ISSN:0885-3185    

DOI:10.1002/mds.870050402

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1990

数据来源: WILEY

摘要:

AbstractAlcohol‐responsive myoclonic dystonia is reported in 26 individuals in a six‐generation family, thus indicating autosomal dominant inheritance. Twenty affected family members aged between 3 and 56 years were examined on one occasion. Myoclonus in arms, shoulder, and neck distribution was seen in 17, with occasional generalized jerks in 14. Leg dystonia/hemidystonia was seen in two infant cases, writer's cramp in seven, torticollis/retrocollis in two, and finger tremor in three. The onset of myoclonus was regularly reported from 2 to 3 years of age, the onset of leg dystonia/hemidystonia from 6 to 18 months of age, writer's cramp from early school age, and neck dystonia from late teenage. The effect of alcohol had been noted in 10 individuals, and seven of them abused alcohol. Once established, the neurological signs did not progress significantly. Leg dystonia resolved in two juvenile members. Two adult members had recovered from myoclonus: one elderly man and one posthemorrhagic spastic hemiplegic man. Extensive family investigation is necessary to clarify the clinical variation of this autosomal dominant disorder of involuntary moveme

ISSN:0885-3185    

DOI:10.1002/mds.870050403

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1990

数据来源: WILEY

摘要:

AbstractIt has been suggested that sleep may have a positive effect on morning motor symptoms in Parkinson's disease (PD). We examined this possibility and also looked at common sleep disorders in PD. Seventy‐eight PD patients and 43 normal elderly subjects answered a questionnaire. Of the PD patients, 43.6% reported improved motor symptoms in the morning, 37.2% worse, and 19.2% unchanged compared to the rest of the day. No difference was found between morning‐better and ‐worse groups with respect to age, duration or stage of PD; antiparkinsonian medications utilized, and predominant motor symptoms. However, the morning‐same group had a shorter duration of PD and less severe disease and required fewer dopaminergic medications. Sleep disorders were seen with equal frequency in the morning‐better and ‐worse groups. Our results suggest that sleep does not have a direct effect on morning motor function. Alterations in morning motor symptomatology probably represent a manifestation of motor fluctuations. Sleep fragmentation and spontaneous daytime dozing occurred much more frequently in PD patients than controls. In addition, nocturnal vocalizations and daytime hallucinations occurred only in t

ISSN:0885-3185    

DOI:10.1002/mds.870050404

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1990

数据来源: WILEY

摘要:

AbstractDystonic movements in a mutation of the Syrian golden hamster, nameddtsz, have several features in common with clinically observed paroxysmal dystonic choreoathetosis. In this study the CNS of the mutant hamsters and age‐matched nondystonic controls was examined for morphological alterations at the age of 30 days, i.e., when the severity of the dystonic syndrome is fully developed. Particular interest was directed to those brain regions (caudate nucleus, putamen, globus pallidus, ventrolateral thalamus) that are presumably involved in symptomatic dystonia of humans, as well as to regions (e.g., spinal cord, dorsal root ganglia, nucleus ruber) for which neuropathologically detectable lesions have been found previously in the dystonia musculorum mouse. The neuropathological investigation was carried out on routine paraffin histology on step sections of the whole brain and spinal cord. In addition, a silver impregnation method was used for detection of pre‐ and/or postsynaptic degeneration. Light microscopic examination, including morphometry, of the nervous tissue failed to reveal any morphological or morphometric differences between control and dystonic hamsters. The only abnormality that was found in several control and dystonic hamsters was hydrocephalus. Breeding studies using magnetic resonance imaging for detection of hydrocephalus showed that hydrocephalus was hereditary but not related to dystonia. Virus infections as a cause of hydrocephalus or dystonia could be excluded by serological analysis with determinations of various virus antibodies in hamster sera. The lack of neuropathological alterations related to dystonic movements in the present study indtszhamsters is comparable to most cases of human hereditary or idiopathic dystonia, which show dystonic movements in the absence of morphological alterati

ISSN:0885-3185    

DOI:10.1002/mds.870050405

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1990

数据来源: WILEY

摘要:

