| Remarks |
Clinically affected but lacking the characteristic pathology of IBM; onset of symptoms began at age 60 years; diagnosed at age 73; prominent weakness of wrist flexors, finger flexors, interossei and quadriceps; marked bilateral atrophy of forearm flexors and moderate quadriceps atrophy (L>R); diminished brachioradialis reflex (R ) absent (L), absent ankle reflexes; unable to use stairs; unable to get up from low chairs; difficulty washing hair, unscrewing caps and turning in bed; right biceps muscle biopsy showed evidence of chronic inflammatory myopathy but no rimmed vacuoles or congophilic inclusions were observed; previous left biceps biopsy showed inflammatory perivascular and endomysial site inflammation; normal creatine kinase and aldolase; abnormal EMG-short duration polyphasic motor unit potentials without resting activity in distal and proximal muscles, reduced insertional activity in the vastus lateralis and medialis; assistive devices: raised toilet seat, bath stool, braces and wheelchair; unresponsive to methotrexate and corticosteroids; son has myasthenia gravis. |