The progressive encephalomyelitis with rigidity syndrome (PEMRS) is a rare neurological disorder that can be considered as the most severe form of the “stiff person” syndrome1 2although other authors suggest that it may be a distinct entity.3 Pathogenesis is far from being clearly defined, but some studies point to humoral immunity as having an important role. Antiglutamic acid decarboxylase (anti-GAD) antibodies have been found to be present in about 40% of patients with stiff person syndrome.4 Treatment is very difficult and in the only case reported so far in which intravenous immunoglobulin has been used the response was poor.2 We report a case of PEMRS with anti-GAD antibodies that had an excellent response to intravenous immunoglobulin.

A 67 year old woman was admitted to our hospital with a 9 month history of progressive gait disturbance and painful leg contractions. Family history was negative for neurological …