J.-C. Cuvellier

Comparison of seizure reduction and serum fatty acid levels after receiving the ketogenic and modified Atkins diet

The ketogenic diet (KD) and the modified Atkins diet are effective therapies for intractable epilepsy. We compared retrospectively the KD and modified Atkins diet in 27 children and also assessed serum long chain fatty acid profiles. After 3 months, using an intent-to-treat analysis, the KD was more successful, with >50% seizure reduction in 11/17 (65%) vs. 2/10 (20%) with the modified Atkins diet, p=0.03. After 6 months, however, the difference was no longer significant: 7/17 (41%) vs. 2/10 (20%) (p=0.24). We observed a preventive effect of both diets on the occurrence of status epilepticus. After 1 and 3 months of either diet, responders experienced a significant decrease in serum arachidonic acid concentration compared to non-responders. The KD and modified Atkins diet led to seizure reduction in this small pilot series, with slightly better results after 3 months with the KD, but not after 6 months. The decrease of serum arachidonic acid levels might be involved in the anticonvulsive effects of KD or modified Atkins diet.

The Prevalence of Premonitory Symptoms in Paediatric Migraine: A Questionnaire Study in 103 Children and Adolescents

The prevalence and characterization of premonitory symptoms have not been rigorously studied in children and adolescents. Using a questionnaire, we retrospectively studied the prevalence of 15 predefined premonitory symptoms in a clinic-based population. In 103 children and adolescents fulfilling the International Classification of Headache Disorders, 2nd edn criteria for paediatric migraine, at least one premonitory symptom was reported by 69 (67%). The most frequently reported premonitory symptoms were face changes, fatigue and irritability. The mean number of premonitory symptoms reported per subject was 1.8 (median 2.2). Age, migraine subtype (with or without aura) and mean attack frequency per month had no effect on the mean number of premonitory symptoms reported per subject. In conclusion, premonitory symptoms are frequently reported by children and adolescents with migraine. Face changes seem to be a premonitory symptom peculiar to paediatric migraine.

Chronic daily headache in French children and adolescents.

To characterize the clinical profile, comorbidity and aggravating factors, and outcomes, a consecutive series of 34 French children and adolescents with chronic daily headache was studied. Of 206 referred over an inclusive interval of 2 years for the evaluation of headaches, 34 merited a diagnosis of chronic daily headache, which was defined as persistent or daily headaches of at least 3 months in duration. The overwhelming majority were female (61.8%), with a mean age at diagnosis of 10.5+/-3.1 years (range, 2.9-14.8 years). According to the Silberstein-Lipton criteria, transformed migraine was the etiology in 61.8%, whereas according to the second edition of the International Classification of Headache Disorders, chronic migraine accounted for 50% of cases. Stressors were recognized in 82%. Analgesic abuse was evident in 52.9%. Of the 29 for whom follow-up information was available, headaches resolved or greatly improved in 93.1%. Children and adolescents with chronic daily headache are thus a small subset of children with headache seen in general ambulatory practice. They tend to be girls in the midteen years experiencing a transformed migraine complicated by analgesic abuse, suggesting potential preventability. Simple measures, which can include reassurance and analgesia education, can be expected to result in improvement and eventual resolution of headache symptoms.

French General Practitioners' Management of Children's Migraine Headaches

Objectives.—This study sought to examine the treatment of children with migraine headaches in a prospective sample of children referred to a tertiary center for headaches.

Clinical features of primary headache in children: a multicentre hospital-based study in France

The aim of this study was to evaluate the concordance between clinical diagnosis and the International Classification of Headache Disorders, 2nd edn (ICHD-II) in children and adolescents with primary headaches. This 6-month prospective multicentre study of 486 patients (mean 9.8 ± 3.1 years; 52.6± girls) assessed the headache features through a structured questionnaire. In 398 patients with a single type of headache, headaches were bilateral (78.1±), frontal (62.4±), pulsatile (56.1±), with associated symptoms in 84.4±. The most frequently assigned diagnoses were migraine without aura (50.8±), probable migraine (14.1±), migraine with aura (11.1±) and frequent episodic tension-type headache (7.5±). For most of the diagnostic categories, the consistency of the investigators diagnosis with the ICHD-II criteria was good (K > 0.6 and ≤ 0.8) or excellent (K > 0.8). We conclude that migraine was predominant with regard to headache diagnoses repartition and that the ICHD-II seems usable in practice for evaluation of primary headache in French children and adolescents.

