Headache Syndromes Flashcards

1
Q

Primary Headaches defined

A

● No obvious underlying cause
● Chronic, recurrent and usually no other signs
or symptoms of neurologic disease.
● Can have Familial relationship
Examples include- Migraines, Cluster
Headache, Tension type Headache, etc.

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2
Q

Secondary Headaches defined

A

● Headache associated with an underlying
cause.
● Headaches that warrant more extensive
work-up
● Neurologic symptoms
● Abnormal Mental status
● Red Flags (More Later)
● Fever

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3
Q

Examples of secondary headaches

A
  • Intracranial lesions, Head
    injury, Dental, Ocular disease, Tumors, Infection, Meningitis, Subarachnoid Hemorrhage, Temporal Arteritis, Sinusitis, Carbon Monoxide Poisoning, Alcohol, Hypoxia, Post- Lumbar puncture, TMJ,
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4
Q

Physical Exam for headaches

A

● Perform and then document a Comprehensive Neurologic Exam
● Fundoscopic exam- Papilledema can be seen with increase in intracranial pressure.
● Intraocular pressure measured with Tonometry- Acute angle closure glaucoma
● Palpate the Temporal Artery- Temporal Arteritis

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5
Q

Diagnostic Studies for headaches

A

● CT
● MRI
● Labs - CBC, CMP, ESR, CRP
● Lumbar Puncture
○ CSF pressure and Analysis
○ Presence of Blood in CSF

Necessary only when the headache pattern has changed recently, the headache
cannot be clearly defined by the clinician as a common primary headache disorder, or neurologic examination reveals abnormal findings”

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6
Q

Types of Headaches - Benign

A

1) Tension Headache
2) Cluster Headache
3) Migraine Headache
4) Analgesic Rebound Headache

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7
Q

Types of Headaches - High Risk

A

1) Subarachnoid Hemorrhage
2) Space-Occupying Lesion
3) Temporal Arteritis
4) CNS Infection

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8
Q

Tension Headache

A

● Most common type of Primary Headache. Range from 30-78% of population
● Etiology is not completely clear, multifactorial, several theories exist.
● Band-like or Viselike, tightness
● More common in Adults, while onset of migraines is usually in younger patients
● High correlation with Depression and Anxiety
● Diagnostic overlap with migraines, similar to migraine without aura
● Worsened with stress, fatigue, noise, glare

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9
Q

Tension Headache
Diagnosis

A

Band-like / Vise-like tightness, bilateral, with predominantly frontal or occipital location. Can be
generalized, and not usually characterized by worsening with activity.

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10
Q

Tension Headache
Treatment

A

● Similar to migraines, often clinicians lump together unless obvious, distinguishing the two can be difficult.
● NSAID, Tylenol
● Dose of Toradol 30-60 mg IM works well in primary care setting
● Amitriptyline prophylactically
● Dihydroergotamine (Problematic) - Pregnancy Cat X, contraindicated in CVD, PVD, HTN, and Macrolides
● Avoid opioids
● Treat comorbid depression or anxiety
● Triptans and Ergotamine not indicated- Why do you think that is?
● Massage, Acupuncture, Improved sleep
● If a person came to ED, we often used a Headache Cocktail

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11
Q

Cluster Headache

A

● Affects predominantly males ages 20-50 yo
● Severe unilateral headache with a patterned timing and/or clusters
● Pathophysiology mechanism is unknown. Thought to be derived from cell
activation of the ipsilateral hypothalamus, which controls circadian rhythms,
which in turn causes triggering the trigeminal autonomic system.
● Can be triggered by alcohol (surprisingly during attacks, but not during periods
of remission) , histamine, nitroglycerin

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12
Q

Cluster Headache presentation

A

● Pain is severe unilateral orbital localization- deep around the eye. Can radiate to temple forehead
and cheek.
● Occurs often during sleep (Can occur during the day), “Alarm Clock Headache” -recurrence daily (15
mins - 3 hrs) at similar times (often 1-2 hrs after sleep onset) lasting for days or weeks. Followed
by resolution of symptoms for months or even years.
● Patient often may arises from bed, agitated, paces, and hold hand to side of the head.

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13
Q

Things that differentiate tension headaches from migraines:

A

+/- photophobia, +/- phonophobia, and without
lateralization of migraines, and typically absent
nausea, vomiting.

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14
Q

Autonomic symptoms during a cluster headache attack

A

● Ptosis, Miosis - can persist after long standing chronic attacks
● Conjunctival Injection, Lacrimation
● Nasal congestion, Rhinorrhea

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15
Q

Cluster Headache
Treatment for acute attack

A

● 100% Oxygen
● Verapamil
● Ergotamine - anticipatory dose when attack is expected
● Intranasal Lidocaine- effect on the sphenopalatine ganglion, Implantable nerve stimulator- experimental
● Vagus nerve stimulation
● Intranasal Triptan

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16
Q

Cluster Headache
Treatment - prophylactic

A

● Verapamil
● Prednisone taper
● Lithium - worry about toxicity
● In intractable cases stimulation of the hypothalamus, and trigeminal nerve ablation has been tried

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17
Q

Migraine Headache

A

● Very common condition, begins typically in adolescence, or childhood and can
improve in advanced years. Can be triggered by stress, certain foods, alcohol,
beverages, noise, menstrual cycle
● Intense, typically unilateral recurrent headache

