A 6-year-old girl presents with six weeks of newly noticed shaky eye movements, clumsiness, and morning headaches with vomiting. Her parents report the nystagmus was never present in infancy — baby photos show still eyes. She has no prior eye disease.
Examination Findings
Acquired jerk nystagmus, disconjugate and variable, with mild bilateral sixth-nerve limitation. Papilloedema present. Gait ataxia. The nystagmus has no null zone and she reports the world ‘jiggles’. Remainder of neurology: no focal weakness.
Investigations
Urgent MRI brain with contrast: an expansile pontine lesion consistent with a brainstem glioma, with obstructive hydrocephalus explaining the papilloedema and morning headaches. Paediatric neuro-oncology referral is immediate; no time is lost on eye-specific tests.
Questions to Think About
- What three features prove this nystagmus is acquired rather than infantile?
- Why are the baby photos clinically useful?
- What is the ophthalmologist’s role once the tumour is found?
Diagnosis
Acquired nystagmus secondary to paediatric brainstem glioma with obstructive hydrocephalus.
Reasoning
Reasoning: New-onset nystagmus in a school-age child is neurological until proven otherwise — and here the oscillopsia (never present in infantile nystagmus), the papilloedema, and the ataxia form a posterior-fossa triad. Baby photos objectively confirm the nystagmus is new, defeating any assumption of a missed infantile case. Diagnosis: acquired nystagmus secondary to brainstem glioma with obstructive hydrocephalus. Management: typical management is urgent paediatric neuro-oncology care — hydrocephalus management, tumour treatment planning (biopsy, radiotherapy, or chemotherapy per protocol; brainstem gliomas are often not resectable), steroids for oedema per protocol, and honest, compassionate family counselling. The ophthalmologist documents baseline vision, fields, discs, and motility, then follows them through treatment — vision monitoring during therapy is the ongoing eye role. The family is supported through a devastating diagnosis with clarity and coordination.
Differential Diagnosis
- Brainstem glioma — confirmed on MRI: acquired nystagmus with brainstem signs and raised pressure in a child.
- Spasmus nutans — ruled out: that is infantile-onset, disconjugate but benign and self-limited; this is new, with papilloedema and ataxia.
- Idiopathic infantile nystagmus — ruled out: the parents’ history and photos prove new onset, plus oscillopsia and papilloedema.
Management
Urgent paediatric neuro-oncology referral; hydrocephalus and tumour treatment per protocol; ophthalmic baseline documentation and vision monitoring through therapy; family support.
Key Learning Points
- New-onset nystagmus in childhood is a neurological emergency until proven otherwise.
- Oscillopsia, papilloedema, and ataxia with nystagmus localise to the posterior fossa.
- After diagnosis, the ophthalmologist’s role is baseline documentation and vision monitoring.
Red Flags
- New nystagmus in a school-age child — image the brain urgently; never call it idiopathic.
- Oscillopsia or papilloedema with nystagmus — acquired disease; the workup is neurological.
- Assuming infantile onset without evidence — old photos objectively date the nystagmus.
Educational content only — not medical advice. Clinical decisions must be made by a qualified professional for the individual patient.