We have reported the case of a 35-year-old white woman suffering from a very slowly progressive bulbar palsy since the age of 13. She shows severe paralysis and wasting of muscles of mastication, face, palate, pharynx, and tongue. After age 30, the motor involvement progressed in a descending manner, with weakness and wasting of muscles of the upper limbs. Muscle biopsy and electromyographic examination demonstrated features characteristic of neurogenic muscular atrophy. With respect to the age of onset, our patient seems to lie between adult-onset progressive bulbar palsy and infantile progressive bulbar palsy (Fazio-Londe) but differs from both by a much more prolonged course. We believe her disease may represent a rare bulbar variant of Kugelberg-Welander-Wohlfart disease, but until we know the cause of these varied disorders, the classification of motor system disease remains speculative.
ASJC Scopus subject areas
- Clinical Neurology