Stiff Leg Syndrome and Myelitis with Anti-Amphiphysin Antibodies: A Common Physiopathology?

  title={Stiff Leg Syndrome and Myelitis with Anti-Amphiphysin Antibodies: A Common Physiopathology?},
  author={Ludivine Chamard and Eloi Magnin and Eric Berger and Beate Hagenk{\"o}tter and Lucien Rumbach and Marc Bataillard},
  journal={European Neurology},
  pages={253 - 255}
culty in walking. [] Key Method Her medical history included type 2 diabetes treated with metformin and a thyroid goiter treated with surgery and levothyroxine. On clinical examination, she presented permanent hypertonia in the lower limb and bilateral dystonia of the extensor hallucis longus muscles. Intermittent spasms were associated with hypertonia. The spasms lasted several seconds and were spontaneous, painful, and made worse by anxiety and cutaneous stimulation. The tibialis anterior muscles were…

Figures from this paper

Effectiveness of Combined Immunoglobulin and Glucocorticoid Treatments in a Patient With Stiff Limb Syndrome: Case Report and Review of the Literature

This report reported on a 55-year-old female who showed typical painful spasms in both lower limbs and abduction of the right eyeball that partially responded to low-dose diazepam and had high-titer anti–glutamic acid decarboxylase (anti-GAD) antibody.

Stiff-Person Syndrome Spectrum Disorders

Stiff-person syndrome was first described in 1956; its further characterization as an autoimmune neurological disorder occurred more than 30 years later with the discovery of glutamic acid

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

Grouping SPS-spectrum cases by pathophysiology provided insights into work-up, treatment, and prognosis, and ruled out malignancy and autoimmunity is appropriate for suspected SPCS cases.

Transverse Myelitis

Advances in Autoimmune Epilepsy Associated with Antibodies, Their Potential Pathogenic Molecular Mechanisms, and Current Recommended Immunotherapies

It is concluded that early diagnosis of autoimmune epilepsy is of great significance, as early immune treatments have useful disease-modifying effects on some epilepsies and can facilitate the recovery.

Amphiphysin-positive paraneoplastic myelitis and stiff-person syndrome

Finding the underlying etiology of transverse myelitis (TM) can be challenging, as several disorders, including multiple sclerosis (MS), neuromyelitis optica, acute disseminated encephalomyelitis,

Non-stiff anti-amphiphysin syndrome: Clinical manifestations and outcome after immunotherapy



[Successful treatment in a patient with a focal form of stiff-person syndrome using plasma exchange and intravenous immunoglobulin therapy].

Stiff-leg syndrome in this case was unresponsive to pharmacotherapy with diazepam and was unchanged for the first month of hospitalization, and Plasma exchange therapy alleviated the clinical symptoms and decreased the anti-GAD antibody titer.

Paraneoplastic stiff limb syndrome

  • I. Silverman
  • Medicine, Biology
    Journal of neurology, neurosurgery, and psychiatry
  • 1999
It is proposed that the nomenclature “stiff limb syndrome” refers to the focal form of SMS when one or more distal limbs are involved; two of their patients were also anti-GAD antibody positive, but none were tested for antibodies.

[A stiff-legged man with a bizarre gait].

  • T. HolmøyM. HornB. Vandvik
  • Medicine, Psychology
    Tidsskrift for den Norske laegeforening : tidsskrift for praktisk medicin, ny raekke
  • 2007
Elevated levels of antibodies against GAD65 in serum and cerebrospinal fluid confirmed the diagnosis SPS three and a half years after symptom debut, and both stiffness and muscle cramps responded excellently to diazepam.

Stiff‐Person Syndrome: A Rare Presentation for Breast Cancer

The patient’s symptoms significantly improved within 4 to 6 months after surgical resection and adjuvant treatment of the cancerous tissues, corroborating the paraneoplastic nature of her clinical syndrome.

Amphiphysin autoimmunity: Paraneoplastic accompaniments

Only 10% of women (some with lung carcinoma) and 4% of men fulfilled diagnostic criteria for stiff‐man syndrome, and patients with isolated amphiphysin‐IgG were more likely to be women and to have myelopathy or stiff‐ man phenomena.


Although the pathophysiology of non-paraneoplastic SPS is incompletely understood, effective treatments include immunomodulating therapies and drugs that enhance GABAergic neurotransmission.

The stiff limb syndrome – a new case and a literature review

The case of a 69 year old woman with clinical, electrophysiological and laboratory findings consistent with a stiff limb syndrome of the right leg, which includes intermittent and painful spasms to sudden auditory or tactile stimuli as well as to emotional upset is reported.

Inflammatory transverse myelitis: evolving concepts

The recent identification of novel biomarkers associated with acute transverse myelitis has led to a better understanding of the spectrum of disorders associated with inflammatory transverseMyelitis, as well as a greater appreciation of its diverse and complex pathogenetic basis.

Stiff limb syndrome: a case report

This clinical case highlights the importance of a therapeutic test to confirm the diagnosis of stiff limb syndrome especially when there is a high clinical suspicion with unremarkable electromyography.