Headache
Primary headaches - Migraines
Cortical spreading depression hypothesis
- Slowly propagating (2-6mm/min) wave of
sustained strong neuronal depolarization travelling from posterior to
anterior
- Correlated with EEG and PET
hyperactivity followed by hypoactivity
- Cortical spreading depression
- Release of K+, H+,
arachidonic acid, prostaglandins, nitric oxide
- Activation of trigeminal afferents
(meningeal branches of V1)
- Local release of CGRP, causing
neurogenic inflammation of meninges and mast cell degranulation
- Activation of pars caudalis of
the trigeminal nucleus
- Projections to the PAG and
intralaminar nuclei of thalamus (perception of pain)
- Activation of superior salivary
nucleus, projecting to meningeal blood vessels to release nitric oxide,
VIP, Ach, leading to more vasodilation
Brainstem generator hypothesis
- Dorsal raphe nucleus, locus ceruleus
for migraine without aura
- Red nucleus, substantia nigra for
migraine with aura
- These nuclei found to be hyperaemic
during and 30 minutes after migraine
- May allow activation of trigeminal
nucleus
- Theory stems from that activation of
PAG can induce migraine-like headaches
Migraine without aura
- At least 5 attacks fulfilling
criteria.
- 4-72 hours (1-72 hours in kids)
- Two of these:
- Unilateral (often bilateral in young
children)
- Pulsating
- Moderate-severe pain
- Aggravated by activity
- At least one:
- Nausea and/or vomiting
- Photophobia and phonophobia
Migraine with aura
- Recurrent attacks of reversible focal
neurological symptoms
- Symptoms develop over 5-20 minutes,
last <60 minutes
- Headache begins during or within 60
min after aura
- Can be migrainous or nonmigrainous, or
no headache
- At least 2 attacks with auras
- Auras can be positive or negative,
visual or sensory
- Aura can also include dysphasic speech
disturbance
Familial hemiplegic migraine (and sporadic hemiplegic
migraine)
- Autosomal dominant inheritance
- Migraine with aura with motor weakness
- Often involve basilar-type symptoms,
decreased LOC, fever, confusion, CSF pleocytosis, MRI normal
- Treatment includes acetazolamide
10-30mg/kg/day or verapamil
- Sporadic form same clinical symptoms
but no family history or known mutation
FHM1
|
19p
|
CACNA1A
|
P/Q voltage
gated Ca channel
|
50%
|
Triggered by
mild head trauma
Progressive
cerebellar ataxia
Episodic ataxia type II, SCA-6
|
FHM2
|
1q
|
ATP1A2
|
A2 subunit of
Na/K pump
|
20%
|
Basilar-type
migraine
Epilepsy
Alternating hemiplegia of childhood
|
FHM3
|
2q24
|
SCN1A
|
voltage gated Na
channel
|
|
Subcortical
infarcts, small vessel arteriopathy, infantile epilepsy
GEFS+, Dravet
|
Basilar-type migraine
- Aura symptoms originating from
brainstem or bilateral hemispheres, without motor weakness
- Symptoms include:
- Dysarthria
- Vertigo
- Tinnitus
- Hypacusia
- Diplopia
- Visual symptoms in both
temporal/nasal fields of both eyes
- Ataxia
- Decreased LOC
- Bilateral paresthesias
Childhood periodic syndromes
- Cyclical vomiting
- Episodic, stereotypical attacks of
intense nausea and vomiting for 1hr-5days
- Associated pallor and lethargy
- Vomiting 4x/hr for at least 1hr
during attack
- Normal between attacks
- Abdominal migraine
- Midline abdominal pain lasting 1-72
hours
- Associated with anorexia, nausea,
vomiting, pallor
- Benign paroxysmal vertigo of childhood
- Attacks of severe vertigo lasting
minutes to hours
- Associated with nystagmus and
vomiting
- Can include unilateral throbbing
headache
Retinal migraine
- Attacks of monocular positive or
negative visual phenomena
- Headache fulfills migraine without
aura criteria
Chronic migraine migraine occurring >15days/month for >3
months
Status migrainosus debilitating migraine lasting >72 hours
Persistent aura without infarction aura lasting >1 week without
radiologic evidence for infarction
Migrainous infaction aura >60 minutes and MRI shows infarct
Migraine-triggered seizure seizure within 60 minutes after migraine aura
Primary headaches Tension-type
Tension-type headache
- Divided into:
- Frequent episodic vs. infrequent
episodic (<1/mo) vs. chronic
(>15d/mo) vs. probable
- With or without pericranial
tenderness
- Most common, but least studied form of
headache
- Bilateral, pressing or tightening, of
mild-mod intensity, not affected by activity
- No nausea. Can have photophobia or
phonophobia
- At least 10 episodes, lasting
30min-7days
Primary headaches Trigeminal autonomic cephalalgias
All forms have
prominent parasympathetic autonomic features, with secondary sympathetic
dysfunction.
