Individuals with<unk>  have muscle cramps and progressive weakness due to degeneration of motor neurons in the brain stem and spinal cord. Ages of onset and severity of manifestations in affected males vary from adolescence to old age, but most commonly develop in middle adult life. The syndrome has neuromuscular and endocrine manifestations. Neuromuscular Early signs often include weakness of tongue and mouth muscles, fasciculations, and gradually increasing weakness of limb muscles with muscle wasting. Neuromuscular management is supportive, and the disease progresses very slowly, but can eventually lead to extreme disability. Further signs and symptoms include: Ideogram of human X chromosome. Homozygous females Homozygous females, both of whose X chromosomes have a mutation leading to CAG expansion of the AR gene, have been reported to show only mild symptoms of muscle cramps and twitching. No endocrinopathy has been described.
Spinal and bulbar muscular atrophy
Infantile progressive bulbar palsy
Ataxic cerebral palsy
Madras motor neuron disease
Multiple system atrophy
Spinal muscular atrophy
Spinal muscular atrophy with lower extremity predominance 1
Babinski–Nageotte syndrome