Table 4 Patients with CMN who died due to neurological complications

Total number of patients who died

34 (of a total of 2339 patients), excluding the studies that only reported neurological signs and symptoms in patients with MRI abnormalities: 24 (of a total of 2107 patients)

Age of neurological diagnosis

Mean: 5.86 years, median: 3 years, range: birth–27 years

Age of death

Mean: 7.44 years, median: 5.15 years, range: 0.7–28 years

Time between diagnosis and death

Mean: 1.43 years, median: 0.76 years, range: 0–4.4 years

Sex

Female: (n = 14), male: (n = 20)

Cause of death

Proliferating melanocytosis of the CNS (n = 19), malignant melanoma (n = 15)

Number of CMN

Multiple (n = 27), single (n = 0), unreported (n = 7)

Symptoms/signs

Neurodevelopmental delay (n = 9), seizures (n = 11), hydrocephalus/increased intracranial pressure (n = 28)