choristoma/seizures

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Multiple choristomas, convulsions and mental retardation as a new neurocutaneous syndrome.

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Functional MRI characteristics of a focal region of cortical malformation not associated with seizure onset.

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Neuroimaging studies have demonstrated that heterotopic tissue of patients with "double cortex" is activated during motor and somatosensory tasks. Activation in patients with malformations of cortical development (MCD) has been variable, likely due to the heterogeneity of the disorder. We examined

Association of epibulbar choristomas with linear cutaneous nevi. Review of the literature.

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The authors describe the association of epibulbar choristomas with linear cutaneous nevi of the epidermal and sebaceous variety in 24 cases reported in the literature. The choristomas are of the bilateral, extensive, multiple and complex type. The associated ocular and systemic findings include lid

Complex limbal choristoma in linear nevus sebaceous syndrome managed with scleral grafting.

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Linear nevus sebaceous syndrome (LNSS) is characterized by nevus sebaceous, mental retardation, seizures, and ocular abnormalities such as complex limbal choistoma. A young male with history of mass in right eye and blackish discoloration of skin over right and left side of forehead since birth

Intradural extracerebral choristoma. Case report and review of the literature.

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BACKGROUND Intracranial extracerebral neuroglial choristoma is a very rare entity. A cleft palate or a nasopharyngeal teratoma has been described as accompanying choristoma, but there are no observations of both anomalies being simultaneously present and combined. METHODS An 8-month-old girl

Complex limbal choristomas in linear nevus sebaceous syndrome.

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OBJECTIVE This study aimed to describe the clinical and histopathologic findings in four patients with complex limbal choristomas associated with linear nevus sebaceous syndrome (LNSS), a rare disorder including nevus sebaceous, seizures, and mental retardation, and often accompanied by ocular

[Pachygyria and laminar heterotopic tissue. A rare case of unique expression of a neuronal migration disorder in twins].

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We report of 16 year old twinsisters with a neuronal migration disorder, twin I with mental retardation and focal epileptic seizures on MRI showed general pachygyria, laminar subcortical heterotopia and mildly dilated lateral ventricles, whereas twin II whose first symptom was a cerebral seizure

Complex choristoma of the gyrus rectus: a distinct clinicopathologic entity?

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We report 2 cases of an unusual central nervous system lesion involving the gyrus rectus and characterized by well-differentiated epithelial and mesenchymal components. One patient was a 30-year-old woman, and the other was a 42-year-old man. Both presented with seizures. Radiologically, the lesions

A Filipino male with encephalocraniocutaneous lipomatosis (Haberland's syndrome).

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BACKGROUND Encephalocraniocutaneous lipomatosis (ECCL), also known as Haberland's Syndrome, is a sporadically occurring neurocutaneous syndrome with no gender or race predilection. ECCL patients present with a broad spectrum of clinical manifestations, often in a unilateral distribution. The

Ectopic cerebellum in anterior cranial fossa: Report of a unique case associated with skull congenital malformations and epilepsy.

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An isolated, well-differentiated ectopic cerebellum arising outside the posterior fossa is extremely rare. We present a unique case of ectopic cerebellum in 25-year-old woman with hypertelorism, skull deformation, and a longstanding history of epileptic seizures. Magnetic resonance imaging revealed

Epileptogenic mineralization: pathological variants with good prognosis.

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Four examples of grossly mineralized lesions of the brain, causing seizures, are described; they include 2 hamartomas, a choristoma (novel associations), and an idiopathic "brain stone." Each was detectable with routine roentgenograms, and the seizures were ameliorated by surgical therapy.

Phenotypic and imaging features of FLNA-negative patients with bilateral periventricular nodular heterotopia and epilepsy.

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OBJECTIVE Periventricular nodular heterotopia (PVNH) is a malformation of cortical development due to impaired neuronal migration resulting in the formation of nodular masses of neurons and glial cells in close proximity to the ventricular walls. We report the clinical characteristics of the largest

Neonatal neoplasms.

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OBJECTIVE To describe neoplasms diagnosed in children

Hypothalamic hamartomas: A comprehensive review of the literature - Part 1: Neurobiological features, clinical presentations and advancements in diagnostic tools

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Hypothalamic hamartomas (HH) are rare, non-neoplastic heterotopic tissues which contains normal neurons and glia including oligodendrocytes and fibrillary astrocytes but in an abnormal distribution. They arise from the floor of the third ventricle, tuber cinereum, or mammillary bodies. Estimated

Hypothalamic Hamartomas: A Comprehensive Review of Literature - Part 3: Updates on Radiotherapy Management

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