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Case report| Volume 27, ISSUE 1, P66-69, January 2005

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Symmetrical thalamic calcifications in a monozygotic twin: case report and literature review

      Abstract

      We report the occurrence of symmetrical thalamic calcifications (STC) in one of a pair of monozygotic twins born at term without evidence of pre- or peri-natal asphyxia. STC is known to be an extremely rare condition in infants. Judging from the few cases reported in the literature, the clinical presentation is very severe: low Apgar score, no spontaneous movements, spasticity or marked hypotonia, impaired suck and swallow, facial diplegia. The prognosis is also very poor. The etiology is still a matter of debate: genetic, infectious, toxic or hypoxic-ischemic insults have been hypothesized. In our case, the presence of the lesion in one of a pair of monozygotic twins would rule out any genetic origin, nor was there any evidence of toxic or infectious disease. The only potential risk factor for fetal damage was hypoxic-ischemic insult related to the twin pregnancy.

      Keywords

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      References

        • Abuelo D.N.
        • Barsel-Bowers G.
        • Tutschka B.G.
        • Ambler M.
        • Singer D.B.
        Symmetrical infantile thalamic degeneration in two sibs.
        J Med Genet. 1981; 18: 448-450
        • Jennekens F.G.
        • Barth P.G.
        • Fleury P.
        • Veldman H.
        • Keuning J.F.
        • Westdorp J.
        Axonal dystrophy in a case of connatal thalamic and brain stem degeneration.
        Acta Neuropathol. 1984; 64: 68-71
        • Di Mario F.J.
        • Clancy R.
        Symmetrical thalamic degeneration with calcifications of infancy.
        Am J Dis Child. 1989; 143: 1056-1060
        • Eicke M.
        • Briner J.
        • Willi U.
        • Uehlinger J.
        • Boltshauser E.
        Symmetrical thalamic lesions in infants.
        Arch Dis Child. 1992; 67: 15-19
        • Bordarier C.
        • Moktari M.
        • Rodriguez D.
        • Adamsbaum C.
        • Robain O.
        Antenatal thalamic lesions in the newborn: two anatomoclinical cases.
        Brain Dev. 1997; 19: 495-498
        • van der Knaap M.S.
        • Barth P.G.
        Discordant infantile encephalopathy with symmetrical thalamic calcifications in identical twins.
        Am J Med Genet. 1994; 52: 218-222
        • Volpe J.J.
        Neurology of the newborn. 3rd ed. Saunders, Philadelphia, PA1999
        • Voit T.
        • Lemburg P.
        • Neuen E.
        • Lumenta C.
        • Stork W.
        Damage of thalamus and basal ganglia in asphyxiated full-term neonates.
        Neuropediatrics. 1987; 18: 176-181
        • Leech R.W.
        • Alvord E.C.
        Anoxic-ischemic encephalopathy in the human neonatal period. The significance of brain stem involvement.
        Arch Neurol. 1977; 34: 109-113
        • Larroche J.C.
        • Droullè P.
        • Delezoide A.L.
        • Narcy F.
        • Nessmann C.
        Brain damage in monozygous twins.
        Biol Neonate. 1990; 57: 261-278