Stiff-Man Syndrome in Childhood

Markandeyulu, V; Joseph, Thomas; Solomon, T; Jacob, Joe Antony; Kumar, Sudhir; Gnanamuthu, C. P.

doi:10.1177/014107680109400614

Cited by 14 publications

(9 citation statements)

References 9 publications

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“…The median age at symptom onset was 40 years (range, 5–70 years), and 5 patients had symptom onset before age 18 years . Clardy et al identified only 8 patients with pediatric‐onset SPS evaluated at the Mayo Clinic from 1984 to 2012, representing 5% of the total SPS cases during that epoch, and an additional 12 individual cases of pediatric SPS reported in the literature were summarized . The median age of onset of the Mayo Clinic pediatric patients was 11 years, half were female, and half had type 1 DM.…”

Section: Epidemiologymentioning

confidence: 99%

“…16 Clardy et al identified only 8 patients with pediatric-onset SPS evaluated at the Mayo Clinic from 1984 to 2012, representing 5% of the total SPS cases during that epoch, and an additional 12 individual cases of pediatric SPS reported in the literature were summarized. [35][36][37][38][39][40][41][42][43][44][45][46][47] The median age of onset of the Mayo Clinic pediatric patients was 11 years, half were female, and half had type 1 DM. In 1 series of GAD65 neurological patients with a broad spectrum of CNS disorders, 23% of affected patients were African-American, considerably higher than the 10% of all registered patients at that institution.…”

Section: Epidemiologymentioning

confidence: 99%

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GAD65 neurological autoimmunity

2017

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The glutamic acid decarboxylase 65-kilodalton isoform (GAD65) antibody is a biomarker of autoimmune central nervous system (CNS) disorders and, more commonly, nonneurological autoimmune diseases. Type 1 diabetes, autoimmune thyroid disease, and pernicious anemia are the most frequent GAD65 autoimmune associations. One or more of these disorders coexists in approximately 70% of patients with GAD65 neurological autoimmunity. Neurological phenotypes have CNS localization and include limbic encephalitis, epilepsy, cerebellar ataxia, and stiff-person syndrome (SPS), among others. Classic SPS is a disorder on the spectrum of CNS hyperexcitability which also includes phenotypes that are either more restricted (stiff-limb syndrome) or more widespread (progressive encephalomyelitis with rigidity and myoclonus). GAD65 antibody is not highly predictive of a paraneoplastic cause for neurological disorders, but diverse cancer types have been occasionally reported. For all phenotypes, responses to immunotherapy are variable (approximately 50% improve). GAD65 autoimmunity is important to recognize for both coexisting nonneurological autoimmune associations and potential immunotherapy-response. Muscle Nerve 56: 15-27, 2017.

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Section: Epidemiologymentioning

confidence: 99%

Section: Epidemiologymentioning

confidence: 99%

GAD65 neurological autoimmunity

2017

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“…No prospective clinical study has been carried out to outline specific modalities for use in pediatric patients. 9 It is a rare but treatable disorder with few case reports in children. Treatment of worsening stiff-person syndrome with intravenous immunoglobulin substantially diminished the neurologic symptoms of our patient.…”

Section: Discussionmentioning

confidence: 99%

Stiff-Person Syndrome and Graves’ Disease

Medeiros

Morais

Primo

et al. 2016

Child Neurology Open

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A 9-year-old female child presented with a history of falls, weight loss, diffuse leg pain, and progressive gait disorder, following 1 previous event described as a tonic–clonic seizure. She had increased thyroid volume, brisk symmetric reflexes, abnormal gait, and painful spasms of the paraspinal musculature. Thyroid function tests indicated biochemical hyperthyroidism, and thyrotropin receptor antibodies were positive. Her electromyography showed continuous activation of normal motor units of the paraspinal and proximal lower extremity muscles. The patient had a diagnosis of Graves’ disease with associated stiff-person syndrome, with elevated anti–glutamic acid decarboxylase antibody levels. After intravenous immunoglobulin therapy, her ambulation was substantially improved and the symptoms of stiff-person syndrome decreased dramatically.

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“…Lately, there has been a strong correlation with striatal lesions and childhood SPS in contrast to spinal and brain lesions in adult SPS [ 37 ]. Most children with SPS also have negative anti-GAD and exhibit acute onset with a transient benign course [ 38 ]. They may also be associated with psychiatric disorders but frequently go unnoticed.…”

Section: Reviewmentioning

confidence: 99%

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

Bhatti

Gazali²

2015

Cureus

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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.

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Stiff-Man Syndrome in Childhood

Cited by 14 publications

References 9 publications

GAD65 neurological autoimmunity

GAD65 neurological autoimmunity

Stiff-Person Syndrome and Graves’ Disease

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

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