Progressive bulbur paralysis (Fazio-Londe disease).
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.
Gulati
Taruna
T
Department of Pediatrics Medicine, Kalawati Saran Children's Hospital and Lady Hardinge Medical College, New Delhi, India.
Dewan
Vivek
V
Kumar
Praveen
P
Ahuja
Bina
B
Anand
V K
VK
D001707:Biopsy, Needle; D010244:Bulbar Palsy, Progressive / Q000175:diagnosis*; D002648:Child; D003391:Cranial Nerves / Q000473:pathology; D004576:Electromyography / Q000379:methods; D005260:Female; D006801:Humans; D007194:India; D008279:Magnetic Resonance Imaging; D009046:Motor Neurons / Q000473:pathology; D011379:Prognosis; D018570:Risk Assessment; D012720:Severity of Illness Index; D014057:Tomography, X-Ray Computed