologists who had evaluated 105 autopsy-confirmed cases (16 MSA and 89 non-MSA disorders). Cases were abstracted from the records of the patients' first visit to an academic center, and were presented as clinical vignettes to six neurologists, each of whom recorded the main clinical features of the presented clinical vignette on a standardized form. Sensitivity and positive predictive values were chosen as validity outcome measures and were calculated by comparing the applied diagnostic criteria to the neuropathologic information. Of note, most MSA patients in this study (mainly those with Shy-Drager type) had not received levodopa therapy since the primary neurologists often had not perceived a need to administer this treatment. The validity of the retrospectively applied criteria for the diagnosis of possible MSA (sensitivity: median, 53%, range, 50-69%; positive predictive value: 30%, 28-39%) and probable MSA (sensitivity: 44%, 31-60%; positive predictive value: 68%, 54-80%) at the first visit was suboptimal. The best, still not perfect, accuracy for this set of diagnostic criteria was obtained when six out of eight features (sporadic adult onset, dysautonomia, parkinsonism, pyramidal signs, cerebellar signs, no levodopa response, no cognitive dysfunction, or no downward gaze supranuclear palsy) were present (median sensitivity, 59%; range, 50-75%; positive predictive value: 67%, 53-83%). This is the first study to validate criteria for the clinical diagnosis of MSA. Our data suggest that it is difficult to achieve an early and accurate clinical diagnosis of this disorder. The probability of correctly diagnosing MSA increases when at least six features of this modified set of criteria are present or when requiring the set for probable MSA.

Adult↗
PubMed1998

Accuracy of the clinical diagnosis of postencephalitic parkinsonism: a clinicopathologic study.

The accuracy of the clinical diagnosis of postencephalitic parkinsonism (PEP) is unknown. We determined the validity of the clinical diagnosis of PEP by presenting 105 records with neuropathologic diagnoses of PEP (n = 7), progressive supranuclear palsy (n = 24), Parkinson's disease (n = 15), dementia with Lewy bodies (n = 14), multiple system atrophy (n = 16), corticobasal degeneration (n = 10), Creutzfeldt-Jakob disease (n = 4), and other dementia disorders (n = 15), as clinical vignettes to six neurologists unaware of the autopsy findings. The neurologists' own clinical diagnoses were compared with neuropathologic diagnoses for measures of diagnostic accuracy, including reliability (kappa statistics), sensitivity and positive predictive values for the first and last visits. The group reliability for the diagnosis of PEP was almost perfect (kappa = 0.91, 0.9). The mean sensitivity at the first visit was 86% (range, 71-100%) with minimal change at the last visit (83%; range, 71-100%). Positive predictive values remained unchanged (100%). The high reliability, sensitivity and positive predictive values of the clinical diagnosis of PEP indicate that neurologists identify this disorder even when they report that they have never evaluated a case. In our data set, the best predictors for the diagnosis of PEP included onset below middle age; symptom duration lasting more than 10 years, and the presence of oculogyric crisis. History of encephalitis lethargica, present in most PEP cases, was an important individual diagnostic predictor. Copyright 1998 Lippincott Williams & Wilkins

Journal Article↗
PubMed1998

Natural history and survival of 14 patients with corticobasal degeneration confirmed at postmortem examination.

OBJECTIVE: To analyse the natural history and survival of corticobasal degeneration by investigating the clinical features of 14 cases confirmed by postmortem examination. METHODS: Patients with definite corticobasal degeneration were selected from the research and clinical files of seven tertiary medical centres in Austria, the United Kingdom, and the United States. Clinical features were analysed in detail. RESULTS: The sample consisted of eight female and six male patients; mean age at symptom onset was 63 (SD 7.7) years, and mean disease duration was 7.9 (SD 2.6) years. The most commonly reported symptom at onset included asymmetric limb clumsiness with or without rigidity (50%) or tremor (21%). At the first neurological visit, on average 3.0 (SD 1.9) years after symptom onset, the most often encountered extrapyramidal features included unilateral limb rigidity (79%) or bradykinesia (71%), postural imbalance (45%), and unilateral limb dystonia (43%). Ideomotor apraxia (64%), and to a lesser extent cortical dementia (36%), were the most common cortical signs present at the first visit. During the course of the disease, virtually all patients developed asymmetric or unilateral akinetic rigid parkinsonism and a gait disorder. No patient had a dramatic response to levodopa therapy. Median survival time after onset of symptoms was 7.9 (SD 0.7) (range, 2.5-12.5) years, and, after the first clinic visit, 4.9 (SD 0.7) (range, 0.8-10) years. Early bilateral bradykinesia, frontal syndrome, or two out of tremor, rigidity, and bradykinesia, predicted a shorter survival. CONCLUSION: The results confirm that unilateral parkinsonism unresponsive to levodopa and limb ideomotor apraxia are the clinical hallmarks of corticobasal degeneration, and only a minority of patients with corticobasal degeneration present with dementia. The study also suggests that a focal cognitive and extrapyramidal motor syndrome is indicative of corticobasal degeneration. Survival in corticobasal degeneration was shortened by the early presence of (more) widespread parkinsonian features or frontal lobe syndrome.

Aged↗