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Recurrent Meningitis in a Patient With Congenital Deficiency of the C9 Component of ComplementFirst Case of C9 Deficiency in Europe
Michele Zoppi, MD;
Matthias Weiss, MD;
Urs E. Nydegger, MD;
Tino Hess, MD;
PeterJ. Späh, PhD
Arch Intern Med. 1990;150(11):2395-2399.
Abstract
• We describe the first cases, to our knowledge, of C9 deficiency in Europe that were detected in a Swiss family, of which two members—one with a complete deficiency and the other with approximately half-normal C9 levels—experienced bacterial meningitis. The index patient, a 56-year-old white man with a history of purulent meningitis at the age of 23 years, presented with an acute meningococcal meningitis. No impairment of cellular immunity or immunoglobulin deficiency could be found. Complement assays showed a complete deficiency of the C9 component, while the other individual component levels were normal and the hemolytic activity (measured using the CH50 assay) was only slightly reduced. A family study revealed complete C9 deficiency in the patient's healthy brother and half-normal C9 concentrations in his sister, his son (who also had experienced an episode of bacterial meningitis), and his niece, consistent with an inherited C9 deficiency. This first case of recurrent meningitis in a white patient with complete C9 deficiency suggests that this complement defect may also be a risk factor for bacterial, especially neisserial, infections.
(Arch Intern Med. 1990;150:2395-2399)
Author Affiliations
From the Medical Division, Anna Seiler-Haus (Drs Zoppi, Weiss, and Hess), and the Hematological Central Laboratory (Dr Nydegger), Inselspital, and the Central Laboratory, Blood Transfusion Service Swiss Red Cross (Dr Späth), Bern, Switzerland.
Footnotes
Accepted for publication November 27,1989.
Presented in part as an abstract at the Second Meeting on Complement in Human Disease, Bari, Italy, September 19,1988.
Reprint requests to the Central Laboratory, Blood Transfusion Service Swiss Red Cross, CH-3000 Bern-22, Switzerland (Dr Späth).
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