Stiff person syndrome nord national organization for. Quantitative clinical and autoimmune assessments in stiff person. Significant improvement of stiff person syndrome after paraspinal injection of botulinum toxin a. Vicari am, folli f, pozza g, comi gc, comola m, canal n, besana c, borri a, tresoldi m, solimena m, et al. Stiff person syndrome is a neurological disease and autoimmune disorder. Stiff person syndrome sps affects only about one or two in a million people. Diagnostic and statistical manual for mental disorders dsmiv axis i. While stiff person syndrome is now considered a compassionate allowance by the ssa, and therefore qualifies for expedited processing, the diagnosis alone is not enough to be found eligible for disability benefits. Multiple similar case descriptions have since followed. Stiff person syndrome sps is a rare and disabling neurological disorder of. Being diagnosed with this very rare condition can leave patients and families with many questions and few answers. Stiff person syndrome sps is characterized by rigidity of the truncal. We report a case of stiffperson syndrome associated with several autoimmune diseases.
Stiff person syndrome living forward home facebook. Find local stiffperson syndrome resources for the top u. It is a postsynaptic cellular protein responsible for clustering of two inhibitory neurotransmitters. However, its also regarded as an autoimmune disease. When sps affects only one specific area of the body, it may also be referred to as focal or partial stiffperson syndrome. Your stiff person syndrome social security disability case. Sps is characterized by fluctuating muscle rigidity in the trunk and limbs and a heightened sensitivity to stimuli such as noise, touch, and emotional distress, which can set off muscle spasms. Introduction stiff man syndrome was first described in 1956 by moersch and woltman. Successful treatment of stiff man syndrome with intravenous. Stiff person syndrome center johns hopkins hospital. The stiff person syndrome center at johns hopkins department of neurology can offer clarity, effective symptom management and hope for the future.
Stiffperson syndrome sps is a rare neurological disorder with features of an autoimmune disease. Stiffperson syndrome sps, formerly called stiffman syndrome is an. The treatment of sps is directed toward the specific symptoms that are apparent in each individual which often requires a multifaceted approach including non. Symptoms may include extreme muscle stiffness, rigidity and painful spasms in the trunk and limbs, severely impairing mobility. Stiff limb syndrome is a clinical feature of the stiff person syndrome, which is a rare and disabling neurologic disorder characterized by muscle rigidity and episodic spasms that involve axial. To raise public stiff person syndrome awareness about the daily challenges living with this rare disease. The term stiff person may be seen to exclude infants, and stiff man is inappropriate.
Backgroundlittle information is available about the incidence of stiffman syndrome sms the classic form or its variants or about longterm treatment respon. Stiff person syndrome sps is a rare, progressive syndrome that affects the nervous system, specifically the brain and spinal cord. Table 1 other autoantibodies associated with stiff person syndrome sps1012 gephyrin present in a very small proportion of the paraneoplastic variant. Monitoring antibody titers during the course of treatment is, hence, unnecessary. Plasmapheresis in the treatment of stiff man syndrome. Jerking stiffperson syndrome is characterized by muscles stiffness and spasms usually affecting the legs. You must include substantial proof of disability in your application. Some individuals may eventually develop classic stiffperson syndrome or variant sps. Stiff person syndrome associated with compartment syndrome.