Cluster headache
- Onset age 20-40 years, males >
females (4:1), 5% autosomal dominant
- Postulated pathophysiology is similar
to migraine, activation of posterior hypothalamic grey matter
- At least 5 attacks fulfilling criteria
- Severe unilateral orbital,
supraorbital, and/or temporal pain lasting 15-180 minutes
- At least one:
- Ipsilateral conjunctival injection /
lacrimation
- Ipsilateral nasal congestion /
rhinorrhea
- Ipsilateral eyelid edema
- Ipsilateral forehead and facial
sweating
- Ipsilateral miosis / ptosis
- Sense of restlessness / agitation
- Frequency q2d to 8/day, often cluster
periods of weeks-months, remission months-years.
- Episodic cluster
clusters of 7-365 days (usually 14-90 days), separated by >1 month
- Chronic cluster
cluster >1 year, no remissions or remissions <1mo
Treatment:
§
Acute:
o
Oxygen by facial mask 7-10L/min for 15 minutes (70%
efficacy)
o
Sumatriptan 6mg SC (75-100% efficacy)
o
DHE
nasal spray/IM, zolmitriptan PO, lidocaine
§
Short
term prevention:
o
Prednisone 60mg qday, taper after 2-3 weeks
o
Methysergide
1mg/d increase by 1mg q3days until 5mg, then q5days until 12mg
§
Long
term prevention:
o
Verapamil 80mg tid, increase 80mg q2weeks
o
Lithium
300mg bid, titrate to high normal levels (600-1200mg qday)
Paroxysmal hemicrania
- Shorter, more frequent, and more in
females
- At least 20 attacks fulfilling
criteria
- Severe unilateral orbital,
supraorbital, and/or temporal pain lasting 2-30 minutes
- At least one:
- Ipsilateral conjunctival injection /
lacrimation
- Ipsilateral nasal congestion /
rhinorrhea
- Ipsilateral eyelid edema
- Ipsilateral forehead and facial
sweating
- Ipsilateral miosis / ptosis
- Frequency ~ 5/day
- Completely prevented by therapeutic doses of indomethacin
- Need dose of 150mg PO/PR qday or 100mg
IV qday to R/O efficacy (lower dose for maintenance)
- Episodic paroxysmal hemicrania (see cluster)
- Chronic paroxysmal hemicrania (see cluster)
Treatment:
§
Indomethacin
25mg tid x10 days
o
Increase
to 50mg tid x10days
o
Increase
to 75mg tid x10days
§
If
works, try weening q6months
§
If
fails, look for different diagnosis!
