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Acanthamoeba rhombencephalomyelitis: Novel white matter tract-based neuroinvasive pattern in a retrospective
Remya Prakash1, Aravind Reghukumar2, K Rammohan1
1Department of Neurology, Government Medical College, Thiruvananthapuram, Kerala, India.
Objective:
To describe a distinct rhombencephalomyelitis pattern in patients with central nervous system infection due to free-living amoebae, differing from the classical granulomatous amoebic encephalitis phenotype.
Methods:
This retrospective observational study included twelve consecutive patients with brainstem and/or spinal cord involvement associated with central nervous system infection due to Acanthamoeba species at a tertiary care centre in Kerala, India. Diagnosis was confirmed by wet mount examination and amoeba species-specific 18S rRNA PCR of cerebrospinal fluid. Comprehensive evaluation excluded bacterial, mycobacterial, fungal, and autoimmune etiologies.
Results:
Twelve patients (seven men; median age 50 years; IQR: 33-60 years) presented with subacute cranial neuropathies (58.3%) and/or gait disturbance (91.7%). Seven (58.3%) were immunocompetent; only two had traditional risk factors (ritualistic nasal ablution). MR scan demonstrated involvement of the spinal cord (66.7%), pons (58.3%), middle cerebellar peduncle (50%), and medulla (41.7%). This distinctive neuroimaging pattern reflected white matter tract-based spread along cerebellar peduncles and/or corticospinal tracts. CSF analysis revealed lymphocytic pleocytosis (median: 12 cells/mm3, IQR: 5-47). with hypoglycorrhachia (CSF-to-blood glucose ratio 0.45 IQR: 0.4-0.5). Acanthamoeba spp. were confirmed by CSF PCR in ten (83.3%) and presumptively by wet mount examination in two (16.7%). Nine patients (75.0%) survived, of whom eight (66.7%) achieved favourable functional outcome (mRS 1-2).
Conclusion:
Acanthamoeba spp. causes distinct white matter tract-based non-granulomatous rhombencephalomyelitis. Neurologists should screen for free-living amoebae CNS infection even without traditional risk factors, as this pattern may mimic demyelinating disorders (NMOSD, MOGAD, ADEM, or MS). When identified early, Acanthamoeba rhombencephalomyelitis carries a favourable prognosis.
Insights
Free-living amoebae, such as Acanthamoeba species, can cause a distinct rhombencephalomyelitis in the central nervous system. Early diagnosis of this non-granulomatous infection is crucial for favorable outcomes.
Area of Science:
- Neurology
- Infectious Diseases
- Neuroimaging
Background:
- Central nervous system (CNS) infections due to free-living amoebae (FLA) typically present as granulomatous amoebic encephalitis.
- A distinct pattern of rhombencephalomyelitis caused by Acanthamoeba species has been observed, differing from classical presentations.
Purpose of the Study:
- To describe a unique rhombencephalomyelitis pattern in CNS infections caused by free-living amoebae.
- To differentiate this pattern from granulomatous amoebic encephalitis.
- To highlight the diagnostic and prognostic implications of this distinct presentation.
Main Methods:
- Retrospective observational study of twelve patients with brainstem/spinal cord involvement due to Acanthamoeba CNS infection.
- Diagnosis confirmed by cerebrospinal fluid (CSF) wet mount and 18S rRNA PCR.
- Exclusion of bacterial, mycobacterial, fungal, and autoimmune etiologies.
Main Results:
- Twelve patients (median age 50 years) presented with subacute cranial neuropathies and gait disturbance.
- MRI revealed white matter tract-based spread in the spinal cord, pons, middle cerebellar peduncle, and medulla.
- Acanthamoeba spp. confirmed by CSF PCR in 83.3% of patients; 75% survived with favorable functional outcomes.
Conclusions:
- Acanthamoeba species cause a distinct, non-granulomatous, white matter tract-based rhombencephalomyelitis.
- Neurologists should consider FLA CNS infection, even without traditional risk factors, as the pattern mimics demyelinating disorders.
- Early identification of Acanthamoeba rhombencephalomyelitis is associated with a favorable prognosis.
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