1/76. 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 = 1keywords = parkinsonism (Clic here for more details about this article) |
2/76. 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 = 6.4937932518858keywords = parkinsonian (Clic here for more details about this article) |
3/76. 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 = 1.6234483129715keywords = parkinsonian (Clic here for more details about this article) |
4/76. 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 = 1.6234483129715keywords = parkinsonian (Clic here for more details about this article) |
5/76. 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 = 6keywords = parkinsonism (Clic here for more details about this article) |
6/76. 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 = 4keywords = parkinsonism (Clic here for more details about this article) |
7/76. 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 = 10.870344938914keywords = parkinsonian, parkinsonism (Clic here for more details about this article) |
8/76. 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 = 4keywords = parkinsonism (Clic here for more details about this article) |
9/76. Phenotypic characterisation of autosomal recessive PARK6-linked parkinsonism in three unrelated Italian families.The clinical features of nine patients (three women and six men) affected by PARK6-linked parkinsonism, belonging to three unrelated Italian families, are reported. The occurrence of affected men and women within one generation suggested an autosomal recessive mode of inheritance in all three families. Mean age at disease onset was 36 /- 4.6 years; all cases except one presented with asymmetrical signs, consisting of tremor and akinesia of one upper limb or unilateral short step gait. Affected individuals had a mean age of 57 /- 8.5 years, and average disease duration was 21 /- 7.8 years. Parkinsonian features included benign course, early onset of drug-induced dyskinesias, and a good and persistent response to levodopa. There were no other associated features (i.e., pyramidal or cerebellar signs, dysautonomia, or diurnal fluctuations unrelated to drug treatment). cognition was unaffected. The clinical picture was remarkably similar in all patients; no relevant family-related differences were found. PARK6 disease is a new form of early-onset parkinsonism without other atypical clinical features.- - - - - - - - - - ranking = 6keywords = parkinsonism (Clic here for more details about this article) |
10/76. Fast orthostatic tremor in Parkinson's disease mimicking primary orthostatic tremor.leg tremor during standing is a rare feature in idiopathic Parkinson's disease (PD). Tremor during standing usually has a low frequency (range, 4-6 Hz), similar to PD rest tremor frequency, and is improved by levodopa. We describe three cases of fast orthostatic tremor (FoT) of legs and trunk mimicking primary orthostatic tremor (OT) in patients treated with levodopa for PD. Asymmetrical akinetorigid syndrome was accompanied by a rest tremor in two cases. We obtained electrophysiological parameters by electromyographic (EMG) polygraphic recording after 16 hours withdrawal of antiparkinsonian treatment and at the maximal effect of levodopa in order to investigate the effect of dopaminergic stimulation upon such cases of orthostatic tremor in PD. Electrophysiological parameters of orthostatic tremor, especially frequency (range 14-18 Hz), were similar to that seen in POT. Severity of tremor was independent of seriousness and duration of PD. levodopa had no effect either on the handicap due to OT or on the amplitude and frequency of the EMG OT activity. In contrast, mild improvement of OT was obtained with benzodiazepines in two cases and parkinsonian syndrome was levodopa-sensitive. These findings suggest that FoT in PD would not be directly controlled by the dopaminergic system. However, increased rhythmicities in basal ganglia or in cerebello-thalamic loops at the rapid frequencies range seen in PD could favor the emergence of a primary orthostatic tremor-like tremor in PD patients.- - - - - - - - - - ranking = 3.2468966259429keywords = parkinsonian (Clic here for more details about this article) |
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