Short-lasting Unilateral Neuralgiform
headache attacks with Conjunctival injection and Tearing (SUNCT)
- Much shorter attacks with prominent
lacrimation and redness of eye
- At least 20 attacks fulfilling
criteria
- Unilateral orbital, supraorbital,
and/or temporal stabbing/pulsating pain 5-240 seconds
- Ipsilateral conjunctival injection and
lacrimation
- Frequency 3-200/day
- May be a subform of Short-lasting Unilateral
Neuralgiform headache attacks with cranial Autonomic
symptoms (SUNA) with other features such as nasal congestion,
rhinorrhea, or eyelid edema
- Refractory to typical headache
therapies, but ?highly responsive to lamotrigine
100-200mg/day
Other Primary Headaches
Primary stabbing headache
- Single or series of stabs,
predominantly in the V1 distribution
- Commonly associated with migraine
(40%) and cluster headache (30%)
- Reports of response to indomethacin
Primary cough headache
- Sudden onset headache <30min in
association with coughing, straining, or Valsalva
- Bilateral, in patients >40years
- Response to indomethacin
- Can be symptomatic of Chiari I
malformation, carotid/vertebrobasilar disease, cerebral aneurysm
Primary exertional headache
- Pulsating headache 5min-48hr brought
on during or after physical exertion
- More in hot weather or high altitude
- May be prevented with ergots, or
treated with indomethacin
- Must exclude subarachnoid hemorrhage
and arterial dissection
Primary headache with sexual activity
- Preorgasmic headache dull ache in head and neck during sexual activity, increasing
with excitement
- Orgasmic headache sudden explosive headache during orgasm
- Must exclude CSF leak, subarachnoid
hemorrhage, arterial dissection
- Associated with primary exertional
headache and migraine
Hypnic headache
- Dull headache awakens patient from
sleep, lasting 15-180 minutes, occurring >15x/mo
- Begins after age 50 years
- Must differentiate with intracranial
disorders and TACs
Primary thunderclap headache
- Severe headpain, maximal intensity at
<1min, lasting 1hr-10d
- May recur within 1 week
- Normal CSF and MRI brain
- Must exclude subarachnoid hemorrhage,
intracerebral hemorrhage, CSVT, vascular malformation (aneurysm), arterial
dissection, CNS angiitis, benign CNS angiopathy, pituitary apoplexy,
colloid cyst, CSF hypotension, acute sinusitis
Hemicrania continua
- Persistent unilateral headache
responsive to indomethacin
- Lasting >3 months
- Side-locked, daily, continuous,
moderate intensity with exacerbations
- Autonomic features:
- conjunctival injection/lacrimation
- nasal congestion/rhinorrhea
- ptosis/miosis
New daily-peristent headache (NDPH)
- Daily headache within 3 days of onset
for >3mo
- Bilateral, pressing/tightening,
mild-mod intensity, not aggravated by activity
- Can have photophobia, phonophobia, or
mild nausea. No vomiting.
- Usually in a patient without prior
headache history
Secondary headaches attributed to head and/or neck
trauma
Secondary headaches attributed to cranial or cervical
vascular disorder
Subarachnoid hemorrhage
- Risk factors: >50, woman, black,
smoking, hypertension, heavy alcohol use
- AVM rupture greatest risk in 3rd
trimester
- Causes:
- Ruptured intracranial saccular aneurysm
(80%)
- Ruptured intracranial AVM (5%)
- Can have sentinel headache hours to months prior to bleed
- Hunt & Hess grading
- Grade 1 asymptomatic, minimal
headache, mild nucchal rigidity
- Grade 2 mod-severe headache +/- CN
deficits
- Grade 3 drowsiness/confusion, mild
focal neuro deficits
- Grade 4 Stupor, mod-severe
hemiparesis
- Grade 5 Coma, decerebrate posturing
- 15% false negative conventional
angiogram
- Watch for cerebral salt wasting and
cardiac stun (high CPK, from catecholamine release)
-
Secondary headaches attributed to non-vascular
intracranial disorder
Headache attributed to high CSF pressure
- Idiopathic intracranial hypertension
- Daily, diffuse, constant pain
aggravated by coughing/straining
- Papilledema, enlarged blind spot,
progressive visual field deficit, CN6 palsy
- Can also have intracranial noises,
tinnitus, transient visual obscurations, diplopia
- Intracranial hypertension secondary to
metabolic, toxic, or hormonal causes
- Endocrine hypoPTH, hypothyroidism,
obesity, Addison disease
- Medications Corticosteroids,
Lithium, vitamin A, tetracyclines, retinoic acid, bactrim, levothyroxine,
growth hormone
- Intracranial hypertension secondary to
hydrocephalus
- Intracranial hypertension also caused
