NYSTAGMUS

RADITYO
DEFINITION

• Nystagmus is defined by rhythmic, abnormal eye movements with a "slow" eye

movement driving the eye off the target followed by a second movement that brings the
eye back to the target.
• The movement can be horizontal, vertical, torsional or a combination of these
movements.
• Nystagmus can be jerk (named for fast phase) or pendular, variable amplitude and
frequency, and can be worsened or improved by gaze position, fixation, or covering one
eye (latent). 
CLINICAL TYPES AND TERMINOLOGY
EPIDEMIOLOGY

Clinical diagnosis and frequency distribution of patients with nystagmus within the
Leicestershire study. The numbers beside the bars represent prevalence per 10,000 (±95% CI),
calculated separately for the 18 years or younger and older than 18 years age groups.
• nystagmus starts by a slow movement of the eye away from the visual target.
• The second movement brings the eye back to the visual target. If the second movement is
slow, the nystagmus is said to be pendular.
• If this second movement is quick, the nystagmus is called jerk nystagmus. 
• By convention, the direction of jerk nystagmus (eg., right-beating nystagmus) is named
after the fast phase of nystagmus. In a right-beating nystagmus, the fast phase is to the
patient's right.
• Dissociated nystagmus refers to the two eyes having nystagmus with the same direction
but with differing amplitudes. 
• Disconjugate nystagmus occurs when the two eyes have different directions of
oscillation, one example of which is sea-saw nystagmus.
• An interesting type of jerk nystagmus is the Periodic Alternating Nystagmus (PAN),
which is characterized by a cycle of unidirectional jerk nystagmus followed by a
dampening or cessation of the abnormal eye movement, then jerk nystagmus occurring in
the opposite direction.
PHYSIOLOGIC NYSTAGMUS

• Physiologic nystagmus (nystagmus that is characteristic of normal oculomotor function)

includes optokinetic nystagmus, vestibular ocular reflex, caloric nystagmus, and post-
rotatory nystagmus.
• As they do not represent pathologic states: .
VESTIBULO OCULAR REFLEX

• Vestibulo-ocular reflex (VOR) is the reflexive movement of the eye that keeps the

visual image stable on the retina during brief, high frequency rotation of the head.
• VORs are controlled by the vestibular system of the inner ears, namely the semicircular
canals, utricle, and saccule.
OPTOKINETIC NYSTAGMUS

• Optokinetic nystagmus (OKN) is a physiologic movement of the eyes in response to

large, moving visual fields (e.g. when one is looking out the window of a moving train).
• The initial movement is a smooth pursuit movement followed by contraversive saccade
back to primary gaze or direction of visual interest.
• Asymmetry of the OKN response to the rotating drum may suggest lesions of the
cerebrum, typically a large lesion of the parietal or parieto-occipital cortex and associated
with homonymous hemianopia. This is in contrast to the lesions of the occipital lobe
which also produce homonymous hemianopia but without the OKN asymmetry
CALORIC NYSTAGMUS

• Caloric nystagmus is a type of VOR (vestibulo-ocular reflex) that is elicited by

stimulating the horizontal semicircle with either warm or cold water in the ear canal to
create a convection current in the endolymph of the semicircle.

• In normal subjects, when cold water is placed in one ear, the eyes will slowly turn toward
the ear with the horizontal fast phase away from the ear. The absence of caloric
nystagmus may indicate brain death.
EARLY ONSET (CHILDHOOD) NYSTAGMUS
INFANTILE IDIOPATHIC NYSTAGMUS

• Infantile idiopathic nystagmus (IIN), also known as congenital (motor) nystagmus, is

the most common type of nystagmus seen in young patients followed by
congenital sensory nystagmus.
• Congenital (motor) nystagmus (e.g. IIN) is by definition idiopathic (e.g. without a known
cause or associated afferent pathway disease) and is therefore a diagnosis of exclusion. It
is present from infancy but usually recognized a few months into life
SENSORY NYSTAGMUS

