[Status dystonicus in childhood]

Background: Status Dystonicus is a rare complication of dystonia. It is a life threatening disorder that needs urgent treatment.The aim of this study is to describe clinical features, management and follow up of children with Status Dystonicus

Methods: - We conducted a retrospective study over an 8-year period including all patients diagnosed with Status Dystonicus. Clinical characteristics, etiologies and management were analyzed

Results: - Ten patients were included. Main features of Status Dystonicus were a severe generalized dystonia with vegetative signs. Laryngeal spasm and swallowing disorders were observed in 4 cases. Several treatments such as Levodopa, Anticholinergics, Baclofen, Benzodiazepines and Neuroleptics were tried. Mechanical ventilation was required in 4 cases. Two patients died due to rhabdomyolysis and respiratory failure. Others returned to their pre-Status Dystonicus

Conclusion: - Status Dystonicus is a life threatening condition that needs an urgent management on an intensive care unit. In fact, patients with Status Dystonicus can develop respiratory failure and metabolic complications.On the basis of our experience, we delineated a therapeutic approach in which the patient with Status Dystonicus needs supportive care, specific therapy of dystonia and intravenous sedative treatment

[Neurological complications of acute intermittent prophyria precipitated by prophyrinogenic drugs and efficiency of heme arginate treatment]

Acute intermittent porphyria [AlP] is a rare n disorder of heme biosynthesis characterized by enzymatic defect of porphobiligen desaminase with accumulation and s excretion of porphyrins and their precursors. Clinical picture is characterized by attacks with a triad of abdominal pain psychiatric disorder and neurological involvement [central and Peripheral nervous system manifestations, often precipitated porphyrinogenic medications are of poor outcome. We report a new cases A 13-year-old girl who: several attacks of AlP and developed acute severe axonal motor neuropathy, three weeks after porphyrinogenic [Famotidin, Phenobarbital and Nifedipine]. We stress on the importance of early diagnosis of AIP to prevent serious neurological complications often precipitated by medications and the efficiency of heme arginate treatment when administrated early during the attacks

L'hemispasme facial et son traitement par la toxine botulique

Authors report results of treatment of hemifacial spasm with Botulinum Toxin. The rationale of this treatment it to create a local and transitory neuro-muscular junction bloc and weakness of the muscles who are hyperactive in facial spasm. between September 1995 and May 1998, 14 patients with hemifacial spasm were treated with Botulinum Toxin. Indications, modalities of utilisation, injection techniques and favourable as well as secondary effect are commented and compared to other series

Le traitment du torticols spasomodique par la toxine botulique

Authors report results of treatment of cervical dystonia with Botulinum Toxin. The rationale of this treatment is to create a local and transitory neuro muscular junction bloc and weakness of the muscles who are hyperactive in cervical dystonia. Between September 1995 and May 1998, 8 patients with cervical dystonia were treated with Botulinum Toxin. indications, modalities of utilisation, injection techniques and favourable as well as secondary effects are commented and compared to other series