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
> 300,000 person-years
9.5% of population consult GP each year re:
neurological symptom
Top 5 symptoms:
1.
2.
3.
4.
5.
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
Episodic Migraine
Triggers – additive effect
headache
Alcohol
lifestyle
Relief of stress /
weekend
Caffeine
Hormone
fluctuation
Preventative
The 4 stages of acute migraine
Aura
(20%)
Prodrome
Postdrome
Headache
+ Associated
features
Hours
Minutes
to hours
Hours
to days
Usually 1-2 days
Acute Migraine – Prodrome
(premonitory features)*
Mental State
Neurological
General
Fatigue
Irritability
Depressed mood
Euphoria
Hyperactivity
Restlessness
Depersonalisation
Derealisation
Yawning
Somnolence
Phonophobia
Photophobia
Osmophobia
Restless Legs
Lightheaded
Food craving
Dizziness
Neck pain /
stiffness
Anorexia
Frequent micturition
Diarrhoea
*prodrome seen in about 60% of patients
Migraine - Aura
“A complex of focal neurological symptoms
(positive or negative phenomena) that
precede or accompany an attack”
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
Migraine - Aura
Visual
– Scotoma
– Photopsia, phosphenes
– Teichopsia (fortification
spectra)
– Metamorphopsia, macropsia,
zoom or mosaic vision
Motor
– Weakness
Sensory
– unilateral or bilateral (<50%),
slow migrating, positive
phenomena
Cheiro-oral migrating
paraesthesiae
Sensory ataxia
– Often reported as
weakness
– Olfactory hallucinations
Cognitive
– Dysphasia / aphasia
– Apraxia
– Agnosia
True weakness is rare and always
unilateral
Dysarthria
– Ataxia
– Chorea
– Movement disorders
Disturbed consciousness / delusions
– Acute confusional state
– Multiple conscious trance-like
states
– Delirium
– Coma
– Déjà vu / Jamais vu
What are migrainous features of
headache ?
Throbbing / pounding
Head, neck and / or face
Unilateral or bilateral
Stimulus Sensitivity
–
–
–
–
–
Movement exacerbation
Noise (photophobia)
Light (phonophobia)
Smell (osmophobia)
Touch (allodynia)
Tenderness
Relieving factors
Nausea +/- vomiting
Icepick (<40%)
(=primary stabbing headache)
–
–
–
–
Flat
Still
Vomit
Sleep
Non-headache symptoms
of acute migraine
Mental State
Depression
Dissociation
Anxiety
Fatigue
Irritability
Agitation
Anger
Rage
Incapacity
Confusion
Exhilaration
Hypomania
[constitutional and mental
changes are almost
universal]
Neurological
Blurred vision
Paraesthesiae
Formication
Vertigo
Acute confusion
Disorientation
Word-finding difficulty
Stuttering
Dysphasia
Autonomic
Syncope
Hemiplegia
Coma
General
Lightheadedness
Flushing, Pallor, skin
change, cold extremities
Oedema / fluid retention
Scalp / face oedema
Hair loss
Neck pain and stiffness
Anorexia
Gastroparesis
Food craving
Nausea (90%)
Vomit (30%)
Eructation
Diarrhoea (16%)
Polyuria
Epistaxis, Ecchymosis
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
Distortion of reality as a
manifestation of migraine
“Alice in Wonderland Syndrome”
– 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
Macrosomatognosia
Macrosomatognosia of head, neck, both arms and hands.
(Podoll and Robinson, Acta Neurolo Scand 2000;101:413-416)
Migraine Autonomic Symptoms
“Migraine is the commonest cause of
facial autonomic disturbance”
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
Differentiating Migraine from other
pathology with history
Acute Migraine may masquerade as
– Stroke
– SAH
– Seizure / NEAD
– Bells palsy
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
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
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
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
Chronic Migraine
Markers to suggest Chronic
(vs episodic) Migraine
1. Loss of prior efficacy of
– Acute attack medications (“painkillers stopped working”)
– Preventative
2. Ask about number of “crystal clear” headache-free days
per month and look for migrainous features in milder
less specific headaches
3. 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
Chronic Migraine
Frequent headaches with migrainous features
+
< 15 days per month headache-free
Gradual characteristic evolution from acute to chronic state
1.
2.
3.
4.
