Stiff person syndrome

Stiff person syndrome, previously known as stiff man syndrome, was first described in 1956 by Moersch and Woltman of Mayo Clinic 1 and is a very rare neuromuscular disease characterised by:

  • progressive muscle stiffness of the spine and lower extremities
  • muscle spasms triggered by external stimuli or emotional stress
  • symptoms typically relieved by benzodiazepines

Onset is usually between 30 and 50 years of age, and diagnosis is made usually with EMG and anti-GAD (glutamic acid decarboxylase) antibodies only present in 60% of patients, but which are very specific.

It appears to be predominantly autoimmune in nature. The current hypothesis is that stiff-man syndrome occurs due to increase in oligoclonal bands in CSF of patients which destroyed GABAergic nerve cells 2, 3.

It is seen as one of the manifestations of a number of autoimmune encephalitides (e.g. anti-GAB antibodies).

Associations

Imaging is only really needed to exclude other unrelated pathology.

In addition to benzodiazepines, Baclofen has been used for treatment. More recently intravenous immunoglobulins (IVIG) have been used in combination with other drugs 4.

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Article information

rID: 2088
Section: Syndromes
Synonyms or Alternate Spellings:
  • Stiff person syndrome

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