Our results support the use of PLEX in severe relapses unresponsive to corticosteroids, since it was an effective and relatively safe treatment for most of our patients.

A 69-year-old Caucasian woman with a 15-year history of refractory chronic lymphocytic B-cell leukaemia (CLL), treated with alemtuzumab in the past 10 months presented with a subacute right foot drop. Initial evaluation with a brain CT scan, lumbosacral MRI, nerve conduction studies and LP was negative. In the following months, progressive right hemibody weakness and dysarthria developed. Brain MRI showed a bilateral parasagittal frontal lesion. Alemtuzumab treatment was withdrawn. Progressive multifocal leukoencephalopathy (PML) was confirmed by PCR. Attempted antiviral therapies proved fruitless. Inexorable clinical deterioration ensued and the patient passed away 10 months after the presentation. This case report intends to call attention for PML as a potential fatal complication of severe immunosuppression, including the possible role of new monoclonal antibodies (such as alemtuzumab) in its pathogenesis.

Results: Sixty-seven patients fulfilled the inclusion criteria. They were mainly Caucasian, 55 female. Median age at onset was 32.0 years and mean follow-up 7.4 ± 6.0 years. Twenty-one patients were definite NMO and optic neuritis (ON) the most frequent initial presentation. Forty-six were classified as NMO spectrum disorders. The main subtypes were recurrent ON and single longitudinally extensive transverse myelitis. Twenty-four patients had positive AQP4-IgG. Twenty-three had other circulating autoantibodies. Fifteen out of 67 patients had concomitant autoimmune disease. There was a significant correlation between the presence of autoimmune disease and the positivity for AQP4-IgG. Five patients died, all definite NMO. Conclusion: This is the first study about this rare disease in Portugal. Demographic features were similar to other studies. The existence of concomitant autoimmune disease was significantly associated with seropositivity for AQP4-IgG.

Our study suggests that cerebrovascular ultrasound is a highly specific method for the diagnosis of cerebrovascular vertigo. However, its low sensitivity makes it a poor candidate for screening.

Neuromyelitis optica spectrum disorder (NMOSD) is a rare disorder in which astrocyte damage and/ or demyelination often cause severe neurological deficits. Objective: To identify Portuguese patients with NMOSD and assess their epidemiological/clinical characteristics. Methods: This was a nationwide multicenter study. Twenty-four Portuguese adult and 3 neuropediatric centers following NMOSD patients were included. Results: A total of 180 patients met the 2015 Wingerchuk NMOSD criteria, 77 were AQP4-antibody positive

No abstract

Introdução: A cardioembolia por fibrilhação auricular assume particular destaque etiológico nas doenças vasculares cerebrais devido à sua crescente incidência, elevado risco embólico e particularidades dos eventos clínicos causados. São objectivos deste trabalho analisar a frequência da fibrilação auricular numa população de doentes com acidente vascular cerebral isquémico observados num hospital nacional, estudar o impacto vital e funcional dos acidentes vasculares cerebrais causados por diferentes etiologias, e avaliar as opções antitrombóticas prévias e posteriores ao acidente vascular cerebral. Metodologia: Realizámos um estudo observacional retrospectivo incluindo todos os doentes internados num hospital central por acidente vascular cerebral isquémico em 2010 (pelo menos um ano de seguimento). A etiologia do acidente vascular cerebral foi definida pela classificação Trial of ORG 10172 in Acute Stroke (TOAST) modificada e o resultado funcional pela escala Rankin modificada. Realizámos análise descritiva das diferentes etiologias de acidente vascular cerebral e das prescrições antitrombóticas a doentes com fibrilhação auricular. Realizámos ainda um estudo de coorte para estudar o impacto clínico das opções antitrombóticas em prevenção secundária após acidente vascular cerebral cardioembólico. Resultados: Na nossa população (n = 631) encontramos frequência de cardioembolia (34,5%) superior à relatada na literatura. Os valores de mortalidade e morbilidade além das opções terapêuticas antitrombóticas em pré e pós-Doença Vascular Cerebral são semelhantes aos de outras séries, confirmando a gravidade dos acidentes vasculares cerebrais cardioembólicos e a subutilização dos antagonistas da vitamina K. A anticoagulação oral foi eficaz em prevenção secundária independentemente do estado funcional sequelar após acidente vascular cerebral. Conclusões: Apesar das recomendações terapêuticas inequívocas a anticoagulação oral continua a ser subutilizada em prevenção de Doença Vascular Cerebral. Confirmamos a eficácia clínica dos antagonistas da vitamina K em prevenção secundária, independentemente das limitações funcionais sequelares.