REVIEW ARTICLE

Central Vertigo and Dizziness

Epidemiology, Differential Diagnosis, and Common Causes
Mehmet Karatas, MD

Background: Dizziness is a common complaint among patients

seen by primary care physicians, neurologists, and otolaryngologists. The most common causes of dizziness are peripheral vestibular disorders, but central nervous system disorders must be excluded. This article provides an overview of the epidemiology of
dizziness, differentiating between central and peripheral vertigo, and
central causes of dizziness.
Review Summary: Dizziness is among the most common complaints in medicine, affecting approximately 20% to 30% of persons
in the general population. Dizziness is a general term for a sense of
disequilibrium. Vertigo is a subtype of dizziness, defined as an
illusion of movement caused by asymmetric involvement of the
vestibular system. Central vestibular lesions affecting the pons,
medulla, or cerebellum cause vertigo, nausea, vomiting, severe
ataxia, multidirectional nystagmus that is not suppressed by optic
fixation, and other neurologic signs. The other types of dizziness are
dysequilibrium without vertigo, presyncope, and psychophysiologic
dizziness, which is often associated with anxiety, depression, and
panic disorder.
Conclusions: Epidemiologic studies indicate that central causes are
responsible for almost one-fourth of the dizziness experience by
patients. The patients history, neurologic examination, and imaging
studies are usually the key to differentiation of peripheral and central
causes of vertigo. The most common central causes of dizziness and
vertigo are cerebrovascular disorders related to the vertebrobasilar
circulation, migraine, multiple sclerosis, tumors of the posterior
fossa, neurodegenerative disorders, some drugs, and psychiatric
disorders.
Key Words: dizziness, central vertigo, epidemiology, differential
diagnosis, central causes

either of the person or the visual surround, (2) dysequilibrium

without vertigo, (3) presyncope (near-faint), and (4) psychophysiologic dizziness, which is often associated with anxiety
and panic.1 4
Vertigo is a subtype of dizziness, defined as an illusory
sensation of movement, and may occur in peripheral and/or
central vestibular disorders. Asymmetric involvement of the
vestibular system leads to vertigo.5 When the vertiginous
sensation is one of horizontal environmental spin or of clear
self-rotation, the lesion is peripheral, mostly in the vestibular
end-organ.3
Dysequilibrium, which is dysfunction in vestibular,
somatosensory, visual systems, and frontal lobes, cerebellum,
and basal ganglia, refers to imbalance or unsteadiness as
dizziness without vertigo.2
Presyncope refers to the lightheaded sensation that
occurs just before fainting. The absence of an illusion of
motion distinguishes it from vertigo. Giddiness, generalized
weakness, and pallor may accompany it. The mechanism is
almost always a reduction in blood flow to entire brain.2,6,7
Psychophysiological dizziness refers to a combination
of symptoms reported as floating, rocking, or swimming
sensations, giddiness, internal spinning, or a feeling of being
removed from ones body. Symptoms may worsen with
stress, fatigue, and some daily activities. It may also develop
after labyrinthine disorders.2,8,9
In this review, the epidemiology of central vertigo and
dizziness, distinguishing central from peripheral vertigo, and
some central causes of dizziness and vertigo are discussed.

(The Neurologist 2008;14: 355364)

izziness is a common complaint among patients visiting

their physicians. Patients use the term dizziness to describe many different sensations. Dizziness can be classified
into 4 groups: (1) vertigo, which is an illusion of movement,

Central causes are responsible for nearly 25%

of the dizziness experienced in patients.

EPIDEMIOLOGY
From the Department of Neurology, Baskent University, Medical School,
Adana Research Center, Adana, Turkey.
Reprints: Mehmet Karatas, MD, Stadyum Cad, 39/1, 01120 Adana, Turkey.
E-mail: drmkaratas@ekolay.net.
Copyright 2008 by Lippincott Williams & Wilkins
ISSN: 1074-7931/08/1406-0355
DOI: 10.1097/NRL.0b013e31817533a3

The Neurologist Volume 14, Number 6, November 2008

Dizziness and vertigo rank among the most common

complaints in medicine, affecting approximately 20% to 30%
of patients in the general population.3 Although dizziness and
vertigo are present in patients of all ages, they are rare
primary complaints in children. Almost 20% of patients older
than 60 years have experienced dizziness severe enough to

