hemiconvulsion-hemiplegia-epilepsy syndrome

نویسندگان

m. r. ashrafi md,associate professor,department of pediatric neurology, children’s medical center,tehran university of medical sciences

j. tafarroji md, resident of pediatrics, children’s medical center,tehran university of medical sciences

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.

برای دانلود باید عضویت طلایی داشته باشید

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

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 کامل

Child 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 کامل

Hemiconvulsion-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 کامل

An 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 کامل

Brief 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 کامل

Stroke-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...

متن کامل

منابع من

با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید


عنوان ژورنال:
iranian journal of child neurology

جلد ۲، شماره ۴، صفحات ۵۵-۵۸

میزبانی شده توسط پلتفرم ابری doprax.com

copyright © 2015-2023