Nystagmus Flashcards
Ocular Motor System controls the _____ and _____ of the eyes
Position and Movement
A dysfunction in the ocular motor system causes…
Diplopia
True/False: EOMs are innervated by lower motor neurons
TRUE
EOMs are attached on one end to ____ and the other end to _______, which allows for eye movements
Sclera; bony orbit
_______ Nucleus corresponds with CN ____ and innervates the Lateral Rectus of the ______ (ipsilateral/contralateral) eye
Abducens Nucleus corresponds with CN 6 and innervates the Lateral Rectus of the ipsilateral eye
_______ Nucleus corresponds with CN ____ and innervates the Superior Oblique of the ______ (ipsilateral/contralateral) eye
Trochlear Nucleus corresponds with CN 4 and innervates the Superior Oblique of the contralateral eye
Oculomotor Complex corresponds with CN ____ and innervates:
- Levator of ______ (ipsilateral/contralateral) eye
- IO of ______ (ipsilateral/contralateral) eye
- IR of ______ (ipsilateral/contralateral) eye
- SR of ______ (ipsilateral/contralateral) eye
- MR of ______ (ipsilateral/contralateral) eye
- LR of ______ (ipsilateral/contralateral) eye
Oculomotor Complex corresponds with CN 3 and innervates:
- Levator of both eyes
- IO of ipsilateral eye
- IR of ipsilateral eye
- SR of contralateral eye
- MR of ipsilateral eye
Interconnections between _______ and ______, via the _______, coordinate vertical movement
Interconnections between Trochlear Nucleus and Oculomotor Nuclear Complex, via the tectospinal tract, coordinate vertical movement
Fixation requires the suppression of _____
Unwanted saccades
Vestibulo-ocular reflex compensates for ____
Head movements
UMN indicates _____ involvement and likely to cause a _____ (unilateral/bilateral) defect
UMN indicates brainstem involvement and likely to cause a bilateral defect
LMN indicates _____ involvement and likely to cause a _____ (unilateral/bilateral) defect
LMN indicates direct EOM or CN involvement and likely to cause a unilateral defect
Nystagmus
Rhythmic and repetitive oscillation of the eye(s)
Direction of nystagmus is defined by the _____ phase
QUICK
New onset nystagmus indicates a lesion in either the ______ or the ______
Either the inner ear or the brain
requires urgent evaluation
What is the most common type of nystagmus?
JERK
Jerk Nystagmus is characterized by…
Slow pathological phase, followed by fast corrective phase in opposite direction
What type of disorders are indicated if there is a torsional component to the nystagmus?
Inner Ear Disorder
Gaze-evoked nystagmus occurs in lateral gaze of ____º or more
40º of more
TRUE/FALSE: In gaze-evoked nystagmus, nystagmus is absent in primary gaze
TRUE
typically
Intoxication causes what type of nystagmus, and thus testing for this nystagmus is often used in sobriety testing?
Gaze-evoked
Unilateral gaze-evoked nystagmus may indicate
Ipsilateral cerebellar or brainstem disease
refer to neuro-ophthalmologist
Gaze-evoked nystagmus in both horizontal and upgaze indicates
Toxic Metabolic Process (aka Intoxication)
Internuclear Ophthalmoplegia
When looking at the contralateral side, aBducting eye exhibits nystagmus, and ipsilateral eye cannot aDduct
INO indicates a lesion where?
MLF
How to differentiate if lesion is in pons or midbrain?
Pons — convergence will be intact
Midbrain — cannot converge (cannot aDduct ipsilateral eye)
INO in an adolescent or younger adult is likely caused by…
MS (demyelination)
INO in an older adult is likely caused by…
Stroke (brainstem infarction)
What 3 arteries are likely to be indicated in vascular-related INO?
- Posterior Cerebral Artery
- Superior Cerebellar Artery
- Basilar Artery
Associated Signs of INO
- Skew Deviation/Ocular Tilt Reaction (hyper ipsilateral to the lesion)
- Vertical Torsional Nystagmus (ipsilateral downbeat and contralateral torsional nystagmus)
- Vertical Gaze Palsy or Nystagmus
In INO, why does the contralateral eye exhibit nystagmus?
Hering’s Law of Innervation
Bilateral INO
Limitation of aDduction with nystagmus of abducting eye in both left and right gaze
TRUE/FALSE: in INO, there will be vertical nystagmus on upgaze
TRUE
Wall Eye Bilateral INO (WEBINO)
Large XT + BINO
What causes WEBINO?
Midbrain lesion that also impacts CN 3 (MR)
One and a Half Syndrome
Ipsilateral gaze palsy + Ipsilateral INO
Where is the lesion in One and a Half Syndrome?
MLF, also impacts CN 6
What is the only residual movement in One and a Half Syndrome?
