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Rapidly Progressive Corticobasal Degeneration Mimicking Brainstem Encephalitis
João Moura1, Vanessa Oliveira1, Ana Sardoeira1
1Department of Neurology Centro Hospitalar Universitário do Porto Porto Portugal.
Movement Disorders Clinical Practice
|February 24, 2023
Summary
Rapidly progressive corticobasal degeneration (RP-CBD) can mimic brainstem encephalitis. Neuropathology confirmed RP-CBD in a patient initially misdiagnosed, highlighting the importance of considering tauopathies in atypical neurological presentations.
Area of Science:
- Neurology
- Neuroscience
- Pathology
Background:
- Corticobasal degeneration (CBD) can present with a rapidly progressive (RP) course.
- This RP-CBD presentation can mimic other neurological disorders, leading to diagnostic challenges.
Purpose of the Study:
- To detail a neuropathologically confirmed case of rapidly progressive corticobasal degeneration (RP-CBD).
- To illustrate how RP-CBD can be initially misdiagnosed as immune-mediated brainstem encephalitis.
Main Methods:
- Retrospective analysis of electronic medical records and authorized video materials.
- Neuropathological examination of brain tissue.
Main Results:
- A 51-year-old man initially diagnosed with immune-mediated brainstem encephalitis showed progressive cognitive decline, asymmetric parkinsonism, and brainstem abnormalities.
- Despite treatment for encephalitis, his condition worsened, and postmortem examination revealed a 4-repeat tauopathy consistent with CBD and extensive brainstem involvement.
Conclusions:
- Rapidly progressive corticobasal degeneration can present with symptoms mimicking brainstem encephalitis.
- The severity of brainstem and upper spinal cord pathology observed in postmortem examination correlated with the patient's clinical and imaging findings.
