Functional MRI (fMRI) is routinely used to non-invasively localize language areas. Magnetoencephalography (MEG) is being explored as an alternative technique. MEG tasks to localize receptive language are well established although there are no standardized tasks to localize expressive language areas. We developed two expressive language tasks for MEG and validated their localizations against fMRI data. Ten right-handed adolescents (μ=17.5 years) were tested with fMRI and MEG on two tasks: verb generation to pictures and verb generation to words. MEG and fMRI data were normalized and overlaid. The number of overlapping voxels activated in fMRI and MEG were counted for each subject, for each task, at different thresholding levels. For picture verb generation, there was 100% concordance between MEG and fMRI lateralization, and for word verb generation, there was 75% concordance. A count showed 79.6% overlap of voxels activated by both MEG and fMRI for picture verb generation and 50.2% overlap for word verb generation. The percentage overlap decreased with increasingly stringent activation thresholds. Our novel MEG expressive language tasks successfully identified neural regions involved in language production and showed high concordance with fMRI laterality. Percentage overlap of activated voxels was also high when validated against fMRI, but showed task-specific and threshold-related effects. The high concordance and high percentage overlap between fMRI and MEG activations confirm the validity of our new MEG task. Furthermore, the higher concordance from the picture verb generation task suggests that this is a promising task for use in the young clinical population.

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Between 1981 and 1993, 41 children were treated for hepatoblastoma. Clinical, radiological and pathological data were reviewed retrospectively, focusing on surgical aspects of treatment and outcome. Fourteen children underwent primary resection of the hepatic tumour. One infant with severe congenital anomalies received only palliative treatment. Of 26 with irresectable disease, pulsed cytotoxic chemotherapy (cisplatin and doxorubicin) enabled subsequent surgical excision in 22 and one child with persistent extensive intrahepatic disease was successfully treated by liver transplantation. Thus, with a policy of selective preoperative chemotherapy, 90 per cent of hepatoblastomas were resectable. There were no perioperative deaths from haemorrhage but one child died from an intraoperative tumour embolus. A total of 28 survivors, 27 of whom are disease-free, were followed for a median of 5 years. The cumulative probability of survival in patients treated with intent to cure was 67 per cent. Analysis of survival data suggested a favourable outcome for those with a pure fetal histological tumour subtype. These results demonstrate significant progress in the treatment of hepatoblastoma.

Misalignment ofthe pulmonary veins with congenital alveolar capillary dysplasia, although rare, has been reported as a cause of persistent pulmonary hypertension of the newborn. Reported cases have been mainly sporadic. Familial occurrence has been reported in only three instances. We present affected sibs with this condition. In addition to pulmonary abnormalities, urogenital abnormalities, including ureteric and urethral obstruction, seem to be common. Autosomal recessive inheritance is suggested. (T Med Genet 1998;35:58-60) Keywords: congenital alveolar capillary dysplasia; misalignment of the pulmonary vessels; persistent pulmonary hypertension of the newborn; autosomal recessive

Patients with primary immunodeficiencies such as the Wiskott-Aldrich syndrome (WAS) are prone to develop Epstein-Barr virus (EBV) related lymphoproliferative disorders (LPDs). EBV LPD is most frequently seen in patients receiving immunosuppressive treatment after organ transplantation (post-transplant lymphoproliferative disorder), but can also arise in the primary immunodeficiencies. Typically, EBV LPD presents as a diffuse systemic disease with lymphadenopathy and organ involvement. A rare angiocentric and angiodestructive form of EBV associated B cell LPD, lymphomatoid granulomatosis (LyG), has also been reported in association with WAS. LyG most commonly involves the lung, but can also be seen in brain, kidney, liver, and skin. This report describes the case of a 16 year old boy with WAS who presented with an isolated non-healing ulcerating skin lesion. Biopsy revealed an EBV related LPD with the histological features of LyG. This cutaneous lesion responded dramatically to treatment with specific anti-CD20 immunotherapy and the patient remains clinically free of LPD at 18 months.A lthough Epstein-Barr virus (EBV) related lymphoproliferative disorders (LPDs) are most frequently seen in patients receiving immunosuppressive treatment after organ transplantation (post-transplant lymphoproliferative disorder; PTLD), they also occur in patients with immunodeficiencies.1 EBV related LPD usually manifests as systemic disease with vague symptomatology, and often shows pulmonary involvement. 2 We present a case of a 16 year old boy with known Wiskott-Aldrich syndrome (WAS) who developed an unusual, non-healing, ulcerating, cutaneous lesion with the clinical and pathological features of lymphomatoid granulomatosis (LyG). Histology confirmed an angiocentric destructive lesion with a mixed infiltrate of T and B cells. The B cells showed nuclear pleomorphism and were EBV positive. There was no evidence of disease elsewhere and this cutaneous lesion responded well to treatment with anti-CD20 immunotherapy. CASE REPORTA 15 year old boy with known WAS presented with an isolated, non-healing, annular 1.5 cm lesion on the left thigh. An initial punch biopsy revealed mild chronic inflammation only, with no specific features, and no organisms could be identified. He remained systemically well but the lesion increased in size to 4 cm and a further incisional biopsy was performed. This showed a focal ulcerating lesion with granulation tissue and fibrosis, and a dense angiocentric lymphocytic infiltrate within the dermis (fig 1). Special stains for fungi, bacteria, and mycobacteria were negative. Immunostaining for CD79a, CD20, CD5, and CD3 demonstrated a mixed T and B cell population, and enabled the identification of a subpopulation of enlarged B cells with moderate nuclear pleomorphism. In situ hybridisation (ISH) for EBV showed highly localised and intense staining for Epstein-Barr encoded viral RNAs (EBERs) in the intralesional lymphocytes, but no positive cells within the surrounding apparently normal skin (fig 1). Polymerase...

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A newborn infant with spontaneous remission of congenital leukemia cutis is described and a literature review of this uncommon phenomenon is provided. In view of the unusual and unpredictable behavior of this disease, chemotherapy should be withheld unless there is evidence of an 11q23 translocation or progressive disease. Otherwise, overall survival does not appear to be affected by adopting a conservative approach. Because of occasional late relapses, long-term follow-up is recommended. The biologic basis underlying spontaneous remission of congenital leukemia is unknown; therefore, molecular or molecular cytogenetic analysis of DNA obtained from a skin biopsy is recommended.

Congenital alveolar capillary dysplasia is a rare cause of irreversible pulmonary hypertension with 100% mortality. We present three cases of congenital alveolar capillary dysplasia with associated gastrointestinal abnormalities. Three full-term neonates presented with pulmonary hypertension needing ventilatory support by oscillation. Of the three, two neonates subsequently needed extracorporeal membrane oxygenation. Abdominal distension associated with bilious aspirates was the gastrointestinal manifestation. One child had duodenal atresia and anorectal anomaly, one with intestinal malrotation and the other with a rare combination of intestinal malrotaion and total colonic Hirschsprung's disease. All three infants succumbed to pulmonary hypertension at mean age 34 days. The etiopathogenesis and pathology of this condition are discussed with a comprehensive review of the literature.