Difference between revisions of "Epilepsy"

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'''Epilepsy''' is a common chronic seizure disorder.
'''Epilepsy''' is a common chronic seizure disorder.


==Etiology==
==General==
*Epilepsy = seizures that are "idiopathic", i.e. no brain tumour, no mass lesion, no brain injury.
*Most common form: ''temporal lobe epilepsy''.<ref>URL: [http://emedicine.medscape.com/article/342150-overview http://emedicine.medscape.com/article/342150-overview]. Accessed on: 20 November 2010.</ref>
 
===Etiology===
*Many.
*Many.
**[[Epilepsy#Focal_cortical_dysplasia_.28FCD.29|Cortical dysplasia]].
**[[Epilepsy#Hamartia|Hamartia]].
**Stroke.
**Infection.
**Head trauma.


Syndromic:
Syndromic:
Line 10: Line 19:
*[[Dysembryoplastic neuroepithelial tumour]].<ref name=pmid15881751>{{Cite journal  | last1 = Cataltepe | first1 = O. | last2 = Turanli | first2 = G. | last3 = Yalnizoglu | first3 = D. | last4 = Topçu | first4 = M. | last5 = Akalan | first5 = N. | title = Surgical management of temporal lobe tumor-related epilepsy in children. | journal = J Neurosurg | volume = 102 | issue = 3 Suppl | pages = 280-7 | month = Apr | year = 2005 | doi = 10.3171/ped.2005.102.3.0280 | PMID = 15881751 }}</ref>
*[[Dysembryoplastic neuroepithelial tumour]].<ref name=pmid15881751>{{Cite journal  | last1 = Cataltepe | first1 = O. | last2 = Turanli | first2 = G. | last3 = Yalnizoglu | first3 = D. | last4 = Topçu | first4 = M. | last5 = Akalan | first5 = N. | title = Surgical management of temporal lobe tumor-related epilepsy in children. | journal = J Neurosurg | volume = 102 | issue = 3 Suppl | pages = 280-7 | month = Apr | year = 2005 | doi = 10.3171/ped.2005.102.3.0280 | PMID = 15881751 }}</ref>
*[[Ganglioglioma]].<ref name=pmid12125968>{{Cite journal  | last1 = Im | first1 = SH. | last2 = Chung | first2 = CK. | last3 = Cho | first3 = BK. | last4 = Lee | first4 = SK. | title = Supratentorial ganglioglioma and epilepsy: postoperative seizure outcome. | journal = J Neurooncol | volume = 57 | issue = 1 | pages = 59-66 | month = Mar | year = 2002 | doi =  | PMID = 12125968 }}</ref>
*[[Ganglioglioma]].<ref name=pmid12125968>{{Cite journal  | last1 = Im | first1 = SH. | last2 = Chung | first2 = CK. | last3 = Cho | first3 = BK. | last4 = Lee | first4 = SK. | title = Supratentorial ganglioglioma and epilepsy: postoperative seizure outcome. | journal = J Neurooncol | volume = 57 | issue = 1 | pages = 59-66 | month = Mar | year = 2002 | doi =  | PMID = 12125968 }}</ref>
*Isomorphic astrocytoma.
==Types==
Features:<ref>MUN. 15 November 2010.</ref>
*Temporal lobe epilepsy
*Mesial temporal sclerosis = scarring of the medial temporal lobe.
**Involves: hippocampus, parahippocampal gyrus and amygdala.
*Granule cell dispersion
===Hamartia===
* Small collection of ectopic glioneuronal cells.
**Morpholology resembling oligodendroglial-like cells. <ref>{{Cite journal  | last1 = Kasper | first1 = BS. | last2 = Stefan | first2 = H. | last3 = Buchfelder | first3 = M. | last4 = Paulus | first4 = W. | title = Temporal lobe microdysgenesis in epilepsy versus control brains. | journal = J Neuropathol Exp Neurol | volume = 58 | issue = 1 | pages = 22-8 | month = Jan | year = 1999 | doi =  | PMID = 10068310 }}</ref>
* Mostly amygdala, less common in hippocampus or temporal lobe.
* Can coexist with focal cortical dysplasia.
===Focal cortical dysplasia (FCD)===
*Localized malformations of the cortex.
*Frequently associated with epilepsy in children.
*Includes cortical dyslamination, cytoarchitectural changes and white matter abnormalities.
*Current consensus: ILAE classification scheme 2011 <ref>{{Cite journal  | last1 = Blümcke | first1 = I. | last2 = Aronica | first2 = E. | last3 = Miyata | first3 = H. | last4 = Sarnat | first4 = HB. | last5 = Thom | first5 = M. | last6 = Roessler | first6 = K. | last7 = Rydenhag | first7 = B. | last8 = Jehi | first8 = L. | last9 = Krsek | first9 = P. | title = International recommendation for a comprehensive neuropathologic workup of epilepsy surgery brain tissue: A consensus Task Force report from the ILAE Commission on Diagnostic Methods. | journal = Epilepsia | volume = 57 | issue = 3 | pages = 348-58 | month = Mar | year = 2016 | doi = 10.1111/epi.13319 | PMID = 26839983 }}
