A new clinico-pathological classification system for mesial temporal sclerosis

Springer Science and Business Media LLC - Tập 113 - Trang 235-244 - 2007
Ingmar Blümcke1,2, Elisabeth Pauli3, Hans Clusmann4, Johannes Schramm4, Albert Becker5, Christian Elger6, Martin Merschhemke7, Heinz-Joachim Meencke7, Thomas Lehmann8, Andreas von Deimling9, Christian Scheiwe10, Josef Zentner10, Benedikt Volk11, Johann Romstöck12, Hermann Stefan12, Michelle Hildebrandt1,2
1Department of Neuropathology, Friedrich-Alexander-University Erlangen-Nuremberg, Erlangen, Germany
2Neuropathological Reference Centre for Epilepsy Surgery, Department of Neuropathology, Friedrich-Alexander-University Erlangen-Nuremberg, Erlangen, Germany
3Department of Neurology, Epilepsy Center (ZEE), University Medical Center, Friedrich-Alexander-University Erlangen-Nuremberg, Erlangen, Germany
4Department of Neurosurgery, University of Bonn Medical Center, Bonn, Germany
5Department of Neuropathology, University of Bonn Medical Center, Bonn, Germany
6Department of Epileptology, University of Bonn Medical Center, Bonn, Germany
7Epilepsy Center Berlin-Brandenburg, Berlin, Germany
8Department of Neurosurgery, Charité-University Medicine Berlin, Berlin, Germany
9Department of Neuropathology, Charité-University Medicine Berlin, Berlin, Germany
10Department of Neurosurgery, University Hospital Freiburg, Freiburg, Germany
11Department of Neuropathology, University Hospital Freiburg, Freiburg, Germany
12Department of Neurosurgery, University Medical Center, Friedrich-Alexander-University Erlangen-Nuremberg, Erlangen, Germany

Tóm tắt

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.

