X-linked recessive bulbospinal neuronopathy: a report of ten cases.

  title={X-linked recessive bulbospinal neuronopathy: a report of ten cases.},
  author={Anita E. Harding and Professor P. K. Thomas and Michael Baraitser and P G Bradbury and John A. Morgan-Hughes and J R Ponsford},
  journal={Journal of neurology, neurosurgery, and psychiatry},
  volume={45 11},
A form of adult onset 'bulbospinal muscular atrophy' of X-linked recessive inheritance is described in 10 patients from eight families. Muscle weakness in the limbs was mainly proximal and developed in the third to fifth decades of life, often preceded by muscle cramps on exertion and tremor of the hands. Weakness and fasciculation of the facial muscles and tongue were prominent. All the patients had gynaecomastia and some were infertile. Two had diabetes mellitus. Motor nerve conduction… CONTINUE READING


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