Neurocutaneous melanosis with hydrocephalus and dandy-walker variant

Sarwar, Mohammad; Tripathy, Laxminarayan; Jain, Harsh; Basu, Sunandan; Sengupta, Ashvini

doi:10.4103/ajns.ajns_542_20

Cited by 2 publications

(1 citation statement)

References 17 publications

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“…We have a male newborn at 36 weeks gestation obtained by cesarean section, with a history in the current pregnancy of poor prenatal control. During The prognosis of NCM is dismal, especially when presented in adults and associated with DWS [7]. associated with ganglioglioma [8]; with Associated Neurofibromatosis [9]; Refractory psychiatric symptoms and seizure [10]; Hemorrhagic Stroke in a Young Adult [11].…”

Section: Clinical Casementioning

confidence: 99%

Hydrocephalus and Dandy-Walker syndrome. Clinical case

Villalobos-Rodríguez

2023

AJMCRR

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Introduction. Dandy-Walker syndrome (DWS) is a developmental abnormality characterized by dilated posterior fossa, cystic enlargement of the fourth ventricle, hypoplasia of cerebellar vermis, and its upward rotation. The affected person may suffer from psychomotor retardation, ataxia, apnea attacks, muscle weakness, occasional muscle spasm, seizures, nystagmus, and macrocephaly. However, half of the cases have average intelligence. Clinical Case. Male newborn at 36 weeks gestation obtained by cesarean section, with a history in the current pregnancy of poor prenatal control. During the fifth month of pregnancy, an ultrasound established a diagnostic suspicion of massive ventriculomegaly. At the sixth month, thinning of the cerebral cortex compression of the cerebellum in the posterior fossa was detected, and a possible ruling out of chromosomopathy related to Dandy-Walker Syndrome was suggested. Discussion. Neurologically, this disease causes multiple anomalies, such as Hemorrhagic Stroke in a Young Adult with Undiagnosed Asymptomatic Dandy–Walker Malformation (5). The embryonic development of the cerebellum and fourth ventricle causes the tentorium to be displaced upwards, and the posterior fossa enlarges. The association of hydrocephalus with DWS is due to blockage of normal cerebrospinal flow, leading to an excessive amount of fluid accumulating in and around the brain and causing an increase in intracranial pressure and head circumference, which ultimately causes neurological impairment. Conclusions. The prognosis of patients with this syndrome depends on the association with intra/extracranial malformations. Early diagnosis of central nervous system abnormalities is considered one of the first objectives of obstetric ultrasound.

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Section: Clinical Casementioning

confidence: 99%

Hydrocephalus and Dandy-Walker syndrome. Clinical case

Villalobos-Rodríguez

2023

AJMCRR

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A Rare Case of Nevus Spilus Type of Giant Congenital Melanocytic Nevus with Classic Proliferative Nodules, Intraparenchymal Melanosis, and Seizures

Das,

Vasudevan,

Kashif

et al. 2024

Indian Journal of Paediatric Dermatology

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Congenital melanocytic nevi, as the name suggests, are present since birth. They are called giant congenital melanocytic nevus (GCMN) when the size exceeds >20 cm in diameter. Due to its unsightly appearance, there is major psychosocial impact on the patient and the family. GCMN in due course of time may give rise to malignant melanoma not only in the GCMN but also in the leptomeninges. Clinical variants of GCMN include nevus spilus type, which is a rare variant. In addition to that with time, GCMN may continue to be affected with benign nodules. We present a rare case of a nevus spilus type of GCMN with classic proliferative nodules associated with intraparenchymal melanosis in brain, leading to seizures.

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Neurocutaneous melanosis with hydrocephalus and dandy-walker variant

Cited by 2 publications

References 17 publications

Hydrocephalus and Dandy-Walker syndrome. Clinical case

Hydrocephalus and Dandy-Walker syndrome. Clinical case

A Rare Case of Nevus Spilus Type of Giant Congenital Melanocytic Nevus with Classic Proliferative Nodules, Intraparenchymal Melanosis, and Seizures

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