hemiconvulsion-hemiplegia-epilepsy syndrome
نویسندگان
چکیده
objective hemiconvulsion-hemiplegia-epilepsy syndrome (hhe) is an initial phase of unilateral or predominantly unilateral convulsive seizures usually of long duration, with a second phase of hemiplegia (usually permanent), immediately following the hemiconvulsions;and then a third stage, characterized by the appearance of partial epileptic seizures. the causes of the initial convulsions in hhe syndrome are multiple but in many patients no cause in obvious. neuroimaging studies demonstrate an acquired atrophy, that often is preceded by swelling and edema of the hemisphere. here we report a case of 18 monthold boy, with an initial phase of repeated episodes of partial seizures, followed by hemiparesis and epileptic seizures, with one episode of generalized convulsive status epilepticus . minor head trauma was the trigger factor of the initial attack. brain neuroimaging showed generalized atrophy , that was more prominent in the left hemisphere.
منابع مشابه
Hemiconvulsion-hemiplegia-epilepsy syndrome: current understandings.
Hemiconvulsion-Hemiplegia (HH) syndrome is an uncommon consequence of prolonged focal febrile convulsive seizures in infancy and early childhood. It is characterized by the occurrence of prolonged clonic seizures with unilateral predominance occurring in a child and followed by the development of hemiplegia. Neuroradiological studies showed unilateral edematous swelling of the epileptic hemisph...
متن کاملChild neurology: hemiconvulsion-hemiplegia-epilepsy syndrome.
Hemiconvulsion-hemiplegia-epilepsy (HHE) syndrome is an uncommon outcome of prolonged focal status epilepticus in childhood. The prolonged focal motor seizure usually occurs during the course of a febrile illness and is followed by hemiplegia ipsilateral to the side of convulsions. This is accompanied by radiologic evidence of acute cytotoxic edema in the affected hemisphere followed by chronic...
متن کاملHemiconvulsion-hemiplegia-epilepsy syndrome: clinical course and neuroradiological features in a 20-month-old girl.
Hemiconvulsion-hemiplegia-epilepsy (HHE) syndrome involves initial sudden and prolonged unilateral convulsive seizures, followed by transient or permanent hemiplegia and epilepsy during infancy or early childhood. Seizures are prolonged, difficult to control and sometimes may require surgery. Hemiplegia varies in intensity, differs from Todd paralysis and disappears in about 20% of cases. Neuro...
متن کاملAn autopsy case of hemiconvulsion-hemiplegia-epilepsy syndrome manifesting as cerebral hemiatrophy in an elderly man.
We report an autopsy case of hemiconvulsion-hemiplegia-epilepsy (HHE) syndrome in a 79-year-old man. HHE syndrome usually occurs in children younger than 4 years of age. Although most HHE syndrome patients live into adult life, only a few cases of the syndrome have been reported in the elderly. In our case, cerebral hemiatrophy, left mesial temporal sclerosis and crossed cerebellar atrophy were...
متن کاملBrief Communication 16p13.11 Microdeletion in a Patient With Hemiconvulsion-Hemiplegia-Epilepsy Syndrome: A Case Report
We describe a patient with hemiconvulsion-hemiplegia-epilepsy syndrome. The pathophysiology of hemiconvulsion-hemiplegiaepilepsy syndrome remains uncertain and there are probably multiple potential contributing factors. Our patient had a chromosomal 16p13.11 microdeletion that confers susceptibility to various types of epilepsy. This is the first report detailing an association of hemiconvulsio...
متن کاملStroke-Like Presentation Following Febrile Seizure in a Patient with 1q43q44 Deletion Syndrome
Hemiconvulsion-hemiplegia-epilepsy syndrome (HHE) is a rare outcome of prolonged hemiconvulsion that is followed by diffuse unilateral hemispheric edema, hemiplegia, and ultimately hemiatrophy of the affected hemisphere and epilepsy. Here, we describe the case of a 3-year-old male with a 1;3 translocation leading to a terminal 1q43q44 deletion and a terminal 3p26.1p26.3 duplication that develop...
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عنوان ژورنال:
iranian journal of child neurologyجلد ۲، شماره ۴، صفحات ۵۵-۵۸
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