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Ask your administrator if you think this is wrong. ====== Evaluation of Childhood Nystagmus ====== =====Workflow overview===== {{:infantile_nystagmus_workup_summary_flowdiagram.jpg?500|}} ===== Infantile Nystagmus Syndrome ===== {{:ins_workup.png?500|}} ===== Acquired Nystagmus in Childhood ===== {{:acquired_nystagmus_workup.png?500|}} * **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 * [[https://www.youtube.com/watch?v=iIgezzbbFgg |Youtube explanation by Fauquier ENT]] * [[https://www.youtube.com/watch?v=aBns2AXi154 |Youtube explanatory Animation of head impulse test by "Zero to Finals"]] * Look for skew deviation with alternate cover testing * Ataxia? * [[https://www.youtube.com/watch?v=vCn1WRR_U3E|Youtube ataxic gait example]] * Initial testing * MRI brain and orbits with contrast * 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 ====== * [[https://www.aao.org/disease-review/clinical-guidelines-childhood-nystagmus-workup|AAO Clinical Guidelines by Dumitrescu AV]] * [[https://aao-resources-enformehosting.s3.amazonaws.com/resources/AAO.LMS/BCSC_Media/BCSC1819/videos/BCSC18_s06_ch13-01_Infantile-Nystagmus-Syndrome.mp4|AAO Video:Infantile Nystagmus]] * [[https://pubmed.ncbi.nlm.nih.gov/32732262/ |Characteristics of Acute Nystagmus in the Pediatric Emergency Department. Garone G, Suppiej A, Vanacore N, et al. Pediatrics. 2020;146(2):e20200484]] * [[https://pubmed.ncbi.nlm.nih.gov/22459007/|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]] * [[https://pubmed.ncbi.nlm.nih.gov/29958590/|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]] * [[https://www.cochranelibrary.com/cdsr/doi/10.1002/14651858.CD015089.pub2/information?cookiesEnabled|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.]] * [[https://www.sralab.org/rehabilitation-measures/head-impulse-test-head-thrust-test| Head inpulse test at Shirley Ryan Ability Lab]] * [[https://pubmed.ncbi.nlm.nih.gov/25226509/|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.]] * [[https://pmc.ncbi.nlm.nih.gov/articles/PMC5610648/| Incidence and Types of Pediatric Nystagmus. Nash DL, Diehl NN, Mohney BG. American Journal of Ophthalmology. 2017;182:31-34]] * [[https://pubmed.ncbi.nlm.nih.gov/27518079/|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]] * [[https://deepblue.lib.umich.edu/items/93eb0aec-f297-4384-b305-1a674c72280a|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]] {{tag>nystagmus}} nystagmus