Syndrome characterized by cerebral hemiatrophy with
marked asymmetry of the cerebral hemispheres and characteristic changes on
skull radiograph. These changes usually are associated with facial
asymmetry, mental retardation, seizures, and contralateral hemiplegia.
Cerebral Hemiatrophy Syndrome.
Unknown. Only a small series of case reports has been
published since the first description in 1933.
Unknown. Normal chromosomal karyotypes and
amino acid excretion patterns on testing. Males and females are equally
Condition results from two different
- Primary: Caused by perinatal asphyxia with symptoms apparent soon after birth
and often resulting in severe white matter lesions and marked asymmetry of
the pyramidal tracts.
- Secondary: Caused by loss of cortical neurons with symptoms presenting later in
childhood after a generally normal early development. Syndrome may be
postictal, particularly if there is a history of early childhood febrile
seizures (multifocal neuronal loss) following a vascular insult such as
middle cerebral artery stroke or trauma (more focal abnormality).
Clinical suspicion is confirmed by the classic changes
on skull radiograph showing ipsilateral osseous hypertrophy of the calvaria
and hyperpneumatization of the paranasal sinuses and mastoid cells. This is
a result of the failure of brain growth leading to inward growth of bony
structures, resulting in increased size of the frontal sinus and elevation
of the greater wing of sphenoid and petrous ridge on the affected side. CT
and MRI scans are used to assess the etiology and extent of cerebral
parenchymal involvement. They usually reveal dilation of the ventricles and
low density of the involved cortical side. Thickening of the calvaria and
midline shift are seen in about half of cases. Temporal sclerosis is
frequent if there is a history of febrile convulsions.
Asymmetry of the face and calvaria and
contralateral hemiplegia are the main characteristics. Moderate-to-severe
mental retardation is often associated with cerebral palsy, visual
perception problems, language (slurred or immature) or speech problems
(repetitive speech patterns, simple jargon), and seizures. Patients may
present with symptoms of schizophrenia without neurologic abnormalities.
Coarctation of the midthoracic aorta has been reported.
General clinical assessment
including the ability to cooperate. Obtain a full history of seizures and
the medication and its efficacy. Look for signs of adverse effects of
antiseizure medication. Antiepileptics should be continued preoperatively.
Separation from parents may be
difficult, so sedative and anxiolytic premedication may be helpful.
Neuroaxial blockade is relatively contraindicated.
If a seizure disorder is present,
potentially epileptogenic drugs (methohexital, ketamine, enflurane,
meperidine, atracurium, and cis-atracurium) should be avoided. Some antiseizure
drugs induce the hepatic enzyme system, which may result in altered
metabolism of predominantly hepatically eliminated drugs. Emergence seizures
on emergence are not uncommon.
Russell-Silver Syndrome: Very rare genetic disorder characterized
by intrauterine growth retardation, hemihypertrophy, and asymmetry of the
head, trunk, arms, and/or legs. The extent and severity ...