[Familial form of cutaneous epitheliomatosis with complex neurological characteristics similar to hereditary spinocerebellar degeneration. Apropos of 4 cases including one case with anatomo-clinical description]

Autor: R, Chateau, M, Tommasi, R, Groslambert, J, Perret, B, Pasquier
Jazyk: francouzština
Rok vydání: 1975
Předmět:
Zdroj: Revue neurologique. 131(6)
ISSN: 0035-3787
Popis: The authors report an observation in which four siblings were affected by both multiple cutaneous epitheliomatosis and complex but relatively stereotyped neurological disorders. Clinically, the main syndrome was cerebello-spinal ataxia with involvement of the anterior horns of the spinal cord with less marked pyramidal and extra-pyramidal features. Neuropathological examination of one of the cases revealed lesions of essentially cerebello-spinal degeneration suggestive of Menzel's disease. The possible connection between the neural and cutaneous lesions is discussed. All the various etiological categories possible have been ruled out; not one being entirely satisfactory, except for the very broad category of genetic neuro-dermatoses.
Databáze: OpenAIRE