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.

Cerebral Venous Thrombosis: A Tunisian Monocenter Study on 160 Patients.

OBJECTIVE: Data regarding cerebral venous thrombosis in North Africa are scarce. This study aims to identify the clinical features, risk factors, outcome, and prognosis of cerebral venous thrombosis in Tunisia. METHODS: Data of 160 patients with radiologically confirmed cerebral venous thrombosis, hospitalized in Mongi Ben Hmida National Institute of Neurology (Tunis, Tunisia), were retrospectively collected and analyzed. RESULTS: The mean age was 37.3 years with a female predominance (83.1%). The mode of onset was subacute in most cases (56.2%). Headache was the most common symptom (71.3%), and focal neurologic symptoms were the main clinical presentation (41.8%). The most common sites of thrombosis were the superior sagittal sinus (65%) and the lateral sinus (60.6%). More than 1 sinus was involved in 114 (71.2%) patients. Parenchymal lesions observed in 85 (53.1%) patients did not correlate with cerebral venous thrombosis extent. Major risk factors were obstetric causes (pregnancy and puerperium) found in 46 (38.6% of women aged <50 years) patients, followed by anemia (28.1%) and congenital or acquired thrombophilia (16.2%). Mortality rate was of 6.6%. Good outcome at 6 months (modified Rankin Scale ≤2) was observed in 105 (87.5%)of 120 patients available for follow-up. Predictors of poor outcome were altered consciousness and elevated plasma C-reactive protein levels. CONCLUSION: Clinical and radiologic presentation of cerebral venous thrombosis in Tunisia was quite similar to other parts of the world with, however, a particularly high frequency of obstetric causes. Plasma C-reactive protein level should be considered as a prognostic factor in CVT.