A Dizzy Patient

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A Dizzy Patient

• Steven Feinberg MD

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HPI

• I have vertigo

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HPI

• Several month duration
• Decreased hearing in left ear, longstanding
• Dizziness started in November after starting
  propranolol for migraine headaches.
• Propranolol was discontinued but vertigo persists

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HPI

• Constant lightheaded feeling
• Intermittent vertigo lasting 1-4 hours
• No temporal relationship to migraines
• No positional relationship
• No dizziness in response to pressure changes or
  loud noises
• Tinnitus in past, none currently

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PMH

• Migraine headaches

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Physical Exam

• Normal ear exam
• Normal neurotologic exam
• Rinne AU
• Weber midline
• Negative Dix-Halpike
• Negative Romberg
• No gaze evoked nystagmus
• Normal finger-nose

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Diagnostic Studies???

• MRI was normal

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Differential Diagnosis

• Vascular
• Vertebrobasilar insufficiency
• CVA
• Brainstem
• Cerebellar
• Labyrinthine
• Infectious/Inflammatory
• Labyrinthitis
• Viral
• Bacterial
• Vestibular neuritis
• Otitis Media
• Otologic syphillis
• Traumatic
• Fistula
• Barotrauma
• Temporal bone fracture

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Differential Diagnosis

• Autoimmune
• Cogans
• Metabolic
• Hypoglycemia
• Wernickes encephalopathy
• Diabetes
• B12 deficiency
• Hypothyroidism
• Hyperventilation

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Differential Diagnosis

• Neoplastic
• Acoustic neuroma
• Glomus
• Congenital
• Inherited ataxias
• Degenerative
• Parkinsons
• Progressive supranuclear palsy
• Multiple systems atrophy
• Normal pressure hydrocephalus

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Differential Diagnosis

• Idiopathic/Iatrogenic
• BPPV
• Menieres
• Vestibular migraine
• Perilymphatic fistula
• Multiple sclerosis
• Cervicomedullary compression
• Superior semicircular canal dehiscence syndrome
• Vestibulotoxic medication
• Recurrent vestibulopathy
• Lupus
• Sarcoid
• Epilepsy (partial seizures)

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What is vertigo?

• Vestibular imbalance
• Asymmetry in tonic vestibular activity within
  vestibular system
• Intense feeling of motion
• Peripheral vs. central
• Vegetative symptoms

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General evaluation

• Central vs. peripheral
• Have patient walk
• Peripheral can walk, lean to side of lesion
• Central often cannot stand, fall in variable
  direction
• Nystagmus
• Central vs. peripheral
• Spontaneous nystagmus, changes with gaze
• Unaffected by fixation
• Purely vertical or torsional almost always
  central
• Absence of head thrust sign
• Other neurologic signs and symptoms

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Vestibular Migraine

• All forms of migraine!!!
• episodic true vertigo
• positional vertigo
• constant imbalance
• movement-associated disequilibrium
• Timing of symptoms
• Presenting symptoms

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What is a Migraine?

• Recurrent
• Nausea
• Light
• Symptom-free
• Throbbing
• Sleep
• Visual symptoms, dizziness, or vertigo.
• Family history

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Incidence

• 18-29 of women
• 6-20 of men
• 25-28 million people in the U.S.
• childbearing age
• Episodic vertigo occurs in 25-35
• 3.0-3.5 of people in the United States
  (Prevalence of Ménière disease is 0.2!)

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Migraine

• 2 categories
• migraine without aura (common migraine, 90)
• migraine with aura (classic migraine, 10)

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International Headache Society Classification of
Migraine (2003)

• Migraine without aura (formally called common
  migraine)
• Headaches last 4-72 hours
• 2-48 hours in children younger than 15 years
• Headache has at least 2 of the following
  characteristics
• Unilateral location
• Pulsating quality
• Moderate or severe
• Aggravation by activity
• During headache, at least 1 of the following
  occurs
• Nausea and/or vomiting
• Photophobia and phonophobia
• At least 1 of the following occurs
• History and physical examination findings do not
  suggest another disorder.
• History and physical examination findings do
  suggest another disorder, but the other disorder
  is ruled out by appropriate investigations (eg,
  MRI or CT scanning of the head

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International Headache Society Classification of
Migraine (2003)

• Migraine with aura (formally called classic
  migraine)
• Aura with at least 2 attacks of the following
• One reversible aura symptom indicating focal CNS
  dysfunction (ie, vertigo, tinnitus, decreased
  hearing, ataxia, visual symptoms in one hemifield
  of both eyes, dysarthria, double vision,
  paresthesias, paresis, decreased level of
  consciousness)
• Aura symptom that develops gradually over more
  than 4 minutes or 2 or more symptoms that occur
  in succession
• No aura symptom that lasts more than 60 minutes
  unless more than one aura symptom is present
• Headache occurring before, during, or up to 60
  minutes after aura is completed