AbstractThe reduced form of nicotinamide adenine dinucleotide coenzyme Q reductase (complex I) activity has recently been shown to be deficient in the substantia nigra of patients dying with Parkinson's disease. This biochemical defect is identical to that produced by the neurotoxin 1‐methyl‐4‐phenyl‐1,2,3,6‐tetrahydropyridine (MPTP), which also produces parkinsonism in humans. Complex I comprises 25 polypeptides, seven of which are encoded by mitochondrial DNA. Restriction fragment analysis of substantia nigra DNA from six patients with Parkinson's disease did not show any major deletion. In two cases, there were different novel polymorphisms that were not observed in control brain (n = 6) or blood (n = 34

ISSN:0885-3185    

DOI:10.1002/mds.870050406

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1990

数据来源: WILEY

摘要:

Abstract4‐‐Propyl‐9‐hydroxynaphthoxazine, or MK‐458 (HPMC), a selective, nonergot D2 agonist administered orally twice a day in sustained‐release form, was studied as adjunctive therapy with carbidopa‐levodopa (Sinemet) in 12 Parkinson's disease patients with motor response fluctuations. The dosage of agonist was gradually increased over 12 weeks to a maximum tolerated level of up to 60 mg/day, and that of Sinemet was reduced concurrently. After 8 weeks, reduction of Sinemet averaged 45.1%, but over the next 4 weeks, despite a continued increase in dosage of the agonist, patients were unable to decrease their Sinemet further, and by 12 weeks mean reduction in Sinemet was only 32%. Only five patients completed the planned 24‐week study, mostly due to progressive loss of efficacy. The MK‐458 is capable of partially substituting for Sinemet in dosages employed in this study. Reduced sensitivity to the drug can appear over a relatively short time, perhaps as a result of downregulation of postsynaptic d

ISSN:0885-3185    

DOI:10.1002/mds.870050407

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1990

数据来源: WILEY

摘要:

AbstractWe examined the influence of maintaining different postural configurations of the upper extremity on the magnitude of tremor in patients with essential tremor and with postural tremor from a variety of other neurological disorders. Patients maintained postures requiring different angles of forward flexion in the sagittal plane, of horizontal flexion, and of elbow extension. The tremor of patients diagnosed with essential tremor was either unaffected or affected only little by changes in limb position. In contrast, patients with pathological tremors, of the cerebellar postural, parkinsonian, and other types, exhibited positional dependence of their tremor. When there was positional dependence of tremor, it was always largest when the hands were near the nose or chin. These observations suggest a practical method for assistance with the clinical discrimination of essential tremor from other postural tremors.

ISSN:0885-3185    

DOI:10.1002/mds.870050408

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1990

数据来源: WILEY

摘要:

AbstractA 28‐year‐old man with a history of congenital hydrocephalus due to aqueductal stenosis shunted at 45 days of age is presented. At age 4 years the valve had to be removed because of septicemia. Twenty‐three years later he developed parkinsonian signs and abnormal, involuntary rhythmic contractions of the eyelids. The latter were elicited on gentle eye closure. Parkinsonism promptly improved after ventriculoperitoneal shunting, but blepharoclonus persisted unch

ISSN:0885-3185    

DOI:10.1002/mds.870050409

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1990

数据来源: WILEY

摘要:

AbstractWe describe a syndrome of spontaneous orofacial dyskinesias and cage stereotypies in a singly housed adult cynomolgus monkey never previously exposed to neuroleptic drugs. Abnormal movements were readily suppressed by acute treatment with haloperidol (0.03–0.24 mg/kg i.m.) or SCH23390 (0.05–0.2 mg/kg i.m.) but not by physostigmine (0.005–0.04 mg/kg i.m.) or scopolamine (0.0025–0.04 mg/kg i.m.). The symptomatology and response to pharmacological manipulations was indistinguishable from that previously attributed to chronic neuroleptic treatment in primates. Our findings indicate that neuroleptic‐induced tardive dyskinesias in most primate studies have not been clearly dem

ISSN:0885-3185    

DOI:10.1002/mds.870050410

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1990

数据来源: WILEY

摘要:

AbstractThe prevalence of dystonia was studied in 125 veterans on chronic antipsychotic therapy using a detailed and systematic examination. Twenty‐seven out of 125 had dystonic manifestations. The most common areas involved were hands and jaw. There was no relation between the presence or absence of dystonia, and duration of neuroleptic therapy. There was a tendency for tardive akathisia to occur more frequently in patients with dystonia than in those without it (Fisher's exact probability test, p = 0.0656). Tardive dystonia in its milder forms may be more common than currently believe

ISSN:0885-3185    

DOI:10.1002/mds.870050411

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1990

数据来源: WILEY