Small vessel abnormalities in alternating hemiplegia of childhood Pathophysiologic implications

Background: The pathophysiology of alternating hemiplegia of childhood (AHC) is unclear. The authors evaluated the skin and muscle biopsies from patients with AHC for vascular abnormalities. Methods: Skin biopsy specimens from four patients ages 18 months, 8 years, 9 years, and 18 years and muscle biopsies from two of these patients were examined by electron microscopy and compared with healthy controls. Results: Vascular abnormalities were found in both skin and muscle. Skin biopsies showed similar abnormalities in all four patients. Vacuoles were visible in the endothelium. The most striking abnormality was the presence in the tunica media of small and unevenly shaped vascular smooth muscle cells (VSMCs) containing intracytoplasmic vacuoles and, occasionally, apoptotic nuclei, with variations according to patient age. Moreover, most VSMCs had lost junctions with neighboring cells, and some were completely isolated. In vessels from muscle biopsies, the VSMCs showed vacuoles, residual osmiophilic deposits, and myofilament loss with substitution by vacuoles. Conclusions: The vascular abnormalities in our patients suggest a primary or secondary vascular pathophysiology to alternating hemiplegia of childhood. The vascular smooth muscle cells may be the initial target of the disease process.

Subdural effusion in a CNS involvement of systemic juvenile xanthogranuloma: a case report treated with vinblastin.

Juvenile xanthogranuloma (JXG) is one of the most common non-Langerhans cell histiocytosis in children. Usually cutaneous, there are disseminated forms. However, neurological localization remains exceptional. A 7-month-old boy had been admitted for subdural effusion due to non-accidental head injury and skin nodular lesions. A biopsy of a skin lesion was considered suggestive of JXG. Skin, eyes, brain, lungs, liver, and testicles were involved. Systemic treatment of JXG was begun with vinblastine. It allowed the regression of skin, lung, and CNS lesions. At age of 11 years, he had not reappearance of the xanthogranuloma. This report emphasizes the possible presentation of xanthogranuloma with subdural effusions, the organs which should be examined in case of disseminated forms and the efficiency of vinblastin.

Bilateral multifocal uveal juvenile xanthogranuloma in a young boy with systemic disease

AbstractBackground. Juvenile xanthogranuloma is a rare non-Langerhans cell histiocytosis that could occasionally produce diffuse systemic disease in young children, but associated posterior uveal lesions are very uncommon. Methods. Case report of an 8-month-old boy with macrocephaly who presented with chronic subdural haematoma and intracranial hypertension. A combination of nodular skin lesions and bilateral yellowish choroidal infiltration with limited retinal involvement suggested juvenile xanthogranuloma. Results. The diagnosis was confirmed by a skin biopsy, and oral corticosteroid therapy was introduced. Progression of the disease to involve an anterior uveitis with hypopyon and numerous other systemic lesions, including the central nervous system, lung, liver, spleen, kidney and testis, was also suggestive of Letterer-Siwe disease. Histiocytes were negative for Langerhans cell markers (S-100 and CD1a) and positive for macrophage marker (CD68). Electron microscopy failed to show Birbeck granules. Ocular lesions regressed under prolonged corticosteroid treatment, but resurgence of the other lesions required chemotherapy with vinblastine. Conclusion. In this atypical systemic variant of juvenile xanthogranuloma with bilateral uveal involvement, the immunohistochemical and ultrastructural findings were crucial in distinguishing juvenile xanthogranuloma from Langerhans cell histiocytosis.

Treatment of primary headache in children: a multicenter hospital-based study in France

The aim of this 6-month, prospective, multicenter study of 398 children and adolescents with primary headaches was to collect data on headache treatment in neuropediatric departments. Treatments were compared before and after consultation. Prior to consultation, the acute treatments that had been prescribed most frequently were paracetamol (82.2% of children) and non-steroidal anti-inflammatory drugs treatment (53.5%); 10.3% had received a prophylactic treatment. No differences in either acute or prophylactic treatment with respect to headache diagnosis were observed. After the neuropediatric consultation, paracetamol was replaced by a non-steroidal anti-inflammatory drug in about three-quarters of cases and by triptan in about one-quarter of cases. The number of children prescribed a prophylactic treatment nearly doubled, whereas there was a 5-fold and 23-fold increase in psychotherapy and relaxation training, respectively, between pre-referral and referral. We conclude that specific treatments were underused for primary headache.

The classification of chronic daily headache in French children and adolescents: A comparison between the second edition of the International Classification of Headache Disorders and Silberstein-Lipton criteria

Few data are available on the applicability of both the criteria proposed by Silberstein and Lipton (S-L) and the International Classification of Headache Disorders-II (ICHD-II) in the classification of children and adolescents with chronic daily headache (CDH). The International Headache Society recently added revised criteria (ICHD-IIR) for chronic migraine to its Appendix. We retrospectively reviewed all charts of 34 children and adolescents (<17 years) with primary CDH presenting to the outpatient clinic of the Universitary Department of Neuropediatrics of Lille between February 2004 and February 2006 and tried to classify their CDH according to both S-L criteria and the recently published ICHD-IIR. Thirty-two children (94%) and 33 children (97%) could respectively be successfully classified into one subtype of CDH according to the S-L classification and the ICHD-IIR. Transformed migraine was the most common diagnosis (61.8%), followed by new daily-persistent headache (20.6%) when the S-L criteria were used. Twenty-three children and adolescents (67.6%) could be classified under one of the migraine categories according to the ICHD-IIR classification. We think that both S-L and ICHD-II classifications, when used with detailed headache histories and diaries, are adequate to classify chronic daily headache in children and adolescents.