Central Vestibular Disorders

CENTRAL CAUSES OF
DIZZINESS AND VERTIGO
1. Vascular
– Ischemic stroke/TIA
• Brainstem
• Cerebellar
• Labyrinthine
– Hemorrhage
• Brainstem
• Cerebellar
– Migraine
• Vertigo
• Dysequilibrium
• Benign paroxysmal vertigo
• Paroxysmal torticollis
– Frontal gait disorder of the elderly
2. Inflammatory
– Multiple sclerosis
– Cerebellitis
– Susac syndrome
– Behçet's syndrome
– Systemic lupus erythematosus
– Sarcoidosis
3. Infectious
– Intracranial complications of suppurative otitis
4. Neoplastic
– Brainstem
– Cerebellar
– Fourth ventricle
– Paraneoplastic
– Paraneoplastic cerebellar
• degeneration
• Opsoclonus/myoclonus
• Craniocervical junction disorders
– Chiari malformation
– Basilar impression
– Syringobulbia
• Inherited ataxias
– Autosomal recessive
• Friedreich ataxia
• Ataxia-telangiectasia
• Vitamin E deficiency
• Refsum disease
– Autosomal dominant
• Spinocerebellar
• ataxias
• Episodic ataxias
• Metabolic
– Wernicke's encephalopathy
– Diabetes
– Vitamin B12 deficiency
– Hypothyroidism
– Hypoglycemia
– Hyperventilation
• Toxic
– Medications
– Alcohol
Aminoglycosides, cisplatin, Vertigo, dysequilibrium Vestibular hair cell toxicity
salicylates, chloroquine, loop
diuretics

Antiepileptics Dysequilibrium, gazeevoked Cerebellar toxicity

(carbamazepine, and downbeat nystagmus
phenytoin, primidone,
lithium)

Tranquilizers: barbiturates, Intoxication CNS depression

antihistamines, tricyclic
amines

Antihypertensives, diuretics Near-faint Postural hypotension

Amiodarone Dysequilibrium, downbeat Unknown, cerebellar toxicity

nystagmus

Alcohol Intoxication, dysequilibrium, CNS depression,

positional vertigo cerebellar toxicity, change
in cupula specific gravity

MTX Dysequilibrium Brainstem and cerebellar

toxicity
• Degenerative
– Parkinson's disease
– Progressive supranuclear palsy
– Multiple systems atrophy
– Normal pressure hydrocephalus
• Epilepsy
– Partial seizures
• Trauma
– Brain contusion
– Post-concussion syndrome
• Physiologic
– Mal de debarquement syndrome
– Motion sickness
• Psychophysiologic
– Chronic anxiety
– Panic disorder
– Phobic postural vertigo
• Psychogenic gait disorder
• Global cerebral hypoperfusion
– Vasovagal presyncope
– Reduced cardiac output
– Autonomic insufficiency
– Hypovolemia
• Multisensory disturbance
– Peripheral neuropathy
– Cervical or thoracic myelopathy
– Visual loss
• Ocular motor disorders
– Superior oblique myokymia
– Voluntary nystagmus
 Central Peripheral

Imbalance severe mild to moderate

Neurologic symptoms frequent rare

Nystagmus changes direction in Unidirectional in all gaze

different gaze positions;
positions; no change with decreases with visual
visual fixation fixation
Hearing loss rare frequent