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Karatas

affect their daily activities.3,4,10 An estimated 7.5 million

patients with dizziness are examined each year in ambulatory
care setting in the United States, and it is one of the most
common principle complaints in the emergency department.11 On the other hand, in Europe, dizziness and vertigo
are also frequent causes of presentation at the emergency
room, with an incidence that approximates 3.5% in Italy.12
Neuhauser et al13 reported that the lifetime prevalence of
vestibular vertigo was 7.8%, the 1-year prevalence was 5.2%,
and the incidence was 1.5% in Germany. Kanashiro et al14
also reported that the most common syndromes in 515 adults
with dizziness were benign paroxysmal positional vertigo
(28.5%), phobic postural vertigo (11.5%), central vertigo
(10.1%), vestibular neuritis (9.7%), Meniere disease (8.5%),
and migraine (6.4%) (Table 1). On the other hand, benign
paroxysmal vertigo of childhood, migraine-associated dizziness, vestibular neuronitis, and otitis media-related dizziness
also accounted for vertigo in children.10 According to Sekine
et al,15 the most common peripheral vestibular disorder was
benign paroxysmal positional vertigo (32%), followed by
Meniere disease (12%), all peripheral vestibular disorders
accounted for 65%, and central vestibular disorders accounted for 7%. Kroenke et al16 also published the results that
dizziness was attributed to a peripheral vestibulopathy in
44%, a central vestibulopathy in 11%, psychiatric causes in
16%, other conditions in 26%, and unknown causes in 13% of
the patients in their series. Thus, it seems that these epidemiologic studies indicate that central causes (certain central
vestibular disorders, migraine, and phobic) are responsible
for nearly 25% of the dizziness experienced in patients.1518
In addition, certain serious causes for central vertigo are
relatively uncommon, including cerebrovascular disease
(6%7%), cardio-circulatory diseases (1.5%3.6%), and posterior fossa tumors (1%).15,19 The prevalence of various
causes of central vertigo has not been well delineated in
epidemiologic studies so far.

DIFFERENTIATING BETWEEN PERIPHERAL AND

CENTRAL VERTIGO
The vestibular system consists of peripheral and central
part. The semicircular canals, the otoliths (utricle and saccule), and the vestibular nerve are peripheral parts of the
TABLE 1. Frequency of Various Causes of Dizziness in
Different Countries (%)
Japan
Brazil
USA
(Sekine 2005) (Kanashiro 2005) (Kroenke 2000)
BPPV
Meniere disease
(Other) peripheral
vestibular
Central vestibular
Psychiatric
Migraine
Unknown
Other

32
12
21

28.5
8.5
9.7

44

10.1
11.5
6.4

11
16

13
26

vestibular system. The vestibular nuclear complex, vestibulocerebellum, brainstem, spinal cord, and vestibular cortex
are also central parts of the vestibular system.20,21 Vertigo
occurs in acute unilateral loss of vestibular function. Autonomic symptoms such as sweating, pallor, nausea, and vomiting are also commonly associated with vertigo, but are rare
with other types of dizziness.5

Central vertigo is generally associated with

severe imbalance, additional neurologic signs,
less prominent movement illusion and nausea,
and central nystagmus.

The physician first must determine whether the vertigo

is of peripheral or central origin, because the presence of
central disorders such as cerebellar infarction or hemorrhage,
basilar artery occlusion, vertebral artery dissection, or a
tumor of the posterior fossa may require emergency management. Central vertigo is generally associated with severe
imbalance, additional neurologic signs, less prominent movement illusion and nausea, and central nystagmus (which is
pure vertical/torsional, multidirectional, and no suppression
with optic fixation).2,5,21 The history usually provides the
basic information for distinguishing between peripheral and
central vertigo.5 Nausea and vomiting are typically more
pronounced in peripheral vertigo than in central vertigo.
Imbalance is always associated with vertigo; more severe
imbalance is especially associated with central causes. Patients with peripheral vestibular lesions also have imbalance,
but they are able to walk. By contrast, many patients with
central vestibular lesions are unable to stand or walk. Auditory symptoms such as hearing loss, tinnitus, fullness, or pain
in the ear are commonly seen in peripheral lesions such as
those affecting the labyrinth or eighth nerve. Besides hearing
loss and tinnitus, lesions of the internal auditory canal may be
associated with ipsilateral facial weakness. Lesions in the
cerebellopontine angle may cause ipsilateral facial numbness,
weakness, and limb ataxia. Vertigo also can be seen as part of
an aura of temporal epilepsy, and during the seizure, the
patient is amnestic. Owing to a rapid compensation process,
acute vertigo due to peripheral lesion tends to improve in
days to weeks, whereas central vertigo may not improve or
may do so more slowly.5,2226 Table 2 outlines guidelines
helpful in the differential diagnosis of vertigo.

Spontaneous nystagmus of peripheral origin

increases in amplitude with gaze in the
direction of the fast phase.

BPPV indicates benign paroxysmal positional vertigo.

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The Neurologist Volume 14, Number 6, November 2008

TABLE 2. Differential Diagnosis of Central and

Peripheral Vertigo
Nausea
Movement illusion
Worse with head movement
Neurologic signs
Imbalance
Hearing loss
Oscillopsia
Caloric test
Recovery

Central Vertigo and Dizziness

TABLE 3. Differentiation Between Spontaneous Nystagmus

of Central and Peripheral Origin

Central

Peripheral

None/mild
Less prominent
No
Common
Severe
Rare
Severe
Hyperexcitability
Months or longer

Severe
More prominent
Yes
Rare
Mild to moderate
Common
Mild
Canal paresis
Days to weeks

Appearance

Suppression with OF
Alexander law
Direction-fixed
Localization

Central

Peripheral

Pure vertical/torsional,
pan, multidirectional,
disconjugate, or
dissociated, may
change direction with
changes in gaze
No or minimal
No
No
Medulla, pontine
tegmentum, cerebellum

Torsional-horizontal,
unidirectional in all
gazes, conjugated

Yes
Yes
Yes
Labyrinth, vestibular
nerve

PAN indicates periodic alternating nystagmus; OF, optic fixation.

