Atypical subacute sclerosing panencephalitis: Case report

Subacute sclerosing panencephalitis (SSPE) is a progressive inflammatory disorder of the central nervous system with both poor prognosis and high mortality. the disease has been related to a persistent and aberrant measles virus infection and no effective treatment has been available. We report a case of SSPE with atypical features including seizures at onset and a fulminant course in a 8 years-old boy who had been previously immunized against measles.Univ Fed Juiz de Fora, Univ Hosp, Dept Internal Med, Neurol Serv, Juiz de Fora, MG, BrazilUniv Fed Fluminense, Niteroi, RJ, BrazilUniv Fed Juiz de Fora, Juiz de Fora, MG, BrazilUniversidade Federal de São Paulo, São Paulo, BrazilUniv Fed Rio de Janeiro, Rio de Janeiro, BrazilUniv Fed Juiz de Fora, Univ Hosp, Dept Internal Med, Juiz de Fora, MG, BrazilUniversidade Federal de São Paulo, São Paulo, BrazilWeb of Scienc

Tetraparesis secondary to cervical ossification of the posterior longitudinal ligament - Case report

Ossification of the posterior longitudinal ligament (OPLL) is a rare cause of myelopathy in non-Oriental populations and relatively unrecognized by general practitioners. A case of an Afro-Brazilian 54-years-old woman presenting with tetraparesis due to cervical OPLL is presented. Emphasis is made for the inclusion of OPLL in the differential diagnosis of compressive cervical myelopathy

Amyotrophic lateral sclerosis type 8 is not a pure motor disease: evidence from a neuropsychological and behavioural study

Objective Amyotrophic lateral sclerosis type 8 (ALS8) is a familial form of motor neuron disease, with predominance of lower motor neuron degeneration, and is caused by mutation of the vesicle-associated membrane protein-associated protein B (VAPB). We aimed to compare the cognitive profile of patients with ALS8 and healthy controls (HC), and to screen for behavioural features in ALS8 patients. Methods The sample was composed of ALS8 patients (n = 22; 14 men; median age 48 years old; median disease duration 6.5 years) and HC (n = 33; 19 men; median age 48 years old). Patients and HC were matched for sex, age and educational level. Participants underwent behavioural, psychiatric (Hospital Anxiety and Depression Scale and Cambridge Behavioural Inventory-Revised) and neuropsychological assessments, focused on executive functions, visual memory, and facial emotion recognition. Results ALS8 patients exhibited subtle deficits in executive functions. Compared to controls, ALS8 patients were significantly impaired in measures of flexibility and inhibitory control. ALS8 patients and HC did not differ in scores of facial emotion recognition. There was clinically relevant anxiety and depression in 36% and 27% of ALS8 patients, respectively. Behavioural disorders such as stereotypic and motor behaviours were present in more than 30% of patients. Conclusions ALS8 patients present mild executive dysfunction and behavioural changes such as mood disorders, apathy and stereotypic behaviour. Our findings suggest that ALS8 is not a pure motor disorder and it is associated with subtle cognitive and behavioural impairments266819801987CNPQ - Conselho Nacional de Desenvolvimento Científico e TecnológicoFAPEMIG - Fundação de Amparo à Pesquisa Do Estado De Minas GeraisFAPESP – Fundação de Amparo à Pesquisa Do Estado De São Paulosem informaçãosem informação2013/01766-