Steroid-responsive Enzephalopathie bei Autoimmunthyreoiditis als Differentialdiagnose der Creutzfeldt-Jakob-Krankheit

Steroid-responsive Enzephalopathie bei Autoimmunthyreoiditis als Differentialdiagnose der Creutzfeldt-Jakob-Krankheit

Steroid-responsive encephalopathy in autoimmune thyroiditis as a differential diagnosis of Creutzfeldt-Jakob disease

Seyma Osmanlioglu

Doctoral thesis

Date of Examination: 2016-03-23

Date of issue: 2016-03-08

Advisor: Zerr, Inga Prof. Dr.

Referee: Siggelkow, Heide Prof. Dr.

Referee: Schön, Margarete Prof. Dr.

Persistent Address: http://hdl.handle.net/11858/00-1735-0000-0028-86F1-D

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Abstract

English

The SREAT is a rare, progressive multiform disease that is not well understood. It is characterized by wide unspecific neuropsychiatric symptoms, elevated thyroid antibodies, and the good response to immunosuppressive terapy. The pathophysiology is still unclear. The aim of this study is to differentiate SREAT from sporadic Creutzfeldt-Jakob disease considering the clinical features, laboratory, imaging, and the course of both diseases. Patients, who were reported to National Reference Centre for the Surveillance of CJD in Göttingen between August 1994 and October 2008, formed the basis for the present dissertation (49 patients with SREAT in the case group and 48 patients with a certain / probable sCJD in the control group). Findings of this dissertation can be summarized as follows. The SREAT patients are younger than the sCJD. The anti-thyroid peroxidase antibodies (anti-TPO) were the most common antibodies in the SREAT, but also in the sCJD. In contrast, thyroglobulin antibodies (TgAb) were significantly more common in the SREAT patients. The exclusive occurrence of anti-TPO points for sCJD and anti-TPO + TgAb combination for SREAT. The epileptic seizure is the symptom that differs SREAT from sCJD. In contrast, the symptoms such as dementia, ataxia, visual symptoms, extrapyramidal disorder, myoclonus and akinetic mutism are still important clues to the sCJD, even if the thyroid antibodies have been detected. The examination of CSF showed an average low pleocytosis in SREAT, whereas this was normal in sCJD. The pathological increase of the protein concentration in CSF is significantly more often in the SREAT. The 14-3-3 protein in cerebrospinal fluid is a good marker to distinguish between the two diseases, since this protein is observed significantly more in sCJD. The basal ganglia hyperintensity in cMRI and signal increase in the frontal, parietal and temporal cortex were observed in the sCJD cases significantly more. The MRI white matter changes in the SREAT cases were areal as opposed to the sCJD cases, which showed more scattered changes. Diffusion weighted imaging (DWI) was better than fluid-attenuated inversion recovery (FLAIR)-weighted images in both groups for representation of cortical hyperintensities. In contrast, the hyperintensities in the white matter in the sense of leukoencephalopathy by FLAIR and T2-weighted images were better representable than using DWI-weighting in both groups. The lack of 14-3-3 protein or the pathologically elevated total protein in CSF are clues for SREAT. The inflammatory changes of cerebrospinal fluid and the areal MRI white matter changes in the SREAT cases could support a cerebral vasculitis as the cause of SREAT.

Keywords: steroid-responsive encephalopathy (SREAT); hashimoto encephalopathy (HE); sporadic Creutzfeldt-Jakob disease (sCJD); autoantibodies; autoimmune diseases; encephalitis; thyroid microsomal antibodies; diagnostic criteria; 14-3-3 protein