Studien Über Erbliche Erkrankungen Des Zentralner Vensystems Ii.: Die Familiäre, Amyotropihsche Lateralsclerose

Munch-Petersen, C. J.

doi:10.1111/j.1600-0447.1931.tb09491.x

Cited by 9 publications

(1 citation statement)

References 13 publications

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“…A number of these reports specifically suggested an autosomal dominant transmission/a high genetic penetrance [ 30 ]. Relevantly, cognitive/behavioral phenotypes were often reported as similar within such familial/genetic cases—e.g., familial cases of progressive bulbar palsy with aphasic dementia [ 94 ], slowly progressing ALS with or without dementia [ 21 ], early-onset psychosis with dementia developing within the fifth/sixth age decades [ 123 ], or slowly progressive, juvenile-onset ALS with psychosis [ 77 ].…”

Section: Familial Incidencementioning

confidence: 99%

Cognitive and behavioral involvement in ALS has been known for more than a century

Zago

Lorusso²,

Aiello

et al. 2022

Neurol Sci

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Background Among clinicians and researchers, it is common knowledge that, in ALS, cognitive and behavioral involvement within the spectrum of frontotemporal degenerations (FTDs) begun to be regarded as a fact in the late 1990s of the twentieth century. By contrast, a considerable body of evidence on cognitive/behavioral changes in ALS can be traced in the literature dating from the late nineteenth century. Methods Worldwide reports on cognitive/behavioral involvement in ALS dating from 1886 to 1981 were retrieved thanks to Biblioteca di Area Medica “Adolfo Ferrate,” Sistema Bibliotecario di Ateneo, University of Pavia, Pavia, Italy and qualitatively synthetized. Results One-hundred and seventy-four cases of ALS with co-occurring FTD-like cognitive/behavioral changes, described in Europe, America, and Asia, were detected. Neuropsychological phenotypes were consistent with the revised Strong et al.’s consensus criteria. Clinical observations were not infrequently supported by histopathological, post-mortem verifications of extra-motor, cortical/sub-cortical alterations, as well as by in vivo instrumental exams—i.e., assessments of brain morphology/physiology and psychometric testing. In this regard, as earlier as 1907, the notion of motor and cognitive/behavioral features in ALS yielding from the same underlying pathology was acknowledged. Hereditary occurrences of ALS with cognitive/behavioral dysfunctions were reported, as well as familial associations with ALS-unrelated brain disorders. Neuropsychological symptoms often occurred before motor ones. Bulbar involvement was at times acknowledged as a risk factor for cognitive/behavioral changes in ALS. Discussion Historical observations herewith delivered can be regarded as the antecedents of current knowledge on cognitive/behavioral impairment in the ALS-FTD spectrum.

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Section: Familial Incidencementioning

confidence: 99%

Cognitive and behavioral involvement in ALS has been known for more than a century

Zago

Lorusso²,

Aiello

et al. 2022

Neurol Sci

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Juvenile type of distal and segmental muscular atrophy of upper extremities

Sobue

Sakai

Iida

et al. 1978

Annals of Neurology

145

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Seventy-one cases of distal and segmental muscular atrophy of the upper extremities with juvenile onset were studied. The clinical features consisted of: juvenile onset, male preponderance, unique distribution of the muscular atrophy in the hand and forearm, tendon reflexes hypoactive in most cases but hyperactive in some, no definite sensory disturbances, no involvement of the cranial nerves, and autonomic nerve disorders in the affected region. There was rapid progression during the 2 to 3 years after onset with a slowly progressive course thereafter. There were no abnormal laboratory findings except for electrophysiological and morphological findings of the affected muscles. The site of lesion was surmised to be from the C5 to T1 spinal segments with intramedullary involvement. The cause is unknown.

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Die chronisch progressiven nuclearen Amyotrophien (Chron. progr. spinale Muskelatrophien, Aran-Duchenne, Werdnig-Hoffmann, chron. progr. Bulbärparalyse und Ophthalmoplegie.) Die amyotrophische Lateralsklerose

Marburg¹

1936

Angeborene Früh Erworbene Heredo-Familiäre Erkrankungen

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Studien Über Erbliche Erkrankungen Des Zentralner Vensystems Ii.: Die Familiäre, Amyotropihsche Lateralsclerose

Cited by 9 publications

References 13 publications

Cognitive and behavioral involvement in ALS has been known for more than a century

Cognitive and behavioral involvement in ALS has been known for more than a century

Juvenile type of distal and segmental muscular atrophy of upper extremities

Die chronisch progressiven nuclearen Amyotrophien (Chron. progr. spinale Muskelatrophien, Aran-Duchenne, Werdnig-Hoffmann, chron. progr. Bulbärparalyse und Ophthalmoplegie.) Die amyotrophische Lateralsklerose

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