Meningeal Melanomatosis: A Challenge for Timely Diagnosis

Table 1

Clinical and paraclinical findings in our three patients.

Pt	1			2			3
Age/gender	17/M			55/M			65/M
Clinical presentation at onset	Headache, nausea and vomiting, diplopia, and weight loss			Left leg monoparesis, headache, nausea and vomiting			Recurrent confusional episodes
MRI
Time from symptom onset (weeks)	4	30	47	8.2		12.9	1	21.4
Hydrocephalus	No	Modest dilation of ventricular system	Tetra-ventricular	Triventricular		Triventricular	No	Triventricular
Leptomeningeal contrast enhancement	No	Contrast not administered	Supra- and infratentorial, cranial nerves, spinal cord, conus, and cauda equina	Supratentorial, conus, and cauda equina		Supratentorial, spinal cord, conus, and cauda equina	No	Supra- and infratentorial, cranial nerves, conus, and cauda equina
Dural melanin deposits	No	No	Yes	No		No	No	Yes
CSF
Time from symptom onset (weeks)	40.6	42.9	44.9	8.1	10.6	14.9	21.4
Glucose (mg/dL)	—	92	83	—	—	29	8
Proteins (mg/dL)	—	2366	2773	370	—	431	535
Cell count (cells/uL)	4	110	174	2	<2	10	36
Cytology	n.p.	Nonspecific	Melanoma cells	n.p.	No identifiable cells	Nonspecific	Melanoma cells
EEG	Poorly organized background activity with bilateral slow abnormalities			Diffuse bilateral slowing			Bilateral fronto-centrotemporal slow abnormalities with left prevalence
Extra CNS visceral met	No			No			No
Final diagnosis	Primary leptomeningeal melanomatosis			Primary leptomeningeal melanomatosis			Leptomeningeal carcinomatosis and cutaneous melanoma
Time to diagnosis (weeks)	47			17			23.4
Clinical evolution	Confusion, visual hallucinations, partial seizures, and behavioral alterations			Urinary retention, progressive paraparesis, and visual hallucinations			Vigilance impairment, generalized seizures, headache, and backache
Treatment	Temozolomide (I cycle)			Dacarbazine (I cycle)			None
Overall survival (weeks)	49.1			23.4			30

Legend to Table 1: CNS = central nervous system, CSF = cerebrospinal fluid, EEG = electroencephalogram, met = metastases, MRI = magnetic resonance imaging, n.p. = not performed, and — = not available.