Kennedy's disease (William R. Kennedy)
- Kennedy's syndrome (William R. Kennedy)
- Kennedy-Stefanis syndrome (William R. Kennedy)
Bulbospinal muscular atrophy, spinal bulbar muscular atrophy.
A slowly progrediating muscular atrophy in males.
DescriptionSynonyms: bulbospinal muscular atrophy, spinal bulbar muscular atrophy. A slowly progrediating muscular atrophy. In males. Onset in 3rd to 4th decade. Muscle weakness, fasciculation, and atrophic changes. Bulbar signs: dysphagia. Absent pyramidal (negative Babinski) sensory and cerebellar signs. Occasionally, gynecomastia. Etiology unknown. X-linked recessive inheritance.
William R. Kennedy first described this entity in an abstract in 1966. The full report followed in 1968 by William R. Kennedy, Miton Alter, and Joo Ho Sung, neurologists in Minneapolis. They reported two families with altogether eleven affected members. All the affected were men with onset of symptoms at 30-40 years of age. According to one author, this condition was first desccribed ine the early 20th century. However, we dop not know by whom.
Dr Paul Delwaide, a Belgian neurologist, first used the eponym Kennedy disease in a 1979 paper.
BibliographyWilliam R. Kennedy, Miton Alter, Joo Ho Sung:
Progressive proximal spinal and bulbar muscular atrophy of late onset: a sex-linked recessive trait. Neurology, Cleveland, Ohio, 1968, 18: 671-680.
C. Stefani, Th. Papapetropoulos, S. Scarpalezos, G. Lygidakis, C. P. Panayiotropoulos:
X-linked Spinal and Bulbar Muscular Atrophy of Late Onset. A separate type of motor neuron disease?
Journal of the Neurological Sciences, Amsterdam, 1975, 24: 493-503.
C. Ertekin, H. Sirin:
X-linked bulbospinal muscular atrophy (Kennedy’s syndrome): a report of three cases.
Acta Neurologica Scandinavica, Copenhagen, 1993, 87: 56-61.
X-bundet recessiv bulbospinal neuronopati - Kennedys syndrom.
K. V. Andersen, Ralf-Peter Michler, Ø. Nilssen, Lisbeth Tranebjærg, Jan Aasly.
Tidsskrift for Dennorske lægeforening, 1999, 119: 1591-1595.
We thank Ansar H. Ahmed, MD, for information submitted.