Characteristics of Acute Nystagmus in the Pediatric Emergency Department

Abstract

OBJECTIVES: Acute nystagmus (AN) is an uncommon neurologic sign in children presenting to pediatric emergency departments. We described the epidemiology, clinical features, and underlying causes of AN in a large cohort of children, aiming at identifying features associated with higher risk of severe underlying urgent conditions (UCs).METHODS: Clinical records of all patients aged 0 to 18 years presenting for AN to the pediatric emergency departments of 9 Italian hospitals in an 8-year period were retrospectively reviewed. Clinical and demographic features and the underlying causes were analyzed. A logistic regression model was applied to detect predictive variables associated with a higher risk of UCs.RESULTS: A total of 206 patients with AN were included (male-to-female ratio: 1.01; mean age: 8 years 11 months). The most frequently associated symptoms were headache (43.2%) and vertigo (42.2%). Ataxia (17.5%) and strabismus (13.1%) were the most common neurologic signs. Migraine (25.7%) and vestibular disorders (14.1%) were the most common causes of AN. Idiopathic infantile nystagmus was the most common cause in infants ,1 year of age. UCs accounted for 18.9% of all cases, mostly represented by brain tumors (8.3%). Accordant with the logistic model, cranial nerve deficits, ataxia, or strabismus were strongly associated with an underlying UC. Presence of vertigo or attribution of a nonurgent triage code was associated with a reduced risk of UCs.CONCLUSIONS: AN should be considered an alarming finding in children given the risk of severe UCs. Cranial nerve palsy, ataxia, and strabismus should be considered red flags during the assessment of a child with AN. WHAT'S KNOWN ON THIS SUBJECT:The diagnostic value of acute-onset nystagmus has been investigated in adults, focusing on the risk of vertebrobasilar strokes. In children and adolescents, possible etiologies have not been investigated, and the diagnostic value of nystagmus in the emergency setting is uncertain.WHAT THIS STUDY ADDS: Migraine is the most common cause of acute nystagmus in the emergency setting. Almost 20% of children with acute nystagmus have an urgent neurologic condition, and the recognition of several red flags conferring a higher risk may help their identification.

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“…Since ataxia and strabismus are considered important findings in a child with nystagmus, neuroimaging was urgently performed to exclude a central lesion. The most common accompanying symptoms of nystagmus are headache and vertigo [5] . Motor deficits are merely the topographic diagnosis in central vestibular syndrome.…”

“…They may find it difficult to characterize the onset of the attack and what provoked the complaint [4] . Moreover, patients diagnosed with this condition mostly do not present with a chief complaint of balance disorders [5] , although the association between balance disorders with sensorineural hearing loss, syncope, and headache in children is significant [6] .…”

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“…Nystagmus is an involuntary abnormal rhythmic oscillation of the eyes. It is a sign of cerebellar, vestibular, or visual pathway impairment 32 . Infantile nystagmus usually manifests during the first 6 months of life, while acquired nystagmus typically appears later 33 .…”

“…About 20% of children who present to the emergency department with acute nystagmus have a significant pathology such as a brain tumor. Other potential etiologies include IIH, demyelinating disorders, degenerative conditions, and infections 32 …”

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“…Between September 2010 and September 2018 in an observational, cross-sectional and retrospective study of 212 children at a mean age of 14,5±3,9 years, benign paroxysmal vertigo of childhood, vestibular dysfunction and vestibular migraine account for more than 65% of the cases (15). In a total of 206 patients at a mean of 8 years 11 months, the most common causes of acute nystagmus are migraine (in 25,7%) and vestibular disorders (in 14,1% of the cases) (10). The prevalence of peripheral vestibular disorders among children aged between 0 and 15 years in Germany is 15,16 per 100000 individuals (14).…”