Abstract | BACKGROUND: DISCUSSION: This condition is usually documented in the Genetics' literature but it has been scarcely disseminated among neurosurgeons. Sudden death of uncertain origin has been reported in children with M-CMTC. Hydrocephalus and tonsillar herniation often occur in this syndrome. The appearance of symptoms and signs of intracranial hypertension or of brain stem compression in M-CMTC patients may herald the development of irreversible neurological damage or death. ILLUSTRATIVE CASES: CONCLUSIONS: Given the high incidence of hydrocephalus and/or tonsillar herniation that occurs in M-CMTC, serial neuroimaging studies and neurosurgical referral seem to be warranted in children with this syndrome.
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Authors | Juan F Martínez-Lage, Encarna Guillén-Navarro, María-José Almagro, Matías Felipe-Murcia, Antonio López López-Guerrero, Marcelo Galarza |
Journal | Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
(Childs Nerv Syst)
Vol. 26
Issue 1
Pg. 13-8
(Jan 2010)
ISSN: 1433-0350 [Electronic] Germany |
PMID | 19763591
(Publication Type: Case Reports, Journal Article)
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Topics |
- Abnormalities, Multiple
(pathology, surgery)
- Arnold-Chiari Malformation
(pathology, surgery)
- Brain
(abnormalities, pathology)
- Encephalocele
(pathology, surgery)
- Female
- Humans
- Hydrocephalus
(pathology, surgery)
- Infant
- Infant, Newborn
- Magnetic Resonance Imaging
- Male
- Treatment Outcome
- Ventriculoperitoneal Shunt
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