Hashimoto’s encephalopathy

Abstract

The clinical features of Hashimoto's encephalopathy have been attributed to a cerebral vasculitis, but pathologic material is rarely available. The authors describe an individual with Hashimoto's encephalopathy complicated by fatal status epilepticus. Postmortem examination demonstrated mild perivascular lymphocytic infiltration throughout the brain and leptomeninges plus diffuse gliosis of gray matter in the cortex, basal ganglia, thalami, hippocampi, and, to a lesser extent, the parenchymal white matter.

Intravenous Immunoglobulin Therapy for Autoantibody-Positive Cerebellar Ataxia

“…Hadjivassiliou (29,30), thrombotic thrombocytopenic purpura (31) and ChurgStrauss syndrome (32) (2,33,34). Furthermore, SPECT studies have reported abnormal cerebral perfusion in celiac disease (35) and Hashimoto's thyroiditis with normal thyroid function (36 …”

Hashimoto’s Encephalopathy: A Diagnosis in Disguise, Case Report and Review of Literature

“…Most evidence suggests an auto-immune vasculitis or other immune-mediated process affecting the cerebral microvasculature. Post-mortem studies of some individuals have shown lymphocytic vasculitis of venules and veins in the brain stem and a diffuse gliosis involving gray matter more than white matter [2][3][4].…”

Steroid-responsive Thalamic Lesions Accompanying Microbleeds in a Case of Hashimoto's Encephalopathy with Autoantibodies Against α-enolase

“…One possible pathologic mechanism of HE is the involvement of an autoimmune inflammatory process because pathological findings of brain biopsy specimens have revealed marked lymphocytic infiltration of parenchymal arterioles and venules (3). Furthermore, in one patient with HE, the autopsy findings demonstrated lymphocytic infiltration throughout the walls of arterioles and venules in the brain stem, white matter, cortex and leptomeninges (4).…”

“…Although autoimmune mechanisms may underlie the pathogenesis, the precise cause of HE remains unclear. Autoimmune cerebral vasculitis is likely because neuropathological examinations of biopsy and autopsy specimens have shown lymphocytic infiltration into the walls of arterioles and venules in brain parenchyma (3,4) and leptomeninges (4). Recently, Yoneda and colleagues reported that serum autoantibodies against the N-terminal region of α-enolase (NAE) are a useful diagnostic marker for HE (5).…”