Nystagmus, which is an involuntary rhythmic oscillation of the eyes, is helpful for localizing vertigo. The direction of the fast phase defines the direction of nystagmus.
Spontaneous nystagmus occurs in patients seated, eyes in
primary position, and without movement of the head. Gazeevoked nystagmus is elicited by changes in gaze position.
Positional nystagmus is present in particular head positions,
not in the sitting position.2125 Typical nystagmus produced
by labyrinth dysfunction is a jerky nystagmus. Spontaneous
nystagmus of peripheral origin increases in amplitude with
gaze in the direction of the fast phase and decreases with gaze
away from the fast phase (Alexander law). Peripheral spontaneous nystagmus is also inhibited with optic fixation, and
that nystagmus usually is prominent for only the first 12 to 24
hours.5,22,25,26 Within a few days, peripheral spontaneous
nystagmus may be inhibited completely, even with gaze in
the direction of the fast phase. Spontaneous nystagmus of
central origin typically changes direction when the patient
looks away from the direction of fast phase. It often persists for
weeks or months. Vertical or pure rotatory nystagmus is always
produced by a central vestibular lesion.5 Neurologic signs such
as diplopia, disconjugate gaze, Horner syndrome, severe gait
ataxia, dysarthria, dysphagia, facial weakness and numbness,
long tractus findings, and limb incoordination also indicate a
central lesion. On the other hand, diplopia can occasionally be
seen in an acute peripheral vestibular lesion for a few days or so
because of deafferentation of otolith inputs.21,22,27

ders. During the maneuver, the patient fixates a visual target,

and eye position is observed immediately after a small thrust
of the head to the left and right. A refixation saccade after the
head thrust indicates a decreased vestibulo-ocular reflex on
the side of head thrust. If the defect is on both sides, the head
thrust test will be positive in both directions.23,28 Postural
testing is important, particularly when evaluating paroxysmal
positional vertigo. In peripheral vestibular disorders, caloric
testing produces an impaired response in one ear often called
canal paresis. Directional preponderance occurs with both
peripheral and central vestibular lesions. Central vestibular
disorders also lead to deficits in the conjugation of eye
movements, saccadic pursuit and horizontal optokinetic abnormalities, central spontaneous or positional nystagmus,
failure of fixation suppression, slowing of the nystagmus fast
phases, a slowing down of the nystagmus slow phases,
perverted nystagmus, vertical optokinetic abnormalities, and
retraction nystagmus.24 28 Table 3 differentiates between
spontaneous peripheral and central nystagmus.
With undiagnosed vertigo, after careful history taking,
general, neurologic, and neuro-otologic examinations, as well
as screening tests for blood count, electrolyte and glucose
levels, and thyroid function are required; neuroradiologic
imaging is indicated when a central lesion is suspected.5,20 23

SELECTED CENTRAL CAUSES OF DIZZINESS

A refixation saccade after the head thrust

indicates a decreased vestibulo-ocular reflex on
the side of head thrust.

The head-shaking nystagmus, which is elicited in response to a vigorous rotation of the head in the horizontal
plane, is also a useful finding to identify patients having
unilateral vestibular hypofunction. On the other hand, the
head-thrust maneuver is used to assess the vestibulo-ocular
reflex, and it is only positive for peripheral vestibular disor 2008 Lippincott Williams & Wilkins

Table 4 outlines common central causes of dizziness. The

selected most common disorders related to dizziness are discussed below.

Cerebrovascular Disorders
The blood supply to the inner ear, brainstem, and
cerebellum arises from the vertebrobasilar system. Vertigo
can occur from occlusion of this system, which includes the
vertebral arteries, the basilar artery, the posterior inferior
cerebellar artery, the anterior inferior cerebellar artery, and
the superior cerebellar artery. Because circulation to the inner
ear arises from the vertebrobasilar system, usually from the
anterior inferior cerebellar artery, vertigo due to cerebrovascular disease can be of peripheral or central origin.2,29 Vascular syndromes related to vertigo or dysequilibrium are
given below.2

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Karatas

TABLE 4. Central Causes of Dizziness

1.
2.
3.
4.
5.
6.
7.
8.
9.
10.
11.

Cerebrovascular disorders
Migraine
Multiple sclerosis
Central positional vertigo/nystagmus
Epilepsy
Craniocervical junction disorders
Neoplastic: primary, metastatic or paraneoplastic
Inherited ataxias
Psychophysiologic
Global cerebral hypoperfusion and hypometabolism
Neurodegenerative disorders: parkinsonism, normal pressure
hydrocephalus
12. Posttraumatic dizziness
13. Toxic: alcohol, Cu, Hg, talium, lead, organic solvents, drugs
(diphenylhydantoin, barbiturates, primidone, carbamazepine,
5-fluorourasil, methotrexate, piperazine nitrofurantoin, lithium)

a.
b.
c.
d.
e.
f.

