Summary
A case of agammaglobulinaemia Bruton-type is reported, in a 5-year-old boy who, after polymyositis treated with corticosteroids, developed an encephalitis.
The observation of intranuclear inclusion bodies in Purkinje cells strongly suggests the viral origin of the encephalitis. The evolution varies at different levels in the central nervous system, viz., lesions are chronic in the brain cortex (where there are superposed anoxic changes), subacute in the brain stem and active in the cerebellar cortex.
An attempt is made to correlate immunological deficiency with viral agression:
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1.
Agammaglobulinaemia may facilitate the penetration of the virus at the respiratory mucosa level;
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2.
Prolonged treatment with corticosteroids results in cellular immunity deficiency superposed to the humoral immunity deficiency already existent, the consequence being an atypical encephalitis.
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Scaravilli, F., Coutinho, P. Encéphalite lymphocytaire dans un cas d'agammaglobulinémie. Acta Neuropathol 25, 188–195 (1973). https://doi.org/10.1007/BF00685198
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DOI: https://doi.org/10.1007/BF00685198