Frequency increases
Severity can increase or decrease
Gaps “fill in” with milder migrainous headaches + PSH
Acute attack medications lose efficacy
e.g. painkillers / triptans
5.
Pervasive non-headache features
usually diminish / disappear on complete headache-free days
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
Caffeine Overuse
Virtue’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”
Chronic Migraine
Triggers and Perpetuating Features
An Inherited
Predisposition:
A “genetic disorder”
+/- Family history
Travel sickness
•Childhood
•Adulthood – with reading
+/- previous migraine
Migrainous hangovers
Undeserved hangovers
Comorbid IBS
Triggers:
Perpetuating
Factors:
Hormones
•Pregnancy
•Postpartum
•OCP
•Menopause
Viral infection
Head injury
Systemic illness
Neurological illness
Neurosurgery
Emotional stress
Idiopathic
Painkillers
Opioids
Paracetamol
NSAIDS
Triptans / Ergot
Caffeine
Coffee
Tea
Cola
Chocolate
Lucozade
Chronic Migraine:
“More Than Just a Headache”
“Evolving” Aura
Stimulus Sensitivity
Migraine Vertigo;
Visual Vertigo;
“Veering”
Light, noise, smell
Chronic Fatigue
Coathanger Neck Pain
Back Pain,
Diffuse
muscle tenderness
Distortion of
Reality – AIWS
Stuttering
Migraine-related
dysequilibrium
Dissociation, lightheaded,
Etc.
Myokymia
+/- Frequent
(+/-severe)
Headache
Mood and Cognitive
Disturbance
Insomnia, poor STM, word
substitutions, irritability,
emotionalism, depression, anhedonia
Sensory Disturbance
(paraesthesiae / formication
Reflex Syncope / POTS
Restless Legs /
PLMS / PLMW
Autonomic symptoms
Chronic Migraine:
Migraine associated symptoms
Ask about “brilliantly crystal clear”
complete headache-free days
e.g. Disappearance of
–
–
–
–
–
–
Post-natal depression
“Chronic fatigue syndrome” or “ME”
Fibromyalgia
Mood disturbance
Vertigo
Neck pain
Migraine and Fatigue
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)
normal (<2.8), MS (5-6.5), depression (4.5), CFS (6.1)
3Center for Disease Control and Prevention
2c.f.
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
IHS ICHD-II
“Headaches attributed to the following
disorders are not sufficiently validated:
– Chronic fatigue syndrome
– Fibromyalgia”
Migraine
and
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
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
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.
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
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
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
Migraine and
Restless Legs (RLS)
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
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
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
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
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!
RLS - Diagnosis
4 essential criteria
1. An urge to move the legs, usually accompanied by
uncomfortable / unpleasant sensations /
paraesthesiae
2. Onset or worsening of symptoms at rest, not
associated with any specific body position
3. Rapid relief by movement such as walking or
stretching
4. 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
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
RLS and sleep
RLS is a major cause
of insomnia
Reduced time asleep
Frequent wakening
Fragmentation of
normal sleep
architecture
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
RLS - descriptions
Creeping
Crawling
Itching
Burning
Searing
Tugging
Drawing
Aching
Electric current
Want to take legs off
Supportive clinical features
Family history
– 60-80% of cases are
familial
– autonomic dominant
with variable
penetrance
Response to
dopaminergic Rx
PLMW or PLMS
Secondary causes and
associations
Iron deficiency
– Reduced ferritin, often normal Hb
– Rx if ferritin less than 50ng/ml – prolonged course
Pregnancy - especially last trimester / ferritin < 50ng/ml
Blood donation
Renal failure - effective Rx with IV Fe
Fibromyalgia
Migraine
Depression
Rheumatoid arthritis
B12 / folic acid deficiency (occasional)
Parkinsons disease, essential tremor
Restless Legs and Migraine
17% of migraine vs 5% of controls had RLS1
RLS reported in 22% migraine subjects vs 8% of controls2
> 60% of RLS patients affected by MOH
Increased dopaminergic premonitory features in those with
comorbid RLS3
RLS and PLMS recognised to be also associated with
fibromyalgia4
Caffeine is “the major aetiological factor in the causation of
restless legs syndrome”5
Rhode et al. Cephalalgia 2007;27(11)1255-60
1
2 d’Onofrio
et al. Neurol Sci 2008 May;29 Suppl1
et al. Neurol Sci 2008 May; 29 Suppl 1
4 Yunus and Aldaq. BMJ 1996. May 25;312(7042)
5 Lutz. J Clin Psychiatry 1978;39(9)693-8
3 Cologno
Periodic Limb Movements and
migraine
Reported in association with migraine1
Other sleep disorders associated with migraine
include2
OSA
Insomnia
Restless Legs
Circadian rhythm disorder
Hypersomnia
1 Bokkala
et al. Pediatr Neurol. 2008 Jul;39(1):33-9
2 Rains and Poceta. Headache. 2006 Oct;46(9):1344-63
Migraine and Dizziness
Migraine-related dizziness
[Personal view]
1.
