ISSN:
1432-0533
Keywords:
Famihal ALS
;
Oculomotor impairment
;
Urinary disturbance
;
Multisystemic degeneration
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
Summary Two sibling cases of familial amyotrophic lateral sclerosis (ALS) revealed degerneration usually associated with other systemic degenerative disorders. The changes in the 41-year-old sister were compatible with those reported in other familial ALS cases affecting the upper and lower motor neurons, posterior columns, and spinocerebellar tracts. The 45-year-old sister revealed more wide-spread degenerative changes involving not only motor neuron systems, but also proprioceptive, general somatic afferent and spinocerebellar afferent systems. Intracytoplasmic hyaline inclusions were observed in the oculomotor nuclei. Clinical manifestations of urinary disturbance and oculomotor imparirment seldom seen in sporadic ALS were interpreted to be due to the unusual distribution of the morbid process. These pathologic findings suggest that familial ALS may be a multisystemic degenerative disorder, frequently involving the spinocerebellar tracts, but occasionally involving other systems as well.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/BF00695602
Permalink
