Kennedy syndrome or bulbospinal muscular atrophy affects mainly an adult population. Clinicians in South Korea have collected clinical and genetic data from 157 patients with this X-linked recessive neuromuscular disorder:
- with a median age of 56.9 years, their cohort is mainly composed of adults (33-83 years);
- a fine tremor of the fingers is very often the first symptom followed progressively by the other cardinal signs of the disease (gynecomastia, peri-oral fasciculations, cramps and fatigability);
- apart from the neurogenic syndrome shared by all, the electromyographic data were quite disparate from one patient to the next, particularly at the sensory level.
This study, which is similar to a natural history of the disease, could serve as a basis for future therapeutic trials.
