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Year : 2022  |  Volume : 70  |  Issue : 2  |  Page : 788--789

Adult Hemimegalencephaly with Migraine as the First Symptom

Qian Liu, Wenjuan Zhao, Guanen Zhou 
 Department of Neurology, Tianjin Huanhu Hospital, 6 Jizhao Road, Jinnan District, Tianjin, 300060, China

Correspondence Address:
Dr. Guanen Zhou
Department of Neurology, Tianjin Huanhu Hospital, 6 Jizhao Road, Jinnan District, Tianjin 300060

How to cite this article:
Liu Q, Zhao W, Zhou G. Adult Hemimegalencephaly with Migraine as the First Symptom.Neurol India 2022;70:788-789

How to cite this URL:
Liu Q, Zhao W, Zhou G. Adult Hemimegalencephaly with Migraine as the First Symptom. Neurol India [serial online] 2022 [cited 2022 Aug 10 ];70:788-789
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Full Text


We report a case of hemimegalencephaly presenting with migraine as the first symptom. A 55-year-old right-handed female patient presented with complaints of recurrent episodes of the left focal headache for the past 2 years. The headache often intermittent attacks lasted for a few minutes with a frequency of 3–4 times per day. Besides, we found her mini-mental state examination score was 18/30 (the patient was illiterate), and Montreal cognitive assessment score was 19/30.

Electroencephalogram was abnormal. Magnetic resonance imaging (MRI) of the brain showed enlarged right hemisphere with smooth thickened cortex and pachygyria, indistinct gray/white differentiation. The patient suffered from intermittent headaches for 2 years, and the effect of medical treatment was poor, thus after finding the abnormality of the brain MRI, she was admitted to the neurosurgery department of another hospital for the stereotactic brain biopsy. Pathologic analysis of the right frontal lobe showed that GFAP(+), Olig2(+), IDH1(-), CD34(-), H3K27M(-), CD56(-), Neu-n(-), P53(-), ki67 labeling index was less than 1%. She was treated with oral carbamazepine 100 mg twice a day. During the follow-up period of one-and-a-half years, there was a decreased recurrence of migraines.


In this case, the patient presented with a contralateral headache rather than an ipsilateral headache. This condition has not been reported before. One study showed that nonaffected hemispheres were found to present cerebral hypoperfusion and hypometabolism, indicating that they might also have developed abnormalities in early development.[1] This may explain the clinical symptoms.

Although the pathological results of this patient showed that GFAP and Olig2 were positive, the clinical symptoms and imaging data were not consistent with glioma. However, It has been found that a patient with hemimegalencephaly treated with vagus nerve stimulation and subsequent glioblastoma development in the hemimegalencephalic hemisphere 6 years after surgery.[2] Jan Chrastina et al. thought that the genetics of hemimegalencephaly suggested a potentially increased risk of malignant glioma growth in the malformed brain.

Adult hemimegalencephaly is a rare condition. The relationship between hemimegalencephaly and brain tumor need our focus and further investigation.{Figure 1}


The authors declare that they have no competing interests.

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Conflicts of interest

There are no conflicts of interest.


1Uematsu M, Haginoya K, Togashi N, Hino-Fukuyo N, Nakayama T, Kikuchi A, et al. Unique discrepancy between cerebral blood flow and glucose metabolism in hemimegalencephaly Epilepsy Res 2010;92:201-8.
2Chrastina J, Novak Z, Brazdil M, Hermanova M. Glioblastoma multiforme in a patient with isolated hemimegalencephaly. J Neurol Surg Rep 2015;76:e160-3.