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Spinal Cord Disease
Published in Philip B. Gorelick, Fernando D. Testai, Graeme J. Hankey, Joanna M. Wardlaw, Hankey's Clinical Neurology, 2020
Inflammatory myelopathies: Demyelinating disease (MS, NMO, etc.).Rheumatologic disorders.Stiff person syndrome.
Neuro-Ophthalmic Literature Review
Published in Neuro-Ophthalmology, 2020
David A. Bellows, Noel C.Y. Chan, John J. Chen, Hui-Chen Cheng, Peter W. MacIntosh, Jenny A. Nij Bijvank, Panitha Jindahra, Michael S. Vaphiades
Stiff-person syndrome (SPS) is a rare neuroimmunological disorder with features of an autoimmune disease. It is characterised by spasm and rigidity of the trunk and limbs. Patients with SPS have elevated serum levels of anti-glutamic acid decarboxylase (anti-GAD65) antibodies, which target GABAergic neurons. The level of GABAergic neurons in the retina is high, particularly in the inner nuclear (INL) and inner plexiform layers. However, visual measurement have not been investigated prospectively in SPS. In this study, SPS patients (n = 23) and healthy controls (n = 28) underwent optical coherence tomography (OCT). SPS patients had significantly lower ganglion cell + inner plexiform layer (GCIPL) thickness and INL thickness as compared to the controls. Additionally, there was a correlation between GCIPL thickness and body regions affected. The measurement of GCIPL thickness could be a biological marker for retinal changes in SPS.
Stiff-person syndrome: an atypical presentation and a review of the literature
Published in Hospital Practice, 2021
Benjamin C. Lin, Jaspreet Johal, Keithan Sivakumar, Alissa E. Romano, Hussam A. Yacoub
Stiff-person syndrome (SPS) is a spectrum of rare autoimmune neurological disorders associated with muscle rigidity and spasms. SPS is characterized by persistent paraspinal and abdominal rigidity and spams triggered by emotional or environmental stimuli [1]. This syndrome was first classified in 1956 by Moersch and Woltman who described progressive muscle rigidity and spasms causing falls and gait disturbance in fourteen patients [2]. Stiff-limb syndrome (SLS) is traditionally considered a subtype of SPS [3]. The pathophysiology of both disorders remains poorly understood, and SLS may be classified as a distinct entity from SPS with some overlapping features.
Managing complications secondary to Waldenström’s macroglobulinemia
Published in Expert Review of Hematology, 2021
Ilias Pessach, Meletios A. Dimopoulos, Efstathios Kastritis
Stiff-person syndrome (SPS) is a paraneoplastic disabling CNS disorder characterized by muscle stiffness and episodic painful spasms that affects the axial and proximal limb muscles through circulating antibodies against glutamic acid decarboxylase (GAD65) [93]. This syndrome can present very rarely together with WM, as a paraneoplastic syndrome probably triggered by IgM proteins behaving as antibodies and can be managed effectively with corticosteroids and rituximab [93].