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Non-headache manifestations of Migraine. Dr Nicholas Silver Consultant Neurologist The Walton Centre for Neurology and Neurosurgery BASH HULL 2011. Copies of slides: anne.mccann@thewaltoncentre.nhs.uk. Migraine Third National Morbidity Survey *. Primary care consultations

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Non-headache manifestations of Migraine


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    1. Non-headache manifestations of Migraine Dr Nicholas Silver Consultant Neurologist The Walton Centre for Neurology and Neurosurgery BASH HULL 2011 Copies of slides: anne.mccann@thewaltoncentre.nhs.uk

    2. Migraine Third National Morbidity Survey* • Primary care consultations • > 300,000 person-years • 9.5% of population consult GP each year re: neurological symptom Top 5 symptoms: • Headache/migraine • Dizziness • Syndromes related to the cervical or lumbar spine • Faints or fits • Symptoms due to cerebrovascular disease. *Anthony Hopkins, JNNP 1989 Apr;52(4):430-3

    3. Episodic Migraine

    4. Triggers – additive effect headache lifestyle Hormone fluctuation Preventative

    5. The 4 stages of acute migraine Aura (20%) ProdromePostdrome Headache + Associated features Hours Minutes Hours Usually 1-2 days to hours to days

    6. Acute Migraine – Prodrome(premonitory features)* *prodrome seen in about 60% of patients

    7. Migraine - Aura • Only present in 20% of migraineurs • Symptoms usually “evolve” over time • Most commonly 20 to 30 minutes • May persist hours to months • “cortical spreading depression” • May occur without headache • “acephalalgic” migraine

    8. Visual Scotoma Photopsia, phosphenes Teichopsia (fortification spectra) Metamorphopsia, macropsia, zoom or mosaic vision Sensory unilateral or bilateral (<50%), slow migrating, positive phenomena Cheiro-oral migrating paraesthesiae Sensory ataxia Often reported as weakness Olfactory hallucinations Motor Weakness True weakness is rare and always unilateral Dysarthria Ataxia Chorea Movement disorders Cognitive Dysphasia / aphasia Apraxia Agnosia Disturbed consciousness / delusions Acute confusional state Multiple conscious trance-like states Delirium Coma Déjà vu / Jamais vu Migraine - Aura

    9. Throbbing / pounding Head, neck and / or face Unilateral or bilateral Tenderness Nausea +/- vomiting Icepick (<40%) (=primary stabbing headache) Stimulus Sensitivity Movement exacerbation Noise (photophobia) Light (phonophobia) Smell (osmophobia) Touch (allodynia) Relieving factors Flat Still Vomit Sleep What are migrainous features of headache ?

    10. Non-headache symptoms of acute migraine

    11. Migraine – postdrome Resolution often associated with: • Fatigue • Listlessness • Fragility • Scalp tenderness Also, following may occur: • Irritable • Impaired concentration • Muscle weakness and aching • Anorexia • Food cravings

    12. Visual aura Teleopsia - “zoom” vision Surroundings may appear very big or very small Body image disturbances body parts appear large, small, distorted, reduplicated or absent Entomopia – “Insect eye” - multiple copies of same image in grid-like pattern Corona phenomena Hallucinations Visual Auditory Olfactory Gustatory Tactile Cognitive deficit apraxia, agnosia acute confusional state Delusions Paranoid psychosis Distortion of reality as a manifestation of migraine“Alice in Wonderland Syndrome”

    13. Macrosomatognosia Macrosomatognosia of head, neck, both arms and hands. (Podoll and Robinson, Acta Neurolo Scand 2000;101:413-416)

    14. Migraine Autonomic Symptoms • Approx 20% of migraineurs Localised facial disturbance • Conjunctival injection (“red eye”) • Lacrimation (“tearing”) • Eyelid / facial swelling • Periorbital swelling and apparent enophthalmos as opposed to ptosis • Nasal congestion / rhinorrhoea (less common) • Objective scalp or facial swellling (oedema) • Flushing (may be unilateral) • Fullness in ear • Ecchymosis (face or limbs) • ? Systemic oedema

    15. Differentiating Migraine from other pathology with history • Acute Migraine may masquerade as • Stroke • SAH • Seizure / NEAD • Bells palsy

