[A childhood case of antiphospholipid syndrome]. / Le syndrome des antiphospholipides à propos d'une nouvelle observation pédiatrique.

UNLABELLED: Many conditions can lead to cerebral strokes in children. The antiphospholipid syndrome widely described in adults in association with systemic lupus erythematosus, is rare in childhood. CASE REPORT: Two months after recovering from varicella and a few days after an episode of bronchitis, a 17-month-old girl developed left facial paralysis associated with right hemiplegia. Brain MRI and angio-scan showed thrombosis in the internal left carotid associated with ischemia in the superficial posterior territory of the left Sylvian artery. Echocardiography and hemoglobin electrophoresis were normal. Tests were negative for protein S, C and antithrombin III deficiencies and no resistance to activated protein C. IgM anticardiolipin antibodies were detected at high level (greater than 25IU/l) initially and six weeks later. In the absence of an evident etiology, mainly systemic lupus erythematosus (negative antinuclear antibodies), the diagnosis of primary antiphospholipid syndrome was retained. The girl was treated by heparin then by salicylate at antiaggregate doses associated with re-habilitation. Twelve months later, the patient had not developed any other thrombosis, in spite of a high level of anticardiolipin antibodies. CONCLUSION: In children with cerebral strokes, antiphospholipid syndrome must be discussed when the usual etiologies have been ruled out.

[Recurrent inflammatory myofibroblastic tumor with renal, retroperitoneal and lymph node involvement]. / Tumeur myofibroblastique inflammatoire récurrente avec triple localisation, rénale, rétropéritonéale et ganglionnaire.

Inflammatory myofibroblastic tumors are uncommon and benign tumors with unknown aetiology. First reported in the lungs, the inflammatory myofibroblastic tumors have been observed in other locations, especially in the abdomen and the pelvis. We report a 14-year-old adolescent female, who presented sequentially an inflammatory pseudotumor of lymph node, the left kidney and the retroperitoneum. Extrapulmonary inflammatory myofibroblastic tumors are mesenchymal solid tumors. They are frequently circumscribed and confined to a single organ. The recurrence of some inflammatory myofibroblastic tumors and their expression of chromosomal abnormalities found in some types of lymphoma suggest that some of these lesions constitute a true neoplastic process.

[Central neurocytoma: three case reports]. / Neurocytome central: à propos de trois observations.

Central neurocytoma is classically recognized as an intraventricular benign brain tumour. Extraventricular localisation is rarer. We report two intraventricular neurocytoma and another bulbar extraventricular neurocytoma. Histologically, central neurocytoma presents remarkable likeness characteristics to oligodendroglioma, but immunohistochemical study distinguishes this tumour. Imaging appearances (CT, MRI) raise the diagnosis and immunohistochemical study confirm it. The purpose of our work is to assess the value of imaging (CT, MRI) in the diagnosis of central neurocytoma.

[Moyamoya and Down syndrome: about 2 cases]. / Syndrome de Moyamoya et trisomie 21 : à propos de 2 cas.

Moyamoya syndrome has rarely been reported in association with Down syndrome. We report on 2 cases in 3-year-old and 6-year-old female children with Down syndrome, who presented with neurological deficit. Imaging (magnetic-resonance angiography and digital-subtraction angiography) revealed the classical Moyamoya pattern. The neurological deficits persisted in both cases. One patient has developed epilepsy.

[Frantz's tumor: anatomoclinical study of six Tunisian cases]. / La tumeur de Frantz: etude anatomoclinique de six cas tunisiens.

Solid and pseudopapillary tumour (Frantz's tumour) is a rare low-grade neoplasm of the pancreas. We report six new cases. Our objective is to specify clinical and pathological characteristics of this rare neoplasm and to discuss its histogenesis. A retrospective review was considered on six Tunisan patients who had solid and pseudopapillary tumor of the pancreas. A review of medical registries and morphological analysis with immunohistochemical study were carried out in all cases. Four patients were female and two patients were male with a median age of 27,5 years (range: 14 - 68 years). Abdominal pain was the most common initial symptoms (5 cases/6). Abdominal computed tomography and/or ultrasonography was used in all the cases. The tumour was in the tail of the pancreas in 4 patients and in the body of the pancreas in one patient; one tumor involved all the pancreas. The median diameter of the tumour was 16,8 cm (range: 8 - 35 cm). Three tumours had an extrapancreatic extension. All patients underwent surgical resection. No adjuvant therapy was recommended. The mean follow up period was 24 months (range: 5 - 78 months). Only one patient died during the surgery. Except for this patient, none experienced tumor recurrence or tumor-related mortality during the follow up period. Solid and pseudopapillary tumour of the pancreas is an uncommon neoplasm which shows distinct clinicopathologically characteristics. Despite diverse studies, its histogenesis remains undetermined. This tumor should be distinguished from other pancreatic neoplasms because its prognosis is excellent after surgical resection.

