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Arch Dis Child. 1975 December; 50(12): 927–932.
PMCID: PMC1545841

Familial dysequilibrium-diplegia with T-lymphocyte deficiency.


A second family is described with a combination of defective thymus-dependent immunity and cerebral palsy. The cerebral palsy comprised nonprogressive dysequilibrium and mild spastic diplegia without limb ataxia. This genetic entity of presumed autosomal recessive inheritance is clearly distinguished from ataxia-telangiectasia. Immunological abnormalities should be sought in other familial or unexplained cerebral palsy syndromes.

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Selected References

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  • Cleveland WW, Fogel BJ, Brown WT, Kay HE. Foetal thymic transplant in a case of Digeorge's syndrome. Lancet. 1968 Dec 7;2(7580):1211–1214. [PubMed]
  • Foroozanfar N, Yamamura M, Watson G, Weaver P, Belton EM, Hobbs JR, Lawler S. Successful thymus graft for T-cell deficiency in a 6-year-old boy. Br Med J. 1975 Feb 8;1(5953):314–315. [PMC free article] [PubMed]
  • Graham-Pole J, Willoughby ML, Aitken S, Ferguson A. Immune status of children with and without severe infection during remission of malignant disease. Br Med J. 1975 May 31;2(5969):467–470. [PMC free article] [PubMed]
  • Hagberg B, Hansson O, Lidén S, Nilsson K. Familial ataxic diplegia with deficient cellular immunity. A new clinical entity. Acta Paediatr Scand. 1970 Sep;59(5):545–550. [PubMed]
  • Moore MJ, Dikkes P, Reif AE, Romanul FC, Sidman RL. Localization of theta alloantigens in mouse brain by immunofluroescence and cytotoxic inhibition. Brain Res. 1971 May 7;28(2):283–293. [PubMed]
  • Terplan KL, Krauss RF. Histopathologic brain changes in association with ataxia-telangiectasia. Neurology. 1969 May;19(5):446–454. [PubMed]

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