Clinical Spectrum of Stiff Person Syndrome: A Review of Recent Reports

Sarva, Harini; Deik, Andres; Ullah, Aman; Severt, William Lawrence

doi:10.7916/d85m65gd

Cited by 32 publications

(8 citation statements)

References 87 publications

(71 reference statements)

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“…Immune-mediated pathomechanisms that target proteins mainly expressed by inhibitory synapses have been associated with stiff person spectrum disorder . Autoantibodies that have been identified include GAD65, GlyR, amphiphysin, dipeptidyl peptidase-like protein 6, gephyrin, γ-aminobutyric acid-A receptor (GABA A R), and GABA receptor associated protein (GABARAP) .…”

Section: Discussionmentioning

confidence: 99%

Spasms and Myoclonus in a Young Woman With Hashimoto Thyroiditis

2020

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Section: Discussionmentioning

confidence: 99%

Spasms and Myoclonus in a Young Woman With Hashimoto Thyroiditis

2020

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“…Benzodiazepines are used for treatment. 58 Paraneoplastic motor neuron syndrome. Paraneoplastic motor neuron disorders are often seen in older patients with progressive myelopathy, which often manifests prior to detection of an underlying cancer.…”

Section: Paraneoplastic Disordersmentioning

confidence: 99%

Autoimmune diseases of the spine and spinal cord

et al. 2023

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Magnetic resonance imaging (MRI) and clinicopathological tools have led to the identification of a wide spectrum of autoimmune entities that involve the spine. A clearer understanding of the unique imaging features of these disorders, along with their clinical presentations, will prove invaluable to clinicians and potentially limit the need for more invasive procedures such as tissue biopsies. Here, we review various autoimmune diseases affecting the spine and highlight salient imaging features that distinguish them radiologically from other disease entities.

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“…As GABA is a major inhibitory neurotransmitter, it is postulated that this reduction results in excessive neuronal activity including an augmented motor neuron drive manifesting as muscle spasm and rigidity. 1,2 A few case reports have described the anesthetic care provided to patients with SPS. Unpredictably, some patients may experience postoperative hypotonia of variable severity and duration (Table 1).…”

Section: Re ´Sumementioning

confidence: 99%

Postoperative hypotonia in a patient with stiff person syndrome: a case report and literature review

Elsherbini

Weingartshofer²,

Backman³

2022

Can J Anesth/J Can Anesth

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Purpose Stiff person syndrome (SPS), an autoimmune disease that manifests with episodic muscle rigidity and spasms, has anesthetic considerations because postoperative hypotonia may occur. This hypotonia has been linked to muscle relaxants and volatile anesthetics and may persist in spite of neostigmine administration and train-of-four (TOF) monitoring suggesting full reversal. We present a patient with SPS who experienced hypotonia following total intravenous anesthesia (TIVA), which was promptly reversed with sugammadex. These observations are considered in light of the relevant medical literature. Clinical features A 46-yr-old female patient with SPS underwent breast lumpectomy and sentinel node biopsy. Anesthesia consisted of TIVA (propofol/remifentanil) with adjunctive administration of rocuronium 20 mg to obtain adequate intubating conditions. Despite return of the TOF ratio to 100% within 30 min, hypotonia was clinically evident at conclusion of surgery two hours later.

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Clinical Spectrum of Stiff Person Syndrome: A Review of Recent Reports

Cited by 32 publications

References 87 publications

Spasms and Myoclonus in a Young Woman With Hashimoto Thyroiditis

Spasms and Myoclonus in a Young Woman With Hashimoto Thyroiditis

Autoimmune diseases of the spine and spinal cord

Postoperative hypotonia in a patient with stiff person syndrome: a case report and literature review

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