抗mog相关性视神经炎前难治性癫痫缺失1例

Katharina Blunschi, P. Avasarala, J. Schreiber, Neha Athale, I. Kahn, T. Lateef
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摘要

儿童期缺失性癫痫(CAE)是儿童癫痫最常见的形式之一。虽然大多数患者在抗癫痫药物治疗后无癫痫发作,但约20%的患者未达到癫痫发作缓解,并被定义为难治性CAE。癫痫通常被认为是一种灰质疾病,但也与白质异常有关。另一方面,视神经炎(ON)是一种典型的白质疾病,其特征是由自身抗体引起的髓鞘炎症和脱髓鞘,如抗髓鞘少突胶质细胞糖蛋白(MOG)抗体。我们提出的情况下,11岁的女性难治性CAE谁发展抗mog抗体阳性ON。CAE和ON通常不是合并症,据我们所知,它们在患者中的合并症以前没有被描述过。然而,在本例中,CAE和ON可能相关,因为她的癫痫发作在对ON进行免疫调节治疗后显着改善。这可能表明ON(可能针对抗mog阳性ON)和CAE之间的关系,或者可能表明CAE存在炎症成分,免疫调节疗法可能在癫痫发作控制中起作用。因此,在治疗难治性癫痫缺席的情况下,免疫工作可能是有帮助的。
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A Case of Refractory Absence Epilepsy Precedes Anti-MOG Associated Optic Neuritis
Childhood absence epilepsy (CAE) is one of the most common forms of pediatric epilepsy. While most patients become seizure free with anti-epileptic drug therapy approximately 20% do not achieve seizure remission and are defined as having refractory CAE. Epilepsy is generally thought of as a grey matter disease but has also been associated with abnormal white matter. Optic neuritis (ON), on the other hand, is typically a white matter disorder characterized by inflammation and demyelination of the myelin sheath due to autoantibodies, such as the anti-myelin oligodendrocyte glycoprotein (MOG) antibody. We present the case of an 11-year-old female with refractory CAE who developed anti-MOG antibody positive ON. CAE and ON are not commonly co-morbid and to our knowledge their co-occurrence in a patient has not been previously described. However, in this case, the CAE and ON may be related as her seizures dramatically improved after initiating immunomodulatory treatments for the ON. This may indicate a relationship between ON (potentially specific to anti-MOG positive ON) and CAE, or may suggest that there is an inflammatory component to CAE and that immunomodulatory therapies may have a role in seizure control. Thus, in cases of treatment resistant absence epilepsy, an immune work up may be helpful.
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