Stiff-Person Syndrome and Related Disorders

A Comprehensive, Practical Guide

Stiff-Person Syndrome and Related Disorders

A Comprehensive, Practical Guide

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This comprehensive title covers all of the broad aspects of stiff-person spectrum disorders (SPSD), ranging from clinical features and laboratory investigations to the basic scientific roles of neuroimmunology and genetics that aid clinicians in understanding the pathogenesis of this disorder.

Organized across 15 chapters, this highly practical book begins with a thorough account of the history and clinical phenomenology of SPSD.  Subsequent chapters then delve into the differential diagnoses of the disorder, as well as its electrophysiology, immunopathogenesis, and neurochemistry. Following this is an analysis of each subtype of SPSD including classic stiff-person syndrome and its variants, correlations between clinical phenotypes and antibodies, as well as SPSD in the pediatric population. Finally, the book concludes with an examination of the various SPSD diagnostic approaches, treatments, and potential emergencies seen in clinical practice.

Developed by a renowned multidisciplinary authorship, Stiff-Person Syndrome and Related Disorders is an indispensable contribution to the clinical literature and will be of great interest to both clinicians and basic science researchers.




Historical background of stiff-person syndromes
Clinical phenomenology and diagnostic criteria of stiff-person syndrome and stiff-person syndrome variants
Differential diagnosis of stiff-person syndromes
Electrophysiology of stiff-person syndromes
Neurochemistry of inhibitory synapses and clinical applications in stiff-person syndromes
Immunopathogenesis of stiff-person syndromes
Antibodies in stiff-person syndrome and their correlations with clinical phenotypes
Anti-glutamic acid decarboxylase 65 (GAD65)-associated syndromes
Progressive encephalomyelitis with rigidity and myoclonus (PERM) syndrome
Emergencies in stiff-person syndrome
Diagnostic approach in stiff-person syndrome and its variants
Treatment of stiff-person syndromes
Pediatric stiff-person syndromes
Peripheral hyperexcitability syndromes
Hyperekplexia.
ISBN 978-3-030-43061-0
Artikelnummer 9783030430610
Medientyp Buch
Auflage 1st ed. 2020
Copyrightjahr 2021
Verlag Springer, Berlin
Umfang XV, 183 Seiten
Abbildungen XV, 183 p. 46 illus., 20 illus. in color.
Sprache Englisch