Stiff-person syndrome

class of disease, rare disease
Subclass of	Movement disorders
Short name	SPS, SPR
Health specialty	neurology
Symptoms and signs	muscle rigidity, myoclonus
Described at URL	https://www.washingtonpost.com/lifestyle/2022/12/08/celine-dion-stiff-person-syndrome/?utm_source=pocket-newtab-global-en-GB
Has phenotype	muscle rigidity
ICD-9-CM	333.91
NCI Thesaurus ID	C85170

Stiff-person syndrome (SPS), dem sanso know as stiff-man syndrome,^[1] be a rare neurological disorder of unclear cause characterized by progressive muscular rigidity den stiffness. De stiffness primarily dey affect de truncal muscles wey be characterised by spasms, wey dey result in postural deformities. Chronic pain, impaired mobility, den lumbar hyperlordosis be common symptoms.^[2]^[3]

SPS dey occur insyd about one insyd a million people wey e be most commonly found insyd middle-aged people. A small minority of patients get de paraneoplastic variety of de condition. Variants of de condition, such as stiff-limb syndrome, wich primarily dey affect a specific limb, often be seen.

Dem first describe SPS insyd 1956. Dem propose diagnostic criteria insyd de 1960s wey dem refine am two decades later. Insyd de 1990s den 2000s, de role of antibodies insyd de condition cam be clearer. SPS patients generally get glutamic acid decarboxylase (GAD) antibodies, wich seldom dey occur insyd de general population. In addition to blood tests for GAD, electromyography tests fi help confirm de condition ein presence.

Benzodiazepine-class drugs be de most common treatment; dem be used for symptom relief from stiffness. Oda common treatments dey include baclofen, intravenous immunoglobin, den rituximab. Limited buh encouraging therapeutic experience of haematopoietic stem cell transplantation dey exist give SPS.^[2]

References

↑ Darras BT, Jones Jr HR, Ryan MM (2014). Neuromuscular Disorders of Infancy, Childhood, and Adolescence (in English). Elsevier Science. p. 188. ISBN 978-0-12-417127-5. The stiff-man syndrome (SMS, also known as stiff-person syndrome) is a rare central nervous system autoimmune disease, but is likely underrecognized.
1 2 "Stiff-Person Syndrome | National Institute of Neurological Disorders and Stroke". www.ninds.nih.gov. Retrieved 2023-01-18.
↑ "Stiff person syndrome - About the Disease - Genetic and Rare Diseases Information Center". rarediseases.info.nih.gov (in English). Archived from the original on 2022-11-15. Retrieved 2023-01-18.

External links

Stiff-Person Syndrome Information Page at NINDS

[Darras2014-1] Darras BT, Jones Jr HR, Ryan MM (2014). Neuromuscular Disorders of Infancy, Childhood, and Adolescence (in English). Elsevier Science. p. 188. ISBN 978-0-12-417127-5. The stiff-man syndrome (SMS, also known as stiff-person syndrome) is a rare central nervous system autoimmune disease, but is likely underrecognized.

[ninds.nih.gov-2] 1 2 "Stiff-Person Syndrome | National Institute of Neurological Disorders and Stroke". www.ninds.nih.gov. Retrieved 2023-01-18.

[3] "Stiff person syndrome - About the Disease - Genetic and Rare Diseases Information Center". rarediseases.info.nih.gov (in English). Archived from the original on 2022-11-15. Retrieved 2023-01-18.

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