Neurocutaneous melanosis and epilepsy surgery

To the Editor: Dr Jean Latrelle, an eminent Parisian neurologist at the time of a meeting in Harriman, N.Y., started his discussion by saying, “Everything has been written, but not everything has been read”. I found the description of a patient with neurocutaneous melanosis by Oliveira de Andrade et al. (Epileptic Disord, 2004; 6: 145-52) interesting. For the past 30 years we have been following a similar patient. She was an intelligent, 29 year old, right-handed female. Birth, development, and family history were normal, and she had no illness that might lead to cerebral seizures. She had a vest type giant nevus from birth, which extended from her back to the extremities. Minor seizures started at the age of 3 years, with a feeling of fear and a painful grabbing sensation in her stomach followed by trembling of the body for 10 to 30 sec. She would call or run to her mother. Minor seizures continued over the years. When she was in her 20s, the attacks started with a sudden uncomfortable feeling in the head described as “high voltage”. She felt cold or hot, shivered, and had “goose pimples”. There was a variable degree of impairment of consciousness, but she was usually not fully unconscious. The episodes lasted a few seconds, and most were purely subjective. Only rarely did she fall in her minor attacks. At times they occurred singly or in brief clusters, but usually they recurred every 2 to 3 min for up to 12 or 15 hours. These prolonged bouts occurred once or twice a month. At such times, her mood changed; she became quiet and irritable. She had a frequent desire to void and was unable to carry on with her work. When these episodes occurred at night, she was unable to sleep since the brief feelings awakened her. She found these prolonged episodes to be uncomfortable, interfering with her work and social life. She had five major tonic clonic attacks in her early 20s. After suitable localization studies she had a right temporal resection. There was no meningeal melanosis. The amygdala was replaced by a dark, brownish yellow, rather discrete mass measuring 7-8 mm in diameter. It was adherent to the tip of the pes hippocampus which was markedly increased in consistency. She had no further seizures with impairment of awareness but has continued to have some auras, presumably coming from the same hemisphere. This patient, with amygdaloid melanosis was described in 1980 (Andermann et al. 1980). She has been fully functional as a hospital social worker since. This lady illustrates very well, like the patient presented by Oliviera de Andrade, that seizures in patients with extensive nevi do not necessarily present with melanoma, nor is there necessarily malignant deterioration. It is unlikely that surgical treatment would lead to development of malignancy and her retardation is also not a reason not to consider surgical treatment. It is much better to be slow without seizures than slow and have seizures as well. As we stated in our 1980 report, melanosis of the amygdala occurring as part of neurocutaneous melanosis may of itself cause symptoms and should be added to the list of small lesions in the temporal lobe leading to complex partial seizures. M