User:Mr. Ibrahem/Stiff-person syndrome
| Stiff-person disease | |
|---|---|
| Other names | Stiff-man syndrome (SMS), Moersch-Woltman syndrome[1] |
| File:Stiff person syndrome wrist.png | |
| Wrist in fist-like position | |
| Specialty | Neurology |
| Symptoms | Stiff muscles, spasms, pain[2][3] |
| Complications | Poor posture, bone fractures, frequent falls, lumbar hyperlordosis[2][1] |
| Usual onset | 20 to 60[3] |
| Duration | Progressive worsening[3] |
| Types | Classic, partial, progressive encephalomyelitis with rigidity and myoclonus (PERM)[3] |
| Causes | Unclear, autoimmune mechanism[2] |
| Diagnostic method | Based on symptoms and supported by blood tests and electromyography[3] |
| Differential diagnosis | Parkinsons, multiple sclerosis, fibromyalgia, psychosomatic illness, anxiety[2] |
| Treatment | Diazepam, baclofen, gabapentin, intravenous immunoglobulin[2][4] |
| Prognosis | Often poor[4][5] |
| Frequency | Rare[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[edit]
- ^ a b c d e f g "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.
- ^ a b c d e f g h i j k l m "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.
- ^ a b c d e f g Muranova, A; Shanina, E (January 2022). "Stiff Person Syndrome". PMID 34424651.
{{cite journal}}: Cite journal requires|journal=(help) - ^ a b c d e f g h 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) - ^ a b c d e 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.