Stiff-Person Syndrome With Respiratory Complications DOI

Elia Gimenez,

Jesús Sancho, Jaime Signes‐Costa

et al.

Archivos de Bronconeumología, Journal Year: 2023, Volume and Issue: 60(3), P. 173 - 174

Published: Dec. 20, 2023

Language: Английский

Stiff Person Spectrum Disorders—An Update and Outlook on Clinical, Pathophysiological and Treatment Perspectives DOI Creative Commons
Benjamin Vlad, Yujie Wang, Scott D. Newsome

et al.

Biomedicines, Journal Year: 2023, Volume and Issue: 11(9), P. 2500 - 2500

Published: Sept. 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.

Language: Английский

Citations

9
Stiff-person syndrome DOI

Smriti Bose,

Saiju Jacob

Practical Neurology, Journal Year: 2024, Volume and Issue: unknown, P. pn - 003974

Published: Sept. 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.

Language: Английский

Citations

2
Stiff Person Syndrome Spectrum Disorders: An Update on Putative Pathophysiology, Clinical Presentations, and Treatment Considerations DOI
Yujie Wang, Scott D. Newsome

Elsevier eBooks, Journal Year: 2024, Volume and Issue: unknown

Published: Jan. 1, 2024

Language: Английский

Citations

0
Stiff-Person Syndrome With Respiratory Complications DOI

Elia Gimenez,

Jesús Sancho, Jaime Signes‐Costa

et al.

Archivos de Bronconeumología, Journal Year: 2023, Volume and Issue: 60(3), P. 173 - 174

Published: Dec. 20, 2023

Language: Английский

Citations

0