Pier Antonio Battistella

Visual outcome of a cohort of children with neurofibromatosis type 1 and optic pathway glioma followed by a pediatric neuro-oncology program

We evaluated the visual outcome of a cohort of children with neurofibromatosis type 1 (NF1) and optic pathway glioma (OPG) treated according to standardized therapeutic guidelines. The study population consisted of all consecutive patients with NF1 and OPG referred to a specialized pediatric neuro-oncology program between 1994 and 2004. Treatment was instituted only in cases of progressive disease or clinical deterioration. Treatment modalities were chemotherapy (based on vincristine/carboplatin) for children younger than 5 years and radiotherapy for all others. Ten boys and 10 girls (seven with a positive family history) entered the trial (median age at diagnosis of OPG, 29 months). At a median follow-up time of 78 months, seven patients had been treated with chemotherapy only, four with radiotherapy, and four with chemotherapy plus radiotherapy. Five patients were observed only. Currently, 18 are alive and two have died. Eight patients were treated for progressive visual loss in the face of stable disease, five for tumor volume increase without visual deterioration, and two for symptomatic tumor volume increase. At referral, six children had a visual acuity (VA) of < 30% in both eyes; eight children had 100% VA bilaterally. At referral, the visual field (VF) could be assessed in three children: One had VF loss in both eyes, one had VF loss in one eye, and one had normal VF. At last follow-up, eight children had VA < 20% in both eyes; only two children had 100% VA in both eyes. Among 11 children who had some visual function, three had VF loss in one eye and three in both eyes, and five had an intact VF. Contrast and color sensitivity were abnormal in seven and six patients, respectively. Thirteen children fell into the WHO hypovision category. In summary, among the 15 children treated, one had a definitive and two a mild improvement in VA. In conclusion, the visual outcome of this selected cohort of NF1 patients with OPG is unsatisfactory. A critical reappraisal of the therapeutic strategy adopted is needed.

A placebo-controlled crossover trial using trazodone in pediatric migraine.

SYNOPSIS

A Placebo‐Controlled Crossover Trial of Nimodipine in Pediatric Migraine

SYNOPSIS

Quality of Life in Young Italian Patients With Primary Headache

Objective.—To compare the quality of life of young Italian patients with headache with that of the juvenile Italian population free of chronic disease.

Quality of life, coping strategies, and family routines in children with headache.

Objective.—To identify the relationship between headache severity, child coping, and quality of life (QoL) in the context of everyday family life.

Juvenile Idiopathic Stabbing Headache

We report on 83 juvenile patients with idiopathic stabbing headache, subjectively perceived as lasting from a fraction of a second to a few minutes. This sample was selected from among 2543 outpatients referred because of recurrent headache to the Paediatric Neurology Services of Ferrara and Padua (Italy). Other painful syndromes were excluded by clinical examination and appropriate tests Idiopathic stabbing headache in the pediatric age group contrary to the adult form, is usually not associated with other primary headache syndromes. We suggest that this clinical picture should be more clearly defined in the future, in order to better understand its relationships with other primary headaches.

Serum and red blood cell magnesium levels in juvenile migraine patients

Recently an important role for magnesium in establishing the threshold for migraine attacks has become evident. Accordingly, we measured serum and red blood cell magnesium levels in juvenile migraine patients with and without aura interictally. In comparison with normal subjects, migraineurs had significantly lower serum and red blood cell magnesium levels.

Unilateral hypomelanosis of Ito with hemimegalencephaly.

A five-year-old white male was referred to the Department of Paediatrics for assessment of mental retardation. On admission, the child’s weight was 19200 kg (50th-75th percentile), height 111 cm (75th percentile), head circumference 56 cm (>97th percentile). Hypopigmented macules were present from birth with a speckled, patchy and bandlike distribution on the right side of the trunk with a clear demarcation at the midline, on the upper right thigh and on the right arm. The patient had a flattened face with frontal bossing and depressed nasal bridge, divergent strabismus, hypertelorism, cup-shaped ears with a thickened antitragus and restricted external auditory meatus and flat feet. Neurological examination showed difficulty in performing fine motor tasks with the right hand, though muscular strength and deep tendon reflexes were normal and symmetrical. The 1.Q. was 71 (Stanford-Binet). The E.E.G. showed repeated sharp waves in the left centrotemporal area and fast activity in the frontal regions. Computed tomography scanning revealed asymmetry of the brain consisting in left megalencephaly with dysmorphic frontal horns of the lateral ventricles (Fig. 1). The family history was negative. Slit lamp and funduscopic examination, audiometry and auditory brainstem evoked responses, electrocardiogram, plain skull and spine roentgenograms, chromosome analysis from blood lymphocyte culture, urinary screening for amino acids and mucopolysaccharides, routine hematochemical investigations and urine analysis were all normal. The histology of a hypopigmented cutaneous lesion was compatible with IPA.

Circadian and Seasonal Variation of Migraine Attacks in Children

Object.—To investigate the rhythmicity of migraine episodes without aura in a pediatric population.

Interictal and Ictal Phase Study With Tc 99m HMPAO Brain SPECT in Juvenile Migraine With Aura

Single photon emission computed tomography (SPECT) was performed on 30 juvenile patients suffering from different types of migraine with aura. SPECT was carried out only during the pain‐free interval in 11 patients. The other 19 patients underwent SPECT ictally and 9 of them also interictally. During the pain‐free interval, the investigation was normal in 16 of 20 cases and revealed hypoperfused areas in 4 of 20 cases. Ictally, regional cerebral blood flow (rCBF) abnormalities were found in 14 of 19 cases: hypoperfusion in 11 patients and hyperperfusion in 3 patients. In most cases, rCBF abnormalities corresponded to the topography of neurologic symptoms. Our intericta period results do not agree with the previous studies in juvenile migraineurs, while during the ictal phase we demonstrated, for the first time, significant rCBF abnormalities in juvenile patients suffering from migraine with aura.