A new clinico-pathological classification system for mesial temporal sclerosis.
We propose a histopathological classification system for hippocampal cell loss in patients suffering from mesial temporal lobe epilepsies (MTLE). One hundred and seventy-eight surgically resected specimens were microscopically examined with respect to neuronal cell loss in hippocampal subfields CA1-CA4 and dentate gyrus. Five distinct patterns were recognized within a consecutive cohort of anatomically well-preserved surgical specimens. The first group comprised hippocampi with neuronal cell densities not significantly different from age matched autopsy controls [no mesial temporal sclerosis (no MTS); n = 34, 19%]. A classical pattern with severe cell loss in CA1 and moderate neuronal loss in all other subfields excluding CA2 was observed in 33 cases (19%), whereas the vast majority of cases showed extensive neuronal cell loss in all hippocampal subfields (n = 94, 53%). Due to considerable similarities of neuronal cell loss patterns and clinical histories, we designated these two groups as MTS type 1a and 1b, respectively. We further distinguished two atypical variants characterized either by severe neuronal loss restricted to sector CA1 (MTS type 2; n = 10, 6%) or to the hilar region (MTS type 3, n = 7, 4%). Correlation with clinical data pointed to an early age of initial precipitating injury (IPI < 3 years) as important predictor of hippocampal pathology, i.e. MTS type 1a and 1b. In MTS type 2, IPIs were documented at a later age (mean 6 years), whereas in MTS type 3 and normal appearing hippocampus (no MTS) the first event appeared beyond the age of 13 and 16 years, respectively. In addition, postsurgical outcome was significantly worse in atypical MTS, especially MTS type 3 with only 28% of patients having seizure relief after 1-year follow-up period, compared to successful seizure control in MTS types 1a and 1b (72 and 73%). Our classification system appears suitable for stratifying the clinically heterogeneous group of MTLE patients also with respect to postsurgical outcome studies.
Blümcke
Ingmar
I
Department of Neuropathology, Friedrich-Alexander-University Erlangen-Nuremberg, Krankenhausstrasse 8-10, 91054, Erlangen, Germany. bluemcke@neuropatho.med.uni-erlangen.de
Pauli
Elisabeth
E
Clusmann
Hans
H
Schramm
Johannes
J
Becker
Albert
A
Elger
Christian
C
Merschhemke
Martin
M
Meencke
Heinz-Joachim
HJ
Lehmann
Thomas
T
von Deimling
Andreas
A
Scheiwe
Christian
C
Zentner
Josef
J
Volk
Benedikt
B
Romstöck
Johann
J
Stefan
Hermann
H
Hildebrandt
Michelle
M
D000328:Adult; D000704:Analysis of Variance; D016923:Cell Death / Q000502:physiology; D016000:Cluster Analysis; D004833:Epilepsy, Temporal Lobe / Q000145:classification* / Q000175:diagnosis* / Q000601:surgery; D005260:Female; D005500:Follow-Up Studies; D006624:Hippocampus / Q000473:pathology* / Q000503:physiopathology* / Q000601:surgery; D006801:Humans; D008297:Male; D008875:Middle Aged; D009474:Neurons / Q000473:pathology*; D010751:Phosphopyruvate Hydratase / Q000378:metabolism; D012189:Retrospective Studies; D012720:Severity of Illness Index
D010751:Phosphopyruvate Hydratase