Migraine can induce ischaemic stroke, and is considered an independent risk factor for stroke in the young. To date, the nature of the link between migraine and stroke is essentially unknown. Forty-five children were studied. Homocysteine levels (fasting and post methionine load), vitamin B12 and plasma folate levels, factor V Leiden, factor II G20210A, methylenetetrahydrofolate reductase (MTHFR) C677T and A1298C mutations were examined. Compared with controls, patients with migraine had higher levels of post-methionine load homocysteine values (19.5 +/- 4.9 vs. 16.9 +/- 1.9; P = 0.025) and significantly lower folate levels (5.8 +/- 2.6 vs. 7.5 +/- 2.1; P = 0.002). We found a trend toward an increased risk of migraine in subjects carrying a homozygous mutant genotype for MTHFR C677T and MTHFR A1298C polymorphisms. Genetic prothrombotic conditions do not seem to be related to migraine in the young, whereas the biochemical differences between migrainous patients and controls are an appealing topic for further investigation.

Summary:Fucosidosis is a rare autosomal recessive lysosomal disorder caused by ␣-fucosidase deficiency. We report a child with fucosidosis, second daughter of non-consanguineous parents, for whom biochemical diagnosis followed clinical evidence of the disease in her older sister. Based on previous experiences, the indication to transplant was considered. Since she lacked a matched sibling, an unrelated marrow donor was found. At prehematopoietic stem cell transplantation evaluation, first signs of neurological involvement were clinically detectable. MRI showed diffuse hypomyelination and auditory brainstem responses and somatic-sensorial evoked potentials were altered. Visual evoked potentials were normal, tortuosity in the retinal veins and peripapillary hemorrhages were detected. Bone marrow transplantation conditioning was with a regimen of busulphan, thiotepa and cyclophosphamide; in vivo Campath 1G, cyclosporin A and short course methotrexate were given to prevent graft-versus-host disease. The patient engrafted rapidly and her post-transplant course was complicated by moderate graft-versus-host disease, transient episodes of idiopathic thrombocytopenic purpura, repeated septic complications and recurrent episodes of Sweet's syndrome. Sequential short tandem repeat polymorphisms on peripheral blood and bone marrow cells documented the persistence of donor engraftment. Follow-up showed a progressive rise of enzymatic levels. Over the last few years, several storage diseases have been treated with hematopoietic stem cell transplantation (HSCT). [3][4][5][6] The enzymatic activity of white blood cells has been proven to rise to normal levels after HSCT. 7 Moreover, the differentiation of donor monocytes into tissue-specific cells such as Kupfer cells, Ito cells, pulmonary or peritoneal macrophages, lymphoid or histiocytic cells of the spleen and lymph nodes or dendritic cells of the skin causes the clearance of the accumulated substrate in many organs. 8 The correction of enzymatic levels has also been demonstrated in the CNS where new microglia and astroglia were shown to originate in the donor's macrophagic system. 9-13 Persistence of engraftment makes it possible to maintain the turnover of these cells and accounts for clinical improvement after HSCT. Intracellular enzyme transfer and uptake of donor-derived enzyme in plasma contribute to improving the advantageous effects of HSCT in these diseases. In the canine model, early HSCT proved to be useful in preventing clinical onset of fucosidosis and in maintaining normal enzymatic levels. 14 Clinical improvement has been reported in human fucosidosis after HSCT. 15,16 In this paper we report on the long-term outcome of fucosidosis in a patient treated with unrelated donor (UD) HSCT. Case reportWe report on the outcome of the second daughter of nonconsanguineous parents, for whom diagnosis followed clinical evidence of the disease in her sister.At the age of 18 months, the patient's older sister underwent cranial MRI which showed a diffuse hypomyelination invo...

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Headache is one of the most common health complaints in children and adolescents. The initial assessment of acute headache aims to recognize whether there is a secondary cause for headache. According to the literature, the secondary headaches due to non-life-threatening diseases are the most frequent ones in pediatrics. In particular, respiratory tract infections and minor head trauma represent the majority. In a small minority of patients, headache is secondary to serious life-threatening intracranial disorders. Meningitis is the most common cause of headache due to serious neurological condition. These patients do not constitute a diagnostic problem, as they usually have clear systemic and neurological signs of intracranial hypertension. Recent onset of headache attacks, occipital location of pain, patient's inability to describe headache characteristics seem frequently recur, together with neurological signs, in intracranial life-threatening conditions.

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