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Pathology of Epilepsy
Mark Cohen
Department of Pathology
University Hospitals Case Medical Center
January 6th
, 2009
'He was thinking, incidentally, that there
was a moment or two in his epileptic
condition almost before the fit itself (if...
Learning ObjectivesLearning Objectives
• Discuss the Blumcke classification of medial
temporal lobe sclerosis, relating
hi...
Pathology of TLEPathology of TLE
5%No Pathology
5%Dual Pathology
25%Focal lesions
65%
Ammon’s Horn
Sclerosis
Blumcke I, Th...
Wyler grading of HS (1992)Wyler grading of HS (1992)
>50% neuronal loss involving
all hippocampal sectorsGrade 4
>50% neur...
Blumcke I, et. Al. A new clinico-pathological classification system
for mesial temporal sclerosis.
Acta Neuropathol (Berl)...
Blumcke I, et. Al. A new clinico-pathological classification
system for mesial temporal sclerosis.
Acta Neuropathol (Berl)...
Blumke classification of MTSBlumke classification of MTS
CA1 preserved, moderate loss in
other sectors (endfolium sclerosi...
Blumcke type
MTS
Age of initial
precipitating injury
or first seizure
(years)
Excellent outcome
at 1 year post
surgery (%)...
Generalized malformations of cortical developmentGeneralized malformations of cortical development
sporadicHME
SPPX2,spora...
Type of cortical
dysplasia
Age at onset of
seizures (years)
Age at surgery (years)
Hemispheric (with
hemimegalencephaly)
0...
MCD pathology by MRI typeMCD pathology by MRI type
00153055
Immature
neurons
8035452045Balloon cells
10253555100
Layer I
n...
Palmini classification
Mild Malformations ofMild Malformations of
Cortical DevelopmentCortical Development
Type I: Ectopic...
• •
Dual Pathology: HS + FCDDual Pathology: HS + FCD
• 12 male patients12 male patients
– Age of onset 10 years (<1 – 29)Age o...
Pathologic diagnosis % of cases
Cortical dysplasia (including
Tuberous Sclerosis)
20
Vascular malformations (including
Stu...
Rasmussen syndromeRasmussen syndrome
• 45 patients (27F, 18M)45 patients (27F, 18M)
• Age at onset: 7 +/- 3 yearsAge at on...
RS: Pathologic stagingRS: Pathologic staging
Pan-laminar cavitation &/or gliosis4
Pan-laminar degeneration & gliosis3
Pan-...
Learning ObjectivesLearning Objectives
• Discuss the Blumcke classification of medial
temporal lobe sclerosis, relating
hi...
" For several instants I experience a" For several instants I experience a
happiness that is impossible in an ordinaryhapp...
Pathology of Epilepsy
Pathology of Epilepsy
Pathology of Epilepsy
Pathology of Epilepsy
Pathology of Epilepsy
Pathology of Epilepsy
Pathology of Epilepsy
Pathology of Epilepsy
Pathology of Epilepsy
Pathology of Epilepsy
Pathology of Epilepsy
Pathology of Epilepsy
Pathology of Epilepsy
Pathology of Epilepsy
Pathology of Epilepsy
Pathology of Epilepsy
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Transcript of "Pathology of Epilepsy"

  1. 1. Pathology of Epilepsy Mark Cohen Department of Pathology University Hospitals Case Medical Center January 6th , 2009
  2. 2. 'He was thinking, incidentally, that there was a moment or two in his epileptic condition almost before the fit itself (if it occurred in waking hours) when suddenly amid the sadness, spiritual darkness and depression, his brain seemed to catch fire at brief moments....His sensation of being alive and his awareness increased tenfold at those moments which flashed by like lightning. His mind and heart were flooded by a dazzling light. All his agitation, doubts and worries, seemed composed in a twinkling, culminating in a great calm, full of understanding...but these moments, these glimmerings were still but a premonition of that final second (never more than a second) with which the seizure itself began. That second was, of course, unbearable.'