Vertebrobasilar transient ischemic attacks

Posterior inferior cerebellar artery syndrome
Anterior inferior cerebellar artery syndrome
Superior cerebellar artery syndrome
Insular infarction
Cerebellar and brainstem hemorrhage

Vertebrobasilar transient ischemic attacks of the cerebellum or brainstem are characterized by episodic vertigo or
dysequilibrium, usually of 1 to 15 minutes duration, with
concurrent diplopia, dysarthria, ataxia, drop attack, and clumsiness of the extremities. Vertebrobasilar transient ischemic
attacks presenting as isolated vertigo are usually related to
vascular occlusion in the distal segment of the vertebral
arteries between the posterior inferior cerebellar artery and
the anterior inferior cerebellar artery, and the subclavian steal
syndrome.5,30 33 Rarely, insular infarction with the middle
cerebral artery territory can cause contraversive tilts, body
lateropulsion, nausea, unsteady gait, and rarely, rotational
vertigo.34,35 Hemorrhage into the brainstem or cerebellum
may produce sudden vertigo. Headache and neck stiffness
suggest hemorrhage rather than infarction.31,36
When evaluating the patient with acute vertigo, the
physician should seek risk factors for stroke, such as hypertension, hyperlipidemia, diabetes, smoking, heart disease. On
the other hand, headache and neck pain followed by vertigo
or unilateral facial paresthesias is an important sign of vertebral artery dissection, and may precede onset of stroke by
several days.29,35,37

Migraine may be associated with many

vestibular symptoms, including episodic vertigo,
chronic motion sensitivity, and
nonspecific dizziness.

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The Neurologist Volume 14, Number 6, November 2008

Migraine
Migraine is estimated to occur in 18% to 29% of
women, 6% to 20% of men, and 4% of children.2 Vertigo
may occur in up to 25% of patients with migraine.5 Headache
and dizziness are 2 of the most frequent symptoms occurring
in the general population. Conversely, migraine and vertigo
are 2 clinical features that tend to occur together.38 Migraine
may be associated with many vestibular symptoms, including
episodic vertigo, chronic motion sensitivity, and nonspecific
dizziness. On the other hand, patients with migraine may
present with benign paroxysmal positional vertigo, Meniere
disease, and motion sickness more often than patients without
migraine. However, persistent cerebellar syndrome may develop in the course of familial hemiplegic migraine.39,40
Dizziness also may be due to orthostatic hypotension, anxiety
disorders, or major depression, all of which have an increased
prevalence in migraineurs.39
It has been believed that vertigo in migraine may arise
from disorders such as spreading depression, regional
changes in brain perfusion, release of neurotransmitters, and
paroxysmal dysfunction of ion channels anywhere along the
peripheral or central vestibular structures.40,41 The principle
clinical vestibular syndromes related to migraine can be
classified into 3 groups39,42:
a. Basilar-type migraine
b. Benign paroxysmal vertigo of childhood
c. Migrainous vertigo (or vestibular migraine)
Basilar-type migraine, as a subtype of migraine with
aura, is characterized by recurrent headaches, usually occipital, associated with aura symptoms localizing to the vascular
territory of the basilar artery.2 The International Headache
Society criteria for basilar-type migraine require the presence
of 1 or more preceding aura symptoms. The aura generally
lasts less than 1 hour and is usually followed by a headache
that may be occipital. The visual aura is usually followed by
vertigo, tinnitus, decreased hearing, diplopia, ataxia, dysarthria, bilateral paresthesia, and paresis and impaired cognition. The headache can be associated with nausea and projectile vomiting.43 Triptans are contraindicated in basilar-type
migraine because of risks of vasospasm and stroke.2
Benign paroxysmal vertigo of childhood, as a subtype
of childhood periodic syndromes in migraine, is characterized
by onset between 1 and 4 years, abrupt randomly occurring
attacks of vertigo and imbalance often with nausea and
vomiting that lasts for 30 seconds to 20 minutes, usually
unaccompanied by headache. These children are healthy
between attacks. This syndrome often subsides by adolescence or evolves into migraine headaches.21 Migraine prophylaxis can be effective at decreasing the frequency and
severity of attacks.43
Migrainous vertigo is a vestibular disorder caused by
migraine, which presents with attacks of spontaneous or
positional vertigo lasting seconds to days and migrainous
symptoms during attack. The prevalence of migrainous vertigo is 7% in the dizziness clinic and 9% in the migraine
clinic.44,45 Although migrainous vertigo is the most common
cause of spontaneous recurrent vertigo, it is not presently
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The Neurologist Volume 14, Number 6, November 2008