</ref>(based on previous classification by Palmini 2004):
*Type I FCD (focal)
**Ia: Abnormal radial cortical lamination.
**Ib: Abnormal tangential cortical lamination.
**Ic: Abnormal radial and tangential cortical lamination.
*Type II FCD (focal)
**IIa: Presence of dysmorphic neurons.
**IIb: Presence of dysmorphic neurons and balloon cells.
*Type III FCD (associated with other lesion)
**IIIa: FCD associated with [[Epilepsy#Hippocampal_sclerosis|hippocampal sclerosis]].
**IIIb: FCD adjacent to a brain tumor.
**IIIc: FCD adjacent to vascular malformation.
**IIIc: FCD associated with previous injury (trauma, inflammation...).
<gallery>
File:FCDIIa dysmorphic neurons HE.jpg|Dysmorphic neurons in FCD (HE)
File:FCDIIa neuronal heterotopia neun.jpg|Heterotopic neurons (NeuN)
</gallery>
===Hippocampal sclerosis===
*Most frequent histopathology in temporal lobe epilepsy (33% of all epilepsy surgery specimen).
*ILAE classification for hippocampus specimen:<ref>{{Cite journal  | last1 = Blümcke | first1 = I. | last2 = Thom | first2 = M. | last3 = Aronica | first3 = E. | last4 = Armstrong | first4 = DD. | last5 = Bartolomei | first5 = F. | last6 = Bernasconi | first6 = A. | last7 = Bernasconi | first7 = N. | last8 = Bien | first8 = CG. | last9 = Cendes | first9 = F. | title = International consensus classification of hippocampal sclerosis in temporal lobe epilepsy: a Task Force report from the ILAE Commission on Diagnostic Methods. | journal = Epilepsia | volume = 54 | issue = 7 | pages = 1315-29 | month = Jul | year = 2013 | doi = 10.1111/epi.12220 | PMID = 23692496 }}</ref>
** ILAE type 1: cell loss predominantly in  CA1 and CA4 sectors.
** ILAE type 2: predominant CA1 neuron loss and gliosis.
** ILAE type 3: CA4 predominant neuronal cell loss and gliosis.
Clinic:
ILAE type 1: benefit from epilepsy surgery.
Notes:
*Gliosis withot neuronal loss is not considered hippocampal sclerosis.
===Granule cell dispersion===
*Affects dentate gyrus.
*Observed in up to 40% specimen with hippocampal sclerosis.
*Clinico-pathological classification:<ref>{{Cite journal  | last1 = Blümcke | first1 = I. | last2 = Kistner | first2 = I. | last3 = Clusmann | first3 = H. | last4 = Schramm | first4 = J. | last5 = Becker | first5 = AJ. | last6 = Elger | first6 = CE. | last7 = Bien | first7 = CG. | last8 = Merschhemke | first8 = M. | last9 = Meencke | first9 = HJ. | title = Towards a clinico-pathological classification of granule cell dispersion in human mesial temporal lobe epilepsies. | journal = Acta Neuropathol | volume = 117 | issue = 5 | pages = 535-44 | month = May | year = 2009 | doi = 10.1007/s00401-009-0512-5 | PMID = 19277686 }}</ref>
**Granule cell pathology (GCP) Type 1: Substantial granule cell loss.
**Granule cell pathology (GCP) Type 2: Cell dispersion, ectopic neurons or clusters of neurons in the molecular layer or bi-lamination.
Clinic:
*Association with longer epilepsy duration.
DDx:
**Epilepsy.
**[[Dementia]].
===Mild Malformation of Cortical Development with Oligodendroglial Hyperplasia===
* Abbreviated: MOGHE <ref>{{Cite journal  | last1 = Schurr | first1 = J. | last2 = Coras | first2 = R. | last3 = Rössler | first3 = K. | last4 = Pieper | first4 = T. | last5 = Kudernatsch | first5 = M. | last6 = Holthausen | first6 = H. | last7 = Winkler | first7 = P. | last8 = Woermann | first8 = F. | last9 = Bien | first9 = CG. | title = Mild Malformation of Cortical Development with Oligodendroglial Hyperplasia in Frontal Lobe Epilepsy: A New Clinico-Pathological Entity. | journal = Brain Pathol | volume = 27 | issue = 1 | pages = 26-35 | month = 01 | year = 2017 | doi = 10.1111/bpa.12347 | PMID = 26748554 }}</ref>.
* Frontal lobe.
* Nonlesional (3.7% of epilepsy case).
* Increase cellularity of Olig2-, and PDGFR-alpha-immunoreactive oligodendroglia.