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International Headache Society Classification of
Migraine (2003)

• Other categories
• Migraine with prolonged aura - Fulfills criteria
  for migraine with aura but the aura lasts more
  than 60 minutes and less than 7 days
• Basilar migraine (replaces basilar artery
  migraine) - Fulfills criteria for migraine with
  aura but 2 or more aura symptoms of the following
  types occur vertigo, tinnitus, decreased
  hearing, ataxia, visual symptoms in both
  hemifields of both eyes, dysarthria, double
  vision, bilateral paresthesias, bilateral
  paresis, and decreased level of consciousness
• Migraine aura without headache (replaces migraine
  equivalent or acephalic migraine) - Fulfills
  criteria for migraine with aura but no headache
  occurs

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International Headache Society Classification of
Migraine (2003)

• Childhood periodic syndromes that may be
  precursors to or be associated with migraine
• Benign paroxysmal vertigo of childhood
• Brief sporadic episodes of dysequilibrium,
  anxiety, and often nystagmus or vomiting
• Normal neurologic examination findings
• Normal findings on electroencephalography
• Migrainous infarction (replaces complicated
  migraine)
• Patient has previously fulfilled criteria for
  migraine with aura.
• The present attack is typical of previous
  attacks, but neurologic deficits are not
  completely reversible within 7 days and/or
  neuroimaging demonstrates ischemic infarction in
  relevant area.
• Other causes of infarction are ruled out by
  appropriate investigations.

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Pathophysiology

• 1992 Cutrer and Baloh
• 2 Mechanisms
• spreading wave of depression and/or transient
  vasospasm.
• neuroactive peptides

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Pathophysiology

• Spreading depression theory
• stimulus (chemical, mechanical) results in a
  transient wave front that suppresses central
  neuronal activity.
• spreads in all directions.
• ion fluxes
• Reduction in cerebral blood flow
• Aura during spreading wave of cortical depression

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Pathophysiology

• Neuropeptide release
• Asymmetric neuropeptide release vertigo.
• Symmetric neuropeptide release increased
  sensitivity to motion
• Cutrer and Baloh - prolonged hormonelike effect.

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Serotonin

• Important substrate in migraine.
• Direct effects on the vestibular nucleus neurons.
  
• Both the serotonergic and the peptidergic
  pathways possibly play a role
• No single hypothesis!

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Pain

• Poorly understood
• Brain insensate
• Large intracranial vessels, extracranial vessels,
  dura all sensate

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Evaluation

• History!!!!
• Menstrual
• Motion intolerance
• The attacks of vertigo may awaken patients and
  usually are spontaneous, but they may be motion
  provoked.
• Triggers
• Concurrent migraine?
• Family history?
• Symptoms
• vertigo, lightheadedness, imbalance, combination.
  
• Bimodal distribution
• Minutes to hours
• Greater than 24 hours
• May last months
• Vertigo at some time in 70
• Hearing loss
• Auditory symtoms (phonophobia in 81, tinnitus in
  15, hearing loss)

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Evaluation

• Symptoms
• The duration of the vertigo variable.
• may be indistinguishable from the spontaneous
  vertigo of Ménière disease.
• rocking sensation may be a continuous feeling for
  many weeks to months.
• Vertigo of Ménière disease does not last longer
  than 24 hours. Seven percent experience vertigo
  for a duration of seconds.
• 31 minutes up to 2 hours.
• 5 for 2-6 hours.
• 8 for 6-24 hours.
• 49 longer than 24 hours.

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Audiologic Evaluation

• Full audiometric evaluation
• Unexplained SNHL in 0-31 of migrainers, up to
  80 of basilar migrainers. .
• Often is of the lower frequencies, may be
  bilateral.
• Fluctuation
• Unlike Ménière disease, rarely progresses.
• Tinnitus rarely obtrusive
• ENG
• not helpful migraine vs Ménière disease.
• normal findings suggest migraine-associated
  vertigo.
• ENG testing not diagnostic
• Reduced vestibular response on calorics (18-60)
• Directional response to rotation testing
• Prolonged response to rotation
• electrocochleography (ECoG).
• may help to differentiate Ménière disease and
  perilymphatic fistula from migraine-associated
  vertigo.

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Physical Exam

• Neurotologic examination often normal.
• Horizontal rotary spontaneous nystagmus may be
  present during an acute attack of vertigo.
• Dix-Hallpike examination may elicit symptoms of
  vertigo or nonvertigo dizziness, each without
  nystagmus.

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Diagnostic Tests

• No diagnostic tests exist!
• diagnosis is made by clinical history
• If unclear diagnosis by therapeutic response to
  treatment.

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Genetic Testing?

