Neurocutaneous melanosis with hydrocephalus and dandy-walker variant

Neurocutaneous melanosis (NCM) is one of the rare, congenital, noninheritable phakomatoses characterized by the presence of large and/or multiple congenital melanocytic cutaneous nevi associated with intracranial leptomeningeal melanocytosis. NCM usually presents before 2 years of age. So far 302 cases have been reported in literature. We report a case of NCM presenting with obstructive hydrocephalus and Dandy-Walker Variant in a young adult.

Key-words:

Dandy-Walker variant - hydrocephalus - neurocutaneous melanosis - tuberculous meningitis

Financial support and sponsorship

Nil.

Publikationsverlauf

Eingereicht: 12. Dezember 2020

Angenommen: 30. Mai 2021

Artikel online veröffentlicht:
16. August 2022

• References

• 1 Barkovich AJ, Frieden IJ, Williams ML. MR of neurocutaneous melanosis. AJNR Am J Neuroradiol 1994;15:859-67.
• 2 Di Rocco F, Sabatino G, Koutzoglou M, Battaglia D, Caldarelli M, Tamburrini G. Neurocutaneous melanosis. Childs Nerv Syst 2004;20:23-8.
• 3 Ruggieri M, Polizzi A, Catanzaro S, Bianco ML, Praticò AD, Di Rocco C. Neurocutaneous melanocytosis (melanosis). Childs Nerv Syst. 2020 Oct;36(10):2571-2596. doi: 10.1007/s00381-020-04770-9. Epub 2020 Oct 13. PMID: 33048248.
• 4 Poe LB, Roitberg D, Galyon DD. Neurocutaneous melanosis presenting as an intradural mass of the cervical canal: Magnetic resonance features and the presence of melanin as a clue to diagnosis: Case report. Neurosurgery 1994;35:741-3.
• 5 Kalayci M, Cağavi F, Bayar U, Gül S, Dursun A, Ermis B, et al. Neurocutaneous melanosis associated with Dandy-Walker malformation. Acta Neurochir (Wien) 2006;148:1103-6.
• 6 Zhang W, Miao J, Li Q, Liu R, Li Z. Neurocutaneous melanosis in an adult patient with diffuse leptomeningeal melanosis and a rapidly deteriorating course: Case report and review of the literature. Clin Neurol Neurosurg 2008;110:609-13.
• 7 Arai M, Nosaka K, Kashihara K, Kaizaki Y. Neurocutaneous melanosis associated with Dandy-Walker malformation and a meningohydroencephalocele. Case report. J Neurosurg 2004;100:501-5.
• 8 Chaloupka JC, Wolf RJ, Varma PK. Neurocutaneous melanosis with the Dandy-Walker malformation: A possible rare pathoetiologic association. Neuroradiology 1996;38:486-9.
• 9 Kadonaga JN, Barkovich AJ, Edwards MS, Frieden IJ. Neurocutaneous melanosis in association with the Dandy-Walker complex. Pediatr Dermatol 1992;9:37-43.
• 10 Cho IY, Hwang SK, Kim SH. Dandy-walker malformation associated with neurocutaneous melanosis. J Korean Neurosurg Soc 2011;50:475-7.
• 11 Gowda VK, Basude A, Srinivas SM, Bhat M. Giant melanocytic nevi with neurocutaneous melanosis masquerading as neurofibromas. J Pediatr Neurosci 2016;11:258-60.
• 12 Fox H. Neurocutaneous melanosis. In: Vinken PJ, Bruyn GW, editors. Handbook of Clinical Neurology. Vol. 14. New York: Elsevier; 1972. p. 414-28.
• 13 Kadonaga JN, Frieden IJ. Neurocutaneous melanosis: Definition and review of the literature. J Am Acad Dermatol 1991;24:747-55.
• 14 Faillace WJ, Okawara SH, McDonald JV. Neurocutaneous melanosis with extensive intracerebral and spinal cord involvement. Report of two cases. J Neurosurg 1984;61:782-5.
• 15 Chander A, Shrestha CD. Cerebrospinal fluid adenosine deaminase levels as a diagnostic marker in tuberculous meningitis in adult Nepalese patients. Asian Pac J Trop Dis 2013;3:16-9.
• 16 Tanaka Y, Satomi K. Cryptococcal meningitis associated with increased adenosine deaminase in the cerebrospinal fluid. Springerplus 2016;5:2093.
• 17 Nakae Y, Kuroiwa Y. A case of listeria meningitis showed high levels of adenosine deaminase in cerebrospinal fluid. Rinsho Shinkeigaku 2009;49:590-3.
• 18 Garcia-Moncó C, Berciano J. Sarcoid meningitis, high adenosine deaminase levels in CSF and results of cranial irradiation. J Neurol Neurosurg Psychiatry 1988;51:1594-6.
• 19 Pettersson T, Klockars M, Weber TH, Somer H. Diagnostic value of cerebrospinal fluid adenosine deaminase determination. Scand J Infect Dis 1991;23:1, 93-100.