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.jpg): Failed to open stream: No such file or directory in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 117 Front+Psychiatry
2017 ; 8
(ä): 212
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Plasmapheresis Responsive Rapid Onset Dementia with Predominantly Frontal
Dysfunction in the Context of Hashimoto s Encephalopathy
#MMPMID29123489
Endres D
; Vry MS
; Dykierek P
; Riering AN
; Lüngen E
; Stich O
; Dersch R
; Venhoff N
; Erny D
; Mader I
; Meyer PT
; Tebartz van Elst L
Front Psychiatry
2017[]; 8
(ä): 212
PMID29123489
show ga
BACKGROUND: Hashimoto's encephalopathy (HE) is a rare immunological
neuropsychiatric disorder characterized by increased antithyroid antibodies and
mixed neurological and psychiatric symptoms. HE has been previously discussed as
a differential diagnosis for rapid progressive dementia. However, most of these
patients suffered from additional neurological symptoms, like ataxia or seizures.
CASE PRESENTATION: Here, we present the case of a 59-year-old female patient
suffering rapid onset dementia with salient frontal executive dysfunction. She
developed rapid onset symptoms, including apathy, verbal depletion up to a
stuporous state, severe working memory deficits, evidence of primitive reflexes,
disturbed Luria's three-step test, and micturition disorder. Analysis of her
cerebrospinal fluid was normal. The serum analyses showed increased antithyroid
(antithyroid peroxidase and antithyroglobulin) antibodies. In the cerebral
magnetic resonance imaging, supratentorial deep and peripheral white matter
lesions were found; the electroencephalography showed intermittent slowing, and
the [(18)F]fluorodeoxyglucose positron emission tomography (FDG-PET) depicted
medial and superior dorsolateral frontal hypometabolism. Several different
psychopharmacological therapeutic approaches with various neuroleptics,
antidepressants, and high doses of lorazepam were unsuccessful. Due to the
organic alterations, including increased antithyroid antibodies, HE was
suspected. Against expectations, treatment with high-dose corticosteroids proved
to be ineffective and was associated with worsening symptoms. However, escalated
treatment with plasmapheresis over 5 days led to significant improvement in all
reported symptoms and in psychometric testing. The neuropsychological improvement
was stable over a 6-month follow-up period, and the FDG-PET normalized.
CONCLUSION: This case report reveals that (1) HE can mimic rapid onset dementia
with predominantly frontal dysfunction; (2) this syndrome can be successfully
treated in the context of HE; and (3) plasmapheresis can be effective in such a
disease constellation. The detection of the immunological causes of rapid onset
dementia and other psychiatric syndromes is important because it opens
opportunities for new, innovative immunosuppressive treatment options.