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User:Mr. Ibrahem/Stiff-person syndrome

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Stiff-person disease
Other namesStiff-man syndrome (SMS), Moersch-Woltman syndrome[1]
Wrist in fist-like position
Medical specialtyNeurology
SymptomsStiff muscles, spasms, pain[2][3]
ComplicationsPoor posture, bone fractures, frequent falls, lumbar hyperlordosis[2][1]
Usual onset20 to 60[3]
DurationProgressive worsening[3]
TypesClassic, partial, progressive encephalomyelitis with rigidity and myoclonus (PERM)[3]
CausesUnclear, autoimmune mechanism[2]
Diagnostic methodBased on symptoms and supported by blood tests and electromyography[3]
Differential diagnosisParkinsons, multiple sclerosis, fibromyalgia, psychosomatic illness, anxiety[2]
TreatmentDiazepam, baclofen, gabapentin, intravenous immunoglobulin[2][4]
PrognosisOften poor[4][5]
FrequencyRare[1]

Stiff-person syndrome (SPS) is a neurologic disorder characterized by stiff muscles and spasms which worsen over time.[1][2] It primarily affects the torso, arms, and legs.[2] Spasms can be triggered by sound, touch, or emotions.[1] Complications may include poor posture, bone fractures, chronic pain, and frequent falls.[1][2][4]

In most cases the cause is unclear, though an autoimmune mechanism is believed to be involved.[2] Many cases are associated with other autoimmune conditions, such as type 1 diabetes.[5] Uncommonly it occurs as a paraneoplastic syndrome.[4][5] Diagnosis is often by finding very high levels of antibodies to glutamic acid decarboxylase (GAD) in the blood or cerebral spinal fluid.[2][4] Electromyography may also be useful.[1]

Symptoms may be treated with medications such as diazepam, baclofen, or gabapentin.[2] Intravenous immunoglobulin or plasmapheresis may also help.[2][4] It generally results in a poor quality of life, with depression or anxiety often occurring as a result.[4][5] Life expectancy is reduced.[5]

Stiff-person syndrome is rare, occurring in about one in a million people.[4] Onset is most often between the ages of 20 and 60, though may uncommonly occur in children.[3] Women are affected twice as often as men.[2] The condition was first described in 1956 by Frederick Moersch and Henry Woltman.[3]

References

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  1. 1.0 1.1 1.2 1.3 1.4 1.5 1.6 "Stiff person syndrome - About the Disease - Genetic and Rare Diseases Information Center". rarediseases.info.nih.gov. Archived from the original on 15 November 2022. Retrieved 9 December 2022.
  2. 2.00 2.01 2.02 2.03 2.04 2.05 2.06 2.07 2.08 2.09 2.10 2.11 2.12 "Stiff-Person Syndrome | National Institute of Neurological Disorders and Stroke". www.ninds.nih.gov. Archived from the original on 8 December 2022. Retrieved 9 December 2022.
  3. 3.0 3.1 3.2 3.3 3.4 3.5 3.6 Muranova, A; Shanina, E (January 2022). "Stiff Person Syndrome". PMID 34424651. {{cite journal}}: Cite journal requires |journal= (help)
  4. 4.0 4.1 4.2 4.3 4.4 4.5 4.6 4.7 Ortiz, JF; Ghani, MR; Morillo Cox, Á; Tambo, W; Bashir, F; Wirth, M; Moya, G (9 December 2020). "Stiff-Person Syndrome: A Treatment Update and New Directions". Cureus. 12 (12): e11995. doi:10.7759/cureus.11995. PMID 33437550.{{cite journal}}: CS1 maint: unflagged free DOI (link)
  5. 5.0 5.1 5.2 5.3 5.4 Hadavi, S; Noyce, AJ; Leslie, RD; Giovannoni, G (October 2011). "Stiff person syndrome". Practical neurology. 11 (5): 272–82. doi:10.1136/practneurol-2011-000071. PMID 21921002.