Hashimoto’s encephalopathy: steroid resistance and response to intravenous immunoglobulins

• Published in: Journal of neurology, neurosurgery and psychiatry Vol. 76; no. 3; pp. 455 - 456
• Main Authors: Jacob, S, Rajabally, Y A
• Format: Journal Article
• Language: English
• Published: England BMJ Publishing Group Ltd 01.03.2005; BMJ Publishing Group LTD; BMJ Group
• Subjects: Adult; Antibiotics; Brain Diseases - drug therapy; Drug Resistance; Female; Hashimoto’s encephalopathy; Humans; Immunoglobulins; Immunoglobulins, Intravenous - therapeutic use; intravenous immunoglobulins; Letters; Medical imaging; Neurology; Patients; steroid resistance; Steroids; Steroids - therapeutic use; Thyroid gland; Thyroiditis, Autoimmune - drug therapy; Treatment Outcome
• ISSN: 0022-3050; 1468-330X
• DOI: 10.1136/jnnp.2004.049395

The initial report of HE suggested a vascular aetiology followed by localised cerebral oedema as a possible mechanism. 1 Some authors suggest that the CSF thyroid autoantibodies may react with a putative CNS antigen and form immune complexes. 2, 4 The immunopathological basis of this syndrome has been compared to a relapsing form of acute disseminated encephalomyelitis. 7 Although reversible MRI findings have been described in HE, 8 neuroimaging (except for isolated patchy uptake by isotope scans) is usually normal in most cases. 3 Cerebral angiography has been found to be normal in several cases of HE, unlike in many other cerebral vasculitides. 1- 3, 5 Thyroid autoantibodies can co-exist with several other forms of autoimmune encephalomenigitis, but the normal MRI scan, the initial dramatic response to steroids, and negative autoantibodies for most other common vasculitides, tends to favour the diagnosis of HE in our case. A recent literature review of 85 patients with encephalopathy and anti-thyroid antibodies suggests that the combination of encephalopathy, high serum anti-thyroid antibody concentrations, and responsiveness to glucocorticoid therapy seems unlikely to be due to chance. 10 The initial meningo-encephalitic type presentation of our patient in 1987 was probably the first manifestation of HE in view of clinical findings and laboratory data (Mild CSF pleocytosis is not unusual in HE. 3 ) There was a delay of 14 years before the diagnosis was first established, in spite of several hospital admissions.