Substantial evidence points to melatonin as playing a role in the regulation of circadian rhythms, sleep, and headache disorders. The objective of the study was to assess 6-sulphatoxymelatonin (aMT6s) levels in a large consecutive series of patients with migraine, comparing with controls. A total of 220 subjects were evaluated—146 had migraine and 74 were control subjects. Urinary samples were collected into the same plastic container since 8:00 p.m. to 8:00 a.m. of the next day (12-h period) and aMT6s was measured with quantitative ELISA technique. Among patients with migraine, 53% presented pain on the day of the urine samples collection. Their urinary aMT6s concentration was significantly lower than in the urine of patients without pain [14.0 ± 7.3 vs. 49.4 ± 19.0; t(143) = −15.1; 95% CI = −40.0 to −30.8; P < 0.001]. There was no significant difference in the aMT6s concentration of patients with migraine without pain on the day of their urine samples collection and controls [49.4 ± 19.0 vs. 42.5 ± 27.9; t(140) = 1.7; 95% CI = −1.2 to 14.8; P = 0.094]. To our knowledge, this is the first study to demonstrate reduction in melatonin levels during attacks in episodic and chronic migraine.

To our knowledge, this is the first study to evaluate the relationship between the urinary concentration of melatonin and migraine comorbidities. These results support hypothalamic involvement in migraine pathophysiology.

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The QOL of adolescent migraine sufferers may be aggravated not only by migraine but also by other factors, such as anxiety and depressive symptoms, which may contribute to the poor QOL in adolescents suffering from migraine.

Spontaneous periodic episodes of hypothermia still defy medical knowledge. In 1969, Shapiro et al. described the first two cases of spontaneous periodic hypothermia associated with agenesis of the corpus callosum. Recently, Dundar et al. reported a case of spontaneous periodic hypothermia and hyperhidrosis without corpus callosum agenesis, suggesting that the periodic episodes of hypothermia might be of epileptiform origin. Here we describe two paediatric patients with spontaneous periodic hypothermia without corpus callosum agenesis and demonstrate, to our knowledge for the first time, altered levels of neurotransmitter metabolites within the cerebrospinal fluid.Hypothermia, defined as a core temperature lower than 35°C, may be the result of several conditions: accidental causes (drugs, intoxication, exposure to extreme cold temperatures); endocrine conditions (hypothyroidism); and central or peripheral neurological disorders. 1,2Spontaneous periodic episodes of hypothermia, however, still defy medical knowledge. In 1969, Shapiro et al.3 described the first two cases of spontaneous periodic hypothermia associated with agenesis of the corpus callosum.Since then, over 50 cases have been reported with several attempts to undercover its pathophysiology.2 Structural, epileptiform, or biochemical abnormalities were suggested as possible causes; however, the definite underlying process remains unclear.Recently, Dundar et al. 1 reported a case of spontaneous periodic hypothermia and hyperhidrosis without corpus callosum agenesis, suggesting that the periodic episodes of hypothermia may be of epileptiform origin.We describe two paediatric patients with spontaneous periodic hypothermia without corpus callosum agenesis and demonstrate, to our knowledge for the first time, altered levels of neurotransmitter metabolites within the cerebrospinal fluid. The São Paulo hospital ethics committee approved this clinical letter and the parents gave informed consent for publication. CASE REPORTS Case 1A 4-year-old female was admitted with spontaneous episodes of dysthermia (hyper-and hypothermia), sweating, and drowsiness of about 2 week's duration. The attacks occurred daily, lasted about 1 to 3 hours and presented spontaneous remission, regardless of any therapeutic attempt (Table I). During the hypothermic attacks, she sweated profusely and felt faint; her skin was pale and cool. The physical examination was otherwise normal. She was born of non-consanguineous parents, with an uneventful gestational and neonatal history. She also showed normal neurodevelopment and there was no family history of neurological disorders.Laboratory findings included hematocrit at 35%, hemoglobin at 13.1g ⁄ dL, a white blood cell count of 9200 ⁄ mL, and a platelet count of 193 000 ⁄ mL. Blood urea nitrogen, serum creatinine, liver function tests, fasting blood sugar, serum electrolytes, serum uric acid, erythrocyte sedimentation rate, C-reactive protein, thyroid function tests, adrenocorticotropic hormone, and urinalysis produced normal results. Elect...

Leukoencephalopathy with brainstem and spinal cord involvement and elevated brain lactate diagnosis is based on its highly characteristic pattern of abnormalities observed by magnetic resonance imaging and spectroscopy. Clinically, affected patients develop slowly progressive cerebellar ataxia, spasticity, and dorsal column dysfunction, sometimes with a mild cognitive deficit or decline. In 2007, the pathophysiology of this disorder was elucidated with the discovery of mutations in the DARS2 gene, which encodes mitochondrial aspartyl-tRNA synthetase, in affected individuals. Here, the authors present a case of leukoencephalopathy with brainstem and spinal cord involvement with normal brain lactate, in which genetic analysis revealed a new mutation in the DARS2 gene not previously described.

-Menkes disease is a rare X-linked disorder related to a defect in the copper metabolism. According to the current literature, the most frequent neuroimaging findings are cortical atrophy, chronic subdural effusion or hygroma, and vascular abnormalities. White matter lesions may be present before other features of the disease and may evolve into atrophy. We hereby report a case of Menkes disease with typical history and progression, and an early phase imaging study with important white matter abnormalities, which could have lead to diagnostic difficulties.KEY WORDS: Menkes disease, neuroimaging. Doença de Menkes: relato de caso com comprometimento da substância branca cerebral RESUMO -A doença de Menkes é patologia ligada ao X relacionada a defeito no metabolismo de cobre. De acordo com literatura atual, os achados mais frequentes em exames de neuroimagem são atrofia cortical, efusão de subdural crônica ou higroma, e alterações vasculares. Lesões em substância branca podem estar presentes antes das alterações clinicas da doença e podem evoluir para atrofia. Apresentamos relato de caso da doença de Menkes com história e evolução típica, descrevendo os achados de neuroimagem na fase inicial da doença que revelaram sinais importantes de comprometimento da substância branca que poderiam conduzir a dúvidas diagnósticas. PALAVRAS-CHAVE: doença de Menkes, neuroimagem.

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