The prevalence of narcolepsy in Catalunya (Spain).

Previous studies have estimated an overall prevalence for narcolepsy between 15 and 70 cases per 100 000 inhabitants. We aimed to estimate the prevalence of narcolepsy in Catalunya (Catalonia), a north-east region of Spain (7 424 754 inhabitants), on 31 December 2014 by identifying all living subjects diagnosed with narcolepsy. First, we identified patients diagnosed by one of the 13 sleep, paediatric or neurological departments that perform tests regularly to diagnose narcolepsy. In a second phase, we searched for additional patients with narcolepsy in a clinical database of the primary health-care system. Clinical files were reviewed and narcolepsy diagnosis validated according to the Brighton Collaboration case definitions. Three hundred and twenty-five patients had a validated diagnosis of narcolepsy in the specialized centres (mean age: 44.6 years, range: 6-89; male: 60.3%; 85% with narcolepsy type 1), including 17.8% cases in Brighton, definition level 1, 62.5% in level 2, 15.4% in level 3 and 4.3% in level 4a. The overall prevalence for narcolepsy was 4.4; 3.7 for narcolepsy type 1 and 0.7 cases per 100 000 inhabitants for narcolepsy type 2. Fifty-six additional narcoleptic patients were identified in the primary health-care system, increasing the overall prevalence to 5.2 cases per 100 000 inhabitants. Prevalence rates for narcolepsy type 1 increased from childhood to adulthood, but in subjects aged more than 50 years there was a substantial drop in prevalence rates, suggesting the presence of a significant pool of undiagnosed cases in this population. Narcolepsy can be considered a rare neurological disorder in Catalunya.

Sordera súbita. Hallazgos en la hemorragia coclear / Sudden deafness: findings in cochlear hemorrhage

La sordera súbita debida a hemorragia laberíntica es una entidad clínica relativamente poco frecuente, y puede ser el único hallazgo demostrable en el estudio de neuroimagen de la sordera súbita, bien como hecho aislado o ligado a una laberintitis, en forma de hiperintensidad laberíntica en secuencias potenciadas en T1 de la resonancia magnética (RM), sin objetivarse refuerzo laberíntico tras la administración de contraste. Presentamos un caso en el que la hemorragia coclear produjo una sordera súbita en una mujer de 22 años previamente asintomática. Revisamos la bibliografía y evaluamos el papel de la existencia de hiperintensidad en los estudios de RM craneal

Sudden deafness owing to labyrinth hemorrhage is a relatively rare clinical entity; labyrinth hemorrhage may be the only demonstrable finding in neuroimaging studies performed for sudden deafness, whether as an isolated finding or associated with labyrinthitis, manifesting as labyrinth hyperintensity in T1-weighted MRI sequences without enhancement after contrast administration. We present a case in which cochlear hemorrhage caused sudden deafness in a previously asymptomatic 22-year-old woman. We review the literature and evaluate the role of the presence of hyperintensity in cranial MRI