A novel assessment and treatment approach to patients with Hashimoto’s encephalopathy

Abstract

SummaryHashimoto’s encephalopathy (HE) is rarely reported with only a few hundred cases published. Diagnosis is made in patients with an appropriate clinical picture and high antithyroperoxidase (anti-TPO) antibodies after infectious, toxic and metabolic causes of encephalopathy have been excluded. There is little objective data on the neurocognitive impairment in patients with HE and their improvement with treatment. We present the case of a 28-year-old woman with HE. Approach to management was novel as objective neuropsychological assessment was used to assess her clinical condition and response to treatment. Intravenous immunoglobulin (IVIg) as the first-line treatment instead of steroids. She responded well. The case illustrates that a different approach is required for the diagnosis and treatment of HE. A new diagnostic criteria is proposed that includes neurocognitive assessment, serum and CSF antibodies, an abnormal EEG and exclusion of other causes of encephalopathy. Furthermore, treatment should be tailored to the patient.Learning points:Neurocognitive assessment should be carried out to assess the extent of brain involvement in suspected Hashimoto’s encephalopathy pre- and post- treatment.Treatment of Hashimoto’s encephalopathy should be tailored to the patient.Unifying diagnostic criteria for Hashimoto’s encephalopathy must be established.

Encefalopatía de Hashimoto resistente a corticoides: reporte de caso y revisión bibliográfica

“…Al revisar la bibliografía, hallamos un estudio de Laycock et al 6 en el cual se utilizó la IgIV como tratamiento de primera línea. En dicho caso, la paciente presentaba un índice de masa corporal (IMC) de 35,7 kg/m², por lo que el tratamiento con glucocorticoides podía conllevar efectos adversos tanto metabólicos como endocrinológicos.…”

A case report on Hashimoto’s encephalopathy: an autoimmune neuroendocrine disorder

“…Our patient was hypothyroid. 2,6,15 The pathogenesis of Hashimoto's encephalopathy is still controversial. 9 Autoimmune vasculitis, autoantibodies against brain-thyroid represent the most probable etiologic pathway.…”

“…9 Autoimmune vasculitis, autoantibodies against brain-thyroid represent the most probable etiologic pathway. 6,9 Recently, Blanchin et al reported that TPOAb from HE patients could bind cerebellar astrocytes in HE patients but not in Hashimoto thyroiditis patients. 11,12 This may support the role of TPOAb in the pathogenesis of HE.…”

“…5 The clinical presentation is highly variable; common clinical features include altered consciousness, tremor, seizures, sleep abnormalities, delusions, hallucinations, stroke-like episodes, and impairment of cognitive function, focal neurological deficits, presenile dementia, psychosis, and ataxia. 6 The specific mechanism of HE is not fully understood, it is believed that brain vasculitis and autoimmune antithyroglobulin or anti-TPO antibodies directed against common thyroid represents the most likely etiologic pathway. 7 Recently International autoimmune encephalitis Society developed clear criteria for, the diagnosis of HE, which are as follows: encephalopathy with seizures, stroke-like episodes, hallucinations, or myoclonus; subclinical or mild overt thyroid disease without severe thyroid dysfunction; normal neuroimaging data or with non-specific abnormalities; presence of serum thyroid peroxidase antibodies, thyroglobulin antibodies; absence of neuronal antibodies in serum and cerebrospinal fluid (CSF); reasonable exclusion of alternative causes.…”

Hashimoto’s encephalopathy: a rare cause of delirium

“…The response to steroids was observed in 98% of patients in a case review and is considered as diagnostic criteria 1. In rare cases, intravenous immunoglobulins or plasma exchange may be required 2–4. Additional epidemiological feature of the condition includes a propensity for it to occur in women with a female to male ratio of 4:1 5.…”