Kaeser's syndrome (Brossard-Kaeser syndrome)

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. 

Kaeser's syndrome

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.

More details: 📖 McGraw-Hill Specialty Board Review Neurology 2th Edition

Heinrich E. Kaeser (1924 -    ), Swiss neurologist.

Jules Brossard (1855 - 1911), French internist and pathologist.

Jules Brossard was an interne at the Paris hospitals from 1882 to 1886, assistant professor of pathology and clinical medicine at the Ecole de médecine de Poitiers from 1889. From 1890 to 1895 he was in charge of clinical teaching, and from 1895 professor of histology. He became titular professor at the chair of internal pathology in 1903, and worked as physician at the Hôtel-Dieu de Poitiers.

References

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.