[Systemic juvenile idiopathic arthriris: diagnosis and management]

Systemic juvenile idiopathic arthritis is a relatively rare disease in childhood. Sometimes, difficulties are encountered because it is a diagnosis of exclusion. The authors describe the clinical, biologic, therapeutic and evolutive characteristics of this affection. It is a retrospective study during a period of 16 years from 1990 to 2005. Inclusion criteria respond to Durban criteria, after exclusion of other etiologies of febrile eruptions. The authors analyse epidemiologic and clinical characteristics of the disease, biologic exams, treatment and evolution. Nine eases are reported during study period. Patients are between 22 month and II year old. Biologic analysis shows an important inflammatory syndrome with a sedimentary rate superior to 80 mm at first hour in average, a white blood cell count superior to ll.000/mm3. the evolution under treatment is characterized by resistance to steroids in one case and dependence to steroids in 2 eases. The complications include a macrophage activation syndrome in 2 cases and complications secondary to steroids in one ease. Systemic juvenile idiopathic arthritis remains a relatively rare disease in childhood. It's management is improved by the emergency of new treatments bnt its evolution is unpredictable

[Doose syndrome or myoclonic-astatic epilepsy: two new Tunisian cases]

Myoclonic astatic epilepsy belongs to the epilepsies with generalized seizures. It occurs in 1-2 per cent of all childhood epilepsies up to age 9. The disease is characterized by age of onset mostly between 2 and 6 years and by various clinical and EEG criteria [myoclonic or astatic seizures, 4-7 Hz background rhythm, polyspikes and waves in electroencephalogram]. The authors report two cases answering these criteria. It is about 2 old boys respectively of 2 years and a half and of 18 months during first seizures. Attacks were polymorphic associating myoclonic seizures, absences and astatic crises causing traumatism. These attacks are associated to clonic generalized seizures in one case. The electroencephalograph showed a thorough rhythm of 4-7 Hz. Cerebral imaging was normal at the 2 childs. The psychomotor development is normal in spite of the rebel character of crises justifying the use of several therapeutic alternatives during the evolution