[Confrontation of Salpétrière hospital. May 1990. A vision and language disorder with sudden appearance in a 70 year old patient].
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Biomedical subjects
Publications and source records attributed to O Sabouraud.
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It is still difficult, in 1991, to evaluate precisely the position occupied by benign forms of multiple sclerosis (MS) in the natural history of the disease. The reality hidden behind the adjective "benign" varies from one author to another, and the estimated frequency of these forms is not the same when one refers to epidemiological studies on whole populations or to clinical studies in major hospitals. There is, however, no doubt that benign MS does exist, since about 10% of MS patients will suffer, throughout the whole duration of the disease (over 30 years), from no more than moderate disablement with few repercussions on their social and professional life. These are patients in who MS began when they were 20 to 30 years' old and evolved by episodes rather than progressively and whose main symptoms are optic neuritis and sensory disorders without pyramidal or cerebellar deficit. Nevertheless, these cases are not easy to recognize a priori: there is no paraclinical examination that can predict a benign course in the long term, and an MS which has long been benign may become worse at any moment.
In a previous study, we established the overall prevalence of multiple sclerosis at 25 per 100,000 inhabitants in the French province of Brittany and found that the geographical distribution was uneven with four circumscribed high prevalence areas with more than 45 per 100,000. We conducted the present study to try to ascertain whether the existence of such clusters of MS could be explained by genetic factors, using two ways: the major histocompatibility markers and the frequency of intermarriage. Among the four areas of high prevalence, we examined the one with the highest prevalence, exempt from migratory movements over the last 100 years. We compared this studied population to a sample of 1005 healthy unrelated individuals coming from all over Brittany, designated as general Brittany population. A large sampling (about 25%) of the population accepted to take part in the study. Regarding HLA markers, we observed in the high prevalence area an increases percentage of B7, B8, B12, DR4 and a decreased percentage of B5, compared with the Brittany control population. The consanguinity coefficient in the population of high MS prevalence area was steadily much higher than the one in neighbouring areas with a low MS prevalence during a long period from 1896 to 1945. Under the reserve of non-randomized sampling of individuals for HLA marker study, our findings yielded a double argument in favor of genetic factors influencing the presence of clusters of MS cases: the particularities of HLA phenotype pattern and the increased consanguinity coefficient. The particularities of HLA polymorphism observed in the high prevalence area are likely to be the consequence of the intermarriage habits of this population.
The symptom described as muscle weakness corrected by exercise appears to be part of the recessive form of congenital myotonia, of which it can constitute the dominant clinical manifestation in some cases. This symptom coincides with decrements of the action of potentials to repetitive stimulations in the electromyogram, which may be particularly severe in these same cases. These observations contribute to the modification and precision of the description of the recessive form of Thomsen's disease, but their pathogenicity remains unclear.
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The selective reduction of PAM cell yield reported by Carp and associates in the presence of tissue from patients with multiple sclerosis (MS) has been attributed to replication in culture of a viral agent associated with MS (MSAA). We investigated the diagnostic potential of the PAM cell effect in MS and in optic neuritis (ON). Six serum and 7 CSF samples from 7 patients with MS, 3 serum and 3 CSF samples from 4 patients with idiopathic ON, and 4 sera from 4 patients with ON induced by ethambutal toxicity were tested. Blind counting showed no reduction in PAM cell yield in the MS group nor any significant difference between the two ON groups. Disturbing inconsistencies in PAM cell growth rates over time and between control flasks were demonstrated.
Clinical and Neuropathological data on sixteen cases of progressive myoclonic encephalopathy are reported. This neurological syndrome appears after an average duration of thirty two months of haemodialysis and leads to death in four and a half months, and is characterized by myoclonus, speech disorder, epileptic seizures, and mental-status changes. At first, clinical signs and symptoms are related to haemodialysis, later they become permanent. An early diagnosis is based on EEG which is the only useful laboratory test, demonstrating bisynchronous slow-wave bursts. The caracteristic histopathologic findings are neuronal depopulation, lipofuscin accumulation, and appearance of Neurofibrillary degeneration, especially in Motor cortex, red nucleus and dentato-olivary systems. It seems to be justified to attribute P.M.D.E. to aluminium chronic poisonning; the source of the aluminium intoxication is not aluminium containing phosphate-binding gels but intravenously administreted tape-water. The intracellular binding of aluminium is shown from a histochemical study employing fluorescent stain Morin.
The authors report two cases of the Shulman's syndrome. Twenty one cases of this new entity are found in the literature. The main signs are the acute painful onset after an unusual physical exertion, the development of fasciitis, and (or) myositis, and (or) scleroderma of limbs, the absence of Raynaud's phenomenon, visceral involvement, constant eosinophilia and hypergammaglobulinemia; the prognosis is good with an improvement sometimes spontaneous or coincident with prednisone therapy. One of the observations is interesting by the association with Gougerot-Sjogren's syndrome, and a familial case of morphea. The prognosis in this case is mediocre: failure of corticotherapy, and incomplete remission with cyclophosphamide.
The case is reported of a boy aged 15 who suffered a bilateral lesion of the temporal convexity after a brain traumatism; he died 5 years later and the symptoms during that period are described. The clinical picture is adequately described neither under the title of dementia, nor under that of aphasia. The concept of an abolition involving the systems of cultural mediation (linguistic, technical) is introduced. The status of echolalia and echo-praxia is discussed.
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The comparison of Histocompatibility Testing in 82 MS Patients and 368 controls is presented. The increase in HL-A7 and decrease in HL-A12 are confirmed. A significant increase in HL-A8 is reported. Mixed Lymphocyte Reaction confirms the LD7a increase (19 out of 24 Multiple Sclerosis patients tested); 100% of the patients bearing the HL-A7 determinant are found to be LD7a. The presence of specific Immune Response genes in Multiple Sclerosis is discussed.
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Two cases of paralysis of the etxernal popliteal sciatic nerve are described. They are secondary to the development of a pseudo-cyst containing mucoid matter within the connective tissue sheath of the nerve itself. The authors describe the pathological anatomy of these lesions and discuss their aetiology, favouring the theory of mucoid degeneration of the connective tissues of the nerve sheath. They give brief indication of the clinical symptoms and emphasize the need for early but simplified surgery, ruling out any nerve resection.
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Thirty-three patients presenting an extra-pyramidal pathology with important involuntary movements have been treated with tiapride. The main beneficial results have been obtained in Huntington's chorea and in certain types of myoclony. The association with L-dopa, must be discussed. Only some L-dopa induced dyskinesias are improved; moreover tiaprid may antagonize L-dopa. The essential familial tremor is never improved.
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