Tài liệu tham khảo

Arruda F, Cendes F, Andermann F, Dubeau F, Villemure JG, Jonesgotman M, Poulin N, Arnold DL, Olivier A (1996) Mesial atrophy and outcome after amygdalohippocampectomy or temporal lobe removal. Ann Neurol 40:446–450 Bien CG, Kurthen M, Baron K, Lux S, Helmstaedter C, Schramm J, Elger CE (2001) Long-term seizure outcome and antiepileptic drug treatment in surgically treated temporal lobe epilepsy patients: a controlled study. Epilepsia 42:1416–1421 Blumcke I, Beck H, Suter B, Hoffmann D, Födisch HJ, Wolf HK, Schramm J, Elger CE, Wiestler OD (1999) An increase of hippocampal calretinin-immunoreactive neurons correlates with early febrile seizures in temporal lobe epilepsy. Acta Neuropathol 97:31–39 Blumcke I, Schewe JC, Normann S, Brustle O, Schramm J, Elger CE, Wiestler OD (2001) Increase of nestin-immunoreactive neural precursor cells in the dentate gyrus of pediatric patients with early-onset temporal lobe epilepsy. Hippocampus 11:311–321 Blumcke I, Thom M, Wiestler OD (2002) Ammon’s horn sclerosis: a maldevelopmental disorder associated with temporal lobe epilepsy. Brain Pathol 12:199–211 Borges K, Gearing M, McDermott DL, Smith AB, Almonte AG, Wainer BH, Dingledine R (2003) Neuronal and glial pathological changes during epileptogenesis in the mouse pilocarpine model. Exp Neurol 182:21–34 Cendes F, Andermann F, Dubeau F, Gloor P, Evans A, Jones GM, Olivier A, Andermann E, Robitaille Y, Lopes CI et al (1993) Early childhood prolonged febrile convulsions, atrophy and sclerosis of mesial structures, and temporal lobe epilepsy: an MRI volumetric study. Neurology 43:1083–1087 Clusmann H, Schramm J, Kral T, Helmstaedter C, Ostertun B, Fimmers R, Haun D, Elger CE (2002) Prognostic factors and outcome after different types of resection for temporal lobe epilepsy. J Neurosurg 97:1131–1141 Davies KG, Hermann BP, Dohan FC, Foley KT, Bush AJ, Wyler AR (1996) Relationship of hippocampal sclerosis to duration and age of onset of epilepsy, and childhood febrile seizures in temporal lobectomy patients. Epilepsy Res 24:119–126 de Lanerolle NC, Kim JH, Williamson A, Spencer SS, Zaveri HP, Eid T, Spencer DD (2003) A retrospective analysis of hippocampal pathology in human temporal lobe epilepsy: evidence for distinctive patient subcategories. Epilepsia 44:677–687 Duvernoy HM (2005) The human hippocampus. Functional anatomy, vascularization and serial sections with MRI. Springer, Heidelberg El Bahh B, Lespinet V, Lurton D, Coussemacq M, Le Gal La Salle G, Rougier A (1999) Correlations between granule cell dispersion, mossy fiber sprouting, and hippocampal cell loss in temporal lobe epilepsy. Epilepsia 40:1393–1401 Engel JJ (1994) Epilepsy surgery. Curr Opin Neurol 140–147 Engel JJ, Van Ness P, Rasmussen TB, Ojemann LM (1993): Outcome with respect to epileptic seizures, In: Engel JJ (ed) Surgical treatment of the epilepsies, Raven, New York, pp 609–621 Fauser S, Schulze-Bonhage A (2006) Epileptogenicity of cortical dysplasia in temporal lobe dual pathology: an electrophysiological study with invasive recordings. Brain 129:82–95 Hildebrandt M, Schulz R, Hoppe M, May T, Ebner A (2005) Postoperative routine EEG correlates with long-term seizure outcome after epilepsy surgery. Seizure 14:446–451 Janszky J, Janszky I, Schulz R, Hoppe M, Behne F, Pannek HW, Ebner A (2005) Temporal lobe epilepsy with hippocampal sclerosis: predictors for long-term surgical outcome. Brain 128:395–404 Janszky J, Schulz R, Ebner A (2003) Clinical features and surgical outcome of medial temporal lobe epilepsy with a history of complex febrile convulsions. Epilepsy Res 55:1–8 Kleihues P, Cavenee WK (2000) Pathology and genetics of tumours of the nervous system. World Health Organization Classification of Tumours, IARC, Lyon Kral T, Clusmann H, Urbach H, Schramm J, Elger CE, Kurthen M, Grunwald T (2002) Preoperative evaluation for epilepsy surgery (Bonn algorithm). Zentralbl. Neurochir 63:101–105 Lee MC, Kim GM, Woo YJ, Kim MK, Kim JH, Nam SC, Suh JJ, Chung WK, Lee JS, Kim HI, Choi HY, Kim SU (2001) Pathogenic significance of neuronal migration disorders in temporal lobe epilepsy. Hum Pathol 32:643–648 Margerison JH, Corsellis JAN (1966) Epilepsy and the temporal lobes: a clinical, electroencephalographic and neuropathological study of the brain in epilepsy, with particular reference to the temporal lobes. Brain 89:499–530 