Brain imaging in cerebellar ataxia associated with autoimmune polyglandular syndrome type 2

J Neuroimaging. 2012 Jul;22(3):308-11. doi: 10.1111/j.1552-6569.2010.00542.x. Epub 2010 Nov 17.

Abstract

Autoimmune polyglandular syndrome (APS) type 2 (Schmidt syndrome) is a disorder characterized by a combination of autoimmune adrenal insufficiency, autoimmune thyroid disease, and type 1 autoimmune diabetes mellitus. We describe the first case of subacute cerebellar syndrome associated with APS type 2. Brain magnetic resonance imaging showed atrophy of the cerebellum and the vermis, as well as of the anterior pituitary gland. Magnetic resonance spectroscopy showed decreased N-acetylaspartate/creatine ratio in the cerebellum and in the pons. Our findings expand the spectrum of neurological deficits in APS type 2 and underlines that cerebellar pathways may be a main target of the disorder.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Brain / pathology*
  • Cerebellar Ataxia / etiology*
  • Cerebellar Ataxia / pathology*
  • Humans
  • Magnetic Resonance Imaging*
  • Male
  • Polyendocrinopathies, Autoimmune / complications*
  • Polyendocrinopathies, Autoimmune / pathology*