Subacute sclerosing panencephalitis and hypoparathyroidism

Subacute sclerosing panencephalitis (SSPE) occurred in 3 unrelated children affected with different genetic forms of hypoparathyroidism (HPT). Clinical or laboratory evidence of an immunological deficit before SSPE onset was ascertained in cases 1 and 3. At the onset of SSPE, all cases showed signs of increased central nervous system (CNS) excitability due to poorly regulated blood calcium levels, and cases 1 and 2 had papilledema. Epidemiological evaluation suggests that the association of SSPE and HPT is not accidental. In HPT, both a subtle immunological deficit and CNS involvement may be predisposing factors of SSPE.