[Parkinson's disease: principal signs and natural development].
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Biomedical subjects
Publications and source records attributed to A Guillard.
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A study of parkinsonian signs was conducted in 29 patients fulfilling Lees' criteria for progressive supranuclear palsy, or Steele-Richardson-Olszewski syndrome, and 2 patients with incomplete ophthalmoplegia but 4 other cardinal features. The patients comprised 17 men and 14 women and their ages at onset ranged from 52 to 77 yrs (mean, 65.1). Parkinsonian features: akinesia, rigidity or resting tremor were present in 29 cases and appeared a mean 0.5 yrs (range 0-3) after onset. In 9 cases (29 p. 100), the parkinsonian features were similar to those of Parkinson's disease, including a resting tremor in 6 cases. The parkinsonian syndrome was initially the sole manifestation in 7 patients, who were diagnosed as having Parkinson's disease. Levodopa was effective in 6 cases, for more than a year in 3. In the other 20 cases (64 p. 100) parkinsonian signs were atypical, usually with a predominantly axial distribution. Levodopa or dopamine agonists showed transient efficacy in 6 cases. In most patients, the treatment with levodopa (n = 27) or bromocriptine (n = 15) was well tolerated. Only two disclosed akinetic fluctuations. The other main clinical features included postural instability with falls (n = 30; mean time after onset 1.8 yrs, range 0-7 yrs); characteristic ophthalmoplegia (n = 29; mean interval 4.4., range 0-18 yrs); intellectual decline and/or frontal signs (n = 22; mean interval 2.4, range 0-8 yrs); axial rigidity (n = 18; mean interval 4.2, range 1-11 yrs); pseudo-bulbar palsy (n = 18; mean interval 3, range 0-8 yrs). An upper limb dystonia was present in an autopsy-proven case.(ABSTRACT TRUNCATED AT 250 WORDS)
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A 39-year-old patient with AIDS presented with a rapidly progressive myelopathy with a partial Brown-Séquard syndrome. He died, 9 weeks after onset of the first neurological signs, from diffuse encephalopathy. Neuropathological examination revealed multiple, usually small, frequently haemorrhagic, infarcts or various ages and numerous fibrin thrombi in medium and small penetrating vessels and capillaries of the brain and spinal cord, characteristic of disseminated intravascular coagulation. There were no inflammatory changes. Immunohistochemical studies for human immunodeficiency virus, cytomegalovirus, varicella zoster virus, herpes simplex virus type 1 and type 2 were negative. Ischaemic spinal cord lesions due to disseminated intravascular coagulation may represent an unusual cause of focal, non-inflammatory, non-tumoral, myelopathic syndrome in AIDS.
A 53-year-old man presented with POEMS syndrome characterized by a solitary plasmocytoma and an angiofollicular lymph node hyperplasia similar to that observed in Castelmann's disease. Two features were prominent: first, a severe hypertriglyceridemia, which could have been related to autoimmune suppression of lipoprotein lipase activity; second, a marked immunostaining of myelin sheaths with occasional widening of myelin lamellae. These data, added to the complete regression of the neuropathy immediately following surgical removal of the plasmocytoma (with subsequent irradiation) suggested that the paraprotein was responsible for the neuropathy.
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No clear general view has emerged from the many recent studies devoted to cognitive disorders in Parkinson's disease. Disparity of the populations and methods, as well as interference from motor disorders and possible psychiatric disorders account for the variability of epidemiological data. Subtle cognitive disorders can be found in most patients at the very beginning of the disease. Recent memory is disturbed in free recall tests, but recognition capacities are preserved. Individualization of visuospatial disorders is discussed. Alteration of conceptual capacities is the predominant disorder. Clinically obvious cognitive disorders appear only in some patients after several years of neurological disease. Some risk factors are agreed upon. The bradyphrenia concept is debated, and the term dementia is not accepted by all authors. The major cognitive alterations in Parkinson's disease differ from those observed in Alzheimer dementia, but the latter is more frequent in parkinsonian patients than in the general population. Patients with Parkinson's disease are also exposed to the risk of transient psychotic episodes. Where parkinsonian cognitive pathology is concerned, modern imaging methods are of no help to clinicians. The anatomical and biochemical changes observed in these patients are reviewed, and their responsibility in the genesis of cognitive disorders is discussed: lesions of brainstem nuclei and alterations in their cortical projections on one side, Alzheimer type dementia lesions and Lewys' bodies on the other side. The diverse and inconstant cognitive disorders of patients with Parkinson's disease cannot be explained by lesions of one single structure; they probably result from variable combinations of multiple known or unknown neuronal and biochemical changes.
Eight cases of Münchhausen syndrome were registered within two years in two hospital neurological departments. Summaries of their clinical history were obtained following an enquiry in 58 hospitals of Paris and suburbs. All patients met Asher's description for the emergency presentation, the truculence-evasiveness manner, the luxuriance of tales, the eclecticism of the alleged symptoms, the vehement request of dangerous or painful procedures and the apparent senselessness. The paucity of scars and modesty of peregrinations recorded are discussed, as well as the lack of self-inflicted injury. Ethical implications and traps set by these tricky patients against consistent medical strategies are developed.
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From December 1988 to September 1989, 973 blood donors, deferred for anti-HBc reactivity, Ag-HBs positivity, elevated ALT, isolated or associated, but negative for anti-HIV, were interviewed in our blood center in the weeks after donation. Among these 973 donors, 53 (5.4%, 46 males, 7 females) were found at risk for HIV infection: intravenous drug abuse: 24 cases; heterosexuality with multiple partners: 17 cases; homosexuality: 8 cases; sexual relations with persons at risk: 4 cases. These 53 donors did not recognize their risk behaviour during the medical talk before donation. 25 out of these 53 donors were seen afterwards and one of them, homosexual man, seroconverted for anti-HIV seven months after the anti HIV negative but anti-HBc positive blood donation. We conclude that, in our experience, director surrogate viral hepatitis markers help to identify donors at risk for HIV infection, and, in one case, earlier in the course of demonstrated HIV infection than the enzyme immunoassays currently licensed.
A case of lobar holoprosencephaly associated with heterotopias of the grey matter is reported. The patient was a young woman whose cerebral malformation was revealed by epileptic seizures at the age of 22. There was no intellectual deficit. Computerized tomography and, chiefly, magnetic resonance imaging, enabled the study of the malformation.