2.
3.
4.
Migraine-related disequilibrium [commonest]
Lightheaded
Dissociation - depersonalisation / derealisation
Hot, sweaty, flushed
Blurred, dim, or spotty vision
Mute and buzzy hearing
+/- secondary panic
+/- situation-specific – hot, bright, noisy, crowded
Migraine vertigo
Visual vertigo
Unexplained veering
Migraine-related dizziness
0.89% of population has migraine vertigo
Total 1 year prevalence of vertigo = 4.9%
Prevalence of BPPV = 1.6% 1
Motion sickness may be treated effectively with Rizatriptan in
migraineurs with migraine vertigo2
NB Motion sickness often associated with stimulus sensitivity to
noise, light and smell
Migraine vertigo presents usually as attacks of spontaneous or
positional vertigo lasting seconds to days and usually with
accompanying migrainous symptoms2
Treat with standard approaches for chronic migraine – lifestyle,
preventative (topiramate, beta blocker, flunarizine, etc,)
1
Neuhauser. Current Opin Neurol 2007
2 Eggers. Curr Neurol Neurosci Rep 2006
FMS and neurotologic symptoms
Fibromyalgia (FMS) (Bayazit et al, 2002)
– 50% otologic symptoms, predominant
dizziness
Rosenhall et al (1996)
– Vertigo / dizziness in 72%
– Auditory evoked potentials suggested
brainstem dysfunction
Considering Migraine in Differential Diagnosis
“The Chameleon in the Neurology Clinic”
Dizziness and Vertigo
? MS
Blackouts / Syncope
? Epilepsy
Sensory disturbance
? NEAD
Fatigue
? TIA
Insomnia
? Stroke
Panic Attacks (+/- panic)
Chronic Fatigue Syndrome
Depression / anxiety
“ME”
Chronic Pain
? Conversion disorder
– Neck pain / Brachalgia
Dementia
– Facial pain
Psychosis
– “Fibromyalgia”
Approaches to
successful
management
of chronic migraine
Selling the concept
Behaviour modification
Preventative
Sleep
Lifestyle
Chronic Migraine - Rx
1.
Acute abrupt withdrawal
–
All acute attack medication (long term)
triptans, analgesics, NSAIDS
–
–
All caffeine
Warn headaches typically worsen+++ for 5-7 days
The “foundation”
1. No painkillers
2. No caffeine
3. Good fluids
4. Regular meals
5. Regular sleep
2.
Lifestyle – sleep, hydration, meals
3.
Rx Restless Legs / Periodic Limb Movements if persist after detox
(disappear in most patients)
4.
After withdrawal, add “preventative” for 1 year
–
–
–
5.
6.