included in the International Headache Society classification

of migraine.39 Migrainous vertigo can be diagnosed according to the following criteria: (1) recurrent vestibular symptoms, (2) migraine according to criteria of International
Headache Society, (3) at least 1 of the following migrainous
symptoms during at least 2 vertiginous attacks: headache,
phonophobia, photophobia, scintillating scotoma, or other auras,
and (4) exclusion of other causes.40,44 Vertigo is occasionally
coincident with headache but more often occurs as an isolated
symptom. Migraine-related vertigo is characterized by varying
motion illusions and motion sensitivity, often with nausea.
Vestibular symptoms associated with migraine are often described as spinning, slow or fast rotation, rocking, tilting, swaying, swimming, to and fro oscillation, or floating. Attack duration often varies from seconds to days. Spell frequencies also
vary from 1 per month to 40 per month. Migraine also may
occur with other causes of vertigo including benign paroxysmal
positional vertigo, psychogenic dizziness, Meniere disease, and
other vestibular disturbances.21,44,46
Treatment of migrainous vertigo currently parallels that
of migraine headache.42 Mild symptoms or brief or infrequent
spells may be left untreated. The long lasting (at least 30
minutes) and frequent symptoms need vestibular suppressants
such as meclizine, diazepam, or promethazine during vertigo
attacks, and a migraine prophylactic drug.21,47

Central Vertigo and Dizziness

frequently have short-lasting paroxysmal vertigo and dizziness

not related to benign paroxysmal positional vertigo.49

Central Positional Vertigo/Nystagmus

Positional vertigo results from a transient excitation
within the vestibular system triggered by a change in position. Positional vertigo has been attributed to lesions of the
semicircular canals and their connections in the vestibular
nuclei and cerebellum. Structural and metabolic factors in the
semicircular canals can alter the specific gravity of the cupula
and trigger positional vertigo during a particular head position. The peripheral and central origins of positional vertigo
have long been known. The most common form of positional
vertigo is benign paroxysmal positional vertigo, and is nearly
always benign and treatable; however, in rare cases, positional vertigo can be a symptom of central nervous system
disorder. Bertholon et al50 reported that benign paroxysmal
positional vertigo was found in 80% of patients with positional vertigo, and central positional vertigo/nystagmus was
diagnosed in 12% of patients with positional vertigo. Patients
with benign paroxysmal positional vertigo have brief episodes of vertigo with position change, typically when turning
over in the bed and getting in and out of bed. The diagnosis
is easily made by Dix-Hallpike test.5

Multiple Sclerosis
Vertigo is the initial symptom in about 5% of patients
with multiple sclerosis and occurs some time during the
disease in 50% of patients. Vestibular symptoms in multiple
sclerosis may be sustained over days to weeks, and may be
paroxysmal or positional. Prolonged spontaneous attacks of
vertigo occur if a demyelinative plaque is located in the root
entry zone of the vestibular nerve or nucleus. These findings
resemble acute peripheral vestibulopathy such as vestibular
neuritis. Vertigo may last for hours to days and be associated
with imbalance, vomiting, direction-fixed horizontal-torsional nystagmus with quick phase beating toward the unaffected side, and suppressed by visual fixation, and unilateral
canal paresis on caloric testing. Sudden hearing loss may
accompany the vertigo when the auditory nerve is also
involved. On the other hand, if central vestibular structures
such as the vestibular nuclei, cerebellar peduncles, or the
cerebellum are affected by demyelinating plaques, then vertigo, severe ataxia, other cranial nerve abnormalities, directionchanging nystagmus, pure vertical nystagmus, intentional
tremor, or pyramidal tract dysfunction may occur. Selective
involvement of the vestibular nuclei may produce a syndrome
indistinguishable from that of a peripheral lesion, except the
nystagmus may not be suppressed by optic fixation, indicating a
central origin.2,48 Pendular nystagmus and internuclear ophthalmoplegia are common findings in multiple sclerosis. Such signs
cause distressing oscillopsia, dizziness, diminished vision, and
diplopia rather than vertigo. Frohman et al49 reported that the
most common cause of vertigo in patients with multiple sclerosis
is benign paroxysmal positional vertigo related to complication
of treatments. Central positional vertigo also can be seen in
multiple sclerosis with lesions located in the region of the fourth
ventricle.5 Conversely, most patients with multiple sclerosis
2008 Lippincott Williams & Wilkins

Central positional vertigo/nystagmus can be

caused by various central nervous system
lesions located in the region of the fourth
ventricle, dorsal vermis, and vestibular nuclei.

Central positional vertigo/nystagmus can be caused by

various central nervous system lesions located in the region
of the fourth ventricle, dorsal vermis, and vestibular nuclei.23,51 The most probable explanation for the central positional vertigo/nystagmus is a vestibular tone imbalance
caused by disinhibition of the vestibular reflexes.51 There are
3 forms of central positional vertigo/nystagmus: (1) positional downbeat nystagmus, (2) positional nystagmus without
concurrent vertigo, and (3) positional vertigo with nystagmus. The most common associated disorders due to central
positional vertigo/nystagmus are cerebrovascular disorders,
spinocerebellar atrophy, multiple sclerosis, Arnold-Chiari
type-1 malformations, tumors of the brainstem and cerebellum, and some drugs.51,52 Differentiation between benign
paroxysmal positional vertigo and central positional vertigo/
nystagmus based on features such as latency, courses and
duration of nystagmus during attack, fatigability, vertigo,
vomiting, and time period during which nystagmus occurs
may be impossible. Only central positional vertigo/nystagmus is typically associated with pure vertical positional
nystagmus that does not remit with repeated positioning.
Other neurologic signs of brainstem and cerebellum can be
seen. Magnetic resonance imaging is the diagnostic tool of

