Beth Brianna Murinson1. 1. Department of Neurology, Johns Hopkins School of Medicine, Baltimore, Maryland 21205, USA. Bethmurinson@hotmail.com
Abstract
BACKGROUND: Stiff-person syndrome (SPS) is a rare neurologic disorder with autoimmune features. It is characterized by progressive, severe muscle rigidity or stiffness most prominently affecting the spine and lower extremities. REVIEW SUMMARY: Superimposed muscle spasms result in simultaneous contraction of agonist and antagonist muscles which are detectable by electromyography (EMG) and relieved by administration of benzodiazepines. The exacerbation of SPS by emotional stressors often results in the referral of these patients for psychiatric assessment although this was more common before the discovery of an association with antibodies to glutamic acid decarboxylase (GAD antibodies). Formerly known as stiff-man syndrome, the female to male ratio is 2:1 and the principle paraneoplastic variant is associated with breast cancer. Although rare, this is a disease of middle age that severely curtails the functional capacity of those it strikes. It is frequently associated with diabetes and other autoimmune diseases. IVIg is recently demonstrated to be effective in the treatment of SPS; diazepam remains useful in managing the symptoms. CONCLUSIONS: This article summarizes the history of SPS, describes important clinical features, discusses management, touches upon areas of uncertainty, and postulates some avenues for research.
BACKGROUND:Stiff-person syndrome (SPS) is a rare neurologic disorder with autoimmune features. It is characterized by progressive, severe muscle rigidity or stiffness most prominently affecting the spine and lower extremities. REVIEW SUMMARY: Superimposed muscle spasms result in simultaneous contraction of agonist and antagonist muscles which are detectable by electromyography (EMG) and relieved by administration of benzodiazepines. The exacerbation of SPS by emotional stressors often results in the referral of these patients for psychiatric assessment although this was more common before the discovery of an association with antibodies to glutamic acid decarboxylase (GAD antibodies). Formerly known as stiff-man syndrome, the female to male ratio is 2:1 and the principle paraneoplastic variant is associated with breast cancer. Although rare, this is a disease of middle age that severely curtails the functional capacity of those it strikes. It is frequently associated with diabetes and other autoimmune diseases. IVIg is recently demonstrated to be effective in the treatment of SPS; diazepam remains useful in managing the symptoms. CONCLUSIONS: This article summarizes the history of SPS, describes important clinical features, discusses management, touches upon areas of uncertainty, and postulates some avenues for research.