Recent Advances and Review on Treatment of Stiff Person Syndrome in Adults and Pediatric Patients

Abstract

Stiff Person Syndrome (SPS) is one of the rarest autoimmune neurological disorders, which is mostly reported in women. It is characterised by fluctuating muscle rigidity and spasms. There are many variants of SPS, these include the classical SPS, Stiff Leg Syndrome (SLS), paraneoplastic variant, gait ataxia, dysarthria, and abnormal eye movements. Studies have shown that the paraneoplastic variant of SPS is more common in patients with breast cancer who harbour amphiphysin antibodies, followed by colon cancer, lung cancer, Hodgkin's disease, and malignant thymoma. Currently, the treatment for SPS revolves around improving the quality of life by reducing the symptoms as far as possible with the use of GABAergic agonists, such as diazepam or other benzodiazepines, steroids, plasmapheresis, and intravenous immunoglobulin (IVIG). There have been random clinical trials with Rituximab, but nothing concrete has been suggested. A treatment approach with standard drugs and cognitive behavioral therapy (CBT) seems to be promising.

Stiff Person Syndrome-The Less Common Antibody: A Case Report

“…Benzodiazepines may be the first-choice treatment (6). Oral baclofen, or for severe cases, intrathecal baclofen can be used (6).…”

“…Benzodiazepines may be the first-choice treatment (6). Oral baclofen, or for severe cases, intrathecal baclofen can be used (6). Other medication options are valproic acid, levetiracetam, tiagabin, and gabapentin for symptomatic treatment (6).…”

“…Oral baclofen, or for severe cases, intrathecal baclofen can be used (6). Other medication options are valproic acid, levetiracetam, tiagabin, and gabapentin for symptomatic treatment (6). Steroids, IVIG, and plasmapheresis are needed for immunosuppression (6).…”

Stiff Person Syndrome and Type 1 Diabetes Mellitus: a Case of the Chicken or the Egg?

“…2,3 Superimposed sudden painful episodic spasms are common 2,3,8 and can be forceful enough to bend surgical pins, dislocate joints, and cause femoral fractures. [10][11][12][13] Spasms are triggered by heightened sensitivities to external stimuli such as unexpected auditory, tactile, or visual stimuli, and to psychological stimuli such as emotional upset, anxiety, or task-specific phobias. 2,3,8,13,14 A subset of spasms involving the respiratory or thoracic musculature can result in lifethreatening restrictive respiratory failure.…”

“…[10][11][12][13] Spasms are triggered by heightened sensitivities to external stimuli such as unexpected auditory, tactile, or visual stimuli, and to psychological stimuli such as emotional upset, anxiety, or task-specific phobias. 2,3,8,13,14 A subset of spasms involving the respiratory or thoracic musculature can result in lifethreatening restrictive respiratory failure. 2,15 The spasms in our patient were linked to the task-specific phobia of tracheostomy capping trials, resulting in repeated bouts of acute hypoxic respiratory failure.…”