[ myoclonic epilepsy in infancy: about 37 cases]

The benign myoclonic epilepsy in infancy [BMEN] is described by Dravet in 1981. It is rare.Defined by the supervene of myoclonic attacks, without other types of attacks in normal infancy aged from 4 months to 3 years old. It is classified among the genaralized idiopathic epilepsies. The purpose of our study is to rate epidemiologic, clinic, electric, therapeutic and evolutive data about BMEN. Our study was retrospective, between 1997 and 2002, based on a card of exploitation including the criteria of inclusion according the definition of this type of epilepsy in the international classification of the epilepsy 1989. We collected 37 cases of BMEN let 1.2% of all the epilepsies. The female sex predominates slightly [55%], the mean age at the begining has been 17 months,the mean age at the consultation has been more than 24 months . About the past, all the children were born at full term, the medicalized delivery noticed in 98% of the cases, normal psychomotor growth in all the cases, the consanguinity represents 11 % of the cases and 19% of similar cases in the family.Clinically, the attacks were myoclonic and recurrent in 30 cases [81%], generalized in 30 cases [81%] and partial in 7 cases [5cases of bending of the head on the trunk, 2 cases at the level of the upper limb] and fall in 6 cases [16%]. The neurologic examination was normal in all the children. The electric signs consist in peaks-waves, and electroencephalographic peaks waves generalized in 43%, with photosensitiveness. The cerebral computerized tomography realized in 7 nursling was normal. The treatement has been based on the valprorate of sodium as monotherapy in the majority of the cases with good evolution in 90% of the cases.the prognosis is usually good. If the myoclonies resist to the valporate of sodium, the adjunction of a Benzodiazepine or ethoxumide may be suggested.The genetic data are unknown.Hence, the signifiant role of the molecular biology

Mediterranean spotted fever revealed by multivisceral damage

We report a case of Mediterranean spotted fever revealed in a 3 year-old girl by severe neurological symptoms as convulsions, feverish coma and generalized hypotonia, associated to acute diarrhoea and digestive haemorrhage. Contact with dogs, wrist bedsore and positive rickettsiosis seroconversion allowed us to have the diagnosis. Encephalic MRI showed an ischemic vasculocerebral accident that raises the problem of rickettsiosis lesions pathogenesis

[Diagnostic difficulties of the atypical onset of Kawasaki disease: a 8 cases report]

Kawasaki's disease is a multisystemic vasculitis that most often affects young children. It is known for inducing adverse cardiac effects, especially the development of coronary aneurysms that could lead to death. The diagnosis is based only on clinical criteria, and needs the presence of typical fever and 4 out of the 5 other clinical criteria. The aim of this study is to highlight the occurrence of atypical onset of the disease that should in no way delay the diagnosis and the start of treatment. We reviewed 13 cases of Kawasaki disease treated at Pediatrics 3 in Children Hospital of Casablanca from 1996 to 2002: 8 had atypical Kawasaki disease with particular clinical onset or biological parameters and/or evolution, that initially led to other diagnosis. The clinical onset manifestations included one case of uveitis with blindness, another case of febrile purpura, the third case with pyretic hepatomegaly and liver cytolysis. A diffuse cervical lymph node enlargement was described in one case and led to the diagnosis of haemopathy. Two patients had neurological involvement, in one case with ataxia in the other with headache and strabismus. A 12 year-old child had polyarthritis and the diagnosis was made retrospectively and the evolution was sub acute. The last patient had respiratory manifestations with coughing, fever and radiological atelectasia. The risk of coronary aneurysms directs to suspect early Kawasaki disease in very young children with persistent fever and inflammatory patterns, even in the absence of all typical manifestations

[Bilateral pulmonary embolism secondary to the venous thrombosis in child nephrotic syndrome]

Nephrotic syndrome is known to be associated with thrombosis in child. These complications are severe. The authors reported a case of a 4 year-old boy who was hospitalized for a corticoresistant nephrotic syndrome. He has developed a bilateral pulmonary embolism secondary to the extension of the thrombosis of the superior vena cava. The treatment consisted in the association of a low molecular weight heparin and vitamin K antagonists for 8 months. The outcome was good after 18 months without sequealea, and the remission of nephrotic syndrome was obtained after using cyclosporine

atypical presentation of juvenile dermatomyositis: about one case

Juvenile dermatomyositis is a multisystem immune-mediated disease that affects the skin, muscles and gastro-intestinal tract. Elevation of serum muscle enzymes, electromyogram findings and muscle biopsy are helpful to confirm the diagnosis of dermatomyositis. The goal of this article is to describe an unusual presentation of juvenile dermatomyositis, associated to celiac disease, with atypical exploration and unexpected evolution to extensive calcinosis. Nora, 14 years old, is followed since the age of 10 for celiac disease successfully treated with a gluten-free diet. At 11 years she is hospitalized because of arthralgia and myalgia. The physical examination shows erythematous facial rash, Gottron papules in metacarpo-phalangian articulations, and desquamatives lesions of elbows. She presents ankles, knees and wrists arthritis with lumbalgia. The diagnosis of dermatomyositis is suspected. Laboratory findings show a moderate inflammation, non auto-immune signs, increased level of muscular enzymes, without any abnormalities neither in the electromyogram nor in the biopsy. The diagnosis of dermatomyositis is confirmed. The patient is treated with high doses of oral corticotherapy, articular kinesi-therapy and gluten-free diet. The outcome was satisfactory, but an extensive calcinosis appeared after 3 years. Dermatomyositis and celiac disease is a rare association; only a few cases have been reported. They have common auto-immune condition that can explain this association. Regarding the nonspecific results of the electromyogram and the muscular biopsy, it can be due to the choice of an area which is not involved. The calcinosis is unusual in this type of monocyclic and stabilized involvement. It can appear due to the poor efficacy of oral corticoids prescribed in the context of malabsorption