    16. Differentiating Migraine from other pathology with history • Aura vs Stroke • Premonitory phase • Evolution • Spread of symptoms • Type of deficit (eg scotoma vs hemianopia) • Positive symptoms with aura

    17. Differentiating Migraine from other pathology with history • Episodic migraine vs SAH • Often very difficult • Err on side of caution • Most useful question - ?Premonitory phase • Check “true” thunderclap, not just like aftermath of being hit by a baseball bat

    18. Differentiating Migraine from other pathology with history • Seizure vs Migraine Syncope • Is migraine syncope a common cause of blackout? • Premonitory phase – often many minutes or hours • Often dissociated and light headed before (eg 10 -15 minutes or more) • Symptoms may resemble panic attack or hyperventilation • May start with primary stabbing headache • Often presence of pain before LOC • Both often followed by migrainous headache

    19. Differentiating Migraine from other pathology with history • Bells Palsy vs Migraine • Migraine may cause facial drooping with apparent weakness • Probable autonomic cause • Loss of frontalis corrugator appearance – oedema • Apparent enophthalmos with periorbital oedema • Can close eye normally; normal blink • Often with prominent numbness, tingling and headache • May have other autonomic disturbance

    20. Chronic Migraine

    21. Markers to suggest Chronic (vs episodic) Migraine • Loss of prior efficacy of • Acute attack medications (“painkillers stopped working”) • Preventative • Ask about number of “crystal clear” headache-free days per month and look for migrainous features in milder less specific headaches • Multisymptomatic patient, even if does not present with headache i.e. presenting with • Fatigue • Other pain syndromes (neck pain, back pain, fibromyalgia, etc) • Vertigo / dizziness • Insomnia • Mood disturbance • Poor memory

    22. Chronic Migraine Gradual characteristic evolution from acute to chronic state • Frequency increases • Severity can increase or decrease • Gaps “fill in” with milder migrainous headaches + PSH • Acute attack medications lose efficacy • e.g. painkillers / triptans • Pervasive non-headache features • usually diminish / disappear on complete headache-free days

    23. Medication Overuse • ? Main cause of lack of response to headache preventatives • All acute attack medications can cause medication overuse, as can caffeine • Usually motivated by patient’s desire to treat their headaches • Commonest cause of chronic daily headache (IHS ICHD II): • “The interaction between a therapeutic agent and a susceptible patient” • If co-morbid neck pain, back pain or “fibromyalgia”, still worth stopping painkillers, as central sensitisation may heighten other bodily pains. • Escalation of acute attack medications, with loss of effectiveness is a big alarm bell to MOH or caffeine overuse headache and chronic as opposed to acute migraine

    24. Caffeine OveruseVirtue’s Household Physician – circa 1920 “Tea and Coffee Headaches. – In the nervous, and often the gouty and rheumatic person, the use of tea and coffee will cause violent headaches. These luxuries of life should be discontinued for at least one month. An extra strong cup of black coffee, to be sure, will stop the headache for the time being, but only adds fuel to the fire in the long run. We would strongly advise anyone that has constant or periodical headaches, if he uses either tea or coffee, and especially coffee, to leave them off entirely for three months. It may be the sole cause, and if caused by tea and coffee, there is no possibility of their cure by medicines while you continue their use”

    25. Chronic MigraineTriggers and Perpetuating Features • Triggers: • Hormones • Pregnancy • Postpartum • OCP • Menopause • Viral infection • Head injury • Systemic illness • Neurological illness • Neurosurgery • Emotional stress • Idiopathic • Perpetuating • Factors: • Painkillers • Opioids • Paracetamol • NSAIDS • Triptans / Ergot • Caffeine • Coffee • Tea • Cola • Chocolate • Lucozade • An Inherited • Predisposition: • A “genetic disorder” • +/- Family history • Travel sickness • Childhood • Adulthood – with reading • +/- previous migraine • Migrainous hangovers • Undeserved hangovers • Comorbid IBS

    26. Chronic Migraine: “More Than Just a Headache” Migraine Vertigo; Visual Vertigo; “Veering” Migraine-related dysequilibrium Dissociation, lightheaded, Etc. “Evolving” Aura Stimulus Sensitivity Light, noise, smell Chronic Fatigue Myokymia +/- Frequent (+/-severe) Headache Coathanger Neck Pain Sensory Disturbance (paraesthesiae / formication Back Pain, Diffuse muscle tenderness Reflex Syncope / POTS Distortion of Reality – AIWS Restless Legs / PLMS / PLMW Mood and Cognitive Disturbance Insomnia, poor STM, word substitutions, irritability, emotionalism, depression, anhedonia Stuttering Autonomic symptoms

