Stiff-Person Syndrome

@article{Murinson2004StiffPersonS,
  title={Stiff-Person Syndrome},
  author={Beth Brianna Murinson},
  journal={The Neurologist},
  year={2004},
  volume={10},
  pages={131-137}
}
Background:Stiff-person syndrome (SPS) is a rare neurologic disorder with autoimmune features. It is characterized by progressive, severe muscle rigidity or stiffness most prominently affecting the spine and lower extremities. Review Summary:Superimposed muscle spasms result in simultaneous contraction of agonist and antagonist muscles which are detectable by electromyography (EMG) and relieved by administration of benzodiazepines. The exacerbation of SPS by emotional stressors often results in… Expand
Stiff-person syndrome: an atypical presentation and a review of the literature.
TLDR
A 79-year-old woman who presented with bilateral lower extremity weakness who was ultimately diagnosed with stiff-limb syndrome, a rare variant of SPS, is presented. Expand
The Stiff-man Syndrome in Children and Adolescents
TLDR
The stiff-man syndrome (SMS) is an autoimmune disorder characterized by stiffness and spasms affecting muscles of limbs and/or axial regions, and both focal and more extensive “whole-body” phenotypes are recognized. Expand
Stiff-person syndrome coexisting with critical illness polyneuropathy
TLDR
A 60-year-old man with SPS associated with critical illness polyneuropathy and CIP was diagnosed during an episode of acute respiratory failure secondary to muscular rigidity and spasms, which has rarely been reported in this condition. Expand
Difficult to Treat Focal, Stiff Person Syndrome of the Left Upper Extremity
TLDR
The need for further understanding of this disorder is outlined in order to provide better symptomatic treatment or potentially more definitive care for Stiff Person Syndrome. Expand
[Two stiff person cases misdiagnosed as conversion disorder].
TLDR
The aim of this case report is to draw attention to the psychiatric presentations of SPS and to emphasize the importance of complete psychiatric and neurological examination, including brain imaging and electrophysiological studies, in the differential diagnosis of CD. Expand
Levetiracetam improves paroxysmal symptoms in a patient with stiff-person syndrome
TLDR
The following case illustrates the therapeutic challenges and demonstrates the successful treatment of paroxysms with the new antiepileptic drug (AED) levetiracetam (LEV). Expand
Stiff-person syndrome presenting as eating disorder: a case report.
TLDR
A 35-year-old female initially treated for anorexia nervosa who was found to have Stiff-Person Syndrome (SPS) is presented to alert practitioners to the potential co-morbidity and symptom overlap between SPS and eating disorders. Expand
Role of Osteopathic Manipulative Treatment in the Management of Stiff Person Syndrome
TLDR
By decreasing somatic dysfunction and reducing the frequency of exacerbations, osteopathic manipulative treatment may alleviate the symptoms and overall morbidity associated with this disease. Expand
Stiff-person syndrome: persistent elevation of glutamic acid decarboxylase antibodies despite successful treatment with rituximab.
  • Eugene A Bacorro, R. Tehrani
  • Medicine
  • Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases
  • 2010
TLDR
It is postulate that rituximab was associated with clinical improvement through mechanisms other than antibody depletion through mechanismsother than antibodies depletion. Expand
Stiff-person syndrome treated with rituximab
TLDR
A case report is presented of a 41-year-old female patient diagnosed with SPS who was treated with rituximab in a public hospital in Brasília, Brazil, showing a good and safe response to the treatment so far. Expand
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References

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Stiff-Person Syndrome: Autoimmunity and the Central Nervous System
TLDR
Both electromyography and the detection of GAD antibodies are useful in establishing a diagnosis of SPS, and the use of immunomodulating therapies and symptomatic treatment with benzodiazepines and baclofen is recommended. Expand
Stiff‐person syndrome: An autoimmune disease
  • P. Blum, J. Jankovic
  • Medicine
  • Movement disorders : official journal of the Movement Disorder Society
  • 1991
TLDR
Two patients with this syndrome who had GAD antibodies in both CSF and serum are described, providing additional evidence of an autoimmune etiology of SPS and of the role of immunotherapy in its treatment. Expand
Stiff-man syndrome updated.
TLDR
Analysis of 13 patients with stiff-man syndrome examined at the Mayo Clinic during the past 30 years revealed that treatment with diazepam decreased the muscle spasms, and rehabilitation is an important adjunct that may further improve function when it is centered on the treatment of low-back pain and hyperlordosis. Expand
Psychiatric consultations in stiff-man syndrome.
TLDR
It is speculated that the GABA system is involved in both the neurologic and psychiatric symptoms of these patients and Psychiatrists have a significant role in the management of patients with stiff-man syndrome and may be expected to manage anxiety, depression, and substance misuse. Expand
Improvement of stiff-person syndrome with tiagabine
TLDR
An antibody-negative patient with clinical SPS whose symptoms improved significantly in response to the anticonvulsant tiagabine, an inhibitor of GABA catabolism is presented. Expand
The Stiff-Person Syndrome: An Autoimmune Disorder Affecting Neurotransmission of -Aminobutyric Acid
TLDR
The stiff-person syndrome causes substantial disability and is therefore of interest to various specialists, including neurologists, internists, rheumatologists, diabetologists, immunologists, and rehabilitation physicians, who study its pathogenetic mechanisms and apply specific therapies. Expand
Stiff-man syndrome with vacuolar degeneration of anterior horn motor neurons
TLDR
A SMS patient with GAD autoantibodies who died 8 month after the onset of the disease, and whose autopsy showed unusual findings which may be relevant to establishing the physiopathological mechanism of this nosological entity is presented. Expand
Psychological factors in the diagnosis and pathogenesis of stiff-man syndrome
TLDR
Retrospective psychological evaluation of patients with stiff-man syndrome revealed a characteristic set of psychological symptoms or features and it is concluded that the common misdiagnosis of SMS as a psychogenic movement disorder is due to the compelling association of a set of salient psychological features, bizarre and fluctuating motor symptoms, and lack of approved neurologic signs. Expand
A critical survey of stiff-man syndrome.
TLDR
A tentative hypothesis is formulated to account for the state of tonic muscle rigidity of stiff-man syndrome, as suggested by the presence of a nonglucose reducing substance in the urine and a rise in inorganic serum phosphorus during glycogen deposition. Expand
The stiff man and stiff man plus syndromes
TLDR
Clinically, stiff persons with ‘plus’ signs may be divided into three groups according to the aggressiveness of the pathology and its relative distribution, while some have a paraneoplastic aetiology, while a non-malignant autoimmune basis seems likely in others. Expand
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