Abstract
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.
Highlights
Childhood absence epilepsy (CAE) is one of the most common forms of pediatric epilepsy, accounting for approximately 10% of epilepsies in children [1]
We present the case of an 11-year-old female with refractory childhood absence epilepsy who developed antiMOG antibody positive Optic neuritis (ON) and had subsequent improvement in her seizures after treatment with steroids and rituximab for ON
This is an interesting case of an 11-year-old female with a history of classic CAE who, after being seizure free for one year, had recurrence of refractory seizures and subsequent development of ON 10 months later. Her seizures were controlled, EEG normalized, and clobazam successfully weaned after the patient received pulse dose steroids and rituximab for ON. This case may indicate a relationship between ON and CAE or may suggest that there is an inflammatory component to CAE, or seizure exacerbation in CAE, and that immunomodulatory therapies may have a role in seizure control
Summary
Childhood absence epilepsy (CAE) is one of the most common forms of pediatric epilepsy, accounting for approximately 10% of epilepsies in children [1]. Epilepsy is generally thought of as a grey matter disease. It can less frequently be associated with abnormal white matter as well [3, 4]. We present the case of an 11-year-old female with refractory childhood absence epilepsy who developed antiMOG antibody positive ON and had subsequent improvement in her seizures after treatment with steroids and rituximab for ON. CAE and ON are not commonly comorbid and to our knowledge their co-occurrence in a patient has not been previously described. In this case, the Clinical Neurology and Neuroscience 2021; 5(2): 25-29.
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