NR AUSP
AU Krasnianski,A.; Meissner,B.; Schulz-Schaeffer,W.J.; Kallenberg,K.; Bartl,M.; Heinemann,U.; Varges,D.; Kretzschmar,H.A.; Zerr,I.
TI Clinical findings and diagnostic tests in the MM2 cortical subtype of sCJD
QU TSE-Forum, 6. Kongress - Nationale TSE-Forschungsplattform, Greifswald 26.6.-28.6.2006, Poster: Diagnostik DIA-05
PT Konferenz-Poster
AB
Objective: To describe clinical features and diagnostic tests of the MM2 cortical subtype in sCJD.
Methods: Clinical symptoms, MRI, EEG, and CSF markers were studied in 12 genetically and neuropathologically verified patients. Histological findings were semiquantitatively evaluated.
Results: Compared with classical sCJD, the disease duration was prolonged (median 14 months). Dementia and early and prominent neuropsychological signs such as spatial disorientation, aphasia and apraxia were found in all patients. Alzheimer's disease was the most frequent initial diagnosis (33%). Increased S-100B protein in the CSF was found in 100%, the 14-3-3 test was positive in 91%. EEG revealed periodic sharp wave complexes in 42%. T2-weighted MRI showed basal ganglia hyperintensities in only one patient. Cortical hyperintensities were not obligatory. Severe cortical damage was the most prominent histological feature.
Conclusions: The S-100B (100%) and 14-3-3 investigation (91%) were the most sensitive diagnostic tests. Prolonged disease duration, dementia as the only CJD typical symptom for longer time, and low sensitivity of MRI and EEG make the diagnosis in the MM2 cortical subtype difficult. Therefore, the detailed clinical investigation is especially important in this sCJD subtype. We suggest that rapidly progressive dementia with early and prominent neuropsychological deficits in older patients should lead to suspicion of the MM2 cortical subtype even if other neurological deficits are absent. It may be proposed that at least some MM2 cortical patients are misdiagnosed as rapidly progressive Alzheimer's disease.
AD A.Krasnianski, B.Meissner, M.Bartl, Uta Heinemann, D.Varges, I.Zerr, Department of Neurology, Georg-August University Göttingen, Germany; W.J.Schulz-Schaeffer, Department of Neuroradiology, Georg-August University Göttingen, Germany; K.Kallenberg, Department of Neuropathology, Georg-August University Göttingen, Germany; H.A.Kretzschmar, Department of Neuropathology, Ludwig-Maximillian University Munich, Germany
SP englisch
PO Deutschland
OR Tagungsband