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Isolated hemimegalencephaly (iHME) is a rare form of congenital malformation of cortical development characterized by enlargement of all or part of one cerebral hemisphere. It typically presents with intractable seizures, developmental delay, contralateral hemiparesis, and hemianopia. We report a
Hemimegalencephaly (HME) is a developmental brain lesion consisting of a unilateral enlarged, dysplastic, and often highly epileptogenic cerebral hemisphere. Most patients exhibit early onset intractable seizures, status epilepticus, hemiplegia, hemianopsia, and developmental delay. Major surgical
Brain single-photon emission computed tomography (SPECT) findings during clinical and subclinical seizures were compared in two neonates with hemimegalencephaly. Interictal and ictal brain SPECT were performed in two neonates. The ictal studies were performed during a clinical seizure in one neonate
A girl with Klippel-Trenaunay-Weber syndrome with partial motor seizures is reported. She had hemimegalencephaly and band heterotopia on MRI of the brain.
We report a 3-year-old boy with the neurocutaneous combination of unilateral alopecia, ipsilateral hemimegalencephaly, and intractable seizures. He was born with an asymmetric hair pattern consisting of absent patches of hair, a small left eyebrow, and less eyelashes on the left eye; he had normal
The epidermal nevus syndrome (ENS) is a sporadic neurocutaneous disorder that consists of epidermal nevi and congenital anomalies involving the brain and other systems. From among over 60 patients with ENS presenting with neurologic manifestations, we identified 17 who had hemimegalencephaly based
The timetable for normal myelination in the developing brain on MRI is well described. Beyond the genetically determined myelination process, environmental or other incidental factors may modulate the actual myelination, globally or regionally. Delayed myelination is well known in many pathological
OBJECTIVE
To explore seizure outcome after surgery for focal epilepsy due to malformation of cortical development (MCD), with focus on the role of MRI.
METHODS
Thirty-five patients who had surgery for intractable focal epilepsy due to MCD identified by preoperative MRI and confirmed by
Clinical and electroencephalographic (EEG) studies were performed in two children with hemimegalencephaly. The ages of seizure onset were 44 hours after birth in one infant and 33 days of postnatal life in the other patient. In both children, infantile spasms (IS) associated with hemihypsarrhythmia,
Three boys with hemimegalencephaly are reported. Two suffered neonatal convulsions and the third presented with seizures at seven months. In each case the EEG was grossly abnormal, with spike and wave activity. All three have significant developmental delay and demonstrate other manifestations of
Hemimegalencephaly is a rare congenital malformation characterised by overgrowth of one hemisphere. Although it is commonly thought to be associated with neurological deficits, developmental delay, and intractable epilepsy, the clinical expression of hemimegalencephaly, can vary widely. This patient