• The genetic cause of a rare type of migraine has
  been discovered.
• Familial hemiplegic migraine, a form of migraine
  with aura, is associated with mutations in the
  CACNA1A gene located on chromosome arm 19p13.
• This gene codes for a neuronal calcium channel.
• Defects involving this gene are also involved
  with other autosomal dominant disorders that have
  neurologic symptoms
• Episodic ataxia type 2 (EA2)
• Familial hemiplegic migraine
• Spinocerebellar ataxia type 6
• The CACNA1A gene may be the link between
  vestibular disorders and migraine.

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Imaging

• An MRI of the brain with gadolinium
• If unilateral sensorineural hearing loss or
  tinnitus, the MRI should be directed to the
  internal auditory canals.

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Making the Diagnosis

• No Universal Algorithm accepted
• Definite diagnosis
• migraine with aura accompanied by concurrent
  episodes of vertigo
• migraine without aura that is repeatedly
  associated with vertigo immediately, before, or
  during the headache.
• Probable diagnosis
• recurrent or continuous vertigo or dizziness
  sensations without neurologic symptoms
• when the dizziness is not time-locked to
  headache,
• when a past or family history of migraine
  headaches exists, and when the dizziness cannot
  be fully explained by other vestibular disorders.
  
• In these patients, a trial of migraine therapy
  can be started for both diagnostic and
  therapeutic purposes.

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Treatment

• Convincing patients difficult!
• The 3 broad classes of migraine headache
  treatment
• reduction of risk factors
• abortive medications
• prophylactic medical therapy.
• In general abortive drugs not effective in
  treating dizziness secondary to migraine.
• Reduction of risk factors (stress, anxiety,
  hypoglycemia, fluctuating estrogen, certain
  foods, smoking)
• Elimination of birth control pills or estrogen
  replacement products

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The Problem

• General migraine literature focuses on management
  of headache rather than dizziness
• No controlled studies exist evaluating treatments

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Migraine and Menieres

• Association suggested by Meniere himself
• Difficult to distinguish
• Prevalence of migraine 56 in MD patients vs 25
  in controls
• Some patients fit diagnostic categories of both
• Patients who meet the clinical criteria for
  Ménière disease should be treated appropriately
  for Ménière disease, even if a history of
  migraine headache exists.

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Treatment Algorithm (Reploeg et al.)

• Institute dietary manipulation
• Nortriptyline, 10 or 25 mg, titrate to 50 mg
• Atenolol 25 mg, titrate to 50 mg
• Neurologic consultation

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Dietary Avoidance

• Offending foods
• monosodium glutamate (MSG)
• alcoholic beverages (red wine, port, sherry,
  scotch, bourbon)
• aged cheese
• chocolate
• Aspartame
• Effective in fewer than 25-30 of migraine cases
• Food diary helpful

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Lifestyle Modification

• Regular sleep
• Regular meals
• Exercise
• Avoiding peaks of stress, troughs of relaxation
• Relaxation training
• Biofeedback

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When to consult neurology??

• focal neurologic deficits
• migrainous infarction
• physician is uncomfortable

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Prophylaxis

• First-line prophylactic
• calcium channel blockers (verapamil),
• tricyclic antidepressants (nortriptyline)
• beta-blockers (propranolol).
• Second-line treatment includes
• Methysergide
• Valproic acid.
• SSRIs
• Gabapentin
• Acetazolamide has also been reported as an
  effective treatment by several authors.

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Prophylaxis

• Exact mechanism unknown.
• May block the release of neuropeptides into dural
  blood vessel walls
• No class more effective than others.
• Verapamil often used initially because lowest
  side effect profile
• If dizziness is controlled with one of these
  medications, the drug should be administered
  continuously for at least 1 year (except for
  methysergide, which requires a 3- to 4-wk
  drug-free interval at 6 mo).
• The medication can be restarted for another year
  if the dizziness returns after discontinuing
  therapy.
• On average 2/3 with 50 reduction of headache
  frequency

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Treatment of Acute Attacks

• Non-specific
• ASA
• Tylenol
• NSAIDs
• Migraine specific treatments
• Triptans 1992
• Serotonin agonists
• Oral, nasal, suppositories, sub-q
• High cost
• Contraindicated with cardiovascular disease

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Management of Vestibular Symtoms

• Promethazine
• Meclizine
• Dimenhydrinate
• Reglan
• Triptans not well studied, but contraindicated in
  basilar migraine due to theoretical risk of
  vasospasm and stroke.
• Surveys suggest vestibular symtoms respond to
  tripans.

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Other Interventions

• Vestibular rehabilitation therapy
• Increased physical activity

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Bibliography

• Cutrer FM, Baloh RW. Migraine-associated
  dizziness. Headache 1992 32 162-3.
• Reploeg MD, Goebel JA. Migraine-associated
  Dizziness Patient Characteristics and Management
  Options. Otol Neurotol. 200223364-371.
• Cummings