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[Acute necrotizing encephalopathy with horizontal gaze palsy]
Y Kurachi1, H Kawahara, K Hatakeyama
1Department of Pediatrics, Kameda Medical Center.
No to Shinkei = Brain and Nerve
|August 1, 1997
Summary
Acute necrotizing encephalopathy (ANE) of childhood is a rare brain disorder. This case highlights a unique ocular movement disorder as a consequence of ANE, suggesting a complex pathophysiological mechanism.
Area of Science:
- Neurology
- Pediatric Neurology
- Neuroscience
Background:
- Acute necrotizing encephalopathy of childhood (ANE) is a rare neurological disorder.
- It is characterized by symmetrical necrotic brain lesions.
- The exact pathophysiological mechanism remains unknown.
Observation:
- A 4-year-old girl presented with severe psychomotor delay and horizontal gaze palsy following ANE.
- Imaging revealed symmetrical thalamic lesions and asymmetrical lesions in the middle cerebral artery (MCA) and posterior cerebral artery (PCA) territories.
- The patient exhibited impaired horizontal but preserved vertical ocular movements.
Findings:
- MRI demonstrated specific involvement of the pontine tegmentum, including the abducens nucleus and medial longitudinal fasciculus (MLF), while sparing the thalamo-mesencephalic junction.
- This selective involvement correlated with the observed peculiar eye movement disorder.
- The lesion distribution suggests a mechanism beyond single vessel occlusion.
Implications:
- This is the first reported case of ANE associated with this specific selective ocular movement disorder.
- The findings suggest a potential toxic or vasoactive agent disrupting the blood-brain barrier.
- Further research is needed to elucidate the complex pathophysiology of ANE and its varied neurological sequelae.