[Case report Brown-Vialetto-Van laere syndrome].
A case of a young man (36 years old) suffering from a progressive bulbo-pontine palsy, bilateral central deafness and respiratory failure, clinically known as Brown-Vialetto-Van Laere syndrome (BVVL) is reported. The illness occurred at 24 years and, after a period of quiescence of 12 years, led to a serious dependence from mechanical ventilation. In tyhis report the most important points of view about resuscitation and intensive treatment, such as neuromuscular failure and locked-in condition, are discussed.
Introini
S
S
Servizio di Anestesia Rianimazione e Terapia Intensiva, Ospedale Vallecamonica, Esine (Brescia), Italy.
Sasso
G M
GM
Moioli
G
G
Morandini
W L
WL
D000328:Adult; D001921:Brain / Q000473:pathology; D010244:Bulbar Palsy, Progressive / Q000473:pathology* / Q000628:therapy; D006313:Hearing Loss, Central / Q000473:pathology* / Q000628:therapy; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D012121:Respiration, Artificial; D012131:Respiratory Insufficiency / Q000473:pathology* / Q000628:therapy; D013577:Syndrome