  3. 3. Learning ObjectivesLearning Objectives • Discuss the Blumcke classification of medial temporal lobe sclerosis, relating histopathologic features to surgical outcomes • Discuss the Palmini classification of cortical dysplasia, relating histopathologic features to surgical outcomes • Define “dual pathology” in temporal lobe epilepsy, and the relative contribution of hippocampal and neocortical lesions • List two additional neuropathologic lesions which commonly present with epilepsy
  4. 4. Pathology of TLEPathology of TLE 5%No Pathology 5%Dual Pathology 25%Focal lesions 65% Ammon’s Horn Sclerosis Blumcke I, Thom M, Wiestler OD. Ammon's horn sclerosis: a maldevelopmental disorder associated with temporal lobe epilepsy. Brain Pathol. 2002 Apr;12(2):199-211
  5. 5. Wyler grading of HS (1992)Wyler grading of HS (1992) >50% neuronal loss involving all hippocampal sectorsGrade 4 >50% neuronal loss CA1, CA3, and/or CA4; CA2 sparedGrade 3 10-50% neuronal loss in CA1, CA3, and/or CA4Grade 2 0-10% neuronal loss in CA1, CA3, and/or CA4Grade 1 AR Wyler, FC Dohan, JB Schweitzer, AD Berry A grading system for mesial temporal pathology (hippocampal sclerosis) from anterior temporal lobectomy - J Epilepsy, 1992
  6. 6. Blumcke I, et. Al. A new clinico-pathological classification system for mesial temporal sclerosis. Acta Neuropathol (Berl). 2007 Mar;113(3):235-244.
  7. 7. Blumcke I, et. Al. A new clinico-pathological classification system for mesial temporal sclerosis. Acta Neuropathol (Berl). 2007 Mar;113(3):235-244.
  8. 8. Blumke classification of MTSBlumke classification of MTS CA1 preserved, moderate loss in other sectors (endfolium sclerosis) MTS type 3 (5%) Severe CA1 loss, mild loss in other sectors (CA1-sclerosis) MTS type 2 (5%) Severe neuronal loss involving all sectors (= Wyler grade 4)MTS 1b (50%) “Classic” – severe CA1 loss, moderate loss in other sectorsMTS 1a (20%) Neuronal cell loss within 1st SD compared to controls No MTS (20%) A new clinico-pathological classification system for mesial temporal sclerosis. Acta Neuropathol (Berl). 2007 Mar;113(3):235-244.
  9. 9. Blumcke type MTS Age of initial precipitating injury or first seizure (years) Excellent outcome at 1 year post surgery (%) Fair outcome at 1 year post surgery (%) No MTS 16 60 20 MTS type 1a 2 70 10 MTS type 1b 3 70 10 MTS type 2 6 70 10 MTS type 3 13 30 40
  10. 10. Generalized malformations of cortical developmentGeneralized malformations of cortical development sporadicHME SPPX2,sporadicPolymicrogyria Filamin 1FLN 1 PV nodular heterotopia DoublecortinDCX Laminar heterotopia PAF-acetylhydrolase Doublecortin Reelin LIS1 DCX(XLIS) RLN Lissencephalies ProteinGenesMalformation Guerrini R, Dobyns WB, Barkovich AJ. Abnormal development of the human cerebral cortex: genetics, functional consequences and treatment options. Trends Neurosci. 2008 Mar;31(3):154-62.
  11. 11. Type of cortical dysplasia Age at onset of seizures (years) Age at surgery (years) Hemispheric (with hemimegalencephaly) 0.1 1 Hemispheric (without hemimegalencephaly) 0.8 3.5 Multilobar 0.7 4.5 Lobar 1.2 5 Focal 1.7 7.5 Cepeda C, et. Al. Epileptogenesis in pediatric cortical dysplasia: the dysmature cerebral developmental hypothesis. Epilepsy Behav. 2006 Sep;9(2):219-35
  12. 12. MCD pathology by MRI typeMCD pathology by MRI type 00153055 Immature neurons 8035452045Balloon cells 10253555100 Layer I neurons 1025806580PMG 7040703590 Cytomegalic neurons 100909010090 Dysmorphic neurons 100100100100100WM neurons FocalLobarMultilobarHemi CDHMEPath (%)
  13. 13. Palmini classification Mild Malformations ofMild Malformations of Cortical DevelopmentCortical Development Type I: Ectopic neuronsType I: Ectopic neurons in/near Layer 1in/near Layer 1 Type 2: NeuronalType 2: Neuronal heterotopia outside Layer 1heterotopia outside Layer 1 Focal CorticalFocal Cortical DysplasiasDysplasias Type IA: Dyslamination +/-Type IA: Dyslamination +/- mild MCDmild MCD Type IB: IA + giant orType IB: IA + giant or immature neuronsimmature neurons Type IIA: DysmorphicType IIA: Dysmorphic neuronsneurons sanssans balloon cellsballoon cells Type IIB: DysmorphicType IIB: Dysmorphic neuronsneurons avecavec balloon cellsballoon cells Palmini A, Najm I, Avanzini G, Babb T, Guerrini R, Foldvary-Schaefer N, Jackson G, Luders HO, Prayson R, Spreafico R, Vinters HV. Terminology and classification of the cortical dysplasias. Neurology. 2004 Mar 23;62(6 Suppl 3):S2-8