==Sudden unexpected death in epilepsy==
==Sudden unexpected death in epilepsy==

Latest revision as of 14:05, 25 March 2019

Epilepsy is a common chronic seizure disorder.

General

  • Epilepsy = seizures that are "idiopathic", i.e. no brain tumour, no mass lesion, no brain injury.
  • Most common form: temporal lobe epilepsy.[1]

Etiology

Syndromic:

Tumour:

Types

Features:[4]

  • Temporal lobe epilepsy
  • Mesial temporal sclerosis = scarring of the medial temporal lobe.
    • Involves: hippocampus, parahippocampal gyrus and amygdala.
  • Granule cell dispersion

Hamartia

  • Small collection of ectopic glioneuronal cells.
    • Morpholology resembling oligodendroglial-like cells. [5]
  • Mostly amygdala, less common in hippocampus or temporal lobe.
  • Can coexist with focal cortical dysplasia.

Focal cortical dysplasia (FCD)

  • Localized malformations of the cortex.
  • Frequently associated with epilepsy in children.
  • Includes cortical dyslamination, cytoarchitectural changes and white matter abnormalities.
  • Current consensus: ILAE classification scheme 2011 [6](based on previous classification by Palmini 2004):


  • Type I FCD (focal)
    • Ia: Abnormal radial cortical lamination.
    • Ib: Abnormal tangential cortical lamination.
    • Ic: Abnormal radial and tangential cortical lamination.


  • Type II FCD (focal)
    • IIa: Presence of dysmorphic neurons.
    • IIb: Presence of dysmorphic neurons and balloon cells.


  • Type III FCD (associated with other lesion)
    • IIIa: FCD associated with hippocampal sclerosis.
    • IIIb: FCD adjacent to a brain tumor.
    • IIIc: FCD adjacent to vascular malformation.
    • IIIc: FCD associated with previous injury (trauma, inflammation...).


  • Dysmorphic neurons in FCD (HE)

  • Heterotopic neurons (NeuN)

Hippocampal sclerosis

  • Most frequent histopathology in temporal lobe epilepsy (33% of all epilepsy surgery specimen).
  • ILAE classification for hippocampus specimen:[7]
    • ILAE type 1: cell loss predominantly in CA1 and CA4 sectors.
    • ILAE type 2: predominant CA1 neuron loss and gliosis.
    • ILAE type 3: CA4 predominant neuronal cell loss and gliosis.

Clinic: ILAE type 1: benefit from epilepsy surgery.

Notes:

  • Gliosis withot neuronal loss is not considered hippocampal sclerosis.

