Syndrome is characterized by pseudobulbar palsy,
epilepsy, cognitive deficits, and bilateral perisylvian malformations on
Congenital Bilateral Perisylvian Syndrome.
Unknown. Recently recognized following advances in
neuroimaging techniques as a distinct syndrome.
Autosomal recessive; male:female ratio is
Unknown. Disorder of neuronal migration.
Suspected by clinical picture and supported by classical
MRI findings. These are radiological indications of bilateral perisylvian cortical
malformations consistent with polymicrogyria involving the sylvian fissure and opercular
Pseudobulbar palsy, dysarthria, mental
retardation (85% of patients) and cognitive deficits, and seizures
(87% of patients) that are atypical and difficult to control by
medication (55% of patients). Infantile spasms in the first 6 months of
life are often the presenting seizure type. Hypotonia, hemiparesis, apneas.
Pharyngeal and masticatory muscle diplegia, especially tongue. Micrognathia,
Full assessment of the airway,
oromotor function, and degree of pseudobulbar palsy is essential.
Preparation for prolonged intubation should be made if appropriate. Seizure
type, frequency, and control needs to be reviewed by neurology and
optimized. Medication should be continued preoperatively. Assessment of
other neurological deficits should be documented. Evidence of acute and
chronic effects of aspiration should be looked for and a chest radiograph
The potential for gastric regurgitation, recurrent
pulmonary aspiration and irritation with ventilation (restrictive disease) must be
considered. Mouth opening and tongue movement are likely to be significantly impaired. Use
of an antisialagogue to reduce secretions is helpful preinduction. Induction of
anesthesia should be either by spontaneous ventilating inhalational technique or rapid
sequence induction with cricoid pressure once face-mask ventilation has been
established. Seizures should be considered as a cause of delay in emergence. Extubation
should only occur once the child is fully awake; the child may need intubation until
fully recovered. Apneas are more frequent postoperatively and appropriate monitoring
should be used. The need for sustained mechanical ventilation postoperatively must be
considered because of the pseudobulbar palsy.
Use of nondepolarizing agents should
be delayed until the airway has been secured and lung ventilation confirmed. Avoid drugs
that may trigger seizures, such as enflurane and sevoflurane, and consider interactions
with antiseizure medication. Caution with opioids if there is a history of apnea.
Kuzniecky R, Andermann F, Guerrini R: Congenital bilateral perisylvian
syndrome: Study of 31 patients. The CBPS Multicenter Collaborative Study.
Lancet 341(8845):608, 1993.
Kuzniecky R, Andermann F, Guerrini R: Infantile spasms: An early epileptic
manifestation in some patients with the congenital bilateral perisylvian
syndrome. J Child Neurol
Gropman AL, Barkovich AJ, Vezina LG et al: Pediatric congenital bilateral
perisylvian syndrome: Clinical and MRI features in 12 patients.
Neuropediatrics 28(4):198, 1997.