KEGG   DISEASE: 海馬硬化を伴う内側側頭葉てんかん
エントリ  
H01826                                                             
名称    
海馬硬化を伴う内側側頭葉てんかん
概要    
Mesial temporal lobe epilepsy with hippocampal sclerosis (MTLE-HS) is a group of chronic disorders characterized by prominent neuronal loss and gliosis in the hippocampus and amygdala. MTLE-HS is restricted to patients in whom hippocampal atrophy and/or abnormal signal intensity on MRI, anterior temporal interictal epileptiform discharges, and additional evidence of temporal dysfunction on functional images and neuro-psychological assessment are demonstrated. Patients with MTLE-HS often have a history of early risk factors such as febrile seizures, status epilepticus (SE) or infection. A seizure-free period may ensue before uncontrolled partial seizures begin. Epileptic focus is located deeply in the mesial temporal region including mainly hippocampus, parahippocampal gyrus and amygdala. The anatomopathological hallmark is sclerosis of the hippocampus. Since most of the cases are drug resistant, epilepsy surgery is the current gold standard therapy option for cessation of seizures. Familial forms of MTLE-HS have been recognized, but no causal gene has been identified so far.
カテゴリ  
神経系疾患
階層分類  
ICD-11 による疾患分類 [BR:jp08403]
 08 神経系の疾患
  てんかんまたは発作
   8A60  構造的または代謝性の病態または疾患によるてんかん
    H01826  海馬硬化を伴う内側側頭葉てんかん
指定難病 [jp08407.html]
 H01826
リンク   
ICD-11: 8A60.7
ICD-10: G40.2
MeSH: C566903
文献    
  著者
Jin SH, Jeong W, Chung CK
  タイトル
Mesial temporal lobe epilepsy with hippocampal sclerosis is a network disorder with altered cortical hubs.
  雑誌
Epilepsia 56:772-9 (2015)
DOI:10.1111/epi.12966
文献    
  著者
Cersosimo R, Flesler S, Bartuluchi M, Soprano AM, Pomata H, Caraballo R
  タイトル
Mesial temporal lobe epilepsy with hippocampal sclerosis: study of 42 children.
  雑誌
Seizure 20:131-7 (2011)
DOI:10.1016/j.seizure.2010.11.002
文献    
  著者
Yang T, Zhou D, Stefan H
  タイトル
Why mesial temporal lobe epilepsy with hippocampal sclerosis is progressive: uncontrolled inflammation drives disease progression?
  雑誌
J Neurol Sci 296:1-6 (2010)
DOI:10.1016/j.jns.2010.06.002
文献    
  著者
Tekturk P, Erdogan ET, Kurt A, Vanli-Yavuz EN, Ekizoglu E, Kocagoncu E, Kucuk Z, Aksu S, Bebek N, Yapici Z, Gurses C, Gokyigit A, Baykan B, Karamursel S
  タイトル
The effect of transcranial direct current stimulation on seizure frequency of patients with mesial temporal lobe epilepsy with hippocampal sclerosis.
  雑誌
Clin Neurol Neurosurg 149:27-32 (2016)
DOI:10.1016/j.clineuro.2016.07.014

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