A rare case of hypomelanosis of Ito with enlargement of Virchow-Rabin spaces in a 10-year-old child with symptoms of seizures.

Shamim Shafieyoon, Ghazaleh Jamalipour Soufi, Farzaneh Hekmatnia, Andrew Parviz Zarei, Ali Hekmatnia, Zahra Mohajeri
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Abstract

Hypomelanosis of Ito (HI) syndrome is a complex neuro-dermatological disorder that affects many organs in the body, including the skin, brain, eyes, and skeleton. This disease has been reported to present with seizures in a few rare cases. Seizures are seen in all age groups but are more common in children and the elderly. Virchow-Robin spaces (VRSs) are spaces around small arteries and the arteries that pierce the surface of the brain and are spread throughout the rest of the brain. As individuals age, the number and size of VRSs increase. A relationship between dilated VRSs and neuropsychiatric disorders has been observed above a 2 mm threshold. The patient is a 10-year-old child who was referred to the neurology ward of Imam Hossein Children's Hospital in Isfahan about 2.5 months ago due to seizures. The last seizure occurred four days before the visit, and the patient was sent for a brain computed tomography (CT) scan, which revealed diffuse bilateral hypopigmented lesions in the brain's white matter. The results of the para-clinical tests were relatively unremarkable. In the early stages of hospitalization, the child received treatment such as fluid therapy and anticonvulsant drugs to stabilize their vital condition. The patient's para-clinical tests, including brain CT, electroencephalogram, complete blood count, liver function test, and magnetic resonance imaging, showed the presence of HI syndrome and bilateral diffuse hypopigmented lesions in the white matter.

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罕见的伊藤低黑素症伴Virchow-Rabin间隙增大的10岁儿童,有癫痫症状。
伊藤黑素减退症(HI)综合征是一种复杂的神经皮肤疾病,会影响身体的许多器官,包括皮肤、大脑、眼睛和骨骼。据报道,这种疾病在少数罕见病例中表现为癫痫发作。癫痫发作见于所有年龄组,但在儿童和老年人中更为常见。Virchow-Robin间隙(VRSs)是小动脉和动脉周围的间隙,这些动脉穿过大脑表面并扩散到大脑的其余部分。随着个体年龄的增长,vrs的数量和规模也在增加。在2毫米以上的阈值上观察到vrs扩张与神经精神疾病之间的关系。患者是一名10岁儿童,约2.5个月前因癫痫被转诊到伊斯法罕伊玛目侯赛因儿童医院的神经内科病房。最后一次癫痫发作发生在就诊前4天,患者被送去做脑部计算机断层扫描(CT),结果显示双侧大脑白质弥漫性低色素病变。准临床试验的结果相对不显著。在住院初期,儿童接受了液体疗法和抗惊厥药物等治疗,以稳定其生命状况。患者的临床旁检查,包括脑CT、脑电图、全血细胞计数、肝功能检查和磁共振成像,均显示HI综合征和双侧弥漫性白质低色素病变。
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