About: Stiff-person syndrome following tick-born meningoencephalatis.

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About: Stiff-person syndrome following tick-born meningoencephalatis. Goto Sponge NotDistinct Permalink

An Entity of Type : http://linked.opendata.cz/ontology/domain/vavai/Vysledek, within Data Space : linked.opendata.cz associated with source document(s)

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rdf:type	skos:Concept http://linked.opendata.cz/ontology/domain/vavai/Vysledek
Description	Stiff-person syndrome (SPS) is a rare disorder characterized by muscle stiffness and painful spasms. Misdiagnosis may occur due to the fact that the clinical picture of SPS is often atypical. The main pathophysiologic mechanism underlying the development of SPS is insufficient inhibition at the cortical and spinal levels. There is good evidence for a primary autoimmune etiology. A 61-year-old man was admitted to a neurological department due to muscle hypertonia with episodic attacks of painful spasms predominantly affecting axial muscles. The symptoms developed shortly after tickborne meningoencephalitis. Electromyography (EMG) revealed signs of continuous motor unit activity. Antibodies against glutamate decarboxylase (anti-GAD) were highly elevated. We present a case of a man who developed clinically severe anti-GAD positive SPS, provoked by tick-borne encephalitis. After therapeutic plasma exchange (TPE) a rapid, temporary improvement of the clinical and neurophysiological findings was noted. Only after being placed on long-term immunosuppression did the patient achieve stable recovery. This case supports the importance of EMG findings and demonstrates the effect of TPE as well as the need for chronic immunosuppression in severe cases of SPS. Stiff-person syndrome (SPS) is a rare disorder characterized by muscle stiffness and painful spasms. Misdiagnosis may occur due to the fact that the clinical picture of SPS is often atypical. The main pathophysiologic mechanism underlying the development of SPS is insufficient inhibition at the cortical and spinal levels. There is good evidence for a primary autoimmune etiology. A 61-year-old man was admitted to a neurological department due to muscle hypertonia with episodic attacks of painful spasms predominantly affecting axial muscles. The symptoms developed shortly after tickborne meningoencephalitis. Electromyography (EMG) revealed signs of continuous motor unit activity. Antibodies against glutamate decarboxylase (anti-GAD) were highly elevated. We present a case of a man who developed clinically severe anti-GAD positive SPS, provoked by tick-borne encephalitis. After therapeutic plasma exchange (TPE) a rapid, temporary improvement of the clinical and neurophysiological findings was noted. Only after being placed on long-term immunosuppression did the patient achieve stable recovery. This case supports the importance of EMG findings and demonstrates the effect of TPE as well as the need for chronic immunosuppression in severe cases of SPS. (en)
Title	Stiff-person syndrome following tick-born meningoencephalatis. Stiff-person syndrome following tick-born meningoencephalatis. (en)
skos:prefLabel	Stiff-person syndrome following tick-born meningoencephalatis. Stiff-person syndrome following tick-born meningoencephalatis. (en)
skos:notation	RIV/00216275:25520/11:39892721!RIV12-MSM-25520___
http://linked.open...avai/riv/aktivita	I
http://linked.open...avai/riv/aktivity	I
http://linked.open...iv/cisloPeriodika	4
http://linked.open...vai/riv/dodaniDat	2012
http://linked.open...aciTvurceVysledku	Mandysová, Petra Latta, Ján Ehler, Edvard
http://linked.open.../riv/druhVysledku	J - Článek v odborném periodiku
http://linked.open...iv/duvernostUdaju	S - Úplné a pravdivé údaje nepodléhající ochraně podle zvláštních právních předpisů
http://linked.open...titaPredkladatele	Univerzita Pardubice / Fakulta zdravotnických studií
http://linked.open...dnocenehoVysledku	232387
http://linked.open...ai/riv/idVysledku	RIV/00216275:25520/11:39892721
http://linked.open...riv/jazykVysledku	eng - angličtina
http://linked.open.../riv/klicovaSlova	Antibodies against glutamic acid decarboxylase (anti-GAD); Painful muscle cramps; Tick-borne meningoencephalitis; Stiff-person syndrome (en)
http://linked.open.../riv/klicoveSlovo	Antibodies against glutamic acid decarboxylase (anti-GAD) Painful muscle cramps Stiff-person syndrome Tick-borne meningoencephalitis
http://linked.open...odStatuVydavatele	CZ - Česká republika
http://linked.open...ontrolniKodProRIV	[D149AF4A9705]
http://linked.open...i/riv/nazevZdroje	Acta Medica
http://linked.open...in/vavai/riv/obor	FH
http://linked.open...ichTvurcuVysledku	3 (xsd:int)
http://linked.open...cetTvurcuVysledku	5 (xsd:int)
http://linked.open...UplatneniVysledku	2011
http://linked.open...v/svazekPeriodika	2011
http://linked.open...iv/tvurceVysledku	Ehler, Edvard Latta, Ján Mrklovský, Milan Mandysová, Petra Havlasová, Jana
issn	1211-4286
number of pages	5 (xsd:int)
http://localhost/t...ganizacniJednotka	25520

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