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Published on: October 29, 2014
Description of six cases of child cauda equina leptomeningitis: an emerging disease
Franca Dicuonzo1, Michele Palma, Michele Tangari
1Department of Neuroradiology, University of Bari, Bari, Italy. f.dic@neurorad.uniba.it
Insights
Child cauda equina leptomeningitis (CCEL) is an emerging aseptic meningitis affecting children. This study details six cases, highlighting lower limb weakness and diagnostic MRI findings, suggesting viral or immune-mediated causes.
Area of Science:
- Pediatric Neurology
- Infectious Diseases
- Neuroimmunology
Background:
- Child cauda equina leptomeningitis (CCEL) is an emerging aseptic meningitis affecting children aged 2-9.
- CCEL presents with prodromal symptoms followed by acute lower limb hypoasthenia, hyporeflexia, and ataxia.
Purpose of the Study:
- To describe clinical features and diagnostic findings in six pediatric cases of CCEL.
- To explore potential pathogen mechanisms of CCEL.
Main Methods:
- Case series description of six children diagnosed with CCEL.
- Spinal magnetic resonance imaging (MRI) to evaluate cauda equina and conus medullaris.
- Differential diagnosis exploration with Guillain-Barre syndrome.
Main Results:
- All patients exhibited pathological enhancement of cauda equina and conus medullaris leptomeninges on MRI.
- MRI facilitated differentiation between CCEL and Guillain-Barre syndrome.
- One case presented with generalized seizures and coma.
Conclusions:
- CCEL is a distinct pediatric neurological condition requiring careful diagnosis.
- Hypotheses for pathogenesis include direct viral damage, immunomediated mechanisms, or a combination.
- Further research is needed to elucidate the exact etiology of CCEL.
Abstract:
Child cauda equina leptomeningitis (CCEL) is a typical clinical example of aseptic meningitis with patterns of an emerging disease, and it affects children aged 2-9. Here we will describe six cases of CCEL. After the prodromes, all children underwent an acute phase with hypoasthenia of the lower limbs, hyporeflexia, staggering and ataxia with steppage. Only in one case there were generalized fits and coma of grade 1-2 too. All children underwent a spinal magnetic resonance imaging (MRI), proving pathologic enhancement of cauda equina and conus medullaris leptomeningitis. At the same time, MRI made possible the differential diagnosis between cauda equina leptomeningitis and isolated minor forms of Guillain-Barre syndrome involving the lower limbs. Three hypotheses will be formulated for understanding the pathogen mechanism(s) of CCEL. The first one is based on the presence of an immediate viral damage on the meninges, the second one, the more likely, contemplates the occurrence of an immunomediated mechanism in a host genetically prone to react in an abnormal way from an immune viewpoint. The third hyphotesis consists in a two-time damage: an early immediate damage from the virus, and a later immunomediated reaction.
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