Case report| Volume 27, ISSUE 6, P458-461, September 2005

Longitudinal MR findings in a patient with hemimegalencephaly associated with tuberous sclerosis


      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.


      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 access
      One-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 to Brain and Development
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect


        • Flores-Sarnat L.
        Hemimegalencephaly: part 1. Genetic, clinical, and imaging aspects.
        J Child Neurol. 2002; 17: 373-384
        • Maloof J.
        • Sledz K.
        • Hogg J.F.
        • Bodensteiner J.B.
        • Schwartz T.
        • Schochet S.S.
        Unilateral megalencephaly and tuberous sclerosis: related disorders?.
        J Child Neurol. 1994; 9: 443-446
        • Griffiths P.D.
        • Gardner S.-A.
        • Smith M.
        • Rittey C.
        • Powell T.
        Hemimegalencephaly and focal megalencephaly in tuberous sclerosis complex.
        Am J Neuroradiol. 1998; 19: 1935-1938
        • Galluzzi P.
        • Cerase A.
        • Strambi M.
        • Buoni S.
        • Fois A.
        • Venturi C.
        Hemimegalencephaly in tuberous sclerosis complex.
        J Child Neurol. 2002; 17: 677-680
        • Parmar H.
        • Patkar D.
        • Shah J.
        • Patankar T.
        Hemimegalencephaly with tuberous sclerosis: a longitudinal imaging study.
        Australas Radiol. 2003; : 438-442
        • Inoue Y.
        • Nemoto Y.
        • Murata R.
        • Tashiro T.
        • Shakudo M.
        • Kohno K.
        • et al.
        CT and MR imaging of cerebral tuberous sclerosis.
        Brain Dev. 1998; : 209-221
        • Baron Y.
        • Barkovich A.J.
        MR imaging of tuberous sclerosis in neonates and young infants.
        Am J Neuroradiol. 1999; : 907-916
        • Wolpert S.M.
        • Cohen A.
        • Libenson M.H.
        Hemimegalencephaly: a longitudinal MR study.
        Am J Neuroradiol. 1994; : 1479-1482
        • Roach E.S.
        • Smith M.
        • Huttenlocher P.
        • Bha M.
        • Alcorn D.
        • Hawley L.
        Diagnostic criteria: tuberous sclerosis complex. Report of the diagnostic criteria committee of the National Tuberous Sclerosis Association.
        J Child Neurol. 1992; : 221-224
        • Barkovich A.J.
        • Kuzniecky R.I.
        • Bollen A.W.
        • Grant P.E.
        Focal transmantle dysplasia: a specific malformation of cortical development.
        Neurology. 1997; : 1148-1152
        • Barkovich A.J.
        • Chuang S.H.
        Unilateral megalencephaly: correlation of MR imaging and pathological characteristics.
        Am J Neuroradiol. 1990; : 523-531