Agreement among neurologists on the clinical diagnosis of dystonia at different body sites.

OBJECTIVE: To study the reliability of the diagnosis of blepharospasm, oromandibular dystonia, cervical dystonia, and writer's cramp among neurologists. METHODS: 12 patients with adult onset focal segmental dystonia were videotaped in a standardised way. The tape was sent to six neurologists who are involved in clinical practice without a specific interest in movement disorders (general neurologists), and to four neurologists expert in movement disorders. The observers had to recognise whether the patients were affected by dystonia and to distinguish among blepharospasm, oromandibular dystonia, cervical dystonia, and writer's cramp. Interobserver reliability was assessed by kappa statistics, and the degree of agreement was classified according to the Landis classification. RESULTS: The 10 neurologists reached slight to moderate agreement on the diagnosis of these four disorders. When the observers were subdivided according with their professional experience in the field, a moderate to perfect agreement on the diagnosis was achieved by specialists in movement disorders, and a fair to moderate agreement by the general neurologists. CONCLUSIONS: Neurologists may have different ability to recognise adult onset focal dystonia, depending on their experience and on the type of dystonia.

Occipital hemorrhage in a child with Schönlein-Henoch syndrome.

The involvement of the Central Nervous System (CNS) in the Schönlein-Henoch Syndrome is quite rare. It seems to be connected with a systemic vasculitis (deriving from Circulating Immuno-Complex), clinically manifested by generic symptoms of meningeal irritation or convulsions, hemiparesis and choreas, due to hemorrhagic lesions. The Authors report a Schönlein-Henoch's syndrome in a 7-year-old boy characterized by diffuse vascular involvement with petechial purpura on his legs, arms and face, intestinal hemorrhages, gross hematuria and serious cerebral involvement. CT-scan showed a right occipital hematoma. A pathogenetic as well as a symptomatic therapy was carried out. The Authors discuss the diagnostic validity of CT.