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Neurocutaneous melanosis (NCM) is a rare neuroectodermal dysplasia that includes both central nervous system (CNS) and integumentary melanocytic abnormalities. NCM can present with varied clinical and imaging findings, classically presenting with large melanocytic nevi involving the posterior axial
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Neurocutaneous melanosis is a rare congenital syndrome characterized by the presence of large or multiple congenital melanotic nevi and benign or malignant pigment cell tumors of the leptomeninges. A 2-month-old female infant was referred to our department with widespread pigmented nevi, numerous
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History A 3-month-old boy presented with new onset of seizure that subsided when he arrived at our institution. There was no reported fever or family history of seizure. Physical examination did not reveal any neurologic abnormalities. Multiple skin lesions of varying sizes were identified on the
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A young boy presented with mental retardation and seizures with extensive hairy naevi. After a fluctuating clinical course over the next 2 years, he worsened rapidly with signs of rising intracranial pressure. A CT scan revealed unsuspected features of Dandy-Walker syndrome. The clinical course is
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BACKGROUND
Patients with giant congenital melanocytic nevi (GCMN) have increased risk of neurocutaneous melanosis (NCM). Brain MRI is mandatory in GCMN.
OBJECTIVE
To present the age related changes in brain MRI in children with NCM during several-year follow-up.
METHODS
In 7 NCM children following
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Melasma is a common disorder of hyperpigmentation that is present most commonly in reproductive age women with brown or black skin colors. The lesions of melasma are characteristically distributed in a symmetric manner on the sun-exposed areas of the face and neck. The precise pathogenesis of the
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The authors present 2 months old child with giant congenital melanocytic naevus of thoraco-lumbosacralis region and numerous congenital pigmented nevi disseminated on the skin of the whole body, slight right hemiparesis and epileptic seizures. Magnetic resonance imaging (MRI) of the head performed
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Melasma is a common disorder in women of reproductive age with darker skin tones, but may also affect adolescents, older women on certain medications, and sometimes men. It usually appears as hyperpigmented macules and patches distributed symmetrically on the face, neck and rarely the upper limbs.
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Neurocutaneous melanosis is a rare, congenital, non-inherited syndrome characterized by numerous and/or large congenital nevi with intracranial leptomeningeal melanocytosis. This report describes two patients, presenting with a giant congenital nevus involving a major portion of the posterior trunk
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Neurocutaneous melanosis (NCM) is rare and is characterized by the proliferation of melanocytes in the central nervous system. A 6-day-old infant boy was referred to our department with giant congenital melanocytic nevi and convulsions. On physical examination the patient had a giant black-brown
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Six patients with congenital giant or multiple melanocytic nevi are studied retrospectively. The clinical, electroencephalographic and neuro-imaging findings for the four patients with neurocutaneous melanosis are described. The other two patients had only cutaneous melanosis. Three of the four with
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OBJECTIVE: Describe the morbidity associated with a rare disease due to an embryological defect. METHODS: Retrospective revision of medical and necropsy reports. Bibliographic research using MEDLINE, LILACS and Index Medicus databases. RESULTS: 1 year-old male patient, admitted with generalized
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A newborn baby boy presented with giant melanocytic nevi on the face, trunk and extremities, and focal cortical dysplasia on MRI. At 3 months of age, he developed intractable epilepsy, and MRI at 2 years of age revealed a high-intensity area in the bilateral cerebellum on T1-weighted images,
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Neurocutaneous melanosis is congenital melanocytic nevus with neurological manifestations. We report a 4-year-old female child presenting with hyperpigmented and nodular skin lesion associated with developmental delay and convulsions. The child had multiple brownish-black nevi on the face and chest
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BACKGROUND
Neurocutaneous melanosis is an infrequent condition characterized by the presence of numerous gigantic cutaneous naevi and melanocytic infiltration of the central nervous system and/or the leptomeningeal layers. Different clinical features may be seen: endocranial hypertension due to
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