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Evaluation of Childhood Nystagmus

Workflow overview

Infantile Nystagmus Syndrome

Acquired Nystagmus in Childhood

  • Less than 20% of childhood nystagmus is acquired
    • Urgent conditions 19%
    • Brain tumors 8.3%

Common Causes

  • Migraine/vestibular migraine — 26%
    • often accompanied by headache and vertigo.
    • Spontaneous nystagmus in adolescents with vestibular migraine tends to be variable in form
  • Peripheral vestibular disorders 14%
    • Vestibular neuritis and BPPV
      • characterized by unidirectional horizontal-torsional nystagmus
      • positive head impulse test
      • suppression with visual fixation.
    • Medication/substance-induced
      • Anticonvulsants (phenytoin, carbamazepine)
      • Sedatives
      • Dextromethorphan- most common drug reported to poison control centers.
      • Alcohol/illicit substances

Serious Causes

  • Intracranial tumors
    • Posterior fossa tumors (medulloblastoma, ependymoma, cerebellar astrocytoma)
      • Vertical nystagmus (downbeat or upbeat) is particularly suggestive
    • Anterior optic pathway tumors (optic nerve glioma)
  • Demyelinating disease (multiple sclerosis)
    • Can present as an acute vestibular syndrome in adolescents.
    • Central oculomotor signs (direction-changing nystagmus, normal head impulse test, skew deviation) help distinguish it from peripheral causes.
  • Posterior circulation stroke/hemorrhage
    • Rare in children but possible with underlying vascular anomalies, coagulopathy, or cardiac disease.
    • Cerebellar stroke can closely mimic vestibular neuritis.
  • Infectious/inflammatory encephalitis
    • Brainstem or cerebellar encephalitis (viral, autoimmune/paraneoplastic)

Other Important Causes

  • Chiari malformation —
    • Found in 3.4% of children undergoing MRI for nystagmus
    • typically causes downbeat nystagmus.
  • Metabolic diseases
    • Leukodystrophies
    • mitochondrial disorders
    • vitamin deficiencies (B12, thiamine)
  • Hereditary/degenerative cerebellar ataxias
    • Episodic ataxia type 2
    • spinocerebellar ataxias
  • Late-recognized congenital nystagmus — Occasionally, infantile nystagmus is not identified until adolescence; key distinguishing features include absence of oscillopsia and absent optokinetic nystagmus.[2]

Work Up

  • Targeted history:
    • Medication/substance exposure
    • headache pattern
    • prior neurologic episodes
    • family history of neurologic disease
    • timeline of onset (acute vs. subacute vs. chronic).
  • Focused exam:
    • Characterize the nystagmus (direction, effect of fixation, gaze-evoked vs. spontaneous),
    • Cranial nerve palsies ?
    • Papilledema?
    • RAPD?
    • Visual acuity
    • Perform head impulse test and test of skew
    • Ataxia?
  • Initial workup
    • MRI brain and orbits with contrast
    • Ophthalmologic evaluation including
    • Visual electrophysiology if visual pathway pathology is suspected
    • Toxicology screen if substance exposure is a concern.
  • Red Flags Suggesting Urgent Pathology: urgent neuroimaging and neurology consultation
    • Cranial nerve deficits
    • Ataxia
    • Oscillopsia
    • Dissociated (asymmetric) nystagmus
    • Papilledema
    • Afferent pupillary defect

Resources

  • AAO Clinical Guidelines by Dumitrescu AV
  • AAO Video:Infantile Nystagmus
  • Characteristics of Acute Nystagmus in the Pediatric Emergency Department. Garone G, Suppiej A, Vanacore N, et al. Pediatrics. 2020;146(2):e20200484. doi:10.1542/peds.2020-0484.
  • Infantile and Acquired Nystagmus in Childhood. Ehrt O. European Journal of Paediatric Neurology : EJPN : Official Journal of the European Paediatric Neurology Society. 2012;16(6):567-72. doi:10.1016/j.ejpn.2012.02.010.
  • Clinical Significance of Spontaneous Nystagmus in Pediatric Patients. Zhou G, Goutos C, Lipson S, Brodsky J. International Journal of Pediatric Otorhinolaryngology. 2018;111:103-107. doi:10.1016/j.ijporl.2018.06.007.
  • Head Impulse, Nystagmus, and Test of Skew Examination for Diagnosing Central Causes of Acute Vestibular Syndrome. Gottlieb M, Peksa GD, Carlson JN. The Cochrane Database of Systematic Reviews. 2023;11:CD015089. doi:10.1002/14651858.CD015089.pub2.
  • Vestibular Neuritis. Baloh RW. The New England Journal of Medicine. 2003;348(11):1027-32. doi:10.1056/NEJMcp021154
  • Common Ocular Effects Reported to a Poison Control Center After Systemic Absorption of Drugs in Therapeutic and Toxic Doses. Slattery A, Liebelt E, Gaines LA. Current Opinion in Ophthalmology. 2014;25(6):519-23. doi:10.1097/ICU.0000000000000103.
  • Pearls & Oy-Sters: Positional Vertigo and Vertical Nystagmus in Medulloblastoma: A Picture Is Worth a Thousand Words. Kronenbuerger M, Olivi A, Zee DS. Neurology. 2018;90(4):e352-e354. doi:10.1212/WNL.0000000000004866.
  • Multiple Sclerosis as a Cause of the Acute Vestibular Syndrome. Pula JH, Newman-Toker DE, Kattah JC. Journal of Neurology. 2013;260(6):1649-54. doi:10.1007/s00415-013-6850-1.
  • Acute Vestibular Syndrome. Hotson JR, Baloh RW. The New England Journal of Medicine. 1998;339(10):680-5. doi:10.1056/NEJM199809033391007.
  • Incidence and Types of Pediatric Nystagmus. Nash DL, Diehl NN, Mohney BG. American Journal of Ophthalmology. 2017;182:31-34. doi:10.1016/j.ajo.2017.07.006.
  • The Role of MR Imaging in Investigating Isolated Pediatric Nystagmus. Batmanabane V, Heon E, Dai T, et al. Pediatric Radiology. 2016;46(12):1721-1727. doi:10.1007/s00247-016-3669-9.
  • Guidelines for Reasonable and Appropriate Care in the Emergency Department 3 (GRACE-3): Acute Dizziness and Vertigo in the Emergency Department. Edlow JA, Carpenter C, Akhter M, et al. Academic Emergency Medicine : Official Journal of the Society for Academic Emergency Medicine. 2023;30(5):442-486. doi:10.1111/acem.14728.