1/100. levodopa may improve orthostatic tremor: case report and trial of treatment.Primary orthostatic tremor is a regular fast lower limb tremor causing unsteadiness on standing. Treatment is generally unsatisfactory. A patient with primary orthostatic tremor who 9 years later developed levodopa responsive idiopathic Parkinson's disease is described. The patient exhibited the classic features of primary orthostatic tremor with relief of the tremor by walking or sitting while treated with levodopa. However, in the "off" state, when the benefits of levodopa disappeared, this orthostatic tremor was continuous and severely compromised the patient's gait. On the basis of this finding eight patients with primary orthostatic tremor were treated with levodopa. Five patients experienced benefit and elected to remain on long term treatment. This study is the first trial of therapy in primary orthostatic tremor and suggests that levodopa can lead to good symptomatic relief in this potentially disabling condition.- - - - - - - - - - ranking = 1keywords = idiopathic (Clic here for more details about this article) |
2/100. Clinical genetics of familial progressive supranuclear palsy.Recent studies have shown that progressive supranuclear palsy (PSP) could be inherited, but the pattern of inheritance and the spectrum of the clinical findings in relatives are unknown. We here report 12 pedigrees, confirmed by pathology in four probands, with familial PSP. Pathological diagnosis was confirmed according to recently reported internationally agreed criteria. The spectrum of the clinical phenotypes in these families was variable including 34 typical cases of PSP (12 probands plus 22 secondary cases), three patients with postural tremor, three with dementia, one with parkinsonism, two with tremor, dystonia, gaze palsy and tics, and one with gait disturbance. The presence of affected members in at least two generations in eight of the families and the absence of consanguinity suggests autosomal dominant transmission with incomplete penetrance. We conclude that hereditary PSP is more frequent than previously thought and that the scarcity of familial cases may be related to a lack of recognition of the variable phenotypic expression of the disease.- - - - - - - - - - ranking = 8761.2452157315keywords = parkinson, parkinsonism (Clic here for more details about this article) |
3/100. Exogenous levodopa is not toxic to elderly subjects with non-parkinsonian movement disorders: further clinical evidence.We report three women between 80 and 87 years of age who had longstanding essential tremor. Due to an erroneous diagnosis of Parkinsonism they had received levodopa preparations for 13, 16 and 25 years, respectively, with cumulative levodopa equivalent doses of 18.0, 9.0, and 4.1 kg. The patients showed neither dyskinesias nor signs of Parkinsonism. These observations do not support a detrimental role of chronic levodopa exposure in elderly individuals with essential tremor.- - - - - - - - - - ranking = 19241.257615498keywords = parkinson (Clic here for more details about this article) |
4/100. Function of the cerebellum in Parkinsonian rest tremor and Holmes' tremor.We describe a patient who developed Parkinson's disease (PD) 17 years after resection of his right cerebellum because of a Lindau tumor. He showed a classic 4.3-Hz resting tremor on the left side but a 3.1-Hz resting, postural, and intention tremor on the right side compatible with midbrain tremor (Holmes' tremor). We conclude that the generator of the tremor in PD cannot be located within the olivocerebellar loop. The cerebellum, however, seems to modulate the tremor frequency of parkinsonian rest tremor and may prevent the rest tremor from transforming into a postural and goal-directed tremor.- - - - - - - - - - ranking = 4810.3144038746keywords = parkinson (Clic here for more details about this article) |
5/100. Posteroventral pallidotomy for midbrain tremor after a pontine hemorrhage. Case report.This 49-year-old man gradually developed a disabling action tremor in the proximal right upper extremity 8 months after suffering a pontine tegmental hemorrhage. The intraoperative microrecording in the nucleus ventralis intermedius (VIM) of the left thalamus revealed tremor-synchronous grouped discharges with a vigorous (2.7 Hz) action tremor predominantly in the shoulder and upper arm. High frequency electrical stimulation in the VIM did not affect the tremor. A posteroventral pallidotomy (PVP) was performed and resulted in the successful alleviation of all tremor activity. Posteroventral pallidotomy is known to alleviate parkinsonian tremors, especially those occurring in the contralateral lower extremity, trunk, and proximal segment of the contralateral upper extremity. The authors consider the pallidoreticular pathway to be an important tremor-mediating pathway for the proximal segment of the upper extremities and believe it can be controlled more effectively by PVP than by VIM thalamotomy, as demonstrated by the PVP-induced resolution of the midbrain tremor observed in this case.- - - - - - - - - - ranking = 4810.3144038746keywords = parkinson (Clic here for more details about this article) |
