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Neurology India, Vol. 52, No. 1, January-March, 2004, pp. 127-128 Letter To Editor Neurocutaneous melanosis: Authors reply Karande S, Kulkarni MV Division of Pediatric Neurology, Department of Pediatrics, L.T.M. Medical College and L.T.M.G. Hospital, Sion, Mumbai - 400022 Code Number: ni04040 Sir, We thank the author(s) of the letter for taking interest in our article "Multiple giant congenital melanocytic nevi with central nervous system melanosis: a case report".[1] We would like to reply to the three points raised as follows. Firstly, the diagnosis of neurocutaneous melanosis in our patient is ′definite′ and is based on guidelines mentioned in ′recent literature′.[2],[3] The diagnosis of multiple giant congenital melanocytic nevi (GCMN) is straightforward, a spot diagnosis, and doesn′t require a skin biopsy. On examining our patient, the same clinical diagnosis of "multiple GCMN" was made by a senior dermatologist and plastic surgeon in our institute. Although the neonate was neurologically normal, the presence of GCMN on scalp and dorsal spine prompted us to recommend a MRI brain study to screen for central nervous system (CNS) melanosis and operable presymptomatic non-melanocytic anomalies of the CNS.[2] Eventually, MRI in our patient could be done at six weeks of age, as the parents took time to arrange money for this investigation. The diagnosis of CNS melanosis was made in our patient because of the presence of highly characteristic melanin deposits in the MRI study.[3] This is the only method to diagnose CNS melanosis in a living patient.[3] Secondly, in our patient there were no clinical indicators suggestive of a developing cutaneous melanoma, viz. changes in colour, size, shape, rapid growth rate, proliferative nodules or ulceration, in any of the GCMN. Hence doing a skin biopsy to rule out a developing cutaneous melanoma was not indicated. We would like to mention that malignant transformation within GCMNs is exceptionally rare in the neonate and has been reported in only three neonates.[4] This recent review has stated that even if clinical indicators of cutaneous melanoma are present, careful histological evaluation may eventually reveal the lesion to be benign.[4] Also, the melanin deposits detected in our patient′s MRI did not reveal any perilesional edema or necrosis or enhancement with gadolinium contrast; ruling out malignant degeneration.[3] Thirdly, the presence of melanin deposits in our patient′s amygdala and thalami is not ′strange′. A recent report has stated that the most common area of involvement in their case series was the amygdala; detected in eight out of 10 MRIs.[5] Also presence of melanin deposits in thalami in cases of neurocutaneous melanosis is common.[2] References
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