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hemimegalencephaly/спазм
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Infantile spasms associated with hemihypsarrhythmia and hemimegalencephaly.
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A case of unilateral megalencephaly in a four-week-old child with infantile spasms and hemihypsarrhythmia is presented. The histological and histometrical investigations showed signs of immaturity in the cortex of the megalencephalic hemisphere.
Surgery for the treatment of medically intractable infantile spasms: a cautionary case.
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The most appropriate time to consider cortical resection to treat medically intractable infantile spasms has not been clearly defined. The risks that need to be reconciled to make this decision are: What is the risk of loss of developmental potential if surgery is delayed too long versus what is the
[Clinical and electroencephalographic studies in children with hemimegalencephaly].
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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,
Posterior quadrantic dysplasia or hemi-hemimegalencephaly: a characteristic brain malformation.
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BACKGROUND
Posterior quadrantic dysplasia (PQD), a developmental malformation involving the temporal, parietal, and occipital lobes of one cerebral hemisphere, leads to intractable epilepsy.
OBJECTIVE
To characterize the clinical features of 19 patients with PQD and analyze the postsurgical outcome
Hypomelanosis of Ito associated with hemimegalencephaly: a clinicopathological study.
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A girl with hypomelanosis of Ito was studied both clinically and at postmortem examination. She manifested severe epilepsy early after birth. Magnetic resonance imaging demonstrated left-sided hemimegalencephaly. The seizures were secondarily generalized or unilateral initially, followed by
Prenatal diagnosis of hemimegalencephaly.
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BACKGROUND
In recent literature, there have been case reports of prenatal diagnosis of hemimegalencephaly, an extremely rare entity characterized by enlargement of all or portions of 1 cerebral hemisphere and intractable seizures. A unique case is presented of hemimegalencephaly of a fetus diagnosed
Longitudinal MR findings in a patient with hemimegalencephaly associated with tuberous sclerosis.
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A patient with an unusual association between tuberous sclerosis complex and hemimegalencephaly is reported. At 2 days of age, CT scan disclosed right hemimegalencephaly with periventricular faint calcification. At 1 month of age, magnetic resonance imaging showed right hemimegalencephaly with T1
Morphological substrates of infantile spasms: studies based on surgically resected cerebral tissue.
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Extensive surgical resections of neocortical cerebral tissue (including hemispherectomies) from 13 infants and children with infantile spasms showed that 12 of 13 specimens contained either malformative and dysplastic lesions of the cortex and white matter (sometimes with associated hamartomatous
Serial IMP-SPECT and EEG studies in an infant with hemimegalencephaly.
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An 8-month-old girl of hypomelanosis of Ito associated with hemimegalencephaly had frequent seizures beginning 44 h after birth. The seizures were secondarily generalized or unilateral initially, followed by infantile spasms at about 1.5 months of age. Frequent partial seizures appeared at 4 months
Clinical spectrum of epileptic spasms associated with cortical malformation.
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The spectrum of clinico-electrical characteristics of epileptic spasms associated with cortical malformation was studied in detail. The subjects were 15 patients suffering from spasms and cortical malformation demonstrated by MRI. The types of cortical malformation causing spasms were various,
Electroencephalographic aspects of hemimegalencephaly.
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The authors retrospectively studied the electroencephalograms (EEGs) of 12 patients with hemimegalencephaly-a unilateral brain malformation which is often overlooked, despite having typical CT-scan and MRI aspects. They were characterized by three types of abnormal tracing, which correlated with
Focal dysplasia of the cerebral cortex and infantile spasms associated with somatic 1q21.1-q44 duplication including the AKT3 gene.
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Somatic and germline duplications or activating mutations of AKT3 have been reported in patients with hemimegalencephaly and megalencephaly. We performed array comparative genomic hybridization on brain tissue and blood in 16 consecutive patients with symptomatic epilepsy due to focal or multilobar
[Hypomelanosis of Ito associated with hemimegalencephaly].
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A girl aged 1 year and 2 months with hypomelanosis of Ito was reported. She suffered from intractable epileptic seizures since the second day after birth. Characteristic bizarre hypopigmented skin lesions were seen on her left shoulder and extended in a linear fashion to the flexion side of the left
Hemimegalencephaly with polymicrogyria - a case report.
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Hemimegalencephaly on magnetic resonance imaging scan (MRI) consists of cortical gray matter almost uniformly abnormal, areas of increased thickness of the cortical gray matter (GM), abnormal gyral patterns, blurring of the grey-white matter transition, atrophy or hemispheric hypertrophy,
Epileptic negative myoclonus in a newborn with hemimegalencephaly.
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OBJECTIVE
We report the case of a male newborn with Ohtahara syndrome and right hemimegalencephaly who presented epileptic negative myoclonus in the first days of life.
METHODS
Prolonged polygraphic studies were performed, as well as MRI and a full clinical examination.
RESULTS
EEG showed a constant
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