The neurocutaneous melanosis (NCM) is a rare syndrome, characterized by the association of giant or multiple non malignant pigmented cutaneous nevi with leptomeningeal melanosis or melanoma. The most common clinical features are convulsive seizures, psychomotor delay, intracranial hypertension and deficit of cranial nerves with onset early in life. We describe a 3.9-years old patient of with pharmacoresistant focal and secondarily generalized seizures. EEG showed focal temporal right sharp waves and spikes. He presented with giant and multiple non malignant pigmented cutaneous nevi and he showed typical neuroimaging features without infiltration of the leptomeninges. The peculiarity of this case is represented by a neurological symptomatology consisting only of focal epilepsy associated with psychomotor delay. Surgical treatment is proposed.
|Translated title of the contribution||Neurocutaneous melanosis and pharmacoresistant epilepsy: A difficult diagnosis|
|Number of pages||3|
|Journal||Bollettino - Lega Italiana contro l'Epilessia|
|Publication status||Published - Apr 2011|
ASJC Scopus subject areas
- Clinical Neurology