A Flow Chart For Classification Of Nystagmus
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1 A Flow Chart For Classification Of Nystagmus Is fixation impaired because of a slow drift, or an intrusive saccade, away from the target? If a slow drift is culprit Jerk Pendular Unidrectional (constant velocity) Central Peripheral (Use effect of fixation and vector of nystagmus) Changes direction with viewing eye = Latent Nystagmus Changes direction with direction of gaze (gaze-evoked) Acquired (velocity decreasing e.g. cerebellar) Acquired (MS or OPT) or Congenital Congenital (velocity increasing)
2 A Flow Chart For Classification Of Nystagmus If a saccade away from the fixation target (saccadic intrusion) is the culprit? With an intersaccadic interval (~200 ms) before a return saccade Without an intersaccadic interval (back to back saccades) Square-wave jerks (small saccades away from and back to fixation), macrosquare wave jerks (much larger saccades) Macrosaccadic oscillations, Large saccades around fixation (extreme degree of saccade hypermetria) Opsoclonus (multiaxis) Ocular Bobbing Single vertical saccade away from fixation followed by slow drift back Flutter (one axis) Includes voluntary nystagmus
3 Waveforms of Nystagmus Jerk or linear (vestibular) Velocity decreasing (acquired gaze-evoked, congenital latent) Velocity increasing (congenital) Pendular
4 SCC Organization: a guide to nystagmus Arrows indicate direction of slow phase with stimulation Central Patterns Peripheral Pattern Flourens
5 Summary table of nystagmus treatment (Strupp et al, J Neurology, 2011) Downbeat nystagmus (DBN) Upbeat nystagmus (UBN) Acquired pendular nystagmus (APN) Period alternating nystagmus (PAN) Infantile (congenital) nystagmus Direction of nystagmus (quick phase) downward, may be diagonal with lateral gaze upward mainly horizontal, may have vertical and/or torsional components horizontal mainly horizontal; may have torsional and small vertical components Waveform (slow phase) Special features jerk, constant, increasing, or decreasing slowphase velocity increased intensity during lateral and downward gaze; sometimes suppressed by convergence jerk, constant, increasing, or decreasing slowphase velocity increased intensity during upward gaze; may convert to DBN on convergence pendular, sinusoidal slowphase associated with other oscillations (e.g., palate) and with hypertrophic degeneration of the inferior olive Jerk, mostly constant slowphase velocity changes direction every 90 to 120 sec accelerating slowphases; foveation periods when the eye is transiently still null zone, in which nystagmus is minimal; often suppressed with convergence Sites of lesion cerebellum (bilateral floccular hypofunction); rarely lower brainstem lesions medial medulla, pontomesencephalic junction, rarely cerebellum pontomedullary, probably affecting components of neural integrator for gaze holding cerebellum (nodulus, uvula) uncertain; some cases are associated with afferent visual system anomalies Etiology cerebellar tumors, degenerations, and stroke; idiopathic; often associated with bilateral vestibulopathy and neuropathy brainstem or cerebellar stroke and tumors; Wernicke s encephalopathy multiple sclerosis, oculopalatal tremor due to brainstem or cerebellar stroke cerebellar degenerations, cranio-cervical anomalies, multiple sclerosis, cerebellar tumors and stroke uncertain; may be associated with afferent visual system anomalies; hereditary in some patients (e.g., FRMD7 mutations) Treatment 4-aminopyridine (5-10 mg tid), 3,4- diaminopyridine (10-20 mg tid), baclofen (5 mg tid) clonazepam (0.5 mg tid) often transient, treatment often not necessary; baclofen (5-10 mg tid) 4-aminopyridine (5-10 tid) memantine (10 mg qid) gabapentin (300 mg qid) baclofen (5-10 mg tid) gabapentin (300 mg qid) memantine (10 mg qid)
6 A Phone Call from an Acutely Vertiginous Patient: Key Diagnoses Stroke BPPV Neuritis Migraine Menieres Headache, Neck pain, Other Neurological Symptoms (diplopia, numbness, weakness, hiccoughs, dysphagia, dysarthria, incoordination), Hearing loss, Risk Factors (age, hypertension, lipid disorder, heart disease, diabetes) Positional, Usually turning over or getting in and out of bed, Transient, Recent inciting events (dentist, hair dresser, trauma, bed rest), Happened before? Sustained vertigo even when at rest though worsened by movement, No hearing change, Viral illness Neurological symptoms? Headache or neck pain? Any previous episodes? Duration of the spell? Is it positional? Hearing symptoms? Age? Vascular risk factors? Headache, Family or personal history of migraine, Headaches may only be remote, Triggers, aura Light and noise sensitivity, Relieved by sleep Aural symptoms (pain, pressure, fullness in ear, seashell tinnitus, Fluctuating hearing loss (low frequency) Otolithic crises of Tumarkin Reversals of direction of vertigo, Lermoyez (hearing improves as vertigo begins)
7 Stroke The Acutely Vertiginous Patient: Key Findings BPPV Neuritis Migraine Menieres Spontaneous jerk nystagmus that does not suppress with fixation, Gaze-evoked, direction - changing nystagmus, Skew deviation, Absent head impulse sign (though if present does not r/o stroke) Other neurological signs including hearing loss Positioning nystagmus, Transient, after a latency, usually mixed vertical torsional if posterior canal or horizontal if lateral canal. Posterior canal BPPV changes direction on sitting up, Lateral canal may be apogeotropic (beats to sky) or geotropic (beats to ground) When most intense is beating toward involved ear Spontaneous, sustained, mixed horizontaltorsional nystagmus in straight ahead gaze. Obeys Alexander s law (more intense with gaze in the direction of quick phase) More intense when lying with bad ear down. Suppressed with fixation, Positive head impulse sign May mimic BPPV or Neuritis or have central ocular motor signs (gazeevoked or vertical nystagmus) Spontaneous nystagmus similar to vestibular neuritis, Nystagmus may spontaneously change direction in the first minutes and then hours after onset. Loss of hearing, especially low frequency, Henneberts sign (tragal compression produces nystagmus)
8 Epley Semont Helminski, Zee et al.
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