Beta blocker (eg propranalol)
AED (eg valproate, topiramate, gabapentin
Tricyclic antidepressant
Change preventative if no response at 4/12
Aim for maximum “tolerated” dose – reduce if persistent sedation
Migraine Preventatives
Reasons for failure
1. Ineffective
– Acute attack drugs still used
– Caffeine
2. Not used to high enough dose
– Aim for “maximum tolerated dose”
3. Used at too high dose
– Often ineffective if patient persistently sedated
– Fatigue with migraine distinguished by pre-existing before drug
started or absence of fatigue on headache-free day
4. Not used for long enough, i.e. 4 months at top level
reached
5. Underlying sleep disorder – RLS, PLM, OSA
Chronic Migraine
Preventatives
Evidence
Poor evidence for many traditionally used preventative
drugs
Best evidence for topiramate and propranalol
Licensing varies between countries
Poor evidence for any individual approaches to Rx for
migraine variants
Much anecdotal “advice”
Rationale in Clinical Situations
Poor sleep – insomnia, RLS, PLMS,
wake unrefreshed
–
–
Gabapentin to 900-1200mg tds,
Pregabilin to 300-400mg bd
AVOID tricyclics and SSRI drugs
Obesity, weight gain on
preventatives
–
Topiramate
Eating disorder
–
Avoid all preventatives if possible
Consider GON Block
If necessary, amitryptilline or
propranalol
Females of reproductive age
–
–
–
Consider avoiding beta blockers
Agitated psychiatric state and/or
suicidal ideation
–
Consider short term olanzepine
Severe migraine vertigo – short
term help
–
Consider olanzepine short term
Use most weight-neutral drugs
Pregnancy / planning pregnancy
–
–
–
Major Depression
Avoid Sodium Valproate and other
anticonvulsants if possible
Counsel all patients who intend to use
anticonvulsants to take good
contraceptive measures, take regular
folate 5mg, and beware induction of
OCP (eg topiramate)
Severe anxiety or hypertension
–
Consider beta blocker
Hemiplegic Migraine
–
–
Consider topiramate, acetazolamide
Flunarizine
Migraine vertigo
–
Consider topiramate, flunarizine
Comorbid diabetes or OSA
–
topiramate
Summary
Chronic Migraine accounts for more than 90% of
referrals to a specialist headache clinic
It is frequently misdiagnosed in hospital and primary care
Chronic migraine may present with features other than
headache
Always consider sleep disorders in your history
It is highly worthwhile taking full migraine history in
patients presenting with unexplained neurological
symptoms
It is worth treating chronic migraine with lifestyle
strategies, attention to sleep quality / disorder, and
rational approach to drug use and preventative
strategies, even if headache is not a main presentation
Copies of slides: anne.mccann@thewaltoncentre.nhs.uk
Patient info sheets - http://www.thewaltoncentre.nhs.uk/patients-and-visitors/patient-leaflets.asp#
Appendix
Alice in Wonderland syndrome
Migraine postdromal features
Migraine Variants (basilar migraine, FHM,
ophthalmoplegic migraine etc)
Fatigue
PLMS
Medication and caffeine overuse
Walton Centre Audit of non headache symptoms
of migraine
Guides for detoxification
Alice in Wonderland Syndrome
Lippman 1952: Certain Hallucinations peculiar to Migraine
– 1 patient with left ear ballooning out 6 inches or more
– Body split in 2 halves as if by vertical line, with right size twice the size of
left.
Syndrome named by Todd, 1955, in relation to migraine and epilepsy:
– Characterised by body schema disturbances and facultative symptoms,
including depersonalisation, derealisation, visual illusions and illusory
alterations in the passage of time
Bizarre visual illusions and spatial distortions
– Macropsia – world appears larger than normal / subject appears smaller
– Micropsia – opposite of macropsia
– Metamorphosia - sensation of formed body distortions
– Zoom vision (e.g. teleopsia)
Sense of time speeding up or slowing down
More commonly reported in children
Often before the headache
Also reported with infectious mononucleosis, epilepsy,
and drugs
Migraine Variants
Migraine variant or migraine equivalent are terms applied to migraine
exhibiting itself predominantly in form other than head pain
Characterized by paroxysmal episodes of
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prolonged visual auras,
atypical sensory, motor or visual aura,
confusion, dysarthria, focal neurological deficits,
gastrointestinal symptoms,
other constitutional symptoms
with or without headache
Many migraine variants recognised in IHS IHCD-II, including:
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hemiplegic migraine
basilar migraine
childhood periodic syndromes
retinal migraine
complicated migraine
ophthalmoplegic migraine
Migraine Variants
Basilar Migraine
Ophthalmoplegic Migraine
