Progressive bulbur paralysis (Fazio-Londe disease)

Indian J Pediatr. 2004 Jan;71(1):101-2. doi: 10.1007/BF02725667.

Abstract

Progressive bulbar paralysis of childhood is characterised by progressive paralysis of muscles innervated by cranial nerves. The authors report a case of progressive bulbar paralysis of childhood in a 12-year-old child. Child was admitted with the complaints of drooping of eyelids, difficulty in swallowing and hoarse voice. She had involvement of III, VII, IX, X, XI and XII cranial nerves and the corticospinal tracts. Electromyography revealed spontaneous activity in the form of fasciculations, giant motor unit potential and discrete recruitment of motor neurons suggestive of denervation pattern. Hearing assessment was normal. Muscle biopsy was also suggestive of neurogenic atrophy.

Publication types

  • Case Reports

MeSH terms

  • Biopsy, Needle
  • Bulbar Palsy, Progressive / diagnosis*
  • Child
  • Cranial Nerves / pathology
  • Electromyography / methods
  • Female
  • Humans
  • India
  • Magnetic Resonance Imaging
  • Motor Neurons / pathology
  • Prognosis
  • Risk Assessment
  • Severity of Illness Index
  • Tomography, X-Ray Computed