Bilateral medial medullary and spinal cord infarctions presenting as locked-in syndrome in an adolescent with primary antiphospholipid syndrome

Bilateral medial medullary infarction (BMMI) is a rare stroke subtype in the paediatric population characterised by quadriplegia, cranial nerve dysfunction and respiratory failure. MRI typically reveals a 'heart-shaped' sign in the medial medulla on diffusion-weighted imaging (DWI). Paediatric stroke aetiologies, including vasculopathy, thrombophilia and cardioembolic disease, differ from those in adults. Antiphospholipid syndrome (APS) is a rare but important cause of paediatric stroke.We report an adolescent male presenting with acute quadriplegia, bulbar dysfunction and respiratory failure with intact cognition, consistent with locked-in syndrome. MRI confirmed BMMI with concurrent spinal cord infarction, and APS was diagnosed based on persistently elevated anticardiolipin antibodies. Anticoagulation therapy and rehabilitation were initiated. However, minimal neurological improvement was observed at 1-year follow-up. This case highlights the rarity of BMMI in children and underscores the importance of early diagnosis and management as well as the recognition of uncommon causes such as APS.

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Publication Details

Journal
BMJ Case Reports
Published
2025-02-01
DOI
https://doi.org/10.1136/bcr-2024-263223
Citations
1
Primary Topic
Bone and Joint Diseases
Type
article
Field-Weighted Citation Impact
1.72
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Bilateral medial medullary and spinal cord infarctions presenting as locked-in syndrome in an adolescent with primary antiphospholipid syndrome

Benjamin Wei-Liang Ng, Geri Ling-Na Wong, Rou Chen Jee
1 citations
BMJ Case Reports
Bone and Joint Diseases
1.72
article

Bilateral medial medullary and spinal cord infarctions presenting as locked-in syndrome in an adolescent with primary antiphospholipid syndrome

Benjamin Wei-Liang Ng, Geri Ling-Na Wong, Rou Chen Jee
article en
1 citations

Abstract

Bilateral medial medullary infarction (BMMI) is a rare stroke subtype in the paediatric population characterised by quadriplegia, cranial nerve dysfunction and respiratory failure. MRI typically reveals a 'heart-shaped' sign in the medial medulla on diffusion-weighted imaging (DWI). Paediatric stroke aetiologies, including vasculopathy, thrombophilia and cardioembolic disease, differ from those in adults. Antiphospholipid syndrome (APS) is a rare but important cause of paediatric stroke.We report an adolescent male presenting with acute quadriplegia, bulbar dysfunction and respiratory failure with intact cognition, consistent with locked-in syndrome. MRI confirmed BMMI with concurrent spinal cord infarction, and APS was diagnosed based on persistently elevated anticardiolipin antibodies. Anticoagulation therapy and rehabilitation were initiated. However, minimal neurological improvement was observed at 1-year follow-up. This case highlights the rarity of BMMI in children and underscores the importance of early diagnosis and management as well as the recognition of uncommon causes such as APS.

BMJ Case ReportsVol. 18(2)
Ministry of Health (MY), Sibu Hospital (MY), SEGi University (MY)
Good health and well-being
Openalex Percentile: Top 23%
Bone and Joint Diseases
1.72
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