Occipital dermoid cyst associated with dermal sinus complicated with meningitis: A case report.

BACKGROUND: Occipital dermal sinus, usually associated with dermoid cyst, is a rare entity; it results from the persistence of an abnormal embryonal communication between the skin and the intradural space. Its main complication is intracranial infection. CASE DESCRIPTION: This 2-year-old girl was hospitalized for meningitis. Neuroradiological studies revealed a cystic mass of the posterior fossa communicating with the skin and hydrocephalus. The diagnosis of dermoid cyst associated with dermal sinus was established at surgery. The patient was treated with radical excision of both the occipital cyst and the dermal sinus associated with systemic antibiotic therapy. She had a good outcome. CONCLUSION: Posterior fossa dermoid cyst should be considered in all children with chronic occipital skin lesion, especially a dermal sinus. We emphasize the importance of early neurosurgical treatment of dermoid cysts to prevent the development of severe complications.

[Trans-ethmoid encephaloceles]. / Les céphalocèles transethmoïdales.

We report two cases of ethmoidal cephaloceles. Ethmoidal cephaloceles are very rare and most commonly present with recurrent episodes of meningitis or sometimes as a nasal mass. Diagnosis is made at MR and CT. Such imaging studies should be obtained in patients with recurrent meningitis or patients with polypoid nasal lesions prior to biopsy.

Early-onset of multiple sclerosis in a 5-year-old girl.

Childhood multiple sclerosis is a rare demyelinating autoimmune disease with particular features. Onset of multiple sclerosis is extremely uncommon in early childhood, particularly before 6 years of age. We report the case of a 5-year-old girl admitted to the hospital for altered consciousness and rapid onset of right hemiparaplegia. Magnetic resonance imaging (MRI) of the brain showed multifocal white matter disease with T2 hyperintense oval lesions in subcortical, periventricular, and cerebellar hemispheres. Treatment with high dose intravenous methylprednisolone (30 mg/kg/day for 3 days) improved symptoms. Intravenous corticosteroid therapy was followed by 1mg/kg/day of oral prednisone. A second MRI, 40 days later, revealed new disseminated T2 hyperintense lesions in the frontal periventricular white matter, corpus callosum, left middle cerebellar peduncle, and dorsal spinal cord, leading to the diagnosis of multiple sclerosis. Azathioprine (2.5 mg/kg/day) was started and the steroid dose was tapered before being stopped after 3 months. After 2 years of follow-up, the patient has remained asymptomatic with a normal neurological exam and with no relapse or side effects of azathioprine. This work shows the particularities in clinical and radiological features of multiple sclerosis in a child aged less than 6 years.

[Hereditary hemorrhagic telangiectasia. Report of a pediatric case]. / La maladie de Rendu-Osler-Weber : à propos d'une nouvelle observation pédiatrique.

Hereditary hemorrhagic telangiectasia, or Rendu-Osler-Weber syndrome, is an autosomal dominant multiorgan disorder. This multisystemic vascular dysplasia is determined by a mutation of one of two main genes, endoglin (ENG) or HHT1, or ACVRL1 or HHT2. These mutations induce vascular disorders that cause recurrent epistaxis and eventually multiple telangiectasia and arteriovenous visceral malformations. We report the case of a 7-year-old girl who developed severe hypoxemia due to multiple pulmonary arteriovenous malformations.

[Neonatal renal candidiasis: a case report]. / Candidose rénale chez le nouveau-né : à propos d'une observation.

UNLABELLED: Neonatal renal candidiasis is an increasingly common condition affecting predominantly premature infants receiving neonatal intensive care or term infants with urogenital tract anomalies. CASE REPORT: a female infant was born by cesarian section at 30 weeks' gestation to a mother whose pregnancy had been complicated by rupture of membranes for 5 days. The infant was admitted at birth, maternofetal bacterial infection was suspected, and intravenous antibiotics were prescribed. The patient developed sepsis caused by Klebsiella pneumoniae at the age of 13 days. She was referred to our hospital at the age of 50 days because of renal abscess. At admission, the baby presented with hypothermia and abdominal distention. Renal ultrasonography findings were compatible with fungal disease. Blood, urine, and cerebrospinal fluid cultures were negative for fungi; however, galactomannan antigen blood levels were increased. Amphotericin B was administered for 6 weeks. End-organ evaluation of the heart, brain, and eyes did not demonstrate disseminated infection. The patient was discharged with a prescription for oral fluconazole and remained well at follow-up.

