[X-chromosomal recessive spinobulbar muscular atrophy (Kennedy type). Description of a family, clinical aspects, molecular genetics, differential diagnosis and therapy].

@article{Kuhlenbaeumer1998XchromosomalRS,
  title={[X-chromosomal recessive spinobulbar muscular atrophy (Kennedy type). Description of a family, clinical aspects, molecular genetics, differential diagnosis and therapy].},
  author={Gregor Kuhlenbaeumer and Michelina Bocchicchio and Wolfram Kress and Peter Young and Christoph Oberwittler and Florian St{\"o}gbauer},
  journal={Der Nervenarzt},
  year={1998},
  volume={69 8},
  pages={660-5}
}
The Kennedy-Syndrome is a X-linked recessive bulbospinal muscular atrophy, in some cases associated with endocrinological disturbances such as androgen resistance and diabetes mellitus. The age of onset is usually between 20 and 40. Presenting symptoms are proximal flaccid weakness, fasciculations, cramps or tremor. Disease progression is usually slow and live expectancy is normal. It is important to distinguish the Kennedy-Syndrome from amyotrophic lateral sclerosis, spinal muscular atrophy… CONTINUE READING