Bulbar Palsy, Progressive

Progressiv bulbärpares

Svensk definition

Motorisk nervcellssjukdom med fortskridande försvagning av de muskler som styrs av nedre hjärnstammens kranialnerver. Kliniska tecken kan vara dysartri, dysfagi, förslappning av och ryckningar och sti ckningar i ansiktsmusklerna och tungan. Hos vuxna utvecklar sig sjukdomen från bulbär svaghet till att omfatta hela axelcylinderutskott, och kan inte skiljas från amyotrofisk lateralskleros (ALS). Faz io-Londesyndromet är en ärftlig form.

Engelsk definition

A motor neuron disease marked by progressive weakness of the muscles innervated by cranial nerves of the lower brain stem. Clinical manifestations include dysarthria, dysphagia, facial weakness, tongue weakness, and fasciculations of the tongue and facial muscles. The adult form of the disease is marked initially by bulbar weakness which progresses to involve motor neurons throughout the neuroaxis. Eventually this condition may become indistinguishable from AMYOTROPHIC LATERAL SCLEROSIS. Fazio-Londe syndrome is an inherited form of this illness which occurs in children and young adults. (Adams et al., Principles of Neurology, 6th ed, p1091; Brain 1992 Dec;115(Pt 6):1889-1900)

Svenska synonymer

Fazio-Londes syndrom Bulbärparalys Progressiv bulbärförlamning i barndomen

Engelska synonymer

Bulbar Palsies, Progressive Palsies, Progressive Bulbar Palsy, Progressive Bulbar Progressive Bulbar Palsies Progressive Bulbar Palsy Bulbar Palsy Bulbar Palsies Palsies, Bulbar Palsy, Bulbar Paralysis, Bulbar Bulbar Paralyses Bulbar Paralysis Childhood Progressive Bulbar Palsy Bulbar Palsy, Progressive, Of Childhood Progressive Bulbar Palsy of Childhood Fazio-Londe Syndrome Fazio Londe Syndrome Fazio-Londe Disease Fazio Londe Disease