Abstract
Neurocutaneous Melanocytosis (NCM) is a rare congenital disorder characterized by multiple melanocytic nevi scattered all over the body, in addition to leptomeninges. It is suggested that NCM is developed due to abnormal proliferation of melanin-producing cells following dysmorphogenesis in the embryology life. The clinical symptoms of NCM depend on the size and site of the lesion. One of the most common sites is the anterior temporal lobe. Management options are limited in such patient and the prognosis is generally poor. Clinical, radiological, along with CSF cytology can establish the diagnosis of NCM. Surgical intervention carries temporary better results than adjuvant therapy in patients with NCM.
Highlights
Neurocutaneous Melanocytosis (NCM) is an uncommon fatal congenital disorder described by the presence of large melanocytic nevi along with benign or malignant melanocytic proliferation of the leptomeninges [1,2,3]
The exact pathogenesis of NCM is not entirely perceived, it is believed to result from dysregulation and impaired production of the neural crest during embryogenesis [4] which eventually leads to anomalous multiplication of melanin-producing cells in the skin and leptomeninges [5,6]
We report an 11 -month- old female patient, a product of Normal Spontaneous Vaginal Delivery (NSVD), full term, with
Summary
Neurocutaneous Melanocytosis (NCM) is an uncommon fatal congenital disorder described by the presence of large melanocytic nevi along with benign or malignant melanocytic proliferation of the leptomeninges [1,2,3]. NCM, Leptomeninges, Dysmorphogenesis, Temporal lobe, Atypical melanocytes Fewer than 200 cases of NCM have been reported in the literature [16], since the first time it was described by Rokitansky in 1861 [16,17].
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