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TABLE 5. Features Distinguishing BPPV From CPVN

Features

BPPV

CPVN

Latency
Duration of
nystagmus
Direction of
nystagmus

330 s
530 s

None
30 s

Torsional/vertical (P,
A canals),
horizontal (H canal)

Pure vertical/torsional
inappropriate with
canal

No

()

Yes
Always
()
Rare

No
Can not be seen
()
Common

Persistent downbeat
nystagmus in any
position
Fatigability
Vertigo
Severe nausea
Vomiting in first
maneuver
Worse with
nonspecific head
movement
Evoked by DixHallpike
Resolves after
maneuvers
Other neurologic
sign

No

()

()

()

()

No

No

May be

BPPV indicates benign paroxysmal positional vertigo; CPVN, central positional

vertigo/nystagmus; P, posterior; A, anterior; H, horizontal.

choice for identifying the posterior fossa lesion related to

central positional vertigo/nystagmus.5,23 Table 4 provides the
features differentiating benign paroxysmal positional vertigo
from central positional vertigo/nystagmus (Table 5).

Epilepsy
Epilepsy is a rare cause of vertigo. Vertigo associated
with epilepsy can be classified as vertiginous epilepsy, rotatory seizures, vestibulogenic seizures, and dizziness and vertigo due to anticonvulsants.53
Recurrent vertigo attacks, and imbalance or dizziness
with nausea, vomiting, or unilateral tinnitus during simple
partial seizures may occur without other symptoms of epilepsy.2 Such attacks also can be an epileptic aura for other
seizures. Such seizures can be triggered by stimulation of the
vestibular cortex in fronto-temporo-parietal region.5355
Rotatory seizures (gyratory seizures) are defined as
circling behavior characterized by a rotation around the body
axis during a seizure for at least 180 degree, speech arrest,
and secondarily generalized seizures. These seizures are relatively uncommon and may occur more frequently in frontal
lobe epilepsy than in temporal lobe epilepsy. Thalamic stimulation also has been shown experimentally to induce circling
movements.56,57
Seizures evoked by vestibular stimulation (during caloric testing) are called vestibulogenic seizures and are complex partial or secondarily generalized seizures. Vertigo can
not be always seen during such evoked seizures.58,59
Anticonvulsants such as diphenylhydantoin, carbamazepine, barbiturates, diazepam, primidone, and others can

360

frequently cause dizziness, vertigo, imbalance, and double

vision as an adverse effect.2

Craniocervical Junction Disorders

Spontaneous and positional vertigo, tinnitus, hearing
loss, dysarthria, dysphonia, ataxia, shortness of the neck,
lower neck hair line, limited range of motion in the neck,
lower cranial nerve signs, and sometimes hydrocephalus are
typically seen in craniocervical junction anomalies. Symptoms worsen with neck extension and cough.60 62 The anomalies associated with vertigo/dizziness are listed below:
a. Congenital fusion of the atlas and foramen magnum: This
is the most common anomaly at the craniocervical junction. The anteroposterior diameter of the canal behind the
odontoid process is less than 19 mm. There are signs of
upper cervical spinal cord compression.61,62
b. Atlantoaxial dislocation: This indicates instability of C1
(atlas) on C2 (axis) of more than 3mm from the C1 arc and
odontoid process. It may be congenital; however, it is
frequently associated with rheumatoid arthritis and Down
syndrome.21
c. Platybasia and basilar invagination: Platybasia refers to a
flattening of the base of the skull and the angle formed by
intersection of the plane of the clivus; the plane of the
anterior fossa is greater than 135 degrees. Basilar invagination is an upward bulging of the occipital condyles.
These anomalies give rise to a characteristic shortness of
the neck and a combination of cerebellar and spinal signs.
A normal-pressure hydrocephalus also may be seen.61,62
d. Chiari type-1 malformation: It is defined as more than 5
mm of cerebellar tonsillar herniation below the foramen
magnum, most easily visualized on a midline sagittal
T1-weigthed magnetic resonance image. Patients may
present with constant or slowly progressive dizziness or
gait instability that may worsen with neck extension. A
Chiari type-1 malformation is frequently associated with
spontaneous or positional vertigo, tinnitus, hearing loss,
and lower cranial nerve signs. Downbeat nystagmus may
occur spontaneously or may be induced with head hanging. Spontaneous vestibular nystagmus also can occur on
a central basis as a result of compression of medullary
vestibular structures. The severity of symptoms of a Chiari
type-1 malformation may progress over time owing to
aging, trauma, and degenerative effects on the
junction.21,63 65

