What are the management options for nystagmus?

Management of Nystagmus

Management of nystagmus depends critically on distinguishing between benign positional nystagmus (BPPV-related), which requires repositioning maneuvers, versus pathological forms requiring pharmacological, optical, or surgical interventions based on the specific nystagmus type and underlying etiology. 1, 2

Initial Diagnostic Differentiation

The first step is determining whether nystagmus represents benign paroxysmal positional vertigo (BPPV) versus other pathological causes:

BPPV-Related Nystagmus (Requires Repositioning Maneuvers)

• Posterior canal BPPV: Diagnosed when torsional, upbeating nystagmus is provoked by the Dix-Hallpike maneuver (bringing patient from upright to supine with head turned 45° to one side and neck extended 20°) 1
• Lateral canal BPPV: Diagnosed with the supine roll test, which reveals direction-changing horizontal nystagmus (either geotropic or apogeotropic) 1
• Treatment for BPPV involves canalith repositioning procedures, not pharmacological management 1

Central or Pathological Nystagmus (Requires Further Workup)

Key red flags indicating non-BPPV nystagmus requiring neuroimaging and specialized management 1, 3:

• Downbeat nystagmus without torsional component (strongly suggests cerebellar or cervicomedullary junction pathology) 3
• Direction-changing nystagmus not fitting BPPV patterns 1
• Gaze-evoked nystagmus (indicates brainstem/cerebellar pathology) 4
• Baseline nystagmus present without positional provocation 3
• Nystagmus that does not fatigue or is not suppressed by visual fixation 4

Management Algorithm by Nystagmus Type

Downbeat Nystagmus

First-line pharmacological options 5, 6:

• 4-aminopyridine: 3 × 5 mg/day or 1-2 × 10 mg sustained-release formulation 7
• 3,4-diaminopyridine (alternative potassium channel blocker) 5, 6
• Clonazepam (if aminopyridines contraindicated or ineffective) 5

Mandatory neuroimaging: MRI of brain to identify structural lesions affecting cerebellum or cervicomedullary junction 3

Upbeat Nystagmus

Pharmacological options 5, 7:

• Memantine: up to 40 mg/day 7
• 4-aminopyridine: 3 × 5 mg/day 7
• Baclofen: 3 × 5-10 mg/day 5, 7

Acquired Pendular Nystagmus (Multiple Sclerosis)

First-line agents 5, 8:

• Gabapentin: up to 2400 mg/day 7, 8
• Memantine: up to 40 mg/day (alternative or adjunct) 5, 7

Acquired Pendular Nystagmus (Oculopalatal Tremor)

Treatment options 5, 8:

• Gabapentin: up to 2400 mg/day 7
• Memantine: up to 40 mg/day 7
• Trihexyphenidyl (if above ineffective) 5

Periodic Alternating Nystagmus

Definitive treatment 5, 6:

• Baclofen: 3 × 5-10 mg/day (often completely suppresses this form) 5, 7
• Memantine: for refractory cases 5

Infantile Nystagmus Syndrome (INS)

Management approach depends on symptom severity 5, 8, 9:

Pharmacological options for symptomatic patients 5, 7, 8:

• Gabapentin: up to 2400 mg/day 7
• Memantine: up to 40 mg/day 7, 8
• Acetazolamide (in select cases) 5
• Topical brinzolamide 5

Optical interventions 7, 8:

• Base-out prisms to induce convergence (reduces nystagmus intensity) 5, 7
• Contact lenses (overcome negative effects of spectacles in abnormal head posture) 7
• Spectacle prisms to shift eccentric null zone 7

Surgical options (requires preoperative evaluation of visual acuity and nystagmus intensity in different gaze positions) 5, 7:

• Kestenbaum operation for eccentric null zone with abnormal head posture 7
• Recess-resect surgery to produce artificial exophoria 7
• Unilateral recess-resect surgery in infantile esotropia with latent nystagmus 7

Torsional Nystagmus

Pharmacological option 5:

• Gabapentin may be effective 5

Seesaw Nystagmus

Treatment options 5:

• Alcohol 5
• Clonazepam 5
• Memantine 5

Pediatric-Specific Considerations

Infantile Nystagmus (Onset First 6 Months)

Immediate referral to pediatric ophthalmologist at time of detection 2

Initial workup 2:

• Clinical ophthalmological examination 2
• Genetic workup 2
• Evaluation for underlying causes: albinism, retinal disease, low vision, vision deprivation (congenital cataracts) 2, 9

Acquired Nystagmus in Children

Neuroimaging indications (MRI brain without and with IV contrast) 2:

• Acquired or late-onset nystagmus 2
• Concurrent neurological symptoms 2
• Asymmetric, unilateral, or progressive nystagmus 2
• Associated oscillopsia, head bobbing, or torticollis 2

Critical finding: 15.5% of children with isolated nystagmus have abnormal intracranial findings on MRI, including Chiari malformation (3.4%) and optic pathway glioma (2%) 2

Additional Treatment Modalities

Botulinum Toxin

• Injections into extraocular muscles or retrobulbar space (for select refractory cases) 5

Emerging Technologies

• Electro-optical devices currently under development to noninvasively negate visual consequences of nystagmus 5, 8

Critical Pitfalls to Avoid

Do not treat nystagmus without proper diagnostic evaluation: Eye movements themselves do not require treatment if patient is asymptomatic; therapy is necessary only for visual disability (decreased visual acuity, oscillopsia) 6

Do not confuse spasmus nutans with benign infantile nystagmus: Spasmus nutans (characterized by nystagmus, head bobbing, torticollis at 1-3 years) requires MRI to exclude anterior visual pathway tumors 2

Do not use CT imaging for nystagmus evaluation: MRI provides superior soft tissue detail for posterior fossa and brainstem structures 2, 3

Do not miss central causes masquerading as BPPV: Downbeat nystagmus without torsional component, direction-changing nystagmus not fitting BPPV patterns, and gaze-evoked nystagmus all indicate central pathology requiring neuroimaging 1, 3, 4

Recognize that most pharmacological treatments are off-label use: Clinical efficacy is generally sought through trial-and-error approach depending on nystagmus type, as few controlled treatment trials exist 6, 7