PROGRESSIVE PROXIMAL MYELOPATHIC MUSCULAR ATROPHY
\pɹəɡɹˈɛsɪv pɹˈɒksɪmə͡l mˌa͡ɪɪləpˈaθɪk mˈʌskjʊləɹ ˈatɹəfi], \pɹəɡɹˈɛsɪv pɹˈɒksɪməl mˌaɪɪləpˈaθɪk mˈʌskjʊləɹ ˈatɹəfi], \p_ɹ_ə_ɡ_ɹ_ˈɛ_s_ɪ_v p_ɹ_ˈɒ_k_s_ɪ_m_əl m_ˌaɪ_ɪ_l_ə_p_ˈa_θ_ɪ_k m_ˈʌ_s_k_j_ʊ_l_ə_ɹ ˈa_t_ɹ_ə_f_i]\
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A group of disorders marked by progressive degeneration of motor neurons in the spinal cord resulting in weakness and muscular atrophy, usually without evidence of injury to the corticospinal tracts. Diseases in this category include Werdnig-Hoffmann disease and later onset SPINAL MUSCULAR ATROPHIES OF CHILDHOOD, most of which are hereditary. (Adams et al., Principles of Neurology, 6th ed, p1089)
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