Atypical Aicardi's syndrome on two sisters not twins

Aicardi's syndrome is a rare genetic disorder characterized by heterogeneity of clinical findings and prognosis. The characteristic features include infantile spasms, agenesis of the corpus callosum and chorioretinal lacunae. This syndrome is seen almost exclusively in female and is often sporadic. However, some familial cases were reported in the literature. The prognosis is unpredictable. It is grave in most of cases with severe psychomotor retardation. The authors report two sisters not twins, from a consanguineous familily with atypical Aicardi syndrome diagnosed at 2 months of age. Clinical feautures were infantile spasms, partial agenesis of the corpus callosum and atypical ophthalmologic abnormalities. Evolution was marked by severe psychomotor retardation, scoliosis and severe recurrent pneumonia entailing the death of one of the patients at 3 years of age

L'electro-encephalogramme chez l'enfant epileptique: interet et limites: a propos de 317 cas

664 electro- encephalogram [E.E.G.] practiced in 317 children [190 boys and 127 girls] followed in hospital consultations for epilepsy have been analysed. The first E.E.G was pathological in 63.4% of cases; fits were recorded in 12 patients [vertigo being the most frequent]. Good concordance between localisation of electrical abnormalities and clinical fit's were observed in 54.2% of patients. Comparison of E.E.G abnormalities with CT scan data 93 patients shows good concrodance in 57% fo them. discordance abnormal E.E.G/ normal scanner is the more frequent [37.6% of this group]. Pathological E.E.G. are more frequent in severe epilepsy [71% of the group]

L'electromyographie chez l'enfant: indications et apport - a propos de 383 observations

Electromyographic techniques [E.M.G.], not yet spread enough in Tunisia, represent a certain relevance in the case of neurological affections among children. 383 E.M.G. examinations have been carried out within three years on 347 children [200 boys and 147 girls] aged less than sixteen. Diagnostic indications prevail [69,7%] of patients for clinical symptoms most frequently walking disorders [38,6%] and hypotoniae [12,6%]. Among 255 examinations with diagnostic purpose, 56,5% are pathological while 43,5% have no diagnostic contribution. The most frequent affections are facial palsies [15,6% of patients]; myopathic syndromes [14,1%]; degenerative and/or hereditary diseases of the nervous system [13,3%] furthered by consanguinity encephalopathies and cerebral disorders [12,1%], nerve injuries [5,8%] especially sciatica ones resulting from intramuscular injections; the after-effects of poliomyelitis [5,8%] and brachial plexus ailments, of obstetrical origin in most cases