Stiff-man syndrome: From the bedside to the bench

Helfgott, Simon M.

doi:10.1002/1529-0131(199907)42:7<1312::aid-anr2>3.0.co;2-w

Cited by 40 publications

(21 citation statements)

References 80 publications

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“…SPS presents with progressive muscle rigidity and spasms of axial muscles and extremities 2. The majority of SPS patients have serum autoantibodies against glutamic acid decarboxylase (GAD)65,3 an enzyme responsible for the synthesis of γ-aminobutyric acid (GABA), the major inhibitory neurotransmitter of the central nervous system (CNS).…”

mentioning

confidence: 99%

Monozygotic twins with stiff person syndrome and autoimmune thyroiditis: rituximab inefficacy in a double-blind, randomised, placebo controlled crossover study

et al. 2009

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mentioning

confidence: 99%

Monozygotic twins with stiff person syndrome and autoimmune thyroiditis: rituximab inefficacy in a double-blind, randomised, placebo controlled crossover study

et al. 2009

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“…Although classic stiff-person syndrome includes muscle rigidity throughout the entire body, the axial musculature as well as the musculature of the limbs are almost always involved [2][3]. A variant of classic stiff-person syndrome is partial stiff-person syndrome, where the muscular spasms are limited to either the upper or lower limbs, and the trunk is spared [4].…”

Section: Discussionmentioning

confidence: 99%

“…Due to this autoimmune etiology, stiffperson syndrome is also thought to be associated with other autoimmune conditions such as diabetes mellitus type 1 [7]. SPS has also been seen in concurrence with conditions such as vitiligo, pernicious anemia, and thyroiditis [2,8].…”

Section: Discussionmentioning

confidence: 99%

The Stiff People: Two Rare Cases of Stiff-person Syndrome

Siddiqui

Tohid

Brown

et al. 2017

Cureus

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“…Less than 1% of patients with this disease have an underlying malignancy. 55 Glutamic acid decarboxylase (GAD) antibodies are usually present in PSPS. 56 A related disorder, progressive encephalomyelitis with rigidity and myoclonus (PERM) causes stiffness and stimulus-sensitive spasms of the muscles leading to brain stem dysfunction.…”

Section: Neurological Paraneoplastic Phenomenamentioning

confidence: 99%

Paraneoplastic Manifestations of Lymphoproliferative Neoplasms

2016

Lymphoma and Chronic Lymphocytic Leukemias

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Paraneoplastic syndromes, although rare, have been associated with lymphoproliferative disorders. Hodgkin's lymphoma (HL) is the most common lymphoid neoplasm known to cause paraneoplastic syndromes. These syndromes can often be the earliest manifestation of the underlying malignancy, and treating the underlying lymphoma can cure the paraneoplastic disease. Paraneoplastic diseases reported in lymphoid malignancies can be broadly classified into hematological, neurological, and dermatological syndromes based on the organ systems that are predominantly involved. In addition, renal and hepatobiliary involvement has also been reported. The pathogenesis of the hematological paraneoplastic conditions primarily involves the production of autoantibodies by the neoplastic lymphocytes, which then subsequently leads to cytopenias. Cytoses are a result of cytokine produced by the neoplasms. The administration of corticosteroids along with chemotherapy for the underlying malignancy is the treatment of choice. Neurological paraneoplastic phenomena have been reported in both HL and non-Hodgkin's lymphoma (NHL). They are thought to be secondary to an immune-related process that is triggered by the underlying process. Both the central and the peripheral nervous systems can be affected. Often, treatment of the underlying malignancy with chemotherapy can result in reversal of the paraneoplastic syndrome. In peripheral neuropathies, muscle relaxants and analgesics are often used for symptomatic relief. Dermatological manifestations, pemphigus vulgaris in particular, often precedes the malignancy. Renal and hepatobiliary manifestations, although rare, are also associated with lymphomas.

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Stiff-man syndrome: From the bedside to the bench

Cited by 40 publications

References 80 publications

Monozygotic twins with stiff person syndrome and autoimmune thyroiditis: rituximab inefficacy in a double-blind, randomised, placebo controlled crossover study

Monozygotic twins with stiff person syndrome and autoimmune thyroiditis: rituximab inefficacy in a double-blind, randomised, placebo controlled crossover study

The Stiff People: Two Rare Cases of Stiff-person Syndrome

Paraneoplastic Manifestations of Lymphoproliferative Neoplasms

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