[Chronic spinal muscular atrophy of facioscapulohumeral type with congenital facial palsy in twins (author's transl)].
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A progressive neurologic disease was identified in a family of closely related Britanny Spaniel dogs. The disease developed in dogs less than 1 year old and characteristically resulted in paraspinal and proximal pelvic limb muscular atrophy. Neurologic examination, electrodiagnostic testing, and histologic studies indicated that the disease results from motor horn cell degeneration. A colony of affected dogs was established, and breeding studies incriminated an inherited basis for the disease.
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A unique association of two rare genetic diseases--progressive muscular atrophy of the spine and rheumatic pelvic spondylitis--observed in a clinical case is described.
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