Chronic neurogenic quadriceps amyotrophy

Two patients with chronic neurogenic quadriceps amyotrophy are reported. A 61-year-old woman had symmetrical wasting and weakness of the quadriceps femoris of eight years' duration. A biopsy revealed neuropathic changes, and electromyograms showed neurogenic as well as myopathic patterns in the quadriceps and other muscles. A 29-year-old woman had long-standing wasting limited to the quadriceps and hip muscles. EMG and biopsy were compatible with neurogenic atrophy. She had a brother suffering from a more widespread and severe form of the disease. These cases suggest that chronic neurogenic quadriceps amyotrophy is a forme fruste of Kugelberg-Welander disease.