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Časopis Neurology – Článok Stiff person syndrome

Neurology

Reviewed, postgraduate scietific medical journal.
Period 3x per year
1336-8621
The journal is indexed in the Slovak National Bibliography, Bibliographiia Medica Slovaca (BMS) and listed to citation database CiBaMed. All articles are reviewed. The publisher does not bear any responsibility for data and opinions of particular authors of the articles or advertisements. The articles on grey pages are company promotions or non reviewed information, an author is responsible for the content. Any reproduction of the content is allowed only with direct consent of the editorial office.
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Neurology
Neurology
Reviewed, postgraduate scietific medical journal.
Period 3x per year
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Téma: Case studies

Stiff person syndrome

Petra Došeková, Georgi Krastev, Jozef Haring, Rastislav Lackovič

Objective: Two case reports of Stiff person syndrome (SPS) and SPS variant- Stiff limb syndrome.
Background: Stiff person syndrome is a rare autoimmune disorder of central nervous system characterized by progressive
rigidity of axial and limb muscles and episodic painful spasms. SPS can be classified into classic SPS, paraneoplastic
SPS and its variants- e.g. Stiff limb syndrome. Diagnostic criteria are based on typical clinical signs and EMG findings
showing contionuous motor unit activity at rest. In most cases antibodies against acid decarboxylase (GAD) are
observed. Treatment is symptomatic using drugs that enhance GABA transimission and immnodomudulatory therapy.
Methods: Recommended guidelines for SPS therapy with use of symptomatic treatment and immunomodulatory therapy.
Results: Two case report: Patient 1 is a 41 year old patient with classis SPS. The paraneoplastic etiology in this patient
was ruled out, also there was no evidence for any autoimmune disorder. Symptomatic therapy with baclofen (3 x 25 mg)
and diazepam (3 x 5 mg), following with 5 sessions of plasma exchange showed no benefit. Afterwards he was treated
with IVIG (0,4 g/kg/day given over 5 days) with significant improvement lasting 7 months when treatment with IVIG
was repeated. After 2 months the treatment with IVIG was repeated. Eventually we started chronic immunosuppresive
treatment with azathioprine (100 mg/d) and methylprednisolone (16 mg/d). We continue symptomathical treatment using
GABA-analogs. Treatment is well tolerated, patient is with no significant disability. Patient 2 is a 52 year old woman
treated for Hashimoto´s thyroiditis, diagnosed with SPS variant- Stiff limb syndrome affected her right upper limb. The
symptomatic therapy with baclofen (30 mg/d) and diazepam (3 x 5 mg) was not tolerated, plasma exchange showed no
benefit. We started treatment with IVIG (0,4 g/kg/day given over 5 days) with significant benefit. Afterwards we started
chronic treatment with azathioprine (100 mg/d) and methyprednisolone (16 mg/d), symptomatical treatment using
GABA-analogs is continued. Treatment is well tolerated with good clinical benefit with no need to repeat IVIG therapy.
Conclusions: SPS is a rare disorder, often misdiagnosed, highly heterogeneous in its manifestation. If left untretaded it
could lead to serious disability or eventually to death. Treatment is difficult and change from case to case.

stiff person syndrome, anti-GAD antibodies, Moersch-Woltmann syndrome, IVIG
Neurológia 2016; 11 (2): 95-100

Ročník 2016  Témy časopisu Neurology 2 / 2016

Original works

Peter Jankovič, Miroslav Galanda, Stanislav Okapec
Anna Kružlíková, Viera Jančulová

Overview works

Ján Necpál
Klaudia Kalmárová, Vladimír Nosáľ, Juraj Sokol, Ingrid Škorňová, Egon Kurča
Radomír Taláb, Marika Talábová

Case studies

Ivan Martinka, Andrea Hergottová, Katarína Sitárová, Miriam Sosková, Peter Špalek
Petra Došeková, Georgi Krastev, Jozef Haring, Rastislav Lackovič
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Nie sú dostupné žiadne voľne dostupné články
CHAIRMAN OF THE EDITORIAL BOARD
doc. MUDr. Miroslav Brozman, CSc.

MEMBERS OF THE EDITORIAL BOARD
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doc. MUDr. Eleonóra Klímová, CSc.
doc. MUDr. Pavol Kučera, PhD.
MUDr. Marian Kuchar, PhD.
doc. MUDr. Robert Mikulík,Ph.D., FESO
MUDr. Vladimír Nosáľ, PhD.
MUDr. Ľubica Procházková, CSc.
prof. MUDr. Bruno Rudinský, CSc.
doc. MUDr. Daniel Šaňák, Ph.D.
doc. MUDr. David Školoudík, Ph.D., FESO
prof. MUDr. Karel Šonka, DrSc.
doc. MUDr. Peter Špalek, PhD.
Dr. Milan R. Voško, PhD.

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Eva Stachová
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OVERVIEW PAPERS
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Citations are numbered chronologically in bold, references in the text are stated by the number of citations in parentheses. Use maximum 20 citations.

Examples of citations:
1. Pitt B, et al. The effect of spironolactone on morbidity and mortality in patients with severe heart failure. Randomized Aldactone Evaluation Study Investigators. N Engl J Med 1999; 341: 709-717.
2. Stenestrand U, Wallentin L. Swedish Register of Cardiac Intensive Care (RIKS-HIA): Early statin treatment following acute myocardial infarction and 1-year survival. JAMA 2001; 285(4): 430-436.
3. LIPID Study Group. Prevention of cardiovascular events and death with pravastatin in patients with coronary heart disease and a broad range of initial cholesterol levels. N Engl J Med 1998; 339: 1349-1357.
4. Jurkovičová O, Spitzerová H, Cagáň S. Komorové arytmie a náhla srdcová smrť pri akútnom infarkte myokardu. Bratisl Lek Listy 1997; 98: 379-389.
5. Osborne BE. The electrocardiogram of the rat. In: Budden R, Detweiler DK, Zbinden G. The rat electrocardiogram in pharmacology and toxicology. Oxford: Pergamon Press 1981: 15-27.

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Which of following factors is not related to rosacea?
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b. Scandinavian origin
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d. endothelial growth factor

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Period 3x per year
1336-8621
The journal is indexed in the Slovak National Bibliography, Bibliographiia Medica Slovaca (BMS) and listed to citation database CiBaMed. All articles are reviewed. The publisher does not bear any responsibility for data and opinions of particular authors of the articles or advertisements. The articles on grey pages are company promotions or non reviewed information, an author is responsible for the content. Any reproduction of the content is allowed only with direct consent of the editorial office.
Predplatné
Neurology
Neurology
Reviewed, postgraduate scietific medical journal.
Period 3x per year
Predplatné