Stiff-person syndrome: insights into a complex autoimmune disorder

  title={Stiff-person syndrome: insights into a complex autoimmune disorder},
  author={Jos{\'e} Fidel Baizabal-Carvallo and Joseph Jankovic},
  journal={Journal of Neurology, Neurosurgery \& Psychiatry},
  pages={840 - 848}
Stiff-person syndrome (SPS) is characterised by progressive rigidity and muscle spasms affecting the axial and limb muscles. Since its initial description in 1956, marked progress has been made in the clinical characterisation, understanding of pathogenesis and therapy of this disorder. SPS can be classified according to the clinical presentation into classic SPS and SPS variants: focal or segmental-SPS, jerking-SPS and progressive encephalomyelitis with rigidity and myoclonus. Most patients… 

Stiff Person Syndrome: A Rare Neurological Disorder, Heterogeneous in Clinical Presentation and Not Easy to Treat

Background. Stiff person syndrome (SPS) is a rare neurological disorder characterized by progressive rigidity of axial and limb muscles associated with painful spasms. SPS can be classified into

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

Stiff-person syndrome: an atypical presentation and a review of the literature

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.

Association of stiff-person syndrome with autoimmune endocrine diseases

While typical clinical symptoms are crucial for the diagnosis of SPS, the presence of anti-GAD autoantibody may consolidate the diagnosis and predict the association with other autoimmune diseases.

Stiff Person Syndrome: A Case Report with Sudden Onset and Coexistence of Sero-Positive Antibodies to Glutamic Acid Decarboxylase and Anti-SOX1 Antibodies

A case of a 25-year-old Vietnamese female patient with SPS presenting with unusual clinical manifestations of sudden onset, rapidly progressive spinal, abdominal, and lower limb rigidity accompanied by painful spasms, autonomic disorders, and severe, multiple bone fractures is reported.

Stiff-Person Syndrome: Seeing Past Comorbidities to Reach the Correct Diagnosis

The case of a 65-year-old African-American female with a past medical history of hypothyroidism, anxiety, and depression with psychotic features who presented with axial muscle rigidity and lactic acidosis is reported, solidifying the diagnosis of SPS, a rare autoimmune and/or paraneoplastic syndrome.

Stiff-person syndrome coexisting with critical illness polyneuropathy

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.



Stiff‐person syndrome: An autoimmune disease

  • Philip BlumJ. Jankovic
  • Medicine, Biology
    Movement disorders : official journal of the Movement Disorder Society
  • 1991
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.

Stiff person syndrome: Advances in pathogenesis and therapeutic interventions

  • M. Dalakas
  • Biology, Medicine
    Current treatment options in neurology
  • 2009
Increased awareness of SPS among practicing physicians is necessary to recognize the disease early and prevent permanent disability, and new immunomodulating agents are being explored to treat difficult cases and to induce long-lasting remissions.

Glycine receptor antibodies in stiff-person syndrome and other GAD-positive CNS disorders

The aims were to search for GlyR antibodies in a large number of patients with well-characterized SPS and other CNS autoimmune controls and other GAD-positive disorders; and to correlate anti-GlyR titers with clinical symptomatology using quantitative scales of stiffness and spasms.

Stiff-man syndrome with vacuolar degeneration of anterior horn motor neurons

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.

Childhood Stiff-Person Syndrome Improved with Rituximab

Rituximab is an important treatment strategy in SPS and the persistence of anti-GAD antibody positivity even with clinical remission remains to be elucidated.

Glycine receptor autoimmune spectrum with stiff-man syndrome phenotype.

Glycine receptor α1-IgG aids identification of autoimmune brainstem/spinal cord hyperexcitability disorders and may extend to the glycinergic visual system.

Psychiatric consultations in stiff-man syndrome.

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.