Hemiplegic Migraine
Episodic ataxia
Vertiginous Migraine
Alternating Hemiplegia of Childhood
Cyclical Vomiting Syndrome
Confusional Migraine
Abdominal Migraine
Benign paroxysmal vertigo of childhood
Retinal migraine
Migraine infarction
? Migraine-triggered seizures (Migralepsy)
Migraine Variants
Basilar Migraine
Aura usually < 1 hour, headache typically follows
Typical hemianopia expands to all visual fields,
sometimes temporary blindness
Many neurological features are bilateral
Visual deficit typically followed by one or more of:
Vertigo
Tinnitus
Decreased hearing
Diplopia
Ataxia
Bilateral paraesthesiae, weakness
Impaired cognition
confusion
Migraine Variants
Confusional Migraine
Boys > girls
Usually in children
Aura
Headache (may be insignificant)
Confusion
Inattention
Distractibility
Difficulty maintaining speech / activities
Sedation
Agitation / violent behaviour
Usually relieved by sleep
Migraine Variants
Ophthalmoplegic Migraine
At least 2 attacks with ocular palsy
Typically IIIrd nerve with dilated pupil and unilateral eye pain
IV and VI palsy (occasional)
Ophthalmoplegia – hours to months
Differential diagnosis includes
Tolosa-Hunt
Aneurysm
Cavernous sinus / middle cranial fossa lesion
Sphenoid sinusitis
Lyme, syphilis, coccidiomycosis, HIV
Sarcoid, Leukaemia, CNS inflammatory disorder
Needs intensive investigation
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MRI+Gd and MRA
DSA
LP
Bloods
Migraine Variants
Hemiplegic Migraine
Sporadic or Familial
Often starts in childhood
Attacks frequently precipitated by minor head injury
Change in conscious level often seen (confusion to coma)
Differential diagnosis includes
Focal seizure
Stroke
MELAS
Homocystinuria
FHM
Autosomal Dominant with variable penetration
Includes episodes with or without motor aura
Episodes
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Days to weeks
May include reduced conscious level (confusion to coma), fever, meningism
May occur without headache
20% of families have patients who develop fixed cerebellar deficits (linked to Chr 19, eg
CACNA1A)
Other gene mutations also recognised (eg on Chr 1, ATP1A2 gene mutations)
Migraine Variants
Episodic Ataxia type 2
Autosomal dominant
Paroxysmal ataxia
Provocation:
– Physical, emotional stress, alcohol, caffeine
Interictal nystagmus
Responds to acetazolamide
Chr 19 (CACNA1A)
Migraine Variants
Vertiginous Migraine
Vertigo present in approx 1/3 of
migraineurs
Recurrent vertigo episodes with or without
other migraine features, e.g.
– Prodromal symptoms
– Nausea
– Stimulus sensitivity to noise, light, smell
– Autonomic disturbance, etc.
Migraine Variants
Retinal Migraine
Not uncommon cause of transient monocular blindness
in young adults
Recurrent attacks of unilateral visual disturbance / loss
with minimal or no headache
Gradual enlarging scotoma enlarging to total monocular
visual loss
? Due to transient vasospasm of choroidal or retinal
arteries
Need to exclude vascular (carotid) disease and other
ocular conditions
Fatigue Severity Scale
During the past week, I have found that:
Score
1. My motivation is lower when I am fatigued.
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2. Exercise brings on my fatigue.
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3. I am easily fatigued.
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4. Fatigue interferes with my physical functioning.
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5. Fatigue causes frequent problems for me.
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6. My fatigue prevents sustained physical functioning.
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7. Fatigue interferes with carrying out certain duties and
responsibilities.
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8. Fatigue is among my three most disabling symptoms.
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9. Fatigue interferes with my work, family, or social life.
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MOH - How much is overuse?
Limitations of IHS guidance
Dietary caffeine not included as component of overuse in IHS
guidance
Increased caffeine consumption has been associated with increased
risk of developing chronic daily headache1
Literature on detoxification from AAM does not take account of
dietary caffeine
How much is overuse according to IHS2?
– Simple analgesics > 15 days per month
– Triptans or combination analgesics3 > 10 days per month
– Opioids or ergotamine > 10 days per month
? Depends on individual pharmacogenetics
1 Sholz
et al, 1988
et al, 1990, Diamond and Dalessio 1982, Mathew 1990, Saper 1987, Wilkinson 1988,
2 Mathew
3 i.e.
with caffeine
How much is overuse?
