Über das Vorkommen “amyloider Substanzen” im Gehirn bei der Encephalitis epidemica.

Weimann, Waldemar

doi:10.1159/000190558

Cited by 9 publications

(2 citation statements)

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“…On the other hand, intracellular local deposits resembling Lafora bodies have also been described in other affections. This is pointed out in some older publications (2,19,25,28). Again and again, even in the most recent literature, these 4 publications are referred to as an argument against the pathognomonic significance of Lafora bodies.…”

Section: Discussionmentioning

confidence: 96%

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Progressive Myoclonus Epilepsy with Lafora Bodies. Clinical‐Pathological Features

Ham

Jäger

1963

Epilepsia

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SUMMARY A sister and brother, children of consanguineous parents, were suffering from progressive myoclonus epilepsy. In the boy, the myoclonic jerks of the Unverricht type remained very discrete. In both cases there were numerous Lafora bodies in the brain; one patient in addition showed cardiac and hepatic changes in the form of accumulations of unidentified material. Patients with progressive myoclonus epilepsy who belong to the Lafora group, have similar clinical symptoms and similar microscopic abnormalities in the brain. The clinical picture is characterized by the onset of generalized seizures at age 10–17, myoclonus of the Unverricht type, early dementia, normal ocular fundi and a duration of illness not exceeding 10 years; EEG findings are to some extent characteristic, and remain practically constant during the course of the illness; the disease often is a familial condition; the parents are free of the affection, but often consanguineous. Numerous Lafora bodies in the substantia nigra, dentate nucleus, the entire cerebral cortex and the thalamus are considered by us to be pathognomonic for a particular subgroup of the clinical syndrome progressive myoclonus epilepsy; this probably also applies to the changes in the heart and liver. RÉSUMÉ Un frère et une soeur, issus de parents consanguins, sont atteints d'une épilepsie myoclonique progressive dont les secousses du type Unverricht sont très discrètes chez le garçon. L'examen anatomique montre de nombreux corpuscules de Lafora dans les deux cerveaux et, chez l'un des malades seulement, des altérations cardiaques et hépatiques représentées par l'accumulation d'une substance non identifyée. Ces deux observations confirment le fait que les malades souffrant d'une épilepsie myoclonique progressive du type Lafora présentent une séméiologie clinique et des anomalies microscopiques cérébrales semblables. L'aspect clinique est caractérisé par l'apparition de crises généralisées entre 10 et 17 ans, par des myoclonies du type Unverricht, une démence relativement précoce et un fond d'oeil normal; l'évolution n'excède jamais dix ans. Les données de L'E.E.G. sont relativement caractéristiques et restent pratiquement constantes pendant le cours de la maladie. L'affection est souvent familiale, mais elle respecte les parents qui sont fréquemment consanguins. L'existence de nombreux corpuscules de Lafora dans la substance noire, dans le noyau dentelé, dans le thalamus et dans tout le cortex cérébral est, à notre avis, pathognomonique d'un subgroupe du syndrome clinique d'épilepsie myoclonique progressive; il en va probablement de méme en ce qui concerne les altérations cardiaques et hépatiques.

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Section: Discussionmentioning

confidence: 96%

“…The correctness of the latter supposition is irrelevant to this paper. Weimann (28) found amyloid deposits in the ganglion cells of the sensory trigeminal nucleus in a case of epidemic encephalitis. In the case described by Spielmeyer (25) there was clinical dementia and muscular atrophy of the upper limbs.…”

Section: Discussionmentioning

confidence: 99%