grey matter heterotopia<\/a>\u00a0within the tuber cinereum. It is usually a non-neoplastic congenital abnormality that can result in stereotypical seizures and behaviors.<\/p>\n <\/p>\n
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The classic clinical picture is of a male (slightly more frequent than in females) with precocious puberty and fits of uncontrolled energy accompanied by either laughter or crying – the so-called gelastic seizures. A gelastic seizure can often be followed by other types of seizures including atonic (drop-seizures), simple partial, or complex partial seizures. Gelastic seizures are difficult to control completely with anti-epileptic drugs but the medications used for partial complex epilepsy can often reduce seizure frequency.<\/p>\n
About half the cases of hypothalamic hemartoma are associated with Pallister-Hall Syndrome. Pallister-Hall results from an autosomal dominant mutation of the gene-regulator GL13. In addition to hypothalamic hemartoma, children with Pallister-Hall can have polydactyly, cutaneous syndactyly, bifid epiglottis, kidney abnormalities and an imperforate anus.<\/p>\n","protected":false},"excerpt":{"rendered":"
The MR images show an abnormal growth hanging down from the hypothalamus. This is a hypothalamic hemartoma, which in some cases can be a type of grey matter heterotopia\u00a0within the tuber cinereum. It is usually a non-neoplastic congenital abnormality that can result in stereotypical seizures and behaviors.<\/p>\n","protected":false},"author":2,"featured_media":365,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"om_disable_all_campaigns":false,"_monsterinsights_skip_tracking":false,"_monsterinsights_sitenote_active":false,"_monsterinsights_sitenote_note":"","_monsterinsights_sitenote_category":0,"footnotes":""},"categories":[14],"tags":[119,118,120,117,121],"aioseo_notices":[],"yoast_head":"\n
Hypothalamic Hemartoma and Gelastic Seizures - DocNeuro<\/title>\n \n \n \n \n \n \n \n \n \n \n \n \n \n\t \n\t \n\t \n \n \n \n \n \n\t \n\t \n\t \n