Nystagmus Can Indicate Either Central or Peripheral Problems
Nystagmus can arise from both central (brainstem/cerebellar) and peripheral (vestibular) pathology, but specific characteristics of the nystagmus pattern allow reliable differentiation between the two etiologies. 1
Key Distinguishing Features
Central Nystagmus Characteristics
- Does NOT fatigue with repeated testing and is NOT suppressed by visual fixation 1
- Often accompanied by severe postural instability and additional neurological signs 1
- Gaze-evoked nystagmus is definitively central, indicating brainstem or cerebellar pathology 1, 2
- Does not have a torsional component in most cases 1
Peripheral Vestibular Nystagmus Characteristics
- Typically has a torsional component 1
- Suppressed by visual fixation 1, 3
- Fatigues with repeated maneuvers 1
- Associated with Ménière's disease, vestibular neuritis, labyrinthitis, and superior canal dehiscence syndrome 1
Red Flag Patterns Indicating Central Pathology
The following nystagmus patterns strongly suggest central nervous system pathology and require urgent neuroimaging: 1
- Downbeat nystagmus - most concerning pattern, strongly suggests cerebellar or cervicomedullary junction disorders 1, 4
- Direction-changing nystagmus without head position changes - indicates central pathology rather than benign peripheral causes 1
- Baseline nystagmus without provocative maneuvers - suggests neurological cause 1
- Gaze-evoked nystagmus - definitively central, does not fatigue 1, 2
- Convergence retraction nystagmus - associated with dorsal midbrain syndrome and multiple sclerosis 1
Critical Diagnostic Pitfalls
Do NOT Mistake Central for Peripheral BPPV
- Downbeat nystagmus on Dix-Hallpike maneuver without a torsional component indicates central pathology, NOT benign paroxysmal positional vertigo 1
- Only the direction of nystagmus during an attack reliably differentiates central from peripheral causes when other features overlap 5
Do NOT Rely on Symptom Resolution
- Central nystagmus from vertebrobasilar insufficiency may present with isolated transient vertigo lasting less than 30 minutes, potentially preceding stroke by weeks or months 1
Recognize Transient Peripheral PAN
- Periodic alternating nystagmus (PAN) can occur with peripheral vestibular disorders (Meniere's disease, acute labyrinthitis) but is transitory, progressing to unidirectional nystagmus within 48 hours 3
- Peripheral PAN shows visual suppression, normal oculomotor tests, and absence of central signs - unlike central PAN 3
Neuroimaging Indications
MRI of the brain (without and with IV contrast) is indicated for: 1, 6
- Any acquired or late-onset nystagmus 1
- Concurrent neurological symptoms 1
- Asymmetric, unilateral, or progressive nystagmus 1
- Any of the red flag patterns listed above 1
- 15.5% of children with isolated nystagmus have abnormal intracranial findings on MRI, including Chiari malformation (3.4%) and optic pathway glioma (2%) 6