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Encephalopathy associated with haemophagocytic lymphohistiocytosis following rotavirus infection
S Takahashi1, J Oki, A Miyamoto
1Department of Paediatrics, Asahikawa Medical College, Japan. staka@ncnaxp.ncnp.go.jp
Insights
A rare case of haemophagocytic lymphohistiocytosis (HLH) associated encephalopathy in a child following rotavirus infection showed neurological recovery with treatment. This highlights the importance of considering HLH in acute neurological conditions.
Area of Science:
- Pediatric Neurology
- Immunology
- Infectious Diseases
Background:
- Haemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome.
- Encephalopathy can be a severe neurological manifestation of HLH.
Observation:
- A 2-year-old boy developed encephalopathy with coma, seizures, and spastic quadriplegia post-rotavirus infection.
- Cerebrospinal fluid (CSF) and serum interferon-gamma levels were elevated.
- Brain MRI showed white matter abnormalities and volume loss; proton MR spectroscopy revealed elevated lactate.
Findings:
- The patient recovered neurologically with treatment including steroids, etoposide, and cyclosporin A.
- Elevated interferon-gamma normalized with clinical remission.
- White matter abnormalities and brain lactate resolved, suggesting reversible CNS immune response.
Implications:
- HLH-associated encephalopathy may result from cytokine overproduction and local immune responses in the CNS.
- Early consideration of HLH in acute encephalopathy is crucial for timely diagnosis and treatment.
- Understanding the CNS immune response in HLH can guide therapeutic strategies.
Unlabelled:
A 2-year-old Japanese boy with a haemophagocytic lymphohistiocytosis (HLH) associated encephalopathy which developed after rotavirus infection is described. The neurological symptoms consisted of coma, seizures and spastic quadriplegia. On therapy with steroids, etoposide and cyclosporin A, the patient recovered without any neurological deficits. The interferon-gamma levels in serum and CSF were elevated at onset of the disease but had returned to normal at the time of clinical remission. Brain MRI revealed diffuse white matter abnormalities and parenchymal volume loss. Proton magnetic resonance spectroscopy revealed elevated lactate in the abnormal lesions observed on MRI, indicating that macrophages not exhibiting aerobic metabolism had infiltrated the CNS. At the time of clinical remission, the white matter abnormalities and brain lactate had disappeared. These findings suggested that the neurological symptoms resulted from the overproduction of cytokines by activated T-cells and macrophages. The pathophysiology of a HLH associated encephalopathy was considered to be a local immune response within the CNS, because interferon-gamma can induce the expression of major histocompatibility complex class I and II antigens on glial cells in the CNS.
Conclusion:
Haemophagocytic lymphohistiocytosis associated encephalopathy should be considered early in the differential diagnosis of cases with acute onset neuropathy.