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Acute flaccid weakness with myelopathy and peripheral nerve involvement in 2 children: Recent characterization of a
Gemma Olive1, Antonia Clarke2, Katja Doerholt3
1Children's Neurosciences, Evelina London Children's Hospital, Kings Health Partners Academic Health Science Centre, UK.
Insights
Acute flaccid weakness in infants can signal central nervous system inflammation or peripheral nerve disease. Early diagnosis requires integrated clinical, imaging, and nerve study assessments for effective management of these overlapping syndromes.
Area of Science:
- Neurology
- Pediatrics
- Immunology
Background:
- Acute flaccid weakness can be an initial symptom of acute transverse myelitis (ATM), an immune-mediated central nervous system disorder.
- It may also indicate anterior horn cell syndrome or peripheral nervous system disease.
Observation:
- Two previously healthy female infants presented with acute flaccid paralysis and encephalopathy.
- Neuroimaging showed central cord signal abnormalities in both infants.
- Electrophysiological studies unexpectedly revealed a generalized axonal motor neuropathy.
Findings:
- The cases highlight overlapping inflammatory syndromes affecting both central and peripheral nervous systems in infants.
- Combined neuroimaging and electrophysiological findings suggest a complex neurological presentation.
Implications:
- Integrated clinical, radiological, and neurophysiological assessments are crucial for diagnosing and managing children with these complex neurological conditions.
- Understanding these overlapping syndromes is vital for timely and appropriate therapeutic interventions in pediatric neurology.
Background:
Acute flaccid weakness may be the first presentation of acute transverse myelitis (ATM), an immune-mediated central nervous system disorder or may be the first presentation of anterior horn cell syndrome or peripheral nervous system disease.
Case Reports:
We describe two previously healthy female infants who presented with acute flaccid paralysis and encephalopathy. Neuroimaging revealed central cord signal changes in both cases and surprisingly electrophysiological studies performed revealed a generalized axonal motor neuropathy as well.
Conclusion:
Clinical, radiological and neurophysiological assessment are important to aid in the diagnosis and subsequent management of children with overlapping inflammatory peripheral and central nervous system syndromes.
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