Stiff person syndrome with diffuse white matter lesions – case presentation

Written by Szatmári Szabolcs, Para János Szabolcs, Fazakas Noémi, Hojda Ancuţa Laura, Szász József Attila

We present the case of a 57 years old male patient with generalised muscle rigidity, hyperlordosis, robot like movements and gait. Only an autoimmune cause could be suspected as any demonstration of a malignant process failed during ten years of evolution. A high antiGAD titre was found, the EMG examination revealed a continuous activity of the motor unit during the relaxation phase inclusively. The brain MRI showed T2 and FLAIR subcortical hyperintensity in the hemispheres. Corticotherapy and increasing doses of diazepam were efficient but the patient developed diabetes mellitus, so we stopped the treatment with medrol and peroral antidiabetic treatment was initiated. We discuss some aspects related to this rare syndrome considered nowadays as a heterogeneous group of autoimmune conditions and movement disorders.


Keywords: stiff person, MRI hyperintensity.

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