CHILDHOOD PROGRESSIVE BULBAR PALSY
\t͡ʃˈa͡ɪldhʊd pɹəɡɹˈɛsɪv bˈʌlbɑː pˈɔːlzi], \tʃˈaɪldhʊd pɹəɡɹˈɛsɪv bˈʌlbɑː pˈɔːlzi], \tʃ_ˈaɪ_l_d_h_ʊ_d p_ɹ_ə_ɡ_ɹ_ˈɛ_s_ɪ_v b_ˈʌ_l_b_ɑː p_ˈɔː_l_z_i]\
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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 intially 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)
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