BACKGROUND: Herpes zoster meningitis and meningoencephalitis, rare complications of herpes zoster, may follow the onset of rash, precede it, or even occur without rash. There have been few studies describing the order of occurrence of neurological symptoms and zoster. OBJECTIVE: We compared the clinical and laboratory features, treatments, and progress of the conditions among patients with herpes zoster meningitis and meningoencephalitis according to the timing of zoster onset in relation to neurological symptoms and also according to the immune status. METHODS: We performed a retrospective review of 21 patients with meningitis and 3 patients with meningoencephalitis who showed the characteristic rash of herpes zoster at National Health Insurance Service Ilsan Hospital between March 2000 and June 2015. RESULTS: The 11 patients in whom zoster preceded neurological symptoms had a shorter duration of neurological symptoms (median 3 days; range 1~10) than did the 13 patients who experienced neurological symptoms first (median 10 days; range 4~27) (p<0.05). The intervals between the onset of neurological symptoms and a) presentation to the hospital, b) cerebrospinal fluid tests, and c) intravenous acyclovir treatment were all shorter in the patients with zoster preceding neurological symptoms (p<0.05). No significant differences in age or immune status were observed between the two groups. In the 9 immunocompromised patients, higher incidence of meningoencephalitis and neurologic complications was noted in comparison to the 15 immunocompetent patients (p<0.05). CONCLUSION: Antecedent zoster rash in varicella zoster virus-induced meningitis and meningoencephalitis appears to lead to more rapid initiation of diagnostic tests and antiviral treatment. The possibility of central nervous system complications should not be overlooked even in patients with herpes zoster.
Acyclovir ; Central Nervous System ; Cerebrospinal Fluid ; Chickenpox* ; Diagnostic Tests, Routine ; Exanthema ; Herpes Zoster ; Herpesvirus 3, Human* ; Humans ; Immunocompetence ; Immunocompromised Host ; Incidence ; Meningitis* ; Meningoencephalitis* ; National Health Programs ; Retrospective Studies

Background: Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematologic disorder characterized by uncontrolled terminal complement activation. Eculizumab, a monoclonal antibody C5 inhibitor was introduced in Korea in 2009 and has been the standard treatment option for PNH. Methods: This study assessed the long-term efficacy/safety of eculizumab in PNH using real-world data from the Korean Health Insurance Review and Assessment Service. Eighty patients who initiated eculizumab from 2009–2020 were enrolled. Results: At eculizumab initiation, the median age was 51.5 years, lactate dehydrogenase (LDH) 6.8 × upper limit of normal, and granulocyte clone size 93.0%. All patients had at least one PNH-related complication before eculizumab initiation, including renal failure (n = 36), smooth muscle spasm (n = 24), thromboembolism (n = 20), and pulmonary hypertension (n = 15). The median (range) duration of eculizumab treatment was 52.7 (1.0, 127.3) months (338.6 total treated patient-years). Despite high disease activity in the study population before treatment initiation, overall survival was 96.2% and LDH levels were stabilized in most patients during treatment. PNH-related complications at treatment initiation were resolved in 44.4% of patients with renal failure, 95.8% with smooth muscle spasm, 70.0% with thromboembolism, and 26.7% with pulmonary hypertension. Extravascular hemolysis occurred in 28.8% of patients (n = 23; 0.09 per patient-year) and breakthrough hemolysis in 18.8% (n = 15; 0.06 per patient-year). No treatment discontinuation cases related to eculizumab were observed. Conclusion These data provided evidence for the long-term efficacy and safety of eculizumab in Korean PNH patients with high disease burdens.