Stiff-Person Syndrome and Related Disorders

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.

A first-of-its-kind, comprehensive book on this dedicated topic Authored by two neuromuscular and one movement disorder experts Covers both clinical and basic science aspects of these disorders, suitable for a wide range of audience including clinicians and basic scientists

Autorentext

Pichet Termsarasab, MDFaculty of Medicine Ramathibodi HospitalMahidol UniversityBangkok, Thailand Thananan Thammongkolchai, MDFaculty of Medicine Ramathibodi HospitalMahidol UniversityBangkok, Thailand

Bashar Katirji, MDAcademic affiliation: Professor of Neurology, University Hospitals Cleveland Medical Center, Cleveland, OH, USA. bashar.katirji@uhhospitals.orgPhone number: (216) 844-4854

Inhalt
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.