cMRT-Auffälligkeiten und Symptomatik bei CJK-Patienten - Gibt es eine Korrelation zwischen cMRT-Veränderungen und der klinischen Symptomatik
Is there a correlation between MRI findings and symptoms in CJD patients
by Stefanie Manger
Date of Examination:2018-05-02
Date of issue:2018-05-07
Advisor:Prof. Dr. Margarete Schön
Referee:Prof. Dr. Inga Zerr
Referee:PD Dr. Marios-Nikos Psychogios
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Abstract
English
Already early in the history of CJD research, even before the detection of CJD subtypes on the basis polymorphism of codon 129 of the prion protein gene and the different types of pathological prion protein (type 1 and 2), a correlation between symptoms and histopathologic findings have been found, e.g. in so-called “Heidenhain variant” typical for visual disturbances and occipital cortical histopathologic findings. Later case reports and studies confirmed a correlation between MRI and histopathologic findings. In routine work of the National Reference Center for transmissible spongiform encephalopathies, there seems to be a correlation even between clinical symptoms and MRI hyperintensities, for example, unilateral MRI hyperintensities are often found in patients with unilateral symptoms. This doctoral thesis systematic study was performed to investigate a possible correlation between MRI hyperintensities and symptoms which is new in literature. Therefore MR images of 138 Patients with probable and definite sporadic CJD and no sign of other disease were evaluated following a standardized scheme. Only DW images where used because they had been proven to be the most sensitive in CJD imaging. Using literature other than CJD literature, symptoms were matched with the loss of function of different brain areas to be investigated in those CJD patients. In this matter some symptoms seemed to match better than others and some symptoms and brain regions didn’t match at all. The reason for this is most likely the complexity of human brain. Essential in this study was also the fact that MR images change in the course of the disease. So for this investigation different periods of time were defined relating to the time of MRI performance. Correlation analysis was performed graphically and using contingency table including the calculation of phi-coefficient and cramers V. Also general data about the included patients was acquired as well as frequency of symptoms, MRI hyperintensities and information about EEG and spinal fluid analysis. Results: In this study there could be shown a positive correlation between symptoms and DW imaging. This was more obvious regarding some brain regions, e.g. basal ganglia, and less obvious regarding others, e.g. temporal cortex. This is most likely due to the difficulties of matching symptoms with areas of deficit brain function and the frequency of symptoms and MRI hyperintensities. Noticeable was the fact that correlation was best if MR imaging and symptom onset were coincidental. Only in thalamus no correlation was found. Causal might be the fact that DWI seems to be inappropriate for finding thalamic hyperintensities and even in FFI patients thalamic MRI hyperintensities are rare, even if the main symptoms are sleep disturbances, which have been linked to thalamic deficit. CJD subtypes differ regarding symptoms, clinical course, EEG and MRI findings as well as cerebral fluid analysis. Therefore it was investigated if they also differ in correlation between symptoms and MR imaging. In fact in MM/MV1 and MM2c subtypes an obvious correlation was found, in MV2 patients it was less obvious and in VV1 and VV2 patient there was hardly any correlation with coincidental symptom onset and MRI.
Keywords: CJD; MRI; symptoms; correlation