Clinical characteristics of paediatric Hashimoto's encephalopathy

https://doi.org/10.1016/j.ejpn.2021.04.006Get rights and content

Highlights

  • •

    Epilepsy is the most frequent symptom of Hashimoto's encephalopathy.

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    There is a wide variety of symptoms in Hashimoto's encephalopathy.

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    Psychological and psychiatric symptoms occur often in Hashimoto's encephalopathy.

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    One in ten children with Hashimoto's encephalopathy experience late sequelae.

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    Determine aTPO in children with unexplained neurological or psychiatric dysfunction.

Abstract

Background

Hashimoto's encephalopathy, also known as steroid responsive encephalopathy, is associated with thyroid antibodies (SREAT) and is a rare but serious form of encephalopathy. In this paper, we describe the signs, symptoms, outcome, and treatments as noted in the case reports reviewed.

Methods

We searched PubMed, Embase, and the Cochrane library for articles in which cases of Hashimoto's encephalopathy were described. The case description had to include the diagnosis, age, sex, presenting symptoms, and diagnostic tests.

Results

We retrieved 360 articles and 65 fulfilled the inclusion criteria. These articles gave reports of 100 cases, with a mean age of 10.9 (range 2.8–19), 78 of whom were female. Epilepsy (79) – including epileptic state [24] – behavioural problems [36], hallucinations [21], headache [21], and decline in school performance [19] were most often reported. Antithyroid peroxidase (aTPO) was reported elevated in all patients. Most children [70] recovered fully, however 16 had late sequelae, mostly epilepsy. Therapies used include steroids, intravenous gammaglobulines, and cytostatics.

Conclusions

Epilepsy, behavioural problems, decline in school performance, and hallucinations are frequent symptoms of Hashimoto's encephalopathy. Steroids are the basis of treatment, although other immunomodulatory drugs seem to be successful. About one in ten children will experience late sequelae. In any child with unexplained neurological, psychiatric, or psychological dysfunction, serum anti-thyroidperoxidase (aTPO) should be determined.

Introduction

Hashimoto's encephalopathy, also known as steroid responsive encephalopathy associated with thyroid antibodies (SREAT) is rare in children and adolescents [1,3]. The estimated prevalence in adults is 2.1/100.000 [2], the prevalence in children is unknown. The condition has a wide variety of severe neurologic symptoms and its pathogenesis is still unknown. In all patients an increased level of serum anti-thyroidperoxidase (aTPO) [4], independent of thyroid status, is found. Despite an elevated level of aTPO in all patients, these antibodies do not play any role in the pathogenesis. In most patients, treatment is effective and leads to disappearance of symptoms [5]. Therefore, early recognition is important and possible. In 2008, we reviewed the presenting signs and symptoms in 25 cases reported [6].

We wanted to determine whether the reported spectrum of signs and symptoms has broadened since 2007, which signs and symptoms have been described most frequently, and whether new presenting features have been described. Therefore, we studied clinical reports of paediatric patients with Hashimoto's encephalopathy.

Section snippets

Methods

We searched the literature for as many paediatric cases of Hashimoto's encephalopathy as possible from 2008/01/01 to 2019/10/01.

To find all paediatric cases of Hashimoto's encephalopathy, we searched for cases and clinical reports in PubMed, Cochrane Library and Embase using the following terms:

(“Hashimoto Disease”[Mesh] OR “Hashimoto's encephalitis” [Supplementary Concept] OR Hashimoto encephalopathy [tiab] OR Hashimoto's encephalopathy [tiab] OR Hashimoto's encephalitis [tiab] OR Hashimoto

Results

We found 360 articles and could include 65 [[7], [8], [9], [10], [11], [12], [13], [14], [15], [16], [17], [18], [19], [20], [21], [22], [23], [24], [25], [26], [27], [28], [29], [30], [31], [32], [33], [34], [35], [36], [37], [38], [39], [40], [41], [42], [43], [44], [45], [46], [47], [48], [49], [50], [51], [52], [53], [54], [55], [56], [57], [58], [59], [60],[61], [62], [63], [64], [65], [66], [67], [68], [69], [70], [71], [72]]. These articles reported on 100 children, their median age was

Discussion

In this study, we expand the clinical understanding of signs and symptoms in children with Hashimoto's encephalopathy. One hundred new cases have been reported since our earlier publication. The most frequent neurological features of Hashimoto's encephalopathy were convulsions, epileptic state, hemiparesis, and coma. Diplopia, dysarthria, and unspecified encephalopathy were reported as other relatively frequent neurological symptoms. The most reported psychiatric symptoms were hallucinations

Conclusions

In conclusion, when the signs and symptoms of all 125 patients of this and our earlier study are aggregated, we suggest including the diagnosis of Hashimoto's encephalopathy in children with an epileptic state, refractory epilepsy, coma, and with rare neurological or psychiatric signs and symptoms. The disease can be found even in young children. We advise including determination of serum aTPO in children with these symptoms, irrespective of thyroid disease. A strongly elevated level of aTPO,

Financial disclosure

The authors have no financial relationships relevant to this article to disclose.

Funding source

This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.

Declaration of competing interest

The authors have no conflicts of interest relevant to this article to disclose.

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