Abstract
A patient with an unusual association between tuberous sclerosis complex and hemimegalencephaly
is reported. At 2 days of age, CT scan disclosed right hemimegalencephaly with periventricular
faint calcification. At 1 month of age, magnetic resonance imaging showed right hemimegalencephaly
with T1 and T2 shortening. The right frontal and parietal lobes were compatible with
transmantle dysplasia. At 6 months of age, the right hemisphere had decreased in size.
Atrophic changes continued to progress until 3 years of age. Periventricular calcification
in the right hemisphere became marked with age. The patient developed frequent partial
motor seizures from 6 weeks of age and infantile spasms from 5 months of age. Electroencephalogram
revealed low amplitude of background activities in the right frontal area. The cause
of this atrophic change in the hemimegalencephalic hemisphere, which could be a hamartomatous
lesion, remains unknown.
Keywords
To read this article in full you will need to make a payment
Purchase one-time access:
Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online accessOne-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:
Subscribe to Brain and DevelopmentAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- Hemimegalencephaly: part 1. Genetic, clinical, and imaging aspects.J Child Neurol. 2002; 17: 373-384
- Unilateral megalencephaly and tuberous sclerosis: related disorders?.J Child Neurol. 1994; 9: 443-446
- Hemimegalencephaly and focal megalencephaly in tuberous sclerosis complex.Am J Neuroradiol. 1998; 19: 1935-1938
- Hemimegalencephaly in tuberous sclerosis complex.J Child Neurol. 2002; 17: 677-680
- Hemimegalencephaly with tuberous sclerosis: a longitudinal imaging study.Australas Radiol. 2003; : 438-442
- CT and MR imaging of cerebral tuberous sclerosis.Brain Dev. 1998; : 209-221
- MR imaging of tuberous sclerosis in neonates and young infants.Am J Neuroradiol. 1999; : 907-916
- Hemimegalencephaly: a longitudinal MR study.Am J Neuroradiol. 1994; : 1479-1482
- Diagnostic criteria: tuberous sclerosis complex. Report of the diagnostic criteria committee of the National Tuberous Sclerosis Association.J Child Neurol. 1992; : 221-224
- Focal transmantle dysplasia: a specific malformation of cortical development.Neurology. 1997; : 1148-1152
- Unilateral megalencephaly: correlation of MR imaging and pathological characteristics.Am J Neuroradiol. 1990; : 523-531
Article info
Publication history
Accepted:
November 22,
2004
Received in revised form:
November 1,
2004
Received:
September 3,
2004
Identification
Copyright
© 2004 Elsevier B.V. Published by Elsevier Inc. All rights reserved.
