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Brossard-Kaeser syndrome
Also known as:
Brossard-Kaeser type of spinal scapulo-peroneal muscular atrophy
Brossard-Stark-Kaeser syndrome
Davidenkov's syndrome
Kaeser's syndrome
Stark-Kaeser syndrome
Associated persons:
Jules Brossard
Sergey Nikolaevich Davidenkov
Heinrich E. Kaeser
Paul Stark
Description:
A rare form of progressive spinal muscular atrophy with system degeneration of the motor anterior horn cells, affecting both sexes, involving mainly the scapular and peroneal muscles. At onset, bilateral foot drop and talipes equinovarus; then in a second phase, the shoulder girdle and finally bulbar involvement may occur. Associated disorders may include heart defect and short heel cord. Autopsy shows muscular atrophy and involvement of the claudal cranial nuclei. Etiology unknown. Inheritance is either autosomal dominant or X-linked. It is clinically indistinguishable from Kugelberg-Welander (recessive).
First described in 1886 by Brossard. Presented as a nosological entity in 1964 by Kaeser.
See also Wohlfart-Kugelberg-Welander syndrome, under Gunnar Wohlfart, Swedish neurologist.
Bibliography:
- J. Brossard:
Etude clinique sur une forme héréditaire d'atrophie musculaire progréssive débutant par les membres inférieurs (type fémoral avec griffes des orteils).
Paris, 1886.
- S. Davidenkov:
Scapuloperoneal amyotrophy.
Archives of Neurology and Psychiatry, Chicago, 1939, 41: 694-701.
- H. E Kaeser:
Die familiäre skapulo-peroneale Muskelatrophie.
Deutsche Zeitschrift für Nervenheilkunde, 1964, 186: 379-394.
- A. R. Sulaiman, et al:
Scapuloperoneal syndrome. Report of two families with neurogenic muscular atrophy.
Journal of the Neurological Sciences, Amsterdam, 1981, 52: 305-325.
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