• Sensory nystagmus, also known as nystagmus associated with afferent visual system
abnormalities, is usually seen in the first 3-4 months and has the same oculomotor
features as infantile nystagmus, but is due to anatomic disorders of the eye that, by
limiting the proper visual sensory input to the eye, limit the visual development of the
patient.
• The causes of sensory nystagmus are many—but a few common etiologies can be
remembered by the 5 A’s mnemonic: Aplasia (hypoplasia) of the optic nerve (optic nerve
hypoplasia), Leber congenital amaurosis, aniridia, Achromatopsia, and ocular albinism.
ACQUIRED FORMS OF NYSTAGMUS

• Elements of the neural

integrator such as the
vestibular nucleus and
the interstitial nucleus
of Cajal are intimately
interconnected via the
oculomotor nucleus.
 ACQUIRED FORMS OF NYSTAGMUS

• The neural mechanism for maintaining eccentric

gaze involves a number of areas of the brainstem
called the neural integrator.
• The horizontal gaze neural integrator consists of
the nucleus prepositus
hypoglossi and medial vestibular nuclei. Vertical
and torsional gaze holding is maintained
bythe interstitial nucleus of Cajal.
• Other components of the neural integrator include
the flocculus and nodulus of the cerebellum. 
GAZE-EVOKED NYSTAGMUS AND REBOUND
NYSTAGMUS
• Gaze-evoked nystagmus has jerk waveform movement that occurs in lateral gaze or upgaze.
• After each eccentric gaze, the eyes move toward the primary position followed by a saccade toward the
eccentric direction, leading to right jerk nystagmus in right gaze and left jerk nystagmus in left gaze, etc.
• This format follows Alexander's law, which states that nystagmus increases in amplitude and frequency as
the patient looks in the direction of the fast phase.
• Unlike end-gaze nystagmus (conjugate, in both right and left directions of gaze, transient, low amplitude of
under 4-degrees, more prominent with age, benign), gaze-evoked nystagmus is sustained, larger in
amplitude, possibly asymmetric, and is often associated with down-beat nystagmus. 
• Gaze-evoked nystagmus is a sign of neural integrator dysfunction. Advanced age can cause degenerative
impairment of the neural integrator, leading to an often symmetric, horizontal gaze-evoked nystagmus. 
PERIPHERAL VESTIBULAR NYSTAGMUS
 CENTRAL VESTIBULAR NYSTAGMUS

• Central forms of vestibular nystagmus arise from dysfunction

in one of the many interconnections between the central
vestibular structures and the neural integrators. In contrast to
peripheral vestibular nystagmus, centrally-derived nystagmus
is not classically inhibited by visual fixation and is typically
confined to one plane (e.g., purely vertical or torsional).
Neuroimaging is crucial in determining the location of the
etiologic 
• Downbeat nystagmus (downward fast phase) is the most common of the central vestibular nystagmuses. Its jerk nystagmus waveform begins with upward drift of the
eyes corrected with a downward saccade. This form of nystagmus follows Alexander's Law and hence is accentuated by downgaze and also by lateral-down gaze, but is
also amplified by convergence and lying prone.[41] Concomitant gaze-evoked nystagmus and rebound nystagmus may be observed.[36] Generally, patients are
symptomatic from vertical oscillopsia. The differential for down-beating nystagmus is broad, but structural lesions can be ruled out with neuroimaging. Cervico-
medullary junction is the most probable location of a structural lesion. 

• Tumors at the foramen magnum

• Arnold-Chiari malformation Type I
• Demyelination
• Stroke
• Cranial trauma
• Drug toxicity (lithium, anticonvulsants)
• Platybasia
• Spinocerebellar degeneration
• Brainstem encephalitis
• Paraneoplastic syndrome
• Impaired nutrition (e.g., Wernicke encephalopathy, parenteral feeding, magnesium deficiency)
• Antibody to glutamic acid decarboxylase (GAD)
• Idiopathic