Anti-GAD negative stiff person syndrome with a favorable response to intravenous methylprednisolone: an experience over evidence.

摘要

A 65-year-old male patient presented with 6 months duration of insidious onset progressive stiffness in both lower limbs followed by upper limbs, trunk and neck rendering him non-ambulatory within 5 weeks. He used to experience severe painful spasms, getting aggravated during the emotional stress. These symptoms were less during sleep. Rest of the history was normal. Examination revealed marked difficulty in lying straight supine. Higher functions and cranial nerves were intact. Motor examination revealed distal amyotrophy in upper limbs with fasciculations in upper and lower limbs both distally and proximally. Along with this patient had a "woody" or "plank like" rigid abdomen muscles [Figure 1a].