holoprosencephaly/crise épileptique

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Epileptic seizures and structural abnormalities in a patient with holoprosencephaly.

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In a patient with holoprosencephaly, partial seizures had various initial ictal symptoms, and ictal EEGs showed epileptogenic foci in the right and left brain. Partial seizures did not culminate in secondary generalized tonic-clonic convulsions. Characteristic malformed structures contribute to the

Gelastic seizure with tectal tumor, lobar holoprosencephaly, and subependymal nodules: clinical report.

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Gelastic seizures are characterized by inappropriate, stereotyped laughter and are often first recognized when other epileptic manifestations occur. They are frequently associated with hypothalamic hamartomas. Central nervous system developmental abnormalities are rarely reported with gelastic

[Lobar holoprosencephaly associated with heterotopic gray matter. A case disclosed in adulthood by epilepsy].

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A case of lobar holoprosencephaly associated with heterotopias of the grey matter is reported. The patient was a young woman whose cerebral malformation was revealed by epileptic seizures at the age of 22. There was no intellectual deficit. Computerized tomography and, chiefly, magnetic resonance

Clinical features and outcomes of holoprosencephaly in Korea.

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The clinical spectrum of holoprosencephaly is broad, and its etiology is heterogeneous. To investigate the clinical spectrum of holoprosencephaly in Korea, we performed a database analysis of 55 cases of holoprosencephaly, including 12 diagnosed postnatally, all from a single institution. The 55

Holoprosencephaly in the west of Scotland 1975-1994.

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Cases of holoprosencephaly which occurred in the west of Scotland over the past 20 years were ascertained from genetics, paediatric, and pathology department records. Fifty cases were identified of which 17 had an underlying cytogenetic abnormality. Of the remaining 33 cases, 26 were delivered after

Holoprosencephaly with neurogenic hypernatremia: a new case.

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BACKGROUND Holoprosencephaly (HPE) is the most common developmental defect of the forebrain and mid-face in humans. It is a disorder of neural induction in which a genetic programming defect results in noncleavage of the forebrain in the sagittal plane and variable hypoplasia of paramedian

Mediobasal and mantle defect of the prosencephalon: lobar holoprosencephaly, schizencephaly and diabetes insipidus.

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An infant is described who had a combination of lobar holoprosencephaly and open-lip schizencephaly. Midline fusion of the basal ganglia was associated with bilateral absence of abundant parts of the brain mantle. Agenesis of the corpus callosum, hypoplasia of the optic nerves and chiasm, absence of

Middle interhemispheric variant of holoprosencephaly: a distinct cliniconeuroradiologic subtype.

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BACKGROUND The middle interhemispheric variant (MIH) is a subtype of holoprosencephaly (HPE) in which the posterior frontal and parietal areas lack midline separation, whereas more polar areas of the cerebrum are fully cleaved. While the neuroradiologic features of this subtype have been recently

The evolution of neurophysiological features in holoprosencephaly.

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The evolution of EEG, visual and auditory evoked responses (VER and AER) and sleep is described in three cases of semilobar holoprosencephaly. During the neonatal period, the waking EEG was characterized by almost continuous high amplitude rhythmic alpha-theta activity in case 1 and 2, which became

[Clinical spectrum and management of holoprosencephaly].

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To study the phenotypic spectrum and management of holoprosencephaly (HPE), we reviewed the findings of eight children with HPE from 3 to 10 years of age, who underwent intervention programs and rehabilitation at our center. One patient had alobar HPE, three semilobar HPE, and four lobar HPE. All

Electroencephalography in holoprosencephaly: findings in children without epilepsy.

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OBJECTIVE To evaluate the electroencephalographic characteristics of patients with holoprosencephaly (HPE) without epilepsy. METHODS We evaluated the electroencephalograms (EEGs) of 18 children with HPE who lacked a history of seizures. Neuroimaging studies were assessed for severity of HPE and

[Perioperative considerations for a holoprosencephaly patient].

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We had twelve anesthetic experiences of seven holoprosencephaly patients for the past thirteen years. We classified these seven patients and compared the difficulty in perioperative control in the patients. Seven patients were divided according to two different holoprosencephaly classifications, one

Middle interhemispheric variant of holoprosencephaly in an asymptomatic adult.

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Middle interhemispheric variant of holoprosencephaly is an uncommon subtype of holoprosencephaly which is characterized by a midline connection of the two cerebral hemispheres in the posterior frontal and parietal regions with the separation of the anterior frontal and occipital lobes. It usually

Middle interhemispheric variant of holoprosencephaly associated with diffuse polymicrogyria.

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An 11-month-old boy was discovered to have a cleft palate, club foot, hypospadias, and myoclonic seizures. No in utero exposure to teratogens was identified. Brain MR imaging revealed a middle interhemispheric fusion variant of holoprosencephaly, diffuse polymicrogyria, and a hypoplastic brain stem;

Hypodipsia-hypernatremia syndrome associated with holoprosencephaly in a child: a case report.

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We report a child with diabetes insipidus and hypodipsia associated with holoprosencephaly. A two-year-old girl with the history of several admittances to hospital during and after the newborn period with hypernatremic dehydration, acute renal failure and convulsions is presented. The patient had

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