[DRESS syndrome, a drug reaction which remains bad known from paediatricians]. / Le DRESS syndrome, une réaction d'hypersensibilité aux médicaments, qui reste mal connue des pédiatres.

The DRESS syndrome (Rash with Eosinophilia and Systemic Symptoms) is a drug hypersensitivity reaction poorly known by paediatricians. It occurs within 1 to 8 weeks of treatment. Clinical features associate in variable patterns, fever, rash, lymphadenopathies, arthritis and potentially life-threatening damage (hepatitis, nephritis, pneumonitis), hyperleucocytosis and eosinophilia. This condition must be early recognized in order to immediately stop suspect drugs. A 6.5 year old girl had a febrile rash, hyperleucocytosis, lymph nodes and cytolitic hepatitis probably due to phenobarbital. Diagnosis of DRESS syndrome was performed only 13 days after the beginning of the eruption. Evolution was favorable but characterized by the recurrence of the febrile eruption with pleuritis. DRESS syndrome is a well described disease that occurs during treatment with a number drugs, particularly anti-epileptic drugs. Steroid therapy and immunoglobulins are proposed for treatment but have not been evaluated.

[Isolated familial corpus callosum agenesis prognosis]. / Les agénésies isolées et familiales du corps calleux sont-elles de bon pronostic?

Agenesia of corpus callosum belongs to a group of cerebral malformations whose prognosis is uncertain. In such cases, assessment of prognosis may benefit from eventual associated fetal, obstetrical or familial features. We report a patient with an isolated corpus callosum agenesia that led to the discovery of a similar malformation in her father. This observation demonstrates that some forms of isolated and familial corpus callosum agenesia could have a favorable outcome. However, the difficulty of the assessment of prognosis in isolated corpus callosum agenesia is emphasized and the question of parental RMI exploration in such a peculiar context is raised.

[Congenital toxoplasmosis with hydrocephalus diagnosed in utero: outcome of treatment]. / Toxoplasmose congénitale avec hydrocéphalie diagnostiquée in utero: évolution sous traitement.

BACKGROUND: The neurological outcome for severe toxoplasmosis can be poor despite appropriate management. CASE REPORT: A maternal toxoplasma infection occurred at 16 weeks of amenorrhoea; prenatal diagnosis was attempted at 20 weeks, fetal infection was confirmed by mouse inoculation at the 30th week. Pyrimethamine plus sulfadiazine treatment was initiated. However, at 37 weeks of amenorrhoea, sonographic examination of the fetus detected hydrocephalus. Despite prompt ventriculo-peritoneal shunting after birth and medical treatment of toxoplasmosis, the neurological developmental outcome was complicated and prognosis is poor at 5 years of age. CONCLUSION: This case shows that parents must be carefully warned about risks of a prenatal toxoplasmosis.

Relapse of herpes simplex encephalitis.

We report five children who had recurrent central nervous system signs after conventional acyclovir therapy for herpes simplex encephalitis. Secondary exacerbation was characterized clinically by severe ballismic movement disorder in all five children, associated with fever, impairment of consciousness, and seizures. Biologic analysis in all children and magnetic resonance imaging and neuropathology studies of the brain in three cases were compatible with inflammatory reaction. In contrast, all viral cultures remained negative, herpes simplex virus antigen in one child and DNA tested by polymerase chain reaction in four children were undetectable in the first samples of cerebrospinal fluid during the relapse, suggesting a postinfectious, immune-mediated mechanism of relapse in these patients.

[Post-traumatic transient cortical blindness]. / Cécité corticale transitoire post-traumatique.

BACKGROUND: Transient cortical blindness is a rare complication of mild head trauma in children. This spectacular manifestation always has a benign outcome. CASE REPORT: A five-year-old girl was admitted for bilateral blindness immediately following a fall with frontal head injury. Neurological examinations and ophthalmological investigations showed no abnormalities. The electroencephalogram showed slow waves in the left occipital area. The blindness resolved completely within 3 hours. CONCLUSION: This bilateral blindness was similar to that of the 40 cases reported since 1964, including a rapid and benign outcome. Its mechanism is not clear, although some suggest a cerebral vasospasm. Our patient's mother has a history of migraine.

Cognitive functions in Duchenne muscular dystrophy: a reappraisal and comparison with spinal muscular atrophy.

In order to clarify cognitive functions in Duchenne muscular dystrophy (DMD), we performed a new controlled neuropsychological study. IQ (WISC-R), verbal skills (fluency, confrontation naming and syntax comprehension) and memory abilities (BEM) were studied in two matched groups; 24 DMD children and 17 spinal muscular atrophy (SMA) children aged 12-16 yr. A significant difference appeared between the DMD and SMA patients: only in the DMD group were there significant disabilities in certain specific functions and normal scores in others. Despite similar education, the DMD children more often had significantly greater learning disabilities. There were more DMD left-handers. Verbal IQ was significantly low whereas performance IQ was at a normal level. DMD children also performed poorly in reading tasks and in some memory functions such as story recall and verbal recognition. Specific cognitive disabilities in certain DMD children, not seen in SMA children, suggest a relationship with a DMD genetic disorder.

Acute measles encephalitis of the delayed type: neuroradiological and immunological findings.

A case of delayed acute measles encephalitis in an immunosuppressed child is reported. Detailed immunological studies have shown defective humoral immunity (defective IgA, IgG2 and IgG3) and decreased natural killer activity. Neuroradiological examination by magnetic resonance imaging revealed several high signal lesions on T2-weighted images in the gray matter without clinical or pathological correlation. The implications of these findings are discussed.

Disseminated cerebral histiocytosis X responding to vinblastine therapy: a case report.

A case of intracranial histiocytosis X with multiple parenchymal brain lesions is described. Despite severe neuroradiological findings, and involvement of a node and vertebra on presentation, the child is doing well clinically, with outpatient chemotherapy, 36 months after diagnosis. The prognosis of cerebral involvement in such atypical cases should probably be reevaluated. Therapeutic problems are discussed.

Schilder's myelinoclastic diffuse sclerosis.

The term "Schilder's disease" has been used to describe conditions as disparate as adrenoleukodystrophy, myelinoclastic diffuse sclerosis, and postinfectious and postvaccinal encephalomyelitis. The eponymic designation should be reserved for instances of myelinoclastic diffuse sclerosis that correspond to the case described by Schilder in 1912. The diagnosis cannot be made unless adrenoleukodystrophy has been ruled out by analysis of the long-chain fatty acids of plasma cholesterol esters. Schilder's myelinoclastic diffuse sclerosis, a variant of multiple sclerosis, is a very rare disease that occurs in children and adults of both sexes and appears to respond to vigorous treatment with corticosteroids and/or corticotropin. A case of this disease is reported and the recent literature of cases that have been called Schilder's disease is reviewed.