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Related Experiment Videos

Lower brainstem dysfunction in an infant with persistent primitive trigeminal artery.

Tohru Okanishi1, Yoshiaki Saito, Shiho Miki

  • 1Division of Child Neurology, Institute of Neurological Sciences, Faculty of Medicine, Tottori University, 36-1 Nishi-cho, Yonogo 683-8504, Japan.

Brain & Development
|September 30, 2006
PubMed
Summary
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Persistent primitive trigeminal artery (PPTA) in an infant caused brainstem compression, leading to developmental delays and neurological deficits. This case highlights potential vascular origins for congenital neurological conditions.

Area of Science:

  • Neuroscience
  • Vascular Neurology
  • Pediatric Neurology

Background:

  • Persistent primitive trigeminal artery (PPTA) is a rare congenital vascular anomaly where the primitive trigeminal artery fails to regress after the formation of the definitive carotid-basilar anastomosis.
  • While often asymptomatic, PPTA can be associated with various neurological symptoms due to its potential to cause mass effect or alter cerebral blood flow.

Observation:

  • A 6-month-old boy presented with stridor, dysphagia, delayed motor development, and postural neck and shoulder dystonia.
  • Imaging revealed PPTA with abnormal flow from the basilar artery to the right internal carotid artery.
  • Further findings included lower brainstem compression by the dilated basilar artery and cerebellar vermis hypoplasia.

Findings:

  • Evoked potentials indicated functional disruption in the lower pons and medulla oblongata.

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  • The observed neurological deficits are potentially linked to the vascular etiology causing lower brainstem compression.
  • Cerebellar vermis hypoplasia was also noted, suggesting a possible congenital malformation syndrome.

    • Implications:

    • This case underscores the importance of considering vascular anomalies like PPTA in the differential diagnosis of pediatric neurological and developmental disorders.
    • The findings suggest that PPTA-induced brainstem compression can lead to significant infratentorial structural and functional abnormalities.
    • Further investigation into the relationship between PPTA, brainstem compression, and conditions like Möbius syndrome is warranted.