Tumors of the Cerebellopontine Angle and

Posterior Fossa
Vestibular schwannoma or acoustic neuroma is a benign tumor arising from Schwann cells of the vestibular nerve
into internal auditory canal. This tumor accounts for 5% to
10% of all intracranial tumors and is the most commonly
occurring tumor of the cerebellopontine angle. The vestibular
schwannoma grows slowly into the internal auditory canal
and cerebellopontine angle, displacing the adjacent cerebellum, pons, or fifth and seventh cranial nerves. Progressive
unilateral hearing loss, tinnitus, and ataxia are common.
Patients sometimes note true vertigo. Bruns nystagmus,
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The Neurologist Volume 14, Number 6, November 2008

which is a combination of peripheral vestibular nystagmus

and gaze-paretic nystagmus, also may be seen. Less often,
facial numbness and weakness, loss of taste and otalgia,
trigeminal neuralgia, and symptoms of increased intracranial
pressure may be seen. These symptoms also may occur with
any mass in the cerebellopontine angle including meningioma, trigeminal neuroma, cholesteatoma, epidermoid cysts,
and metastatic tumors. Gadolinium-enhanced magnetic resonance imaging is the best technique for diagnosing a vestibular schwannoma. Neurofibromatosis type-2 is also an autosomal-dominant disorder characterized by bilateral vestibular
schwannoma, meningioma, or glioma.66 68
Cerebellopontine angle meningiomas cause hearing
loss and facial pain or numbness, dizziness and ataxia, and
are the second most common tumor of the posterior fossa,
after vestibular schwannoma. Magnetic resonance imaging usually differentiates a meningioma from a vestibular
schwannoma because the latter originates from the internal
auditory canal.66
Cerebellar tumors are astrocytomas, ependymomas,
medulloblastomas, hemangioblastomas, and metastases. Medulloblastomas are the most common cerebellar tumors in
children, and metastases are the most common in adults. The
typical history for a posterior fossa tumor is the accumulation
and progression of neurologic symptoms over weeks to
months. Ataxia, nystagmus, and other ocular motor disorders
are common and also depend on the specifically affected part
of cerebellum. Positional vertigo and nystagmus, usually
downbeat, also may occur. Occipital headache, radiating
forward, is the most common symptoms manifesting with
cerebellar tumors. Cyclic vomiting, or vomiting upon lying
down, and lower cranial nerve palsies may occur late in the
course. Compression of the fourth ventricle may cause obstructive hydrocephalus that leads to papilledema.2,66

Paraneoplastic Cerebellar Degeneration

Paraneoplastic neurologic syndromes are nervous
system disorders caused by cancer but not metastatic
disease, vascular or metabolic deficits, infections, or nutritive deficiency. Immunologic factors probably play the
crucial role in the pathogenesis of paraneoplastic neurologic syndromes. Paraneoplastic cerebellar degeneration is
a rare complication of cancer and is most frequently
associated with ovary, breast, and lung cancer as well as
Hodgkin lymphoma. Several paraneoplastic antineuronal
antibodies such as anti-Yo and anti-Ri (breast and gynecologic cancer), anti-Hu (lung cancer), anti-Tr, and antimGluR1 (Hodgkin lymphoma) have been identified in
patients with paraneoplastic cerebellar degeneration. Paraneoplastic cerebellar degeneration typically begins fairly
abruptly and progress rapidly through several months with
a subacute cerebellar syndrome. Patients with paraneoplastic cerebellar degeneration present with dizziness, nausea,
and vomiting followed by gait instability, diplopia, nystagmus, gait and appendicular ataxia, dysarthria, and dysphagia. Opsoclonus/myoclonus also may be seen in children with neuroblastoma.2,69 73
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Central Vertigo and Dizziness

Hereditary Ataxias
The hereditary ataxias are a heterogeneous group of
genetic disorders that can be seen as an autosomal-dominant,
recessive, X-linked, mitochondrial inheritance. The adultonset autosomal dominant ataxias are now classified by
sequential numbers assigned when a new genetic locus is
discovered, currently SCA1 through SCA28. The most common autosomal recessive ataxia is Friedreich ataxia, which is
usually caused by a GAA repeat expansion in the frataxin
gene. Symptoms of Friedreich ataxia begin before 20 years
but may manifest as late as the sixth decade. An axonal
sensory neuropathy with areflexia, positive Babinski sign,
dysarthria, proprioceptive and vibratory sensory loss, optic
atrophy, scoliosis, diabetes, and cardiomyopathy besides typical cerebellar ataxia are characteristic. Other rare causes of
autosomal recessive ataxias include ataxia-telangiectasia,
abetalipoproteinemia, and Refsum syndrome.29,74,75
The familial episodic ataxias are rare, distinct, and
dominantly inherited diseases of early onset characterized by
episodes of ataxia. Most patients recover fully between attacks, but some may develop progressive ataxia with cerebellar atrophy. There are 2 subtypes of episodic ataxia:
episodic ataxia type 1 (EA1), which manifests with interictal
myokymia without vertigo; and episodic ataxia type 2 (EA2),
which often manifests with ataxia, vertigo, nausea, vomiting,
dysarthria, and interictal mainly downbeat and gaze-evoked
nystagmus. Some EA2s develop progressive ataxia later in
life. Ataxic spells lasting minutes to hours are provoked by
stress, exercise, or alcohol, and may respond to acetazolamide. Familial episodic ataxias indeed are channelopathies.
EA1 is caused by mutations in a potassium channel-encoding
gene on chromosome 12p13, whereas EA2 is caused by
mutations in a calcium channel-encoding gene localized on
chromosome 19p, which is highly expressed in the cerebellum, which is also the disease-causing gene in spinocerebellar
ataxia type 6 and several kindreds with familial hemiplegic
migraine.29,76 80