by systemic autoimmune disease SLE, sarcoidosis, Behηet disease
Treatment:
§
Acetazolamide
1-4g/day (start at 250mg bid)
o
Side
effects: distal paresthesias, unpleasant taste, low serum bicarb, allergic
rash, anemia, renal stones, sulfa
allergy
o
Can
use after 20weeks gestation
§
Corticosteroids
4-6wks in severe visual symptoms
§
Topiramate
§
Intermittent
NSAIDs, other migraine prophylactic therapies
§
Optic
nerve sheath decompression
§
CSF
shunting
Headache attributed to low CSF pressure
- Post-dural puncture headache
- Worsens within 15 minutes of
sitting/standing, improves with lying down
- Neck stiffness, tinnitus, hypacusia,
photophobia, nausea
- Develops within 5 days of LP, usually
resolves within a week or after epidural blood patch
- CSF fistula headache
- Occurs after known trauma or
procedure
- MRI pachymeningeal enhancement,
evidence of leak on myelography or cisternography
- CSF opening pressure <60 mmH2O
in sitting position
- Idiopathic low CSF pressure
- No history of known trauma or
procedure
Treatment:
§
Bedrest
and caffeine for 1-2 weeks
§
Blind
blood patch (thoracic/cervical)
§
CT
myelograph to ID leak, then patch or surgery
Syndrome of transient Headache and Neurological
Deficits with CSF Lymphocytosis (HaNDL)
- CSF pleocytosis with lymphocytic
predominance (>15cells/μL), normal cultures and imaging
- Associated headache and transient
neurological deficits
- Neuro deficits include motor/sensory
deficits and aphasia
- Can also include elevated CSF protein
and opening pressure, with papilledema
- Episodes recur over <3 months
Secondary headaches attributed to substance or
withdrawal
Headache induced by acute substance use or exposure
- Nitric oxide donor
- Phosphodiesterase inhibitor
- Carbon monoxide
- Alcohol
- Monosodium glutamate
- Cocaine
- Cannabis
- Histamine
- CGRP
Medication overuse headache (MOH)
- Headache on >15days/mo, regular
overuse for >3mo, resolves after 2mo of discontinuation of drug
- Ergotamine (>10d/mo)
- Triptan (>10d/mo)
- Analgesic (>15d/mo)
- Opioid (>10d/mo
Headache as an adverse event attributed to chronic
medication
- Exogenous hormones contraception or
hormone replacement therapy
Headache attributed to substance withdrawal
- Bilateral, pulsating headache
associated with withdrawal of regular intake
- Improves with taking substance or 7
days of withdrawal
- Caffeine-withdrawal
- Opioid-withdrawal
- Estrogen-withdrawal
Secondary headaches attributed to infection
Secondary headaches attributed to disorder of
homeostasis
- Hypoxia /
hypercapnea altitude, diving, sleep apnea
- Dialysis
- Arterial
hypertension pheochromocytoma, preeclampsia/eclampsia, hypertension
- Hypothyroidism
- Fasting
- Cardiac cephalalgia
associated with myocardial infarction, resolves with managment of MI
Secondary headaches attributed to disorders of cranium,
neck, eyes, ears, nose, sinuses, teeth, mouth
Secondary headaches attributed to psychiatric disorder
Cranial neuralgias and facial pain
Trigeminal neuralgia (tic douloureux)
- Classical trigeminal neuralgia
- Paroxysmal, stereotyped attacks of
pain lasting <2min in a division of the trigeminal
- Intense, sharp, superficial, stabbing
- Pain can evoke spasm of muscles of
face
- Precipitated from trigger area or
trigger factors
- Washing, shaving, smoking, talking,
brushing teeth
- Often has a refractory period after a
paroxysm
- May be due to compression of
trigeminal root or aberrant vessels
- If bilateral, must consider other
etiology, e.g., multiple sclerosis
- Symptomatic trigeminal neuralgia
- Same as Classical but demonstrable
structural lesion found, but no refractory period
Glossopharyngeal neuralgia
- Classical glossopharyngeal neuralgia
- Same idea as trigeminal neuralgia,
but in CNIX distribution
- Posterior tongue, tonsillar fossa,
pharynx, beneath lower jaw, in ear
- Precipitated by:
- Swallowing, chewing, talking,
coughing, yawning
- Symptomatic glossopharyngeal neuralgia
- Aching pain persists between
paroxysms
- Sensory impairment in CNIX
distribution
Nervus intermedius neuralgia
- Paroxysmal brief pain in depth of ear
- Trigger area in posterior wall of
auditory canal
- Accompanying lacrimation, salivation,
taste changes
- Associated with VZV
- Can present like otalgic variant of
glossopharyngeal neuralgia
Superior laryngeal neuralgia
- Same but in superior laryngeal nerve
- Throat, submandibular region, under
ear
- Triggered by swallowing, straining
voice, head turning
a bunch more, check the ICHD if interested.