Granule cell dispersion

  • Affects dentate gyrus.
  • Observed in up to 40% specimen with hippocampal sclerosis.
  • Clinico-pathological classification:[8]
    • Granule cell pathology (GCP) Type 1: Substantial granule cell loss.
    • Granule cell pathology (GCP) Type 2: Cell dispersion, ectopic neurons or clusters of neurons in the molecular layer or bi-lamination.

Clinic:

  • Association with longer epilepsy duration.

DDx:

Mild Malformation of Cortical Development with Oligodendroglial Hyperplasia

  • Abbreviated: MOGHE [9].
  • Frontal lobe.
  • Nonlesional (3.7% of epilepsy case).
  • Increase cellularity of Olig2-, and PDGFR-alpha-immunoreactive oligodendroglia.

Sudden unexpected death in epilepsy

  • Abbreviated SUDEP.

Diagnosis:

Epidemiology:[10]

  • Typically poorly controlled epilepsy.
  • Incidence: 0.09-9 per 1000 patient-years.

See also

References

  1. ↑ URL: http://emedicine.medscape.com/article/342150-overview. Accessed on: 20 November 2010.
  2. ↑ Cataltepe, O.; Turanli, G.; Yalnizoglu, D.; Topçu, M.; Akalan, N. (Apr 2005). "Surgical management of temporal lobe tumor-related epilepsy in children.". J Neurosurg 102 (3 Suppl): 280-7. doi:10.3171/ped.2005.102.3.0280. PMID 15881751.
  3. ↑ Im, SH.; Chung, CK.; Cho, BK.; Lee, SK. (Mar 2002). "Supratentorial ganglioglioma and epilepsy: postoperative seizure outcome.". J Neurooncol 57 (1): 59-66. PMID 12125968.
  4. ↑ MUN. 15 November 2010.
  5. ↑ Kasper, BS.; Stefan, H.; Buchfelder, M.; Paulus, W. (Jan 1999). "Temporal lobe microdysgenesis in epilepsy versus control brains.". J Neuropathol Exp Neurol 58 (1): 22-8. PMID 10068310.
  6. ↑ Blümcke, I.; Aronica, E.; Miyata, H.; Sarnat, HB.; Thom, M.; Roessler, K.; Rydenhag, B.; Jehi, L. et al. (Mar 2016). "International recommendation for a comprehensive neuropathologic workup of epilepsy surgery brain tissue: A consensus Task Force report from the ILAE Commission on Diagnostic Methods.". Epilepsia 57 (3): 348-58. doi:10.1111/epi.13319. PMID 26839983.
  7. ↑ Blümcke, I.; Thom, M.; Aronica, E.; Armstrong, DD.; Bartolomei, F.; Bernasconi, A.; Bernasconi, N.; Bien, CG. et al. (Jul 2013). "International consensus classification of hippocampal sclerosis in temporal lobe epilepsy: a Task Force report from the ILAE Commission on Diagnostic Methods.". Epilepsia 54 (7): 1315-29. doi:10.1111/epi.12220. PMID 23692496.
  8. ↑ Blümcke, I.; Kistner, I.; Clusmann, H.; Schramm, J.; Becker, AJ.; Elger, CE.; Bien, CG.; Merschhemke, M. et al. (May 2009). "Towards a clinico-pathological classification of granule cell dispersion in human mesial temporal lobe epilepsies.". Acta Neuropathol 117 (5): 535-44. doi:10.1007/s00401-009-0512-5. PMID 19277686.
  9. ↑ Schurr, J.; Coras, R.; Rössler, K.; Pieper, T.; Kudernatsch, M.; Holthausen, H.; Winkler, P.; Woermann, F. et al. (01 2017). "Mild Malformation of Cortical Development with Oligodendroglial Hyperplasia in Frontal Lobe Epilepsy: A New Clinico-Pathological Entity.". Brain Pathol 27 (1): 26-35. doi:10.1111/bpa.12347. PMID 26748554.
  10. ↑ Tomson, T.; Nashef, L.; Ryvlin, P. (Nov 2008). "Sudden unexpected death in epilepsy: current knowledge and future directions.". Lancet Neurol 7 (11): 1021-31. doi:10.1016/S1474-4422(08)70202-3. PMID 18805738.
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