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Stiff Person Syndrome (stiff + person_syndrome)
Selected AbstractsDistinct patterns of serum immunoreactivity as evidence for multiple brain-directed autoantibodies in juvenile neuronal ceroid lipofuscinosisNEUROPATHOLOGY & APPLIED NEUROBIOLOGY, Issue 5 2006M. J. Lim Autoantibodies to glutamic acid decarboxylase (GAD65) have been reported in sera from the Cln3,/, mouse model of juvenile neuronal ceroid lipofuscinosis (JNCL), and in individuals with this fatal paediatric neurodegenerative disorder. To investigate the existence of other circulating autoreactive antibodies, we used sera from patients with JNCL and other forms of neuronal ceroid lipofuscinosis (NCL) as primary antisera to stain rat and human central nervous system sections. JNCL sera displayed characteristic patterns of IgG, but not IgA, IgE or IgM immunoreactivity that was distinct from the other forms of NCL. Immunoreactivity of JNCL sera was not confined to GAD65-positive (GABAergic) neurons, but also stained multiple other cell populations. Preadsorption of JNCL sera with recombinant GAD65 reduced the intensity of the immunoreactivity, but did not significantly change its staining pattern. Moreover, sera from Stiff Person Syndrome and Type I Diabetes, disorders in which GAD65 autoantibodies are present, stained with profiles that were markedly different from JNCL sera. Collectively, these studies provide evidence of the presence of autoreactive antibodies within multiple forms of NCL, and are not exclusively directed towards GAD65. [source] Acute ataxia, Graves' disease, and stiff person syndromeMOVEMENT DISORDERS, Issue 13 2007Su-Ynn Chia MD Abstract Stiff person syndrome (SPS) has been associated with autoimmune diseases, such as Type 1 diabetes mellitus and autoimmune thyroid disease (Hashimoto's thyroiditis), among others. The association of SPS with hyperthyroidism is extremely rare. We describe a patient with uncontrolled Graves' disease and undiagnosed SPS, who presented initially with acute ataxia simulating a cerebrovascular accident. Initiation of immunosuppressive therapy dramatically improved the patient's Graves' disease within 2 weeks but the neurological symptoms were not alleviated after a follow-up period of 3 years. © 2007 Movement Disorder Society [source] Stiff Limb Syndrome: End of Spectrum or A Separate Entity?PAIN MEDICINE, Issue 3 2009Usha K. Misra DM 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. [source] Protective and detrimental immunity: lessons from stiff person syndrome and multiple sclerosisACTA NEUROLOGICA SCANDINAVICA, Issue 2009T. Holmøy Background,, The immune system may attack the brain and cause inflammatory disorders like multiple sclerosis (MS). On the other hand, the immune system may protect and support neurons. Methods, There are two obstacles to study this paradox in humans. First, the target antigens in many human central nervous system (CNS) disorders are unknown. Second, it is often difficult to separate pathogenic from protective events, as well as primary from secondary phenomena. Idiopathic stiff person syndrome (SPS) circumvents the first obstacle, because most patients secrete antibodies against glutamic acid decarboxylase (GAD) 65. The immune response against glatiramer acetate (GA) may circumvent the second obstacle. Migration of activated T helper cells to the intrathecal compartment could be a common denominator in GA treatment and SPS. Results, We here discuss recent results on T cells in MS and SPS, showing that GAD65-specific and GA-reactive lymphocytes in the cerebrospinal fluid are not a simple reflection of those in blood. Conclusion, The rules and mechanisms governing T cell selection and maintenance in the CNS may provide a key to the understanding of protective and detrimental aspects of CNS immunity. [source] | ||||||||||