Central nervous system (CNS) involvement is an extremely rare manifestation in eosinophilic
granulomatosis with polyangiitis (EGPA), associated with a poor prognosis. Here we
present a case of 50-year-old female patient with long-term asthma treatment who presented
initially with extreme eosinophilia (56%) and severe progressive ascending paresis,
similar to Guillain–Barré syndrome, leading to tetraplegia. After navigating through
diagnostic mazes, the diagnosis of EGPA was established based on eosinophilia, myeloperoxidase
anti-neutrophil cytoplasmic antibody (MPO-ANCA) positivity, asthma, eosinophil granulomatosis
in the gastrointestinal tract, and severe peripheral nervous system involvement, complicated
with rare central nervous granulomas and ischemia. With combined immunosuppressive
and immunomodulatory treatment including high-dose corticosteroids, rituximab and
intravenous immunoglobulin along with symptomatic treatment and planned rehabilitation
over 6 months, our patient recovered gradually from tetraplegia and adverse events
such as severe infections and osteoporotic fractures. Now, from a 2-year perspective,
we can conclude a successful treatment leading to decrease in all of her symptoms.
Due to persistent eosinophilia after steroid tapering, she was switched to mepolizumab
maintenance treatment and demonstrated continuous improvement of motor and sensory
functions. Thanks to periodically repeated rehabilitation, she became self-sufficient
and returned to her previous job. Our case highlights that EGPA patients should be
treated in a center of expertise due to the rarity of the disease and complexity of
diagnosis and treatment. Careful multidisciplinary cooperation, the huge effort of
the patient, and a supportive environment can show a way back from immune-mediated
tetraplegia.
