RT Book, Section
A1 Bissonnette, Bruno
A1 Luginbuehl, Igor
A1 Marciniak, Bruno
A1 Dalens, Bernard J.
SR Print(0)
ID 58082701
T1 Pelizaeus-Merzbacher Syndrome
T2 Syndromes: Rapid Recognition and Perioperative Implications
YR 2006
FD 2006
PB The McGraw-Hill Companies
PP New York, NY
SN 9780071354554
LK accessanesthesiology.mhmedical.com/content.aspx?aid=58082701
RD 2024/04/24
AB A  very  rare,  slowly  progressive  dysmyelinating  disease  affecting,  in  a  diffuse  pattern,  the  cerebrum,  cerebellum,  brainstem,  and  spinal  cord.  Two  types  are  described:  X-linked  (infantile  form)  and  the  autosomal  dominant  (preadulthood  form).  Clinical  features  also  include  stridor,  muscle  spasticity,  and  nystagmus.  Often  fatal  in  the  first  year  of  life  from  respiratory  complications.