All types of analgesic and acute attack medications reported to be
associated with MOH
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Paracetamol, NSAIDS, Opioids
All known triptans
Ergotamine
Rebound headache may occur acutely in single attack
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stopping AAM = recognised use in first line Rx of status migrainosus:
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Stop acute attack drugs
Rehydration
Treatment of nausea and vomiting
(+/- later IV steroid, neuroleptic, IV dihydroergotamine)
Anecdotally, patients who stop using analgesics or triptans often
report shorter attacks of acute migraine following detox
Clinical Features of rebound headache
Analgesic rebound headache - No placebo-controlled trials
Caffeine rebound headache:
stopping low dose caffeine frequently results in withdrawal headache1
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Double blind placebo-controlled short-term caffeine withdrawal study
N = 64, subjects with low to moderate caffeine intake
32 – placebo; 32 – continued caffeine
50% of those given placebo had headache by day 2
6% of those continuing caffeine had headache by day 2
Nausea, depression, flu-like symptoms common in placebo (detox) group
Does not indicate long term consequences of detoxification
Silverman et al 1992
Caffeine Overuse
Not “proven”, but long recognised to cause
headaches, especially on withdrawal
Caffeine regarded as acute attack medication
Often in combined analgesics
Mild headaches (e.g. regarded as TTH) almost
always disappear with complete elimination of
acute medication and caffeine
Caffeine withdrawal - first line for treatmentresistant depression
Caffeine content in drinks
12 oz drink
mg
Red Bull (8oz) 80
Lucozade
46
Diet coke
46
Dr Pepper
41
Pepsi
38
Diet pepsi
36
Coca cola
34
8 oz drink
mg
Coffee
Tea
Cocoa
Decaf coffee
70-135
40-60
14
2-3
Horlicks, sprite etc are caffeine free
Walton Centre Audit on
Non-headache manifestations of
migraine
Retrospective review of 50 consecutive
patients at WCNN with chronic migraine
100
80
60
40
PTS +
panic
PTS - no
panic
sensory
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wakening
>2
20
fatigue
%
depression
anhedonia
irritability
90
80
70
60
50
40
30
20
10
0
emotionalism
%
memory
(STM)
Retrospective review of 50 consecutive
patients at WCNN with chronic migraine
Appendix
Migraine treatment & preventatives
Approach to successful treatment of chronic migraine
The withdrawal:
Warn of possible severe worsening for 1-2 weeks
Worsening is a good sign and usually heralds reverse to acute migraine
Admit for in-patient detoxification if elderly, diabetes, severe triptan or opioid
overuse, severe depression and/or suicidal ideation
– May assist withdrawal with:
Fluids (+/- IV)
Oral / rectal domperidone up to 120mg per day
5/7 - Naproxen 500mg 8am + 4pm
Clonidine (if opiates ++)
5/7 - IM Chlorpromazine @ 10pm
IV Dihydroergotamine
Steroids
Combined pain syndromes:
Advise that other pains often eventually improve off painkillers (especially
neck and back), due to cessation of central sensitisation
Consider other measures for other pains:
– Back pain – Pilates, Extensor stretch exercises, swimming, pain clinic – epidurals
etc
– Neck Pain – usually improves ++
– Arthritis – glucosamine, large joint revision etc.
Preventative Drugs for Migraine
Licensed
Unlicensed
Beta Blockers*
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Propranalol (best evidence for use)
Timolol, Metoprolol
Antiepileptic Drugs (AED)
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Topiramate***
Others
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Clonidine (antihistamine and serotonin
antagonist) – of no proven efficacy (BNF
states “Clonidine is not recommended and
may aggravate depression and cause
insomnia”)
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Pizotifen - evidence for effectiveness is poor;
adverse effects severely limit use
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Methysergide*** – considered very effective
but concerns about about ergot side effects
(retroperitoneal fibrosis etc)
Beta Blockers
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Atenolol (not licensed, but commonly used)
Nadolol
Tricyclic antidepressants**
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Amitriptyline (best studied)
Dosulepin (commonly used; potentially better
tolerated – beware cardiac arrhythmias)
Nortryptilline (often better tolerated)
Antiepileptic Drugs (AED)
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Sodium Valproate**
Gabapentin (limited evidence of efficacy – 1
study)
Zonisamide
Neuroleptics
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Olanzepine
amisulpiride
Calcium Antagonists
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Verapamil
Flunarizine
SSRI’s, Venlafaxine, Levetiracetam,
Tizanidine, ACE II antagonists
Alternative
* Partial agonists unhelpful; ideal beta blocker is hydrophilic and cardioselective