Tolosa-Hunt Syndrome
- Rare before age 20 years
- Episodic orbital pain with paralysis
of CN III, IV, VI within 2 weeks
- Granulomatous infiltration of
cavernous sinus / superior orbital fissure / orbit
- Symptoms resolve within 72 hours of
corticosteroids
- Treatment: Prenisone 60-120mg qday
x7-10days
- 40% patients have relapse
Ophthalmoplegic migraine
- Recurrent migrainous headaches with
paresis of 1+ ocular cranial nerves (esp. CNIII) within 4 days
- At least 2 attacks, and no
intracranial lesion (MRI may show Gad enhancement of nerve)
- Headache often lasts 7+ days
- Not migraine - ? recurrent
demyelinating neuropathy
Central causes of facial pain
- Anaesthesia dolorosa
- painful anesthesia or hypasthesia in
trigeminal or occipital nerve from central cause
- often related to surgical trauma for
therapy of cranial neuralgia
- Central post-stroke pain
- Facial pain associated with multiple
sclerosis
Other unclassified headaches
Pure menstrual migraine
without aura
- Migraine without aura
occurring exclusively on day 1+/-2 of menstruation in at least 2/3
menstrual cycles
Menstrually-related migraine
without aura
- Also happens at other
times
Non-menstrual migraine without
aura
- Migraine without aura in
a menstruating woman
Migraine aura status
- 2+ auras per day for
>5 days
Alternating hemiplegia of childhood
- Recurrent hemiplegia, progressive
encephalopathy, paroxysmal phenomena and mental impairment
- Onset before age 18 months
- Paroxysmal phenomena associated with
hemiplegia or independently including tonic spells, dystonic posturing,
choreoathetoid movements, nystagmus, ocular motor abnormalities, autonomic
disturbances
- Clinical presentation:
- Phase 1
- Mild developmental delay
- Intermittent attacks precipitated by
excitement, stress, fatigue
- Episodic nystagmus, ocular
deviation, dystonia, hemiplegia
- Lasts minutes-days, at least
once/month
- Right side more often affected than
left
- Phase 2
- Lasts 1-5 years
- Intensified symptoms, more frequent
- Developmental regression, persistent
neurological deficits
- May develop choreoathetosis between
attacks
- Phase 3
- Persistent developmental delay,
fixed neurological deficits
- Decreased in frequency of attacks
- Normal EEG, MRI, CT, metabolic work-up
- Outcome:
- 90% have developmental delay, 50%
mild
- Severity related to age of onset
- Flunarazine can decrease attack
frequency, but does not change outcome
Benign paroxysmal torticollis
- Episodic, self-remitting
head tilt lasting mins-days, recurring monthly
- Associated with pallor,
irritability, malaise, vomiting, ataxia
- Onset in 1st year
- Can evolve into benign
paroxysmal vertigo of childhood, migraine with aura, or nothing
Nummular headache
- Pain in a small
circumscribed area of the head without underlying lesion
- Mild to moderate pain
- Chronic lasting
weeks-months
- Slight female
preponderance
Acute treatments
Acetaminophen and NSAIDs
Triptans
- Serotonin 5HT 1B/D receptor agonists
- Mechanisms include:
- 1B - Selective
intracranial/extracerebral vasoconstriction (counteracts neurogenic
vasodilation)
- 1D inhibits trigeminal sensory
nerve activation and release of vasoactive neuropeptides
- 1B/D/F inhibits neurons of
trigeminal-cervical complex
- Common side effects:
- Hypertension, vasospasm