Nausea variable, may be absent severe

Recovery by central slow rapid

compensation
Lateral Medullary Infarction
Wallenberg’s Syndrome
Lateral Pontomedullary Infarction
Cerebellar Infarction
 Axial T2-weighted brainstem MRI showing high signal
intensity in the region of the eighth nerve root-entry zone.
Note that the vestibular nuclei appeared to be spared.
• TL, temporal bone
• Ch, cochlea
• HSC, horizontal SC
• SO, superior olive
• V(sn), spinal nucleus
• ICP, inferior cerebellar
peduncle
• 4th, fourth ventricle
• VN, vestibular nuclei.
 RECURRENT SPONTANEOUS
ATTACKS OF VERTIGO - Migraine
• Migraine without aura
• Migraine with aura
– Typical aura with migraine headache
– Typical aura with non-migraine headache
– Typical aura without headache
– Familial hemiplegic migraine
– Sporadic hemiplegic migraine
– Basilar-type migraine
• Childhood periodic syndromes that are commonly
– precursors of migraine
– Cyclical vomiting
– Abdominal migraine
– Benign paroxysmal vertigo of childhood
• Retinal migraine
• Complications of migraine
– Chronic migraine
– Status migrainosus
– Persistent aura without infarction
– Migrainous infarction
– Migraine-triggered seizure
 DIAGNOSTIC CRITERIA FOR
MIGRAINE – with aura
• A. At least five attacks fulfilling B–D.
• B. Headache attacks lasting 4 to 72 hours (untreated or unsuccessfully treated).
• C. Headache has at least two of the following characteristics:
– 1. Unilateral location.
– 2. Pulsating quality.
– 3. Moderate or severe intensity.
– 4. Aggravation by or causing avoidance of routine physical activity (e.g., walking or climbing
stairs).
• D. During headache at least one of the following:
– 1. Nausea and/or vomiting.
– 2. Photophobia and phonophobia.
• E. At least one of the following:
– 1. History and physical do not suggest headaches secondary to organic or systemic
metabolic disease.
– 2. History and/or physical and/or neurologic examinations do suggest such disorder, but is
ruled out by appropriate investigations.
– 3. Such disorder is present, but migraine attacks do not occur for the first time in close
temporal relation to the disorder.
 DIAGNOSTIC CRITERIA FOR
MIGRAINE – without aura
• A. At least two attacks fulfilling B–D.
• B. Aura consisting of at least one of the following, but no motor weakness:
– 1. Fully reversible visual symptoms including positive features (e.g., flicker
– lights, spots, or lines) and/or negative features (e.g., loss of vision).
– 2. Fully reversible sensory symptoms including positive features (e.g., pins and
– needles) and/or negative features (e.g., numbness).
– 3. Fully reversible dysphasic speech disturbance.
• C. At least two of the following:
– 1. Homonymous visual symptoms and/or unilateral sensory symptoms.
– 2. At least one aura symptom develops gradually over 􀂕5 minutes and/or
– different aura symptoms occur in succession over 􀂕5 minutes.
– 3. Each symptom lasts 􀂕5 minutes and 􀂔60 minutes.
• D. Headache-fulfilling criteria B–D for migraine without aura begins during
the aura or follows aura within 60 minutes.
• E. Not attributed to another disorder.
 Neurotologic Manifestations
• Associated with many vestibular
symptoms, including episodic vertigo,
chronic motion sensitivity, and nonspecific
dizziness.
• Known as vestibular migraine
 Migraineurs
• Migraineurs often have a lifelong or childhood history of
motion sensitivity, with symptoms provoked by
amusement park rides, riding in the back seat of a car, or
reading in the car.
• They may describe a swimming or rocking sensation
inside their heads.
• Some may complain that busy or moving visual
environments such as supermarkets, shopping malls,
escalators, or passing trains provoke or aggravates their
dizziness.
• Most migraine sufferers will report some motion
sensitivity during a severe headache and will often prefer
to lie still in a dark, quiet room, although these symptoms
alone are not sufficient to be considered vestibular
migraine.
 Nomenclature
episodic vertigo related to migraine
• Migrainous vertigo
• Vestibular migraine
• Basilar migraine
• Benign recurrent vertigo
• Migraine-related vestibulopathy
• Migraine-associated dizziness
 Basilar Migraine
• Basilar migraine was originally described as a subtype of
migraine with aura.
• It is characterized by recurrent headaches, usually
occipital, associated with multiple neurologic symptoms
localizing to the vascular territory of the basilar artery —
the brainstem, cerebellum, and occipital lobes.
• Basilar migraine can occur throughout life in both sexes
with varying symptoms including a high incidence of
impaired consciousness (77%).
• Given how often vertigo, fluctuating hearing, and tinnitus
occur together, basilar migraine may be difficult to
distinguish from peripheral disorders such as Ménière's
disease and from vertebrobasilar TIAs.
 Vertigo
• Vertigo is extremely common in migraine and
occurred in 26.5% of Kayan and Hood's group of
migraine patients, compared with only 7.8% of
those with tension headache.
• Patients with episodic vertigo from vestibular
migraine can be thought of as a subset of basilar
migraine patients.
• Attacks of vertigo usually lasted a few minutes to
several hours, but occasionally were as short as
a few seconds or as long as a few days.
