Stiff Person Spectrum Disorders—An Update and Outlook on Clinical, Pathophysiological and Treatment Perspectives

Biomedicines, Год журнала: 2023, Номер 11(9), С. 2500 - 2500

Опубликована: Сен. 10, 2023

Stiff person spectrum disorders (SPSD) are paradigm autoimmune movement characterized by stiffness, spasms and hyperekplexia. Though rare, SPSD represent a not-to-miss diagnosis because of the associated disease burden treatment implications. After decades as an enigmatic orphan disease, major advances in our understanding evolving diseases have been made along with identification multiple autoantibodies. However, most important recent developments relate to recognition wider affection, beyond classic core motor symptoms, further insights into immunomodulatory symptomatic therapies. In this review, we summarize literature on clinical paraclinical spectrum, current pathophysiological understanding, well possibly future therapeutic strategies.

Язык: Английский

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Stiff-person syndrome

Practical Neurology, Год журнала: 2024, Номер unknown, С. pn - 003974

Опубликована: Сен. 2, 2024

Stiff-person syndrome (SPS) is an autoimmune disease associated mainly with antibodies to glutamic acid decarboxylase (GAD) or glycine, characterised by intermittent painful spasms, stiffness and rigidity of the proximal truncal muscles. Neuro-ophthalmological gastrointestinal symptoms also occur. The are caused neuronal excitability due impaired inhibitory (gamma amino butyric [GABA] glycine) neurotransmission. SPS part a larger spectrum GAD antibody-spectrum disorders, which overlaps epilepsy, cerebellar ataxia, myoclonus, progressive encephalomyelitis, myoclonus (PERM) limbic encephalitis. PERM often against glycine receptor. Some cases paraneoplastic. Diagnostic delay irreversible disability, therefore, clinicians need high degree clinical suspicion make earlier diagnosis. This review updates various presentations that should raise its related conditions includes diagnostic algorithm treatment strategies including immunotherapy GABA-ergic drugs.

Язык: Английский

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Stiff Person Syndrome Spectrum Disorders: An Update on Putative Pathophysiology, Clinical Presentations, and Treatment Considerations

Elsevier eBooks, Год журнала: 2024, Номер unknown

Опубликована: Янв. 1, 2024

Язык: Английский

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Stiff-Person Syndrome With Respiratory Complications

Archivos de Bronconeumología, Год журнала: 2023, Номер 60(3), С. 173 - 174

Опубликована: Дек. 20, 2023

Язык: Английский

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