6/100. Electrophysiological observations in hereditary parkinsonism-dementia with Lewy body pathology.We studied the only two living affected individuals who are part of a previously reported kindred that expresses a hereditary parkinsonism-dementia syndrome with Lewy body pathology. The electrophysiological characteristics of the hyperkinetic movement disorders in these patients were examined to provide physiological insights into the clinical phenotype of this syndrome. Evaluation of both patients showed 7-9 Hz electromyographic discharges in upper extremity muscles during postural activation, and one patient showed a 4-5 Hz discharge pattern correlating to a rest tremor. Brief (<50 ms) myoclonic electromyographic discharges were seen in both patients, and a time-locked relationship to a focal cortical premovement electroencephalographic potential was elicited in one patient. Somatosensory evoked potentials were not enlarged and long latency reflexes were not enhanced. electroencephalography was normal in one patient but showed pathologic slow frequencies in the other. The electrophysiological findings show evolution which correlates with an apparent characteristic evolution of hyperkinetic movement disorders that accompanies the severe progression of parkinsonism-dementia in this kindred. These results have implications for the future study of this and similar syndromes.- - - - - - - - - - ranking = 52568.893122453keywords = parkinson, parkinsonism, body (Clic here for more details about this article) |
7/100. A Yorkshire family with adult-onset cranio-cervical primary torsion dystonia.Although a family history is described in approximately 20% of patients, large families with adult-onset craniocervical primary (idiopathic) torsion dystonia (PTD) are rare. We report a new British family with cranio-cervical dystonia. Seventeen members of the family were examined. Five cases were diagnosed as definite PTD and one as probable PTD. Mean age at onset was 29 years (range, 19-40 yrs). The phenotype was characterized by adult-onset cranio-cervical dystonia in all affected cases. A few cases had additional voice tremor and/or postural arm tremor. The GAG deletion in the DYT1 gene was excluded in the index case. Linkage analysis was performed between the disease and several marker loci spanning DYT6 and DYT7 regions, and haplotypes were reconstructed in all subjects. Although linkage analysis was not completely informative, reconstructed haplotypes excluded linkage between the disease and either DYT6 or DYT7. This report confirms that familial cranio-cervical dystonia is genetically heterogeneous, and further studies of other PTD families with similar clinical features are needed to identify other new genes.- - - - - - - - - - ranking = 1keywords = idiopathic (Clic here for more details about this article) |
8/100. Involuntary movements after anterior cerebral artery territory infarction.BACKGROUND AND PURPOSE: patients with anterior cerebral artery territory infarction presenting with involuntary movements have rarely been described in the literature. CASE DESCRIPTIONS: The author reports 9 such patients: 3 with asterixis, 5 with hemiparkinsonism (tremor, rigidity, hypokinesia), and 1 with both. Asterixis developed in the acute stage in patients with minimal arm weakness, whereas parkinsonism was usually observed after the motor dysfunction improved in patients with initially severe limb weakness. Asterixis correlated with small lesions preferentially involving the prefrontal area; parkinsonism is related to relatively large lesions involving the supplementary motor area. CONCLUSIONS: anterior cerebral artery territory infarction should be included in the differential diagnosis of asterixis and hemiparkinsonism.- - - - - - - - - - ranking = 35044.980862926keywords = parkinson, parkinsonism (Clic here for more details about this article) |
9/100. Zolpidem for antipsychotic-induced parkinsonism.OBJECTIVE: To report a case of antipsychotic-induced parkinsonism that was managed with zolpidem. CASE SUMMARY: A 34-year-old white man who had had antipsychotic-induced parkinsonism with symptoms of repetitive persistent gross tremors of the hands for numerous years was unresponsive to traditional antiparkinsonian medications. With the initiation of zolpidem 10 mg four times daily, the tremors decreased significantly. DISCUSSION: The use of zolpidem for antipsychotic-induced parkinsonian hand tremors in this patient was based on the severity of the symptoms and the lack of response to several trials of traditional medications. When zolpidem was started at 10 mg four times a day, the motor examination score on the Unified Parkinson's disease Rating Scale decreased from 29 at baseline to a score of 9 after one month of use. After four months of zolpidem use, the patient's mental status decompensated, and clozapine was initiated. As the patient experienced excessive sedation, zolpidem was discontinued while clozapine was maintained to help with the psychosis and, potentially, the tremors. The tremors reemerged with a motor examination score of 30. Zolpidem was reinitiated at 5 mg four times daily, and the patient's tremors have been stable for two years. CONCLUSIONS: Further investigation is needed to study the use of nontraditional medications in patients requiring antipsychotic medications who have refractory parkinsonian symptoms.- - - - - - - - - - ranking = 66998.414506013keywords = parkinson, parkinsonism (Clic here for more details about this article) |
10/100. intention tremor, parkinsonism, and generalized brain atrophy in male carriers of fragile X.The authors report five elderly men with the fragile X premutation who had a progressive action tremor associated with executive function deficits and generalized brain atrophy. These individuals had elevated fragile X mental retardation 1 gene (FMR1) messenger rna and normal or borderline levels of FMR1 protein. The authors propose that elevations of FMR1 messenger rna may be causative for a neurodegenerative syndrome in a subgroup of elderly men with the FMR1 premutation.- - - - - - - - - - ranking = 35044.980862926keywords = parkinson, parkinsonism (Clic here for more details about this article) |
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