HERPES-SIMPLEX VIRUS-1 AND VARICELLA VIRUS-INFECTIONS IN FAMILIAL DYSAUTONOMIA PATIENTS

Familial dystautonomia (FD) patients are deficient in type C fibers, s uggesting that there may be a different pattern of infection and clini cal presentation when infected by Herpes simplex virus type 1 (HSV-1) or Varicella-Zoster virus (VZV). These viruses infect and are reactiva ted in the periphery of the body through type C sensory nerve fibers. HSV-1 infects epithelial cells, penetrates into type C fibers, and mig rates to the ganglia to generate latent infection. In reactivation, th e viral DNA migrates through type C fibers, infecting the epidermis at the entry site. VZV infects through the respiratory tract, causing sy stemic viral infection and latency in the ganglia, from which it is re activated and reaches the skin. The study was carried by clinical ques tionnaire and by HSV and VZV IgG antibodies on fifty-one FD patients a nd eighty matched controls. The questionnaire revealed that no FD pati ent had a history of clinical HSV-1 infection, compared to 15% in the control group (P < 0.05), while 50% FD patients had been infected by v aricella, compared to 66% in the VZV control group. However in FD, VZV clinical manifestations were mild in comparison to controls. There wa s no difference in infection rates for some other viral diseases. HSV- 1 antibodies were detected in 24% of the FD patients, compared to 38% in the control group (P < 0.1). VZV antibodies were similar in FD and controls (66%, 63%). We concluded that the rate of HSV infection in FD is low and clinical reactivation is rare. The rate of varicella infec tion appears to be the same for patients and controls, but in FD the c linical presentation is mild. We suggest that these differences are du e to the lack of type C fibers in FD patients. (C) 1998 Wiley-Liss, In c.