Psychiatric Dizziness
a. Psychogenic dizziness: Panic disorder with agoraphobia,
generalize anxiety and personality disorders, and depression are frequently seen in dizzy patients. Patients with
psychogenic dizziness have certain stimuli or social events
as main causes, and a clear dissociation between objective
and subjective dysequilibrium, inappropriately excessive
anxiety or fear, and no spontaneous nystagmus detected.
On the other hand, primary vestibular disorders also can
induce secondary psychiatric symptoms.2,81,82
b. Phobic postural vertigo: Phobic postural vertigo is comparatively the most frequent form of dizziness and is
characterized by a combination of nonrotational dizziness
and subjective disturbance in the upright stance and gait
despite normal results of clinical balance tests. Patients
with phobic postural vertigo often report a particularly
increased unsteadiness when looking at moving visual
scenes. It may be accompanied by anxiety and panic
symptoms. It is almost invariably associated with particular constellations of perceptual stimuli or social situa-

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Karatas

tions. There is a tendency for rapid conditioning, generalization, and avoidance behavior to develop. The
subjective postural imbalance without falls is typical for
phobic postural vertigo. Frequently, onset of the condition
follows periods of particular psychosocial stress.82 84
Phobic postural vertigo should be distinguished from
panic disorder or agoraphobia. Behavioral therapy and
regular physical activity are effective in phobic postural
vertigo. When left untreated, phobic postural vertigo becomes chronic in most cases and leads to considerable
impairments also at work.8,9,25,83,85

Presyncopal Dizziness
Presyncope refers to light-headedness without an illusion of movement that occurs just before fainting or losing
consciousness. Presyncopal signs and symptoms including
generalized weakness, giddiness, headache, blurred vision,
diaphoresis, paresthesia, pallor, nausea, and vomiting are
usually present for seconds or minutes before loss of consciousness. Presyncopal symptoms may be spontaneous, positional, or have specific triggers depending on the cause. The
mechanism is almost always a reduction in blood flow to the
entire brain.
Causes of presyncope have been categorized as cardiovascular, neurologic, neurocardiogenic (vasovagal), metabolic, and psychiatric. Cardiovascular causes can be structural heart disease, coronary heart disease, and arrhythmia.
The most common neurologic causes are orthostatic hypotension and postural orthostatic tachycardia syndrome. Orthostatic hypotension, which is one of the most common
causes of syncope, can be attributed to impaired peripheral
vasoconstriction or to a reduction of intravascular volume.
On the other hand, postural orthostatic tachycardia syndrome
is a type of orthostatic intolerance characterized by excessive
tachycardia and decreased cerebral blood flow in the upright
position. Neurocardiogenic presyncope or vasovagal presyncope as an unexplained cause can be associated with painful
or emotionally stressful situations such as anxiety or fear,
with prolonged standing or specific trigger situations. It is
often noted in individuals receiving sympathetic blocking
agents and vasodilator drugs for hypertension, elderly patients receiving tranquilizers, and patients with anemia. Hypovolemia and hypoglycemia may lead to faintness and
weakness. Hyperventilation is also a common cause of dizziness in anxious patients.7,86,87
Medical history and physical examination including
pulse and blood pressure monitoring both supine and
standing, cardiac auscultation, electrocardiogram, Holter
monitoring, tilt-table test, and blood glucose and hematocrit analyses are the most important parts of the evaluation
for suspected presyncope.6,7,86

CONCLUSIONS
Dizziness and vertigo are among the most common
complaints in medicine, affecting approximately 20% to 30%
of persons in the general population. Dizziness is a general
term for a sense of disequilibrium. Vertigo is a subtype of
dizziness, defined as an illusion of movement caused by
asymmetric involvement of vestibular system. Central ves-

362

tibular lesions affecting the pons, the medulla, or the cerebellum cause vertigo, nausea, vomiting, severe ataxia, multidirectional nystagmus (which is not suppressed by optic
fixation), and other neurologic signs. Epidemiologic studies
indicate that central causes are responsible for almost onefourth of the dizziness experienced by patients. The most
common central causes of dizziness and vertigo are cerebrovascular disorders related to vertebrobasilar circulation,
migraine, multiple sclerosis, tumors of posterior fossa, neurodegenerative disorders, some drugs, and psychiatric disorders. The
other types of dizziness are dysequilibrium without vertigo,
presyncope, and psychophysiologic dizziness, which is often
associated with anxiety, depression, and panic disorder.

ACKNOWLEDGMENT
The author is grateful to Prof. Dr. Tulay Kansu for
editorial assistance with the article.
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