Abducting of contralateral eye (that exhibits nystagmus)
Brun’ Nystagmus is a type of ____ nystagmus
Gaze-evoked
Brun’s Nystagmus is associated with a lesion in _____
The Cerebellopontine Angle (CPA)
Is Brun’s Nystagmus associated with peripheral or central nystagmus? Explain.
BOTH
Peripheral due to CN 8 dysfunction and Central due to pons compression
Brun’s nystagmus:
low frequency and high amplitude when looking _____ (to lesion/opposite of lesion)
low frequency and high amplitude when looking to lesion
Brun’s nystagmus:
high frequency and low amplitude when looking _____ (to lesion/opposite of lesion)
high frequency and low amplitude when looking opposite of lesion
What are some clinical findings you might expect in a patient with Brun’s Nystagmus? (4)
- CN 7 defects: ipsilateral facial palsy
- Bilateral papilledema
- CN 5 defects: sensory loss
- Cerebellar defects: ataxia
The defect in a peripheral vestibular pathway dysfunction is likely to be located ____
In labyrinth of the ear or the vestibular nerve
The defect in a central vestibular pathway dysfunction is likely to be located ____
In brainstem:
1. Root entry zone of CN 7
2. Vestibular nuclei
3. Oculomotor nuclei
Alexander’s Law
Nystagmus increases when looking at direction of fast beating
Peripheral or Central Vestibular Nystagmus:
May respond to medication (e.g. clonazepam)
Central Vestibular Nystagmus
Peripheral or Central Vestibular Nystagmus:
Follows Alexander’s Law
Peripheral
Peripheral or Central Vestibular Nystagmus:
Associated with severe vertigo and possible hearing loss
Peripheral
Peripheral or Central Vestibular Nystagmus:
Unilateral lesions common
Peripheral
Peripheral or Central Vestibular Nystagmus:
Does NOT follow Alexander’s Rule
Central
Peripheral or Central Vestibular Nystagmus:
Nystagmus dampened by fixation
Peripheral
Peripheral or Central Vestibular Nystagmus:
Unidirectional nystagmus (torsional, vertical, or horizontal)
Central
Peripheral or Central Vestibular Nystagmus:
can have mixed horizontal/vertical/torsional
Peripheral
Peripheral or Central Vestibular Nystagmus:
Induced by head movements
Peripheral
Peripheral or Central Vestibular Nystagmus:
May have pursuit/saccadic defects
Central
Peripheral or Central Vestibular Nystagmus:
Nystagmus does not increase in direction of fast-beat
Central
does NOT follow Alexander’s Rule
Possible etiologies of Peripheral Vestibular Nystagmus? (4)
- Acoustic neuroma
- Labyrinthitis
- Meniere’s Disease
- Demyelination
TRUE/FALSE: periodic alternations nystagmus always indicates an acquired nystagmus
FALSE; periodic alternating nystagmus can be congenital or acquired
Periodic Alternating Nystagmus is characterized by…
Direction of fast phase changes in cycles of 60-90 seconds, with (often shifting) null period of 5-10 seconds
TRUE/FALSE: Pendular Acquired Nystagmus can be horizontal, vertical, or torsional
TRUE
What occurs if Pendular Acquired Nystagmus combines a vertical and horizontal phase?
Circular, Elliptical, or even Oblique nystagmus
What is the most common pathology associated with Pendular Acquired Nystagmus?
MS
Oculomasticatory Myorhythmia is characterized by
Pendular convergence nystagmus, associated with slow (involuntary) movements of jaw
Oculomasticatory Myorhythmia is strongly associated with…
Whipple Disease
Whipple Disease is strongly associated with which type of nystagmus?
Oculomasticatory Myorhythmia
What is Whipple Disease?
Rare, bilateral infection that affects the GI and impairs absorption of nutrients
Whipple Disease TRIAD
- Dementia
- Ophthalmoplegia
- Myoclonus
What is Myoclonus?
Brief, sudden twitching of a muscle/group of muscles
Oculopalatal Myoclonus is characterized by…
Pendular nystagmus in combination with palatal tremor
Likely etiology for Oculopalatal Myoclonus?
Brainstem infarction or hemorrhage
TRUE/FALSE: See-Saw Nystagmus responds well to medication
FALSE; responds very poorly to treatment in general
See-Saw Nystagmus is characterized by
Pendular nystagmus with elevations/intorsion of one eye and depression/extorsion of the other
What type of nystagmus is an example of a Dissociated Nystagmus?
INO
Disassociated Nystagmus is commonly caused by…
MS or brainstem stoke
Dissociated Nystagmus is characterized by…
Movement of right and left eye in the same direction but with asymmetrical amplitudes
Two examples of Monocular Nystagmus
- Spasmus Nutans
- Hienmann-Bielschowsky Phenomenon
TRUE/FALSE: Spasmus Nutans can be self-limiting
TRUE, usually by age 5!
