Case Report
Delirious Mania Associated with Autoimmune
Gastrothyroidal Syndrome of a Mid-Life Female: The Role of
Hashimoto Encephalopathy and a 3-Year Follow-Up
including Serum Autoantibody Levels
Udo Bonnet,
1
Claudia Selle,
1
and Ralf Kuhlmann
2
1
Department of Psychiatry, Psychotherapy, and Psychosomatic Medicine, Evangelisches Krankenhaus Castrop-Rauxel,
Academic Teaching Hospital of the University of Duisburg-Essen, Essen, Germany
2
Department of Neurology, Evangelisches Krankenhaus Castrop-Rauxel, Academic Teaching Hospital of
the University of Duisburg-Essen, Essen, Germany
Correspondence should be addressed to Udo Bonnet; udo.bonnet@uni-due.de
Received 11 June 2016; Revised 5 August 2016; Accepted 9 August 2016
Academic Editor: Toshiya Inada
Copyright © 2016 Udo Bonnet et al. Tis is an open access article distributed under the Creative Commons Attribution License,
which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
We report the case study of a 57-year-old Caucasian female with steroid-responsive encephalopathy associated with autoimmune
thyroiditis (SREAT), commonly termed Hashimoto encephalopathy (HE). Tis presentation includes one of the longest lasting
follow-up studies of HE considering the neuropsychiatric symptoms (here delirium, mania, and EEG-slowing) and their relation
to serum autoantibody levels. Antithyroid-peroxidase autoantibodies, the hallmark of autoimmune thyroiditis, were found in the
serum and also in the cerebrospinal fuid. Diagnostic analyses found no evidence of limbic encephalopathies characterized by serum
antibodies against intracellular, synaptic, or further cell surface antigenic targets, neoplasm, and connective tissue or vasculitis
diseases. A potential contribution of bipolar disorder and metabolic encephalopathies due to severe hypothyroidism, glucocorticoid
treatment, accelerated thyroid hormone replacement therapy, or vitamin B defciency is critically discussed. Another special feature
of this case report is the linkage of HE to an autoimmune polyendocrine syndrome (type 3B) afecting the gastroduodenum in
addition to the thyroid gland.
1. Introduction
Hashimoto encephalopathy (HE) is a rare pathological con-
dition with an estimated prevalence of 2/100,000 and vari-
ous neuropsychiatric symptoms (NPS) combined with posi-
tive serum antithyroid-peroxidase autoantibodies (TPO-Abs,
usually >200 U/mL) [1, 2]. Tese antibodies are a hallmark
of Hashimoto thyroiditis that occurs in 1–5% of the general
population [1, 2]. Most patients with HE are euthyroid or
have subclinical hypothyroidism, and 20% of them have overt
hypothyroidism while hyperthyroidism is rare [1, 2]. HE’s
clinical onset is usually subacute and its clinical presentation
is multifaceted including neuropsychiatric symptoms (NPS),
such as cognitive decline, behavioral symptoms, depression,
psychosis, cerebral ischemia, seizure, myoclonus, tremors,
and fuctuating levels of consciousness [1, 2]. Abnormalities
in neuroimaging, electroencephalogram (EEG), and cere-
brospinal fuid (CSF) are not required to diagnose HE
although being present in more than 50% of the cases (usually
elevated CSF protein, generalized slowing of EEG-waves,
and T2-hyperintense foci on brain MR imaging) [1, 2].
HE is characterized by a remission of NPS, followed by
normalization of neuroimaging and EEG afer corticosteroid
(glucocorticoid) therapy (steroid-responsiveness) or, if resis-
tant, escalating immunosuppression [1, 2]. However, cases
with partial remission or progression even up to death were
described [1]. Data regarding the course of HE are scarce
and rely on clinical follow-ups which usually lasted from 6
to 24 months. A review of the 82 cases reported in literature
revealed that, in this time span, most HE patients (>50%)
had not relapsed while 30% had relapsed afer glucocorticoid
discontinuation, 5% of whom had died [1]. Te etiology of
Hindawi Publishing Corporation
Case Reports in Psychiatry
Volume 2016, Article ID 4168050, 7 pages
http://dx.doi.org/10.1155/2016/4168050