Table 1.

Summary table of neurocutaneous melanosis (NCM)

Etiology	Non-familial. Pathogenic mechanism is still unclear; it is believed to be due to dysplasia of the neuroectodermal melanocyte precursor cells.
Incidence	Rare, about 100 cases reported in the English literature. Prevalence is estimated at 1/50,000–1/200,000.
Gender ratio	No predisposition according to gender
Age predilection	Most commonly within the first two years of life
Risk factors	Unknown
Treatment	There is no effective treatment. Chemotherapy and radiotherapy have been shown to be ineffective or little effective. Most therapies are designed to treat the symptoms associated with the disorder, mainly those related to hydrocephalus.
Prognosis	Asymptomatic NCM shows a normal life expectancy. Symptomatic NCM has an extremely poor prognosis.
Findings on imaging	In fact, as melanin pigment is inherently paramagnetic, the typical NCM lesions usually exhibit high intensity on T1W images and low intensity to iso-intensity on T2W images. The amygdala is the most common location of melanocytic deposits. The other frequent areas of melanocytic cell infiltration are cerebellum, medulla, pons, cerebral peduncles, and upper cervical spinal cord.