Presentation of Intrinsic Pontine Gliomas in Children

  • Cranial neuropathies: Bilateral and multiple cranial nerve involvement is frequent in patients with diffuse intrinsic pontine gliomas. Abducens (CN VI) palsy is the most frequent cranial neuropathy at presentation (6, 10, 17, 28). Facial palsy is also frequently seen at initial presentation. Involvement of CN VIII causes deafness and contributes to ataxia. Lower cranial nerve involvement causes aspiration and difficulty in speech, swallowing, and maintaining head positions.
  • Long tract involvement: These children often present with severe limb weakness and hyperreflexia, resulting in difficulty walking and sometimes sitting. Long tract involvement also causes loss of tactile, nociceptive, and proprioceptive sensations, causing numbness and contributing to ataxia.
  • Ataxia: Both CN VIII involvement and loss of proprioceptive sensation contribute to ataxia. Invasion of the cerebellum is common in diffuse intrinsic pontine gliomas, which is another cause of ataxia.
  • Other symptoms at presentation: Patients may have headache, nausea and vomiting, and other symptoms that indicate the presence of increased ICP.