  14. 14. • •
  15. 15. Dual Pathology: HS + FCDDual Pathology: HS + FCD • 12 male patients12 male patients – Age of onset 10 years (<1 – 29)Age of onset 10 years (<1 – 29) – Age at invasive EEG 30 years (6 – 50)Age at invasive EEG 30 years (6 – 50) • 113 seizures + interictal data from113 seizures + interictal data from depth electrodes in HC & subduraldepth electrodes in HC & subdural electrodes over temporal neocortexelectrodes over temporal neocortex • 40% of seizures from AHC, 35% from40% of seizures from AHC, 35% from TN, and 25% from bothTN, and 25% from both Fauser S, Schulze-Bonhage A. Epileptogenicity of cortical dysplasia in temporal lobe dual pathology: an electrophysiological study with invasive recordings. Brain. 2006 Jan;129(Pt 1):82-95
  16. 16. Pathologic diagnosis % of cases Cortical dysplasia (including Tuberous Sclerosis) 20 Vascular malformations (including Sturge-Weber disease) 20 Glioneuronal tumors 20 Other gliomas 10 Gliosis 30 52 patients with occipital lobe epilepsy Binder DK, Von Lehe M, Kral T, Bien CG, Urbach H, Schramm J, Clusmann H. Surgical treatment of occipital lobe epilepsy. J Neurosurg. 2008 Jul;109(1):57-69.
  17. 17. Rasmussen syndromeRasmussen syndrome • 45 patients (27F, 18M)45 patients (27F, 18M) • Age at onset: 7 +/- 3 yearsAge at onset: 7 +/- 3 years • Age at hemispherectomy: 9.5 +/- 4 yearsAge at hemispherectomy: 9.5 +/- 4 years • Duration of symptoms: 0.5 – 14 yearsDuration of symptoms: 0.5 – 14 years Pardo CA, Vining EP, Guo L, Skolasky RL, Carson BS, Freeman JM. The pathology of Rasmussen syndrome: stages of cortical involvement and neuropathological studies in 45 hemispherectomies. Epilepsia. 2004 May;45(5):516-26.
  18. 18. RS: Pathologic stagingRS: Pathologic staging Pan-laminar cavitation &/or gliosis4 Pan-laminar degeneration & gliosis3 Pan-laminar inflammation & gliosis2 Mild focal inflammation1 Normal cortex0
  19. 19. Learning ObjectivesLearning Objectives • Discuss the Blumcke classification of medial temporal lobe sclerosis, relating histopathologic features to surgical outcomes • Discuss the Palmini classification of cortical dysplasia, relating histopathologic features to surgical outcomes • Define “dual pathology” in temporal lobe epilepsy, and the relative contribution of hippocampal and neocortical lesions • List two additional neuropathologic lesions which commonly present with epilepsy
  20. 20. " For several instants I experience a" For several instants I experience a happiness that is impossible in an ordinaryhappiness that is impossible in an ordinary state, and of which other people have nostate, and of which other people have no conception. I feel full harmony in myselfconception. I feel full harmony in myself and in the whole world, and the feeling isand in the whole world, and the feeling is so strong and sweet that for a few secondsso strong and sweet that for a few seconds of such bliss one could give up ten yearsof such bliss one could give up ten years of life, perhaps all of life.of life, perhaps all of life. I felt that heaven descended to earth andI felt that heaven descended to earth and swallowed me. I really attained god andswallowed me. I really attained god and was imbued with him. All of you healthywas imbued with him. All of you healthy people don't even suspect what happinesspeople don't even suspect what happiness is , that happiness that we epilepticsis , that happiness that we epileptics experience for a second before an attack."experience for a second before an attack."
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