Disease Information for Hashimotos Encephalitis

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Clinical Manifestations
Signs & Symptoms
Altered mental status Mental status change
Delirium
Delirium/Agitated delirium
Drowsiness/somnolence
Headache
Irritability/short tempered
Rapidly progressive dementia
Seizures
Seizures/Children/recurrent
Chronic Fatigue
Constitutional symptoms
Fatigue
Fatigue Tiredness Exhaustion
Hypersomnia/Increased need for sleep
Infant peevish/irritable/fretful
Laboratory Tests
Abnormal Lab Findings - Increased
TSI (Thyroid stimulating immunoglobulin Assay (labs)
Associated Diseases & Rule outs
Associated Disease & Complications
Autoimmune disease
Coma/Unconscious
Encephalitis
Treatment
Drug Therapy - Indication
SX/Brain biopsy
Definition

cerebral angiitis causes rapid dementia like j-K disease; responds to steroid; see also Hashimotos encephalitis with anti-microsomal antibodies; Hashimoto"s Encephalopathy;

Hashimoto"s encephalopathy (HE) is a rare, but probably underdiagnosed, treatable autoimmune disorder associated with chronic lymphocytic Hashimoto"s thyroiditis; HE often presents with features that may overlap other rapidly progressive dementias, particularly CJD; Symptoms of HE

include dementia, altered levels of consciousness or confusion, hallucinations/psychosis, personality changes resembling depression, pyramidal and extrapyramidal signs, myoclonus, ataxia, stroke-like episodes,and seizures; Unlike CJD, HE tends to have a fluctuating course and is more frequently associated with seizures; These fluctuations can clinically help distinguish HE from CJD, as the latter uniformly has a progressive downward

course; HE occurs much more commonly in women (85%) than in men; Patients with HE may be euthyroid, subclinically hypothyroid, hypothyroid, and even hyperthyroid; The disorder is suggested based on the presence of elevated

antithyroid autoantibody [antithyroglobulin and/or antithyroid peroxidase (antimicrosomal or anti-TPO)]; EEG characteristically shows nonspecific asynchronous background slowing and intermittent diffuse or focal slow

activity; however, as in CJD, triphasic waves or periodic sharp waves may occur; MRI may show nonspecific abnormalities--most commonly increased subcortical, mesial-temporal, or white-matter signal on T2- or FLAIR-weighted images; CSF often shows a nonspecific increase in CSF

protein; The etiology of this disease is not clear but may involve the presence of a shared antigen in the brain and thyroid, autoimmune vasculitis, and/or reversible leukoencephalopathy; Treatment of HE is immunosuppressive, typically with high-dose glucocorticoids; The prognosis

for outcome of patients with HE who are treated is very good; More than 90% of patients respond favorably to treatment, although some patients have residual symptoms or a fluctuating disease course

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External Links Related to Hashimotos Encephalitis
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PubMed (National Library of Medicine)
NGC (National Guideline Clearinghouse)
Medscape (eMedicine)
Harrison's Online (accessmedicine)
NEJM (The New England Journal of Medicine)
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