A Dizzy Patient

1. �A Dizzy Patient� Steven Feinberg MD

2. HPI �I have vertigo�

3. 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

4. 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

5. PMH Migraine headaches

6. Physical Exam Normal ear exam Normal neurotologic exam Rinne +AU Weber midline Negative Dix-Halpike Negative Romberg No gaze evoked nystagmus Normal finger-nose

8. Diagnostic Studies??? MRI was normal

9. 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

10. Differential Diagnosis Autoimmune Cogan�s Metabolic Hypoglycemia Wernicke�s encephalopathy Diabetes B12 deficiency Hypothyroidism Hyperventilation

11. Differential Diagnosis Neoplastic Acoustic neuroma Glomus Congenital Inherited ataxias Degenerative Parkinson�s Progressive supranuclear palsy Multiple systems atrophy Normal pressure hydrocephalus

12. Differential Diagnosis Idiopathic/Iatrogenic BPPV Meniere�s Vestibular migraine Perilymphatic fistula Multiple sclerosis Cervicomedullary compression Superior semicircular canal dehiscence syndrome Vestibulotoxic medication Recurrent vestibulopathy Lupus Sarcoid Epilepsy (partial seizures)

13. What is vertigo? Vestibular imbalance Asymmetry in tonic vestibular activity within vestibular system Intense feeling of motion Peripheral vs. central Vegetative symptoms

14. 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

15. Vestibular Migraine All forms of migraine!!! episodic true vertigo positional vertigo constant imbalance movement-associated disequilibrium Timing of symptoms Presenting symptoms

16. What is a Migraine? Recurrent Nausea Light Symptom-free Throbbing Sleep Visual symptoms, dizziness, or vertigo. Family history

17. 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 M�ni�re disease is 0.2%!)

18. Migraine 2 categories migraine without aura (common migraine, 90%) migraine with aura (classic migraine, 10%)

19. 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

20. 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

21. 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

22. 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.

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

24. 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

25. Pathophysiology Neuropeptide release: Asymmetric neuropeptide release = vertigo. Symmetric neuropeptide release = increased sensitivity to motion Cutrer and Baloh - prolonged hormonelike effect.

26. 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!

27. Pain Poorly understood Brain insensate Large intracranial vessels, extracranial vessels, dura all sensate

28. 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)

29. Evaluation Symptoms The duration of the vertigo variable. may be indistinguishable from the spontaneous vertigo of M�ni�re disease. rocking sensation may be a continuous feeling for many weeks to months. Vertigo of M�ni�re 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.

30. 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 M�ni�re disease, rarely progresses. Tinnitus; rarely obtrusive ENG not helpful � migraine vs M�ni�re 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 M�ni�re disease and perilymphatic fistula from migraine-associated vertigo.

31. 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.

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

33. 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.

34. 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.

35. 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.

36. 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

37. The Problem� General migraine literature focuses on management of headache rather than dizziness No controlled studies exist evaluating treatments

38. Migraine and Meniere�s 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 M�ni�re disease should be treated appropriately for M�ni�re disease, even if a history of migraine headache exists.

39. 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

40. 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

41. Lifestyle Modification Regular sleep Regular meals Exercise Avoiding peaks of stress, troughs of relaxation Relaxation training Biofeedback

42. When to consult neurology?? focal neurologic deficits migrainous infarction physician is uncomfortable

43. 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.

44. 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

45. 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

46. 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.

47. Other Interventions Vestibular rehabilitation therapy Increased physical activity

48. 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. 2002;23:364-371. Cummings