Background <p>Acute infectious meningoencephalitis is a&#xa0;severe condition caused by bacterial, viral, fungal, or parasitic infections of the central nervous system (CNS). A&#xa0;rare but severe complication is cerebral vasculitis with ischemic infarcts. The available literature is sparse and mainly restricted to case reports, further challenging an accurate and timely diagnosis.</p> Methods <p>We provide a&#xa0;detailed retrospective analysis of five cases of fulminant infectious vasculitis accompanied by ischemic strokes treated at the neurological intensive care unit (ICU) of the Department of Neurology, Medical University of Graz, Austria, from 2017 to 2023 to determine risk factors and possible treatment options.</p> Results <p>We report one case each of CNS vasculitis associated with an infection with <i>Streptococcus pneumoniae</i>, varicella zoster virus (VZV), human herpesvirus&#xa0;6 (HHV-6), <i>Borrelia burgdorferi</i>, and <i>Cryptococcus neoformans</i>, each resulting in devastating disseminated ischemic infarcts. The described patients were between 49 and 73&#xa0;years old, with a&#xa0;male to female ratio of 3:2. The final diagnosis was unfortunately delayed in all cases, ranging from 6–60&#xa0;days after symptom onset. The outcome was fatal in four of the cases.</p> Conclusion <p>Infectious cerebral vasculitis can occur irrespective of the causative agent’s nature. Known typical risk factors are an immunocompromised state of the patient; delayed diagnosis and treatment; and the presence of age-related vascular changes, although this complication can also occur completely unexpectedly. Infarcts may even occur in the weeks following the infection. In case of an infection with <i>Streptococcus pneumoniae</i>, also the possibility of antibiotic resistance must increasingly be kept in mind. To our knowledge, we are the first to report fatal HHV‑6 encephalitis and vasculitis in a&#xa0;previously healthy 49-year-old male treated with oral steroids for alopecia areata without any history of solid organ or stem cell transplantation.</p>

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Cerebral vasculitis as a severe complication of acute infectious meningoencephalitis—a case series of five different pathogens

  • Sonja Hochmeister,
  • Michaela Tanja Haindl,
  • Alexander Pichler,
  • Julia Pointinger,
  • Kujtim Bytyqi,
  • Stefan L. Leber,
  • Juan-Jose Archelos

摘要

Background

Acute infectious meningoencephalitis is a severe condition caused by bacterial, viral, fungal, or parasitic infections of the central nervous system (CNS). A rare but severe complication is cerebral vasculitis with ischemic infarcts. The available literature is sparse and mainly restricted to case reports, further challenging an accurate and timely diagnosis.

Methods

We provide a detailed retrospective analysis of five cases of fulminant infectious vasculitis accompanied by ischemic strokes treated at the neurological intensive care unit (ICU) of the Department of Neurology, Medical University of Graz, Austria, from 2017 to 2023 to determine risk factors and possible treatment options.

Results

We report one case each of CNS vasculitis associated with an infection with Streptococcus pneumoniae, varicella zoster virus (VZV), human herpesvirus 6 (HHV-6), Borrelia burgdorferi, and Cryptococcus neoformans, each resulting in devastating disseminated ischemic infarcts. The described patients were between 49 and 73 years old, with a male to female ratio of 3:2. The final diagnosis was unfortunately delayed in all cases, ranging from 6–60 days after symptom onset. The outcome was fatal in four of the cases.

Conclusion

Infectious cerebral vasculitis can occur irrespective of the causative agent’s nature. Known typical risk factors are an immunocompromised state of the patient; delayed diagnosis and treatment; and the presence of age-related vascular changes, although this complication can also occur completely unexpectedly. Infarcts may even occur in the weeks following the infection. In case of an infection with Streptococcus pneumoniae, also the possibility of antibiotic resistance must increasingly be kept in mind. To our knowledge, we are the first to report fatal HHV‑6 encephalitis and vasculitis in a previously healthy 49-year-old male treated with oral steroids for alopecia areata without any history of solid organ or stem cell transplantation.