Best Practice & Research Clinical Endocrinology & Metabolism
4Hashimoto's encephalopathy: myth or reality? An endocrinologist's perspective
Section snippets
Neurologic features
Most reported cases occurred in girls and women (72%). The female-to-male ratio is almost the same as that for Hashimoto's thyroiditis.4 Hashimoto's encephalopathy follows the age and sex distributions of autoimmune thyroid disease5 and occurs in adults5 as well as in children.18, 19, 20, 21
Clinical manifestations are those of fluctuating encephalopathy. Cognitive impairment22, 23, 24, 25, 26 and behavioral changes2, 27 are common. A focal or generalized seizure disorder is also a
Response to immune modulation
This issue is confounded by the fact that, by definition, the condition of almost all the reported patients improved with high-dose corticosteroid therapy, and patients who were non-responsive have been excluded in most case reports and reports of larger series5 (Castillo PR, Woodruff BK, Boeve BF, et al, unpublished data, 2004). Both high doses of oral prednisone and shorter courses of intravenous methylprednisolone have been used.65 Other forms of corticosteroid-sparing immunomodulation—such
Differential diagnosis
Before the diagnosis of Hashimoto's encephalopathy can be made, all other causes of encephalopathy must be excluded. CSF examination, including bacteriologic and virologic evaluations, is necessary. Metabolic and toxic causes of encephalopathy should also be excluded. In patients with overt hypothyroidism, psychological and behavioral abnormalities and neurologic findings may be present that respond to normalization of thyroid function.
Hashimoto's encephalopathy and Creutzfeldt–Jakob disease
Hashimoto's encephalopathy: A Real Clinical Entity but the Wrong Terminology?
There is no question that a syndrome of autoimmune corticosteroid-responsive encephalopathy associated with positive antithyroid antibodies or mild thyroid failure exists. Reported cases of antibrain antibodies71 and pathologic findings identified in brain biopsy and autopsy specimens also suggest an autoimmune source. The presence of antithyroid antibodies in CSF has raised the possibility of intrathecal synthesis of these antibodies.73, 74 However, no evidence, so far, shows that thyroid
The role of the neurologist
Identification of steroid-responsive encephalopathy is a neurologic diagnosis, which should be reached after exclusion of other known causes of encephalopathy.2, 4, 5 The neurologist considers and rules out systemic connective tissue and autoimmune conditions such as lupus erythematosus95, known vasculitis, and Sjögren's syndrome47 that may have CNS involvement. Thyroid function and thyroid antibodies are analyzed, along with other markers of autoimmunity, and if the results are positive, the
The role of the endocrinologist
Endocrinologists are less likely to see typical cases of autoimmune encephalopathy in their practices. Encephalopathy associated with thyroid autoimmunity occurs in patients who have either asymptomatic subclinical hypothyroidism or isolated positive antithyroid antibodies or who are euthyroid and receiving thyroxine therapy. With increasing recognition of autoimmune encephalopathy, and in particular patients with associated thyroid autoimmunity, it is expected that more cases will be
Summary
Since the original description of a patient with hypothyroidism and multiple episodes of reversible stroke-like events and seizure, neurologists have sought evidence of thyroid dysfunction and autoimmunity in patients who have had encephalopathy of uncertain etiology, after exclusion of infectious and other inflammatory encephalopathies. Then, if the patient has positive antithyroid antibodies or mild or treated thyroid failure, the patient is treated with corticosteroids. If the response is
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