    27. Chronic Migraine: Migraine associated symptoms e.g. Disappearance of • Post-natal depression • “Chronic fatigue syndrome” or “ME” • Fibromyalgia • Mood disturbance • Vertigo • Neck pain Ask about “brilliantly crystal clear” complete headache-freedays

    28. Migraine and Fatigue

    29. Migraine and Fatigue • Fatigue is common in chronic migraine1: • 84% scored >3 on Fatigue Severity Scale (FSS) 2 • 67% met CDC3 criteria for Chronic Fatigue Syndrome • Headache is commonly not volunteered by patients when presenting with other complaints • Chronic migraine should be considered in ALL patients presenting with chronic fatigue – all such patients should also have detailed sleep history. 1Peres et al (Cephalagia 2002:22:720-724) 2c.f. normal (<2.8), MS (5-6.5), depression (4.5), CFS (6.1) 3Center for Disease Control and Prevention

    30. 1994 CDC Criteria for Chronic Fatigue Syndrome • Primary symptoms Clinically evaluated, unexplained, persistent or relapsing chronic fatigue that is: • of new or definite onset • Not result of ongoing exertion; • Not substantially alleviated by rest; and • Results in substantial reduction in previous levels of function • Additional requirements Concurrent occurrence of > 4 of following symptoms: • Self-reported impairment in short term memory / concentration • Muscle pain • Joint pain without joint swelling or redness; • Headaches of a new type, pattern, or severity; • Unrefreshing sleep • Post-exertional malaise lasting more than 24 hours • sore throat; • tender cervical or axillary lymph nodes; • Final requirement • All other known causes of chronic fatigue must have been ruled out

    31. IHS ICHD-II • “Headaches attributed to the following disorders are not sufficiently validated: • Chronic fatigue syndrome • Fibromyalgia”

    32. Migraine and

    33. Migraine and Corpalgia • Cases of acute “migraine of the legs” • Cuadrado et al (Cephalalgia 2008) • 3 patients presenting with spontaneous body pain in association with migraine attacks. All patients had allodynia to mechanical stimuli over the painful areas. • Lovati et al (Expert Review of Neurotherapeutics 2009) • hypothesised that extracephalic allodynia mediated by mechanism of thalamic sensitization

    34. Migraine and Fibromyalgia (FMS) • Comorbidities of Fibromyalgia Syndrome (FMS) 1: • Depression • Anxiety • Headache; migraine and tension-type • IBS • Chronic Fatigue Syndrome • Vertigo • “Sinus” problems • TMJ dysfunction • POTS • Peres (Neurology 2001) reported high rates of FMS in transformed (chronic) migraine patients 1 Waylonis and Heck, Am J Phys Med Rehab 1992

    35. Migraine and Fibromyalgia (FMS) • Peres (Curr Neurol Neuroscience Rep 2003), and Centonze (Neurol Sci 2004) • suggest episodic migraine, chronic daily headache and FMS are continuum of same disorder. • Arguments based upon theories of central sensitisation • Patients with FMS show increased sensitivity to mechanical, thermal and electrical stimuli, with abnormal central pain mechanisms and augmented pain experience.

    36. Medication overuse and other bodily pains • Overuse of painkillers is a risk factor for developing chronic neck and back pain1. The study of 51,383 patients concluded: “Overuse of analgesics strongly predicts chronic pain and chronic pain associated with analgesic overuse 11 years later, especially among those with chronic migraine” • Reports of refractory neck and/or back pain in patients with migraine – same or improved following AAM withdrawal 1 Zwart et al, Head Hunt study, 2003

    37. Migraine and Fibromyalgia (FMS) • De Tommaso et al (Cephalalgia 2008) • FMS in 36% of patients with primary headache • Those with comorbid FMS had: • Highest level of migraine severity • Poor sleep quality • Headache severity heightened intensity of diffuse pain and fatigue • Pamuk and Cakir (Clin Exp Rheumatol 2005) • Increased FMS symptoms with menses (pain / fatigue) • Increased prevalence of FMS starting at menopause

