Stiff limb syndrome: End of spectrum or a separate entity?

Abstract

Background. Stiff-person syndrome is a rare disorder characterized by rigidity of axial or limb muscles with episodes of co-contraction of agonist and antagonist muscles during the spasms. In some patients axial or limb involvement may predominate and may have unusual manifestations. Design. Case report. Setting. Tertiary care teaching hospital. Patient. A 42-year-old farmer presented with seasonal occurrence of hiccup and vomiting during summer months for the last 3 years. He had painful lower limb spasms lasting for 2-3 minutes every 10-15 minutes for the past 20 days. His neurological examination was normal, erythrocyte sedimentation rate (ESR) was 50 mm at 1st hour, and cerebrospinal fluid protein 78 mg/dL without pleocytosis. Radiograph of chest, abdominal ultrasound, and craniospinal magnetic resonance imaging were normal. The patient improved on diazepam. Conclusion. Our patient is a forme fruste of stiff person syndrome with hiccups and vomiting due to diaphragmatic spasm. © 2009 American Academy of Pain Medicine.