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Published on: May 19, 2020
Cerebral vasculitis as a severe complication of acute infectious meningoencephalitis-a case series of five different
Sonja Hochmeister1, Michaela Tanja Haindl2, Alexander Pichler2
1Department of Neurology, Medical University Graz, Graz, Austria. sonja.hochmeister@medunigraz.at.
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.
Insights
Infectious cerebral vasculitis, a rare complication of central nervous system infections, often leads to delayed diagnosis and poor outcomes. This study highlights the diverse causative agents and the critical need for timely intervention in managing this severe condition.
Area of Science:
- Neurology
- Infectious Diseases
- Vascular Neurology
Background:
- Acute infectious meningoencephalitis is a severe central nervous system (CNS) infection.
- Cerebral vasculitis with ischemic infarcts is a rare but serious complication.
- Existing literature is limited, primarily case reports, hindering diagnosis.
Purpose of the Study:
- To analyze risk factors and treatment options for fulminant infectious vasculitis with ischemic strokes.
- To investigate five cases treated in a neurological intensive care unit.
- To contribute to the understanding of this challenging neurological condition.
Main Methods:
- Retrospective analysis of five cases of infectious vasculitis with ischemic strokes.
- Data collected from 2017 to 2023 at the Medical University of Graz, Austria.
- Focus on neurological intensive care unit (ICU) patient data.
Main Results:
- Identified causative agents: Streptococcus pneumoniae, VZV, HHV-6, Borrelia burgdorferi, Cryptococcus neoformans.
- Patients aged 49-73 years; diagnosis delayed 6-60 days; fatal outcome in 4 out of 5 cases.
- Reported a unique fatal case of HHV-6 encephalitis and vasculitis in an immunocompetent individual.
Conclusions:
- Infectious cerebral vasculitis can be caused by various agents and affect patients unexpectedly.
- Risk factors include immunocompromised state, delayed diagnosis/treatment, and age-related vascular changes.
- Early diagnosis and treatment are crucial; antibiotic resistance and post-infectious infarcts are considerations.
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