Unusual intraatrial thrombus in a neonate with coarctation of the aorta.

Left atrial thrombus in neonates is uncommon. We describe a newborn with coarctation of the aorta, in whom a thrombus confined to the left appendage was discovered on the first day of life and was thought to be a myxoma.

[Neonatal cervical artery dissection secondary to birth trauma]. / Dissection artérielle cervicale néonatale secondaire à un traumatisme obstétrical.

Cervical artery dissection is rare in the neonatal period and is most often caused by birth injury during dystocic labor. The severity of this pathology is due to the possibility of serious neurological complications. We report a case of a male newborn who was born vaginally after shoulder dystocia. The extraction was difficult, resulting in a fracture of the right humerus. On the second day of life, the child presented generalized clonic convulsions. Computed tomography of the brain showed an ischemic stroke in the territory of the right middle cerebral artery, the territory of the right posterior cerebral artery, and the right lenticulostriate and capsular regions. Doppler ultrasonography and magnetic resonance angiography showed bilateral carotid artery thrombosis and dissection at the left common carotid artery and its two branches and the right vertebral artery. We discuss the mechanisms of this pathology and we emphasize preventive measures.

[Megalencephaly with dystonia revealing Canavan disease]. / Macrocéphalie avec dystonie révélant une maladie de Canavan.

Canavan disease, or N-acetyl aspartic aciduria, is an autosomal recessive leukodystrophy characterized by spongy degeneration of the brain. The disease results from the accumulation of N-acetyl aspartic acid in the brain, due to aspartoacylase deficiency. We report the case of a 6-month-old girl who presented with megalencephaly, peripheral hypertonia, and a developmental delay noticeable after 4 months of age. Magnetic resonance imaging of the brain with spectroscopy was suggestive of Canavan disease, which was confirmed by chromatography of urinary organic acids.

[Posterior reversible encephalopathy syndrome induced by acute postinfectious glomerulonephritis]. / Syndrome d'encéphalopathie postérieure réversible induit par une glomérulonéphrite aiguë postinfectieuse.

INTRODUCTION: Posterior reversible encephalopathy syndrome (PRES) is a clinical and radiological entity characterized by neurologic symptoms in association with usually reversible bilateral posterior hemispheric edema on neuroimaging. It has been described in adults more than in children. Many pathological conditions and treatments have been associated with this syndrome. CASE REPORT: We report the case of an 11-year-old boy who presented with acute postinfectious glomerulonephritis, renal failure, and arterial hypertension. Two days after admission he developed generalized tonic clonic seizures. His blood pressure was 145/90 mmHg. Computed tomography showed hypodense lesions in the occipital and frontal areas. Based on the clinical manifestations and the typical pattern of brain imaging, the diagnosis of PRES was retained. Clinical manifestations and radiological abnormalities resolved after antihypertensive therapy. Axial FLAIR images and diffusion-weighted magnetic resonance images showed a slight hyperintensity signal in the left occipital area. CONCLUSION: Acute postinfectious glomerulonephritis in children should be considered a risk factor for developing PRES.

[Antisynthetase syndrome: a report of four cases and literature review]. / Le syndrome des antisynthétases : à propos de quatre observations et revue de la littérature.

The antisynthetase syndrome (ASS) includes inflammatory myopathy (polymyositis or dermatomyositis), interstitial lung disease (ILD), arthritis, Raynaud's phenomenon, and mechanic's hands, associated with antibodies against aminoacyl-tRNA-synthetases, the most well-recognized being the anti-Jo1 antibody (anti-histidyl-tRNAsynthetase). We report four cases of antisynthetase syndrome and review the clinical characteristics and prognosis factors dominated by ILD. We report the cases of four women with a mean age of 42 years (28-62 years). The interstitial lung disease was found in four cases and was objectified by CT-scan in all cases. The pulmonary symptoms were consisted of dyspnea (one case) and respiratory distress (one case). The anti-Jo1 antibodies were present in the four patients. The myopathy was concomitant with pulmonary involvement (two cases), preceded it in 6 months (one case) and in the course of evolution and after 1 month (one case). All patients received corticosteroid treatment. The immunosuppressive treatment was necessary for two patients because of the severity of the pulmonary involvement. The outcome was favorable in two patients, partially favorable in a patient who presented pulmonary fibrosis. However, one patient died after developing brain abscesses.