Mathern GW, Babb TL, Mischel PS, Vinters HV, Pretorius JK, Leite JP, Peacock WJ (1996) Childhood generalized and mesial temporal epilepsies demonstrate different amounts and patterns of hippocampal neuron loss and mossy fibre synaptic reorganization. Brain 119:965–987 Mathern GW, Babb TL, Vickrey BG, Melendez M, Pretorius JK (1995) The clinical-pathogenic mechanisms of hippocampal neuron loss and surgical outcomes in temporal lobe epilepsy. Brain 118:105–118 Mathern GW, Leiphart JL, De Vera A, Adelson PD, Seki T, Neder L, Leite JP (2002) Seizures decrease postnatal neurogenesis and granule cell development in the human fascia dentata. Epilepsia 43(Suppl 5):68–73 Mathern GW, Pretorius JK, Babb TL (1995) Influence of the type of initial precipitating injury and at what age it occurs on course and outcome in patients with temporal lobe seizures. J Neurosurg 82:220–227 Mathern GW, Pretorius JK, Babb TL (1995) Quantified patterns of mossy fiber sprouting and neuron densities in hippocampal and lesional seizures. J Neurosurg 82:211–219 Meencke HJ, Veith G (1991): Hippocampal sclerosis in epilepsy, In: Lüders HO (ed) Epilepsy surgery, Raven, New York, pp 705–715 Nadler JV (2003) The recurrent mossy fiber pathway of the epileptic brain. Neurochem Res 28:1649–1658 Parent JM, Elliott RC, Pleasure SJ, Barbaro NM, Lowenstein DH (2006) Aberrant seizure-induced neurogenesis in experimental temporal lobe epilepsy. Ann Neurol 59:81–91 Parent JM, Lowenstein DH (1997) Mossy fiber reorganization in the epileptic hippocampus. Curr Opin Neurol 10:103–109 Proper EA, Jansen GH, van Veelen CW, van Rijen PC, Gispen WH, de Graan PN (2001) A grading system for hippocampal sclerosis based on the degree of hippocampal mossy fiber sprouting. Acta Neuropathol (Berl) 101:405–409 Proper EA, Oestreicher AB, Jansen GH, Veelen CW, van Rijen PC, Gispen WH, de Graan PN (2000) Immunohistochemical characterization of mossy fibre sprouting in the hippocampus of patients with pharmaco-resistant temporal lobe epilepsy. Brain 123:19–30 Sagar HJ, Oxbury JM (1987) Hippocampal neuron loss in temporal lobe epilepsy: correlation with early childhood convulsions. Ann Neurol 22:334–340 Salanova V, Markand O, Worth R (2004) Temporal lobe epilepsy: analysis of patients with dual pathology. Acta Neurol Scand 109:126–131 Schulz R, Ebner A (2001) Prolonged febrile convulsions and mesial temporal lobe epilepsy in an identical twin. Neurology 57:318–320 Seifert G, Schilling K, Steinhauser C (2006) Astrocyte dysfunction in neurological disorders: a molecular perspective. Nat Rev Neurosci 7:194–206 Sommer W (1880) Die Erkrankung des Ammonshorns als aetiologisches Moment der Epilepsie. Arch Psychiat Nervenkr 308:631–675 Stefan H, Pauli E (2002) Progressive cognitive decline in epilepsy: an indication of ongoing plasticity. Prog Brain Res 135:409–417 Sutula TP, Cascino G, Cavazos J, Parada I, Ramirez L (1989) Mossy fiber synaptic reorganization in the epileptic human temporal lobe. Ann Neurol 26:321–330 Sutula TP, Golarai G, Cavazos J (1992) Assessing the functional significance of mossy fiber sprouting. Epilepsy Res Suppl 7:251–259 Sutula TP, He XX, Cavazos JE, Scott G (1988) Synaptic reorganization in the hippocampus induced by abnormal functional activity. Science 239:1147–1150 Tassi L, Colombo N, Garbelli R, Francione S, Lo Russo G, Mai R, Cardinale F, Cossu M, Ferrario A, Galli C, Bramerio M, Citterio A, Spreafico R (2002) Focal cortical dysplasia: neuropathological subtypes, EEG, neuroimaging and surgical outcome. Brain 125:1719–1732 Thom M, Zhou J, Martinian L, Sisodiya S (2005) Quantitative post-mortem study of the hippocampus in chronic epilepsy: seizures do not inevitably cause neuronal loss. Brain 128:1344–1357 von Lehe M, Lutz M, Kral T, Schramm J, Elger CE, Clusmann H (2006) Correlation of health-related quality of life after surgery for mesial temporal lobe epilepsy with two seizure outcome scales. Epilepsy Behav 9:73–82 Wieser HG (2004) ILAE Commission Report. Mesial temporal lobe epilepsy with hippocampal sclerosis. Epilepsia 45:695–714 Wieser HG, Ortega M, Friedman A, Yonekawa Y (2003) Long-term seizure outcomes following amygdalohippocampectomy. J Neurosurg 98:751–763 Wyler AR, Dohan FC, Schweitzer JB, Berry AD (1992) A grading system for mesial temporal pathology (hippocampal sclerosis) from anterior temporal lobectomy. J Epilepsy 5:220–225