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|Year : 2021 | Volume
| Issue : 4 | Page : 1103-1104
Neurocutaneous Melanocytosis in a 24-year-old Woman
Mieszko Zagrajek1, Kinga Belowska-Bień2, Joanna Bladowska3, Agnieszka Hałoń4, Bogusław Paradowski1
1 Department of Neurology, Interventional Radiology and Neuroradiology, Wroclaw Medical University, ul. Borowska 213, Wroclaw, Poland
2 Department of Pharmacology, Interventional Radiology and Neuroradiology, Wroclaw Medical University, ul. Borowska 213, Wroclaw, Poland
3 Department of General Radiology, Interventional Radiology and Neuroradiology, Wroclaw Medical University, ul. Borowska 213, Wroclaw, Poland
4 Pathomorphology and Oncological Cytology, Wroclaw Medical University, ul. Borowska 213, Wroclaw, Poland
|Date of Web Publication||2-Sep-2021|
Department of Neurology, Wroclaw Medical University, ul. Borowska 213, 53-556 Wroclaw
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Zagrajek M, Belowska-Bień K, Bladowska J, Hałoń A, Paradowski B. Neurocutaneous Melanocytosis in a 24-year-old Woman. Neurol India 2021;69:1103-4
Neurocutaneous melanocytosis (NCM) is a rare disorder characterized by abnormal aggregations of nevomelanocytes within the CNS associated with large/multiple congenital melanocytic nevi and elevated risk of malignant transformation. NCM can be asymptomatic or cause progressive neurological impairment, sometimes resulting in death. Diagnosis is according to physical examination, neuroimaging (MR), and biopsy.,,
A 24-year-old woman was born with multiple large dermal pigmented nevi, both hairy and hairless, of different size and degree of melanin saturation, located on the trunk and extremities [Figure 1] and [Figure 2]. As a child, she suffered from paroxysmal abdominal pain with skin blanching and consequential drowsiness (probably complex partial seizures). At the age of 23, headaches and generalized convulsive seizures occurred.
|Figure 1: Large hairy congenital melanocytic nevus located along the central line of the body on the neck and multiple satellite melanocytic nevi on the back|
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|Figure 2: Multiple congenital melanocytic nevi, both hairy and hairless, of different sizes and degrees of melanin saturation on the trunk and extremitieslimb|
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On admission to our Department of Neurology, physical examination revealed positional tremor of hands, right upper limb weakness, diminished deep tendon reflexes, positive Babinski sign on the right side, and wide-based gait.
Brain and cervical spine MRI [Figure 3] showed diffuse enhancement of thickened leptomeninges surrounding the brain and spinal cord. CSF examination revealed increased protein level (169 mg/dL), cell count was normal, and no cancerous cells were found. EEG showed generalized slowing and bilateral paroxysmal sharp waves in parieto-temporal lobes. A consulting psychiatrist diagnosed the patient with organic asthenia. The samples from the intradural extra-medullary lesion (cervical spine C5/C6) were collected for histopathological examination, which confirmed the diagnosis of NCM.
|Figure 3: MR examination of the brain, axial (a) and sagittal (b) T1-weighted images after contrast administration. Diffuse and extensive contrast enhancement of cerebral leptomeninges as well as of leptomeninges within the cervical spinal canal (b) is visible|
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The patient was discharged and instructed to take valproic acid, dexamethasone, and escitalopram. Owing to the risk of developing malignant melanoma, permanent dermatological, oncological, and neurological supervision was recommended.
Written and verbal consent for the publication was obtained from the patient.
Financial support and sponsorship
This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.
Conflicts of interest
There are no conflicts of interest.
| » References|| |
Alikhan A, Ibrahimi OA, Eisen DB. Congenital melanocytic nevi: Where are we now? Part I. Clinical presentation, epidemiology, pathogenesis, histology, malignant transformation, and neurocutaneous melanosis. J Am Acad Dermatol 2012;67:495.e1-17.
Scattolin MA, Lin J, Peruchi MM, Rocha AJ, Masruha MR, Vilanova LC. Neurocutaneous melanosis: Follow-up and literature review. J Neuroradiol 2011;38:313-8.
Sabat SB. Teaching NeuroImages: Neurocutaneous melanosis. Neurology 2010;74:e82.
[Figure 1], [Figure 2], [Figure 3]