** Unlicensed, but recommended for use in BNF!
*** Hospital Supervision or Specialist Introduction only
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Butterbur, coenzyme Q10, riboflavin, feverfew
First Line
Nortryptilline / Amitryptilline / Dosulepin
Propranalol (Inderal LA)
Second Line
Epilim Chrono
Topiramate
Third Line
Gabapentin (first line if sleep disorder)
Paroxetine
Refractory cases
Flunarizine
Olanzepine
Methysergide
Botulinum toxin
GON Block / GON stimulator
Alternative agents
Pizotifen
Lisinopril, Candesartan
Clonidine
Lamotrigine, verapamil, carbamazepine
Butterbur, coenzyme Q10, riboflavin, feverfew
First Line
Dosulepin
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25mg 7-8pm, increase 25mg each 2/52, aim 1mg/kg or maximum tolerated dose; reduce
dose if persistent side effects other than dry mouth
Consider ECG
Beware, may exacerbate restless legs syndrome and poor sleep and be counterproductive
Propranalol (Inderal LA)
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80mg, increase 160-240mg
Avoid if severe depression
Second Line
Epilim Chrono
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200mg, increase 200mg / week, aim 400-800mg bd
+ Folic acid and contraception if young female
Warn – side effects: weight gain, hair loss, tremor (10%), polycystic ovaries
Beware teratogenic++ (learning disabilities etc)
Topiramate
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25mg, increase each week 25mg, aim 50-150mg bd
20% - cognitive side-effects or reduced speech – must stop
Approx 10% - severe mood disorder (depressed / agitated / aggressive / suicidal) must stop
Tingling at higher doses (usually settles).
Risk of renal calculi, acute glaucoma (if myopic)
Drink > 3 Litres per day
Occasional hair loss
Beware induces POP and COCP
Beware teratogenic
Third Line
Gabapentin
– Some evidence of benefit
– Well tolerated in most
– Useful if comorbid restless legs
my first line if RLS persists after detox
– 600-1200mg tds
Paroxetine
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SSRI’s not likely to be as useful as tricyclic antidepressants
10mg, increase 20mg after 1 week
Warn side-effects (dizzy, nausea, drowsy) typically last only 2/52
May exacerbate poor sleep
Refractory cases
Flunarizine
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Off licence calcium antagonist
Licensed in some European countries where may be one of first line drugs
Anecdotal benefits in prolonged aura and migraine-related dizziness
Useful in refractory patients
Beware tardive (extrapyramidal) side effects, weight gain and severe depression
Olanzepine
– Very helpful in emergency situations
very resistant cases (in specialist clinics only)
Short term for important time
Beware weight gain, diabetes and tardive movement disorder
Methysergide
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Good anecdotal evidence
Useful for refractory cases
Safe if <12mg daily dose, drug holidays (1 month off every 5 months)
Monitor U+E, FBC, ESR, CXR (6 monthly) and echocardiograms yearly
Alternative agents
Pizotifen
– Very poorly tolerated – weight gain and sedation
– If tolerated, works reasonably
– Rarely used in headache clinics
Lisinopril, Candesartan
– Small evidence, small effect
Clonidine
– Licensed, but never been studied
Lamotrigine, verapamil, carbamazepine
– Unlikely to work as migraine preventatives
Alternative drugs – butterbur, coenzyme Q10, riboflavin, feverfew
– Small studies, some evidence
– Lack of systematic safety data
– Inconsistency of preparations (espec butterbur)
Alternative treatments
Greater Occipital Nerve (GON) Blocks
– Not proven
– Appear very effective for <3-4/12 in approx 40-50%
– Useful as stopgap strategy
IV Dihydroergotamine
– Not proven
– Appear very effective for <3-4/12 in approx 40-50%
– Useful as stopgap strategy
Botulinum toxin
– ? Role in chronic migraine (ineffective in acute migraine)
– Studies have blinding issues
Occipital Nerve Stimulation
– Experimental
– Anecdotal benefit in number of primary headache disorders including
migraine, cluster headache, SUNCT and hemicrania continua
Migraine Preventatives
Reasons for failure
1. Ineffective
– Acute attack drugs still used
– Caffeine
2. Not used to high enough dose
– Aim for “maximum tolerated dose”
3. Used at too high dose
– Often ineffective if patient persistently sedated
– Fatigue with migraine distinguished by pre-existing before drug
started or absence of fatigue on headache-free day
4. Not used for long enough, i.e. 4 months at top level
reached
5. Underlying sleep disorder – RLS, PLM, OSA