Stiff person syndrome (SPS): Literature review and case report

Abstract

Stiff person syndrome (SPS) is a rare, debilitating condition which presents with progressive and inconsistent neurological features. The main symptoms are stiffness and intermittent, painful muscle spasms, triggered and exacerbated by stressful and emotional stimuli. The fluctuating clinical nature of SPS, and otherwise normal neurological examination, often lead to a misdiagnosis of conversion disorder. Psychiatric symptoms frequently accompany this disorder and patients are often first seen by psychiatrists. SPS is autoimmune-based: antibodies are directed against glutamate decarboxylase, resulting in dysregulation of gamma-aminobutyric acid (GABA) in the brain which is considered the cause of the neuropsychiatric symptomatology. SPS should be considered in the differential diagnosis of conversion disorder. Effective management requires early detection, a collaborative approach with GABA-ergic medication and intravenous immunoglobulins, and management of concomitant psychiatric disorders. We describe a patient with SPS. Only one other case has been reported in South Africa.