 Vertigo
• Most patients reported multiple attacks, most
commonly several per month.
• Of the patients in the series, 62% had only
vestibular symptoms, and another 16% had only
vestibular plus auditory symptoms of tinnitus,
episodic hearing loss, or aural fullness.
• In 68% of patients, the attacks of vertigo were
associated with a moderate headache, most
often holocephalic (28%) or occipital and nuchal
(24%).
 Auditory Symtpoms
• Phonophobia is the most common, occurring in up to
81% of those with migraine.
• 70% of patients had phonophobia during the headache
and 76% during the headache-free interval.
• Significantly lowered thresholds for hearing discomfort in
migraineurs.
• Tinnitus is also common, occurring in 15% of
migraineurs and more than 60% with basilar migraine,
but is usually over-shadowed by other complaints.
• Low-frequency hearing loss in 80% of basilar migraine
patients, bilateral in 46%, and unilateral in 34%.
• In 60%, the hearing loss was fluctuating, making
distinction from Ménière's disease difficult.
 NE
• Usually normal in migraineurs.
• A surprisingly high percentage (65%) of patients with central ocular
motor signs between attacks.
• Most frequent were vertical (48%) and horizontal (22%) impaired
pursuit, gaze-evoked nystagmus (27%), moderate positional
nystagmus (11%), or spontaneous nystagmus (11%).
• With basilar migraine had normal ocular motor testing with
electronystagmography (ENG).
• Various types of positional nystagmus are reported to be common
on ENG of migraineurs.
• Caloric testing may show a reduced vestibular response unilaterally
in 18% to 60% of patients and bilaterally in 4% to 12%
• A directional preponderance to rotation is also a common finding.
• The most common abnormality with rotational testing is a prolonged
duration of response.
 Pathophysiology
• Migraine can be thought of as a neurovascular
disorder: neural events result in the dilation of
blood vessels, which subsequently results in
pain and further nerve activation.
• The basic biologic problem appears to be the
dysfunction of an ion channel in the aminergic
nuclei within the brainstem or diencephalon,
such as the serotonin-producing dorsal raphe
nucleus or norepinephrine-producing locus
ceruleus, that normally modulates sensory input
and exerts neural influences on cranial vessels.
• The brainstem appears to become activated
during attacks of migraine.
• The aura of migraine (such as a scintillating
scotoma) is likely a result of spreading
depression, a wave of decreased cerebral
perfusion that slowly passes across the cortex
as a response to depressed neuronal function
from accumulation of extracellular potassium.
• The wave of depression spreads across the
visual cortex at a rate of 2 to 6 mm/min and
correlates well with the rate of enlargement of
the scintillating scotoma observed by the patient.
 Pain of Migraine
• Incompletely understood.
• The brain is largely insensate, but pain can be generated by large
extracranial vessels, proximal intracranial vessels, or dura mater.
• Vascular stimulation is likely a secondary phenomenon. Trigeminal
nerve fibers around pial arteries on the ventral surface of the brain
are depolarized by the high potassium concentration from spreading
depression. This leads to the release of vasoactive
neurotransmitters such as calcitonin gene-related peptide (CGRP)
and substance P, which in turn increases vascular permeability,
dilates cerebral vessels, and causes a sterile neurogenic
inflammation.
• Vasospasm likely results from the hypometabolism of spreading
depression and is associated with the classic visual aura, as well as
the monocular blindness of retinal migraine.
 Vestibular Ménière's disease
• Recurrent vertigo lasting minutes to hours without
associated auditory or neurologic symptoms
• A partial explanation for the frequent concurrence of MD
and migraine is that some patients may fit the diagnostic
categories for both.
• A defective ion channel with predominant expression in
the brain and inner ear could lead to a local buildup of
extracellular potassium, causing both the spreading
depression in migraine and a paroxysmal osmotic
dysequilibrium that causes endolymphatic hydrops and
increased perilymphatic potassium with toxic effects on
inner ear hair cells.
 Management of Headaches
• Lifestyle changes are aimed at avoiding triggers of migraine.
• Regular sleep, regular meals, exercise, avoiding peaks of stress
and troughs of relaxation, and avoiding dietary triggers can be
helpful.
• Some patients find relaxation training and biofeedback useful.
• Preventive therapies aim to reduce attack frequency, severity, and
duration and are instituted based on a variety of factors, including
the headache frequency, degree of disability caused by headaches,
responsivity to acute treatments, and patient preference.
• Good evidence supports the use of amitriptyline, divalproex sodium
(valproate), propranolol, timolol, fluoxetine, or gabapentin for daily
migraine prophylactic.
• On average, about two thirds of the patients given any of these
drugs will have a 50% reduction in headache frequency.
• Simple nonspecific pain medications such as aspirin,
acetaminophen, nonsteroidal antiinflammatory drugs, or
combination analgesics