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Case Reports
. 2022 Aug 15;8(8):e10198.
doi: 10.1016/j.heliyon.2022.e10198. eCollection 2022 Aug.

The first pediatric anti-lactosylceramide antibody-positive encephalomyeloradiculoneuropathy

Akiko Sasaki  1 , Itaru Hayakawa  1 , Tatsuro Mutoh  2 , Yuichi Abe  1
Affiliations
Case Reports

The first pediatric anti-lactosylceramide antibody-positive encephalomyeloradiculoneuropathy

Akiko Sasaki et al. Heliyon. .

Abstract

Introduction: The anti-lactosylceramide (LacCer) antibody is an anti-neutral glycolipid antibody that is involved in the pathogenesis of encephalomyeloradiculoneuropathy (EMRN). It causes acute and subacute injuries to both the central and peripheral nerves. However, no pediatric cases of anti-LacCer antibody-positive EMRN have been reported so far.

Case: A 12-year-old girl presented with signs of meningitis. She subsequently showed disturbance of consciousness and flaccid tetraplegia and was placed on mechanical ventilation due to respiratory failure. MRI showed lesions in the cerebral white matter, basal ganglia, medulla oblongata, as well as the anterior horn of the spinal cord at the C2 to Th1 and Th11 to L1 levels. Nerve-conduction studies showed axonal neuropathy of the motor nerves. After steroid pulse therapy, high-dose immunoglobulin therapy, and plasma exchange, the lesions gradually regressed, and the neurological symptoms improved steadily. The neurological sequelae were minimal at 6 months after disease onset. Although serum anti-aquaporin 4 and anti-myelin oligodendrocyte glycoprotein antibodies were negative, she showed positive anti-lactosylceramide antibody in both serum and cerebrospinal fluid, indicating that these antibodies may be involved in the pathogenesis of this disease.

Conclusion: The first pediatric case of anti-LacCer antibody-positive EMRN showed similar features to the same disease in adults. Anti-neutral glycolipid antibodies should be measured in children presenting with a wide range of neurological symptoms involving both central and peripheral nerves.

Keywords: Anti-lactosylceramide antibody; Anti-neutral glycolipid antibodies; Combined central and peripheral demyelination; Encephalomyeloradiculoneuropathy.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
MRI of the brain and spinal cord. (A—C) T2-weighted images of the brainstem and spine. (B) Axial image derived from the C2/C3 level in (C). Symmetrical high signal is seen in the anterior horn (arrow). (D, E) FLAIR images of the cerebrum.
Figure 2
Figure 2
Clinical course of the patient. Abbreviations: anti-LacCer antibody, anti-lactosylceramide antibody; CSF, cerebrospinal fluid; IVIG, intravenous immunoglobulin; IVMP, intravenous methylprednisolone; PE, therapeutic plasma exchange; PPV, predicted plasma volume; PSL, prednisolone.
See this image and copyright information in PMC

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