TRUE/FALSE: Spasmus Nutans can cause amblyopia and strabimus
TRUE
Spasmus Nutans Triad
- Torticollis
- Head nodding
- Monocular or Asymmetric nystagmus
With monocular nystagmus, why are we concerned and generally should order an MRI?
Potential Optic Pathway/Chiasmal Glioma
Spasmus Nutans:
Onset — ?
Resolves by —?
Onset: 4-14 months
Resolves by: 5 yrs
Hienmann-Bielschowsky Phenomenon is characterized by…
Infantile Monocular (slow) Pendular Nystagmus, secondary to severe monocular visual loss
TRUE/FALSE: even after vision is restored, nystagmus usually persists in Hienmann-Bielschowsky
FALSE: usually resolves when vision is restored
With downbeat nystagmus, the typical head posture is chin-_____ (up/down)
Chin-down (brings eyes up)
With upbeat nystagmus, the typical head posture is chin-_____ (up/down)
Chin-up (brings eyes down)
Downbeat or Upbeat Nystagmus:
Associated with Metastatic Breast Cancer
Downbeat
Downbeat or Upbeat Nystagmus:
Obeys Alexander’s Law
BOTH
Downbeat or Upbeat Nystagmus:
More difficult to locate
Upbeat
Downbeat or Upbeat Nystagmus:
Typically caused by lesion in cervicomedullary junction
Downbeat
Downbeat or Upbeat Nystagmus:
Minimal nystagmus seen in primary gaze
BOTH
Downbeat or Upbeat Nystagmus:
Associated with anticonvulsants (e.g. phenytoin)
Upbeat
Downbeat or Upbeat Nystagmus:
More commonly associated with cerebellar lesion
Upbeat
Nystagmus associated with Wernicke’s Encephalopathy
ANY
TRUE/FALSE: Wernicke’s Encephalopathy can be self-limiting
Not really; it’s disabling/life-threatening and requires immediate treatment
Etiology of Wernicke’s Encephalopathy?
Severe B1 deficiency, usually secondary to bariatric surgery or alcoholism
Clinical signs of Wernicke’s
- Nystagmus
- Cerebellar dysfunction
- Confusion
When examining nystagmus, what should be noted? (3)
- Nystagmus with near and far target
- Nystagmus in different gazes
- Nystagmus during fixation
All children with nystagmus should undergo ________ to r/o any _____ etiologies
Careful fundus exam to r/o any retinal or ON etiologies
What is the etiology for congenital nystagmus?
Unknown
TRUE/FALSE: congenital nystagmus is often not noted in the child until days after birth
FALSE; usually months after birth
TRUE/FALSE: congenital nystagmus remains present when the child is asleep
FALSE; not present during sleep
FUNBLOCS
Congenital Nystagmus Features:
1. Fixation increases the nystagmus
2. Upgaze (+ all other gazes) — nystagmus remains horizontal
3. Null point is present (and can manifest as head turn)
4. Bilateral and conjugate
5. Latent component
6. OKN not superimposable
7. Convergence dampens nystagmus
8. Symptomless (generally no oscillopsia)
TRUE/FALSE: Monocular nystagmus is often horizontal in nature
FALSE; vertical
Latent Component of Congenital Nystagmus often seen in what 3 patients?
- Infantile ET
- (+) lesion affecting binocular development
- Down Syndrome
Latent Component of Congenital Nystagmus is characterized by…
When either eye covered, uncovered eye beat away from covered eye
Congenital Nystagmus is classified as either _____ or _____?
Motor or sensory
Sensory Congenital Nystagmus is associated with what ocular conditions? (5)
- Albinism
- Congenital Stationary Night Blindness
- ON Hypoplasia
- Retinal Dystrophies
- Cataracts
Congenital Nystagmus — Motor or Sensory:
Stable over lifetime
Motor
Congenital Nystagmus — Motor or Sensory:
Varying visual prognoses
Sensory
Congenital Nystagmus — Motor or Sensory:
Not associated with other neurological anomalies
Motor
Congenital Nystagmus — Motor or Sensory:
Can be AD, AR, or X-linked inheritance
Motor
Congenital Nystagmus — Motor or Sensory:
Can be progressive or static
Sensory
Surgical Options for Congenital Nystagmus?
- Detach/reattach EOMS
- Align eyes to null point
- Recti recession to decrease tension
Non-surgical options for Congenital Nystagmus?
- RE correction
- BO prism (induce convergence)
- Toked prism (to null point)
- 7 BO + -1.00 to compensate for accommodation
- Topical brinzolamide
- Oral memantine or gabapentin
- Botox
What is Mollaret’s Triangle and what type of Nystagmus is it associated with?
Nuclei or midbrain, medulla, and cerebellum
Associated with Oculopalatal Myoclonus
Associated with parasellar lesions or septa-optic dysplasia
Seesaw Nystagmus