The so-called “Vulpian-Bernhardt” type of Spinal progressive muscular atrophy

A 52-year-old man and a 74-year-old man with neurogenic muscular atrophy with scapulo-humeral distribution of late onset were reported.Their clinical neurological features consited of1. onset of the disease at the age of 52 and 732. no heredofamiliar history3. slow progression4. mucular atrophy and muscle weakness with scapulo-humeral distribution5. EMG findings and muscle biopsy showed neurogenic changes.6. fasciculation was observed, and 7. no bulbar palsy.Difference between these cases and Kugelberg-Welander disease was discussed.