Melanomatosis of the Skin and Central Nervous System in Infants

Wilcox, John C.

doi:10.1001/archpedi.1939.01990020149016

Cited by 31 publications

(10 citation statements)

References 4 publications

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“…In two patients (Bjornboe, 1934;Berblinger, 1915) there was an associated neurofibromatosis, and in one case Willis (1960) . 46 F + (Wilcox, 1939) the melanosis was associated with pial telangectasis. All the patients (with one exception) had numerous pigmented skin naevi.…”

Section: Discausionmentioning

confidence: 92%

“…The dura has been normal in all cases. Macroscopic pigmentation of the cerebral cortex has been seen in only 1 case (Wilcox, 1939), but in 7 cases (Berblinger, 1915: Farnell andGlobus, 1931;Lecouturier, Ley, Titeca, and van Bogaert, 1939;Maclachlan, 1914;Grahl, 1906;Russell, 1949;Jiitte, 1939) there was macroscopic pigmentation of the cerebellum and pons. The cerebellar pigmentation was in all cases most marked in the cortex, but striking pigmentation of the dentate nucleus on one or both sides was noted in 3 cases (Berblinger, 1915;Maclachlan, 1914: Lecouturier et al, 1939.…”

Section: Discausionmentioning

confidence: 99%

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Neuro-cutaneous Melanosis

Fox¹,

Emery²,

Goodbody³

et al. 1964

Archives of Disease in Childhood

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Combined congenital abnormalities of the skin and central nervous system are well recognized-such conditions as Sturge-Weber syndrome, tuberous sclerosis, and neurofibromatosis. A very much rarer and less well-known member of this group is neurocutaneous melanosis. posterior fossa decompression was performed, and it was noted at operation that the meninges overlying the cerebellum were thickened and pigmented. The underlying cerebellar cortex also appeared to be pigmented. After operation the child renained well for eight weeks, but it was then noted that he was ataxic and though he was talking well he was emitting a shrill high-pitched cry at frequent intervals. The posterior fossa decompression was tense and bulging, and a Pudenz valve was therefore inserted into the right lateral ventricle draining into the right internal jugular vein. The child then remained fairly well, apart from some degree of ataxia, for a further period of four months, when he was readmitted with increasing restlessness, ataxia, and rigidity. The posterior fossa decompression was again tense and bulging, and at operation the Pudenz valve was found to be blocked by a protein coagulum, and it was replaced. Three days after the operation the child's conscious level began to decline:he developed left-sided Jacksonian epileptiform attacks and, despite repeated ventricular taps, died at 25 months.NECROPSY. There were no abnormal pathological findings outside of the skin and the central nervous system. In particular, no evidence of tumour was found in any other organ. The skin showed numerous large pigmented hairy naevi on the skin of the limbs, chest, trunk, back, and scalp. These varied in size, but ranged up to a maximum diameter of 6 cm.The dura appeared normal and showed no evidence of pigmentation, but the pia-arachnoid showed a diffusely. grey coloration most marked over the right cerebral hemisphere. In addition there were numerous scattered FiG.

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Section: Discausionmentioning

confidence: 92%

Section: Discausionmentioning

confidence: 99%

Neuro-cutaneous Melanosis

Fox¹,

Emery²,

Goodbody³

et al. 1964

Archives of Disease in Childhood

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“…The dense perivascular cellular proliferations in the leptomeninges of the brain and spinal cord act as a barrier for complete résorption of the fluid. 82 However, a diagnosis of malignant melanoma of the brain is difficult since the preexisting melanocyte may appear anaplastic.67…”

Section: Distribution Of Nevusmentioning

confidence: 99%

Giant Pigmented Nevi, Melanoma, and Leptomeningeal Melanocytosis

Reed¹

1965

Arch Dermatol

323

114

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“…15 Support for this assertion may be found in the known association of neurocutaneous melanosis with other developmental anomalies of the neuraxis, such as hypoplasia of the cerebellar vermis, syringomyelia, intraspinal arachnoid cyst, intraspinal lipoma, pial telangiectasia, and Sturge-Weber syndrome. 17,20,23,32,34 If this hypothesis is correct, however, a more severe dysplastic manifestation could occur. 7 The incidence of occipital meningoceles in Dandy-Walker malformation is approximately 11 to 18%, 6 but the pathogenesis is not clear.…”

Section: Discussionmentioning

confidence: 99%

Neurocutaneous melanosis associated with Dandy—Walker malformation and a meningohydroencephalocele

Arai

Nosaka²,

Kashihara³

et al. 2004

Journal of Neurosurgery: Pediatrics

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Neurocutaneous melanosis and Dandy-Walker malformation are both forms of rare congenital neurodysplasia. Interestingly, 8 to 10% of patients with neurocutaneous melanosis also harbor an associated Dandy-Walker malformation, indicating that these developmental abnormalities share a common origin. The authors describe a case of neurocutaneous melanosis associated with Dandy-Walker malformation and an occipital meningohydroencephalocele with a giant melanotic nevus. Multiple congenital liver masses were also observed in the infant. The occipital nevus was totally excised, and ventriculoperitoneal and cyst-peritoneal shunts were created to prevent subsequent hydrocephalus. Findings in this case support the possibility that excessive melanocytes hinder normal mesenchymal development, causing Dandy-Walker malformation and an occipital meningocele.

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Melanomatosis of the Skin and Central Nervous System in Infants

Cited by 31 publications

References 4 publications

Neuro-cutaneous Melanosis

Neuro-cutaneous Melanosis

Giant Pigmented Nevi, Melanoma, and Leptomeningeal Melanocytosis

Neurocutaneous melanosis associated with Dandy—Walker malformation and a meningohydroencephalocele

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