- Atypical sensations (burning,
tingling, warmth)
- Palpitations, syncope
- Contraindications:
- Ischemic cardiac, cerebrovascular,
peripheral vascular syndromes
- Vasospastic coronary artery disease
- Uncontrolled hypertension
- Basilar or hemiplegic migraine
- Other meds in 24 hours ergots
(methylsergide, dihydroergotamine) or other triptans
- Other meds in 2 weeks MAOIs
Ergots
- 5HT (non-specific),
NE, DA agonists
- Common side effects:
- Hypertension,
vasospasm
- Paresthesia, rash,
headache
- Dizziness, anxiety,
dyspnea, flushing, diarrhea, increased sweating
- Severe side effects:
- Long term use pleural
and retroperitoneal fibrosis
- Myocardial infarction
and stroke
Antiemetics
- Prochlorperazine and
chlorpromazine IV
- Side effects:
- Prochlorperazine
(Stemetil) is neuroleptic extrapyramidal symptoms
Opiates
- Butorphanol nasal spray
(atypical opioid)
- High risk for dependency
and abuse
Status migrainosus therapy
- Dark, quiet room, IV
fluids, sedation
- IM/IV ketoralac (Toradol)
0.4-1 mg/kg/dose
- Antiemetics:
- Metoclopramide (Maxeran)
1-2 mg/kg/dose
- Chlorpromazine 0.5-1
mg/kg/dose
- Ondansetron
- IV DHE 0.1-0.2mg/dose q8h
(pretreat with anti-emetic)
- IV steroids
- Methylprednisone
1mg/kg/dose
- Dexamethasone
0.25-0.5mg/kg/dose
- PO prednisone 40-60mg
tapered over 3-5 days
Raskin protocol
- Metoclopramide 10mg IV
q8h x 2-5 days
- Dihydroergotamine 0.5mg
IV q8h x 2-5 days
Prophylactic therapy
Drug
|
Dose
|
Side effects
|
ACE inhibitors and
angiotensin receptor antagonists
|
Candesartan
|
16mg
qd
|
Headache,
dizziness, angioedema, hyperkalemia, hyponatremia, leukopenia, rhabdomyolysis
|
Anticonvulsants
|
Valproate
|
500-1500mg
|
See AEDs page
|
Carbamazepine
|
600-1200mg
|
Gabapentin
|
600-1200mg
|
Topiramate
|
100mg
|
Antidepressants (TCA, SSRI,
MAOI)
|
Amitriptyline
|
10-400mg
|
Dry
mouth, metallic taste, epigastric distress, constipation, dizziness, mental
confusion, tachycardia, palpitations, blurred vision, urinary retention,
orthostatic hypotension, conversion of depression to mania (bipolar), decreased seizure threshold
|
Beta-adrenergic blockers
|
Atenolol
|
50-200mg
|
Drowsiness,
fatigue, lethargy, sleep disorders, nightmares, depression, memory
disturbance, hallucination, GI effects, decreased exercise tolerance,
orthostatic hypotension, bradycardia, impotence, aggravation of intrinsic
muscle disease (myasthenia gravis),
teratogenicity, asthma, insulin-dependent diabetes
|
Propranolol
|
40-400mg
|
Calcium channel antagonists
|
Verapamil
|
120-640mg
|
Increase
in headache, constipation, dizziness, nausea, hypotension, edema, heart block
|
Flunarizine
|
5-10mg
|
Weight
gain, somnolence, dry mouth, dizziness, hypotension, extrapyramidal symptoms, depression
|
Neurotoxins
|
Botulinum
toxin A
|
IM
q3mo
|
Anaphylaxis,
local and adjacent muscle weakness, dysphagia, dyspnea
|
Serotonin agonists
|
Methysergide
|
|
Retroperitoneal
and pleural fibrosis, valvular fibrosis, GI symptoms, edema, flushing, hair
loss, weight gain, neutropenia
|
Others
|
Riboflavin
|
400mg
qd
|
Yellow
urine
|
Magnesium
|
400-600mg
|
GI
effects, arrhythmias
|
Feverfew
|
50-82mg
|
|
Butterbur
(Petasites)
|
50-100mg
|
|
Coenzyme
Q10
|
100-150mg
|
|
Updated: December 31, 2007
Disclaimer: These are personal study notes.
No promises for accuracy or originality.