    38. FMS pathophysiology • Abnormal CNS function1 • Supraspinal central sensitization • fMRI – cortical and subcortical augmentation of pain processing • Evidence for role of autonomic nervous system • Best treatments = antidepressant and anticonvulsant medications. NB One small trial of beta blockers suggested possible effect 1Thimineur and De Ridder, Pain Medicine 2007

    39. Migraine and Restless Legs (RLS)

    40. Chronic Migraine and RLS / PLM[Personal view] • Recognised in my clinic as major factor in CM (> 7 yrs) • Commonly comorbid with chronic migraine and caffeine/medication overuse (approx 80%) • Also provoked by caffeine and painkillers • Disappears after full detox in approx 80% • Frequently see CM in those that present with RLS • Disrupts normal sleep architecture and leads to sleep deprivation • Wake unrefreshed • Frequent wakening and dreaming • Diurnal variation of RLS symptoms (worst towards evening) • PLMS often not obvious • ? Caused by or provokes migraine

    41. Migraine and RLS • If disappears with detox, typically returns in acute episodic migraine attacks • If persists after detox, worth treating before adding migraine preventative • Pregabilin / Gabapentin – restore normal sleep architecture • Sinemet CR – beware augmentation – ? less likely if Rx breaks every 6/12 • Dopamine agonist (beware impulse control disorder and counsel patient) • High remission rate with Rx • Beware RLS / PLM provoked by tricyclic antidepressants and SSRI drugs – cause poor sleep architecture • Full and prolonged replacement of iron if Ferritin <50ng/ml • Replace B12 / folate and exclude renal impairment

    42. Restless Legs Syndrome “Wherefore to some, when abed they betake themselves to sleep, presently in the arms and legs, leapings and contractions of the tendons, and so great a restlessness and tossing of their members ensue, that if the diseased are no more able to sleep, than if they were in the place of the greatest torture” Sir Thomas Willis, 1672

    43. A medical condition? • On Hypochondria: Virtue’s Household Physician, a twentieth century medica: “The skin will twitch in different parts, or feel numb, or have the sensation of spiders crawling on it” ? Associations with migraine: “bright sparks are seen before the eyes…..at one time the person will feel as large as a barrel, at other times not larger than a whip-stock, the head will feel light or heavy, large or small. The smell becomes perverted; the hypochondriac will smell odors where there are none,,,,the persons are subject to fainting turns…..they are irritable, fretful, peevish and fickle” “Eminent Authorities Consulted” included Frances Dercum, William Gowers, F Savary Pearce, Ludwig Hirt, Charles L Dana, early 1920’s

    44. RLS • 1800’s “Anxietas Tibiarum” • sign of hysteria and/or neurosis • 1944 Ekbom “Asthenia Crurum Paraesthetic” (irritable legs) Acta Med Scand • Published observational review of 34 cases • Characterised salient features: • Diurnal pattern of lower extremity paraesthesia coupled with compulsion to move, worsening with rest and alleviated by movement • NB not same as Ekbom’s syndrome referring to delusional parasitosis, same Ekbom though!

    45. RLS - Diagnosis 4 essential criteria • An urge to move the legs, usually accompanied by uncomfortable / unpleasant sensations / paraesthesiae • Onset or worsening of symptoms at rest, not associated with any specific body position • Rapid relief by movement such as walking or stretching • Marked diurnal / circadian pattern, worse in the evening or night. Note that patient may however wake in am with painful legs that disappears on getting up and moving

    46. RLS • Ekbom • Considerable clinical morbidity • No objective evidence of neurological abnormality • Common – 5% of population • Often family history • Noted associations with • Pregnancy • Iron deficiency anaemia • Blood donors • Carcinoma

    47. RLS and sleep • RLS is a major cause of insomnia • Reduced time asleep • Frequent wakening • Fragmentation of normal sleep architecture

    48. RLS – clinical features • Characterised by unpleasant, deep within lower legs, most commonly distal to knees • May note sensations in thighs, feet, arms • If occur in arms, usually less severe there • Most commonly bilateral • May be unilateral • Only experienced after rest • Almost irresistible urge to move legs or stretch • May need to walk around to get relief • Most severe in late evening (diurnal) • May complain of true pain / dull ache