Neurocutaneous melanosis: follow-up and literature review.

Neurocutaneous melanosis is a rare, congenital, non-inherited syndrome characterized by numerous and/or large congenital nevi with intracranial leptomeningeal melanocytosis. This report describes two patients, presenting with a giant congenital nevus involving a major portion of the posterior trunk with satellite congenital nevi scattered all over the body, who developed seizures at 4 and 6 months of age, respectively. Changes in follow-up magnetic resonance (MR) examinations over an 8-year period were seen in case 1, while parenchymal melanocytic accumulation was reported in the region of the amygdala in case 2. These cases emphasize that neurocutaneous melanosis should be suspected in patients with giant congenital nevus with or without neurological symptoms. Also, neuroaxial MR screening should be performed in all cases and, ideally, before myelination of the brain to provide the highest sensitivity for detecting melanin deposits in the leptomeninges.

Idiopathic intracranial hypertension with and without papilloedema in a consecutive series of patients with chronic migraine.

Chronic migraine (CM) has been associated with idiopathic intracranial hypertension without papilloedema (IIHWOP), a significant percentage of these cases occurring in obese patients with intractable headache. A prospective study from February 2005 to June 2006 was made of 62 CM patients who fulfilled International Headache Society diagnostic criteria and had cerebral magnetic resonance venography (MRV) and lumbar puncture (LP) done. Two patients were excluded, six (10%) with elevated cerebrospinal fluid (CSF) open pressure (OP), five with body mass index (BMI) > 25. None of the patients had papilloedema or abnormal MRV. BMI and CSF OP were significantly correlated (r = 0.476, P < 0.001, Pearson's correlation test). Obesity (defined as BMI > 30) was a predictor of increase in intracranial pressure (defined as OP > 200 mmH(2)O) (f = 17.26, 95% confidence interval 6.0, 8.6; P < 0.001). From our study we strongly recommend that not only intractable CM patients with high BMI, but also first diagnosed patients with BMI > 30 should be systematically evaluated by a LP to rule out IIHWOP.

Side-shifting hemicrania continua with aura (migraine with aura with autonomic symptoms responsive to indomethacin?).

Atypical features of hemicrania continua (HC), including both visual aura and side shifting, have been reported previously. However, auras and variable unilaterality have never been reported together in HC. We report two patients with side-shifting HC with aura. These patients' symptoms are unilateral headaches, visual aura, autonomic features, throbbing pain, nausea and photo/phonophobia. One could speculate that the unilaterality and/or the autonomic symptom modules are indomethacin responsive. The patients can also be classified as chronic migraine with aura, with autonomic symptoms, responsive to indomethacin. Neither migraine subtype nor side-shifting HC with aura is included in the current International Headache Society (IHS) classification, so these patients are not classifiable. Side-shifting HC with aura implies the need to revisit the traditional IHS categorization of headaches into unique diagnostic groups. The modular headache theory may be a tool for the understanding of these rare and complex cases.