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18
Q

Pathophysiology of Migraine headaches

A

● Pathophysiology not totally clear. Considered to have strong genetic relationship
to autosomal dominance has been seen. Thought in the past thought to be
related solely to distention of the intracranial arteries. This idea has diminished
over time with newer neuroimaging technology
● Currently the relationship to dysfunction of the trigeminal system causing release
of neuropeptides such as calcitonin gene-related peptide (CGRP), which leads to
an inflammatory cascade. Leads to neurogenic inflammation into vessel walls
● Role of serotonin (5-HT) as a mediator of migraine propagation, hence use of
serotonin agonist eg. sumatriptan (Imitrex) reducing neurogenic inflammation

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19
Q

Two main Categories of migraine headaches

A

● Migraine with Aura - Traditionally referred to as “Classic Migraine”
● Migraine without Aura - “Common Migraine” appropriately named, as it is more common. 5:1

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20
Q

Phases of a migraine headache

A

● Prodrome- 60% of people will report pre-symptoms- Light, Sound sensitivity, Fatigue, Food cravings, Mood changes, Excessive thirst, Anorexia, etc.
● Aura- For those who get it. Usually visual disturbance, but can be sensory (numbness to face hands, etc.) or motor (Sense of heaviness in the limbs, Speech Disturbance). Associated with scintillating scotoma (enlarging blind spot with shimmering edge.)
● Migraine Phase- The actual headache
● Postdromal Phase- Up to 24 hours of feeling tired, or euphoric, weakness, food cravings, anorexia

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21
Q

Migraine Headache
Diagnosis

A

● Headache, usually pulsatile, typically lateralized throbbing
● Recurrent- To diagnose, must have repeated attacks, lasting 4-72 hrs, not attributable to secondary cause
● Characteristic nausea, vomiting, photophobia, phonophobia
● Patient often will want to lay still, in a darkened room
● Can be preceded by an Aura ( often described as a visual disturbance).
● Headache can build gradually and last for hours
● Important to note, that migraines for some can be somewhat individualized and predictable.

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22
Q

Migraine Headache
Abortive Treatments

A

● The earlier the better, prodrome phase
● Various categories eg. Triptans, NSAIDs, Ergots, CGRP, 5HT1F agonists
● Headache Cocktail- various combinations of meds, that can be given to treat migraines,
varies w/ facility- Hospital based vs Outpatient setting
● Darken the room, limit noise
● Acupuncture - Some consider as nearly effective as medication use.
● May take some trial and error to find what works best

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23
Q

Headache Cocktail

A

○ IV Normal Saline 1 liter
○ Diphenhydramine (Benadryl) 25 mg IV
○ Metoclopramide (Reglan) 10 mg IV, or you can substitute
■ Phenergan 25 mg IV or Zofran 4-8 mg IV
○ Ketorolac (Toradol) 30 mg IV
○ +/- Magnesium 2 mg IV
○ Oxygen

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24
Q

Triptans

A

● serotonin receptor- namely 5-hydroxytryptamine (5-HT1b/1d) receptor agonist
eg. Sumatriptan (oral and intranasal), Eletriptan, Rizatriptan, Zolmitriptan
● Triptans used nearly exclusively for Migraines- other primary headaches may respond, should be taken early in the migraine
● Because migraines are not completely understood, the actual mechanism of triptans in not totally understood. Thought to activate serotonin 5-HT1B receptors which inhibit
release of vasoactive peptides. Resulting in vasoconstriction

25
Q

Serotonin 5-HT1f Agonist - Lasmiditan (Reyvow)

A

● New and novel class of medication.
● Lasmiditan (Reyvow) approved in 2019
● Although not completely understood, selective to serotonin 5-HT1F thus reducing
trigeminal nerve firing, without the vasoconstriction seen in triptans

26
Q

Dihydroergotamine

A

Ergot alkaloid derived from a fungus that affects grain, rye.
● Often combined with caffeine (eg. Cafergot)
● Lots of drug interactions
● Similar action to triptans. Effects α- adrenoceptors and 5-HT1 agonist

27
Q

Black Box for Dihydroergotamine

A

Avoid use with potent CYP3A4 inhibitors due to increased risk of ischemic
events, avoid use in those with cardiovascular disease.
○ FYI- Just make sure you run a med interaction check. CYP3A4 inhibitors (eg.
Ketoconazole, clarithromycin, protease inhibitors (HIV drugs), etc.)

28
Q

CGRP Antagonists

A

● Calcitonin-gene related peptides orally given as small molecule CGRP
antagonists
● Ubrogepant (Ubrevly) in 2019, Rimegepant (Nurtec) in 2020,
Zavegepant (Zavzpret) in 2023
● Those with refractory or contraindications to triptans
● Not to be confused with CGRP monoclonal antibodies used for
prophylaxis

29
Q

Chronic headaches defined

A

● 8 days per month, with 15 HAs in a month

30
Q

Migraine Headache
Prophylactic Medications

A

Antidepressants
● Amitriptyline- Tricyclic antidepressant (TCA) often given as first-line prophylaxis in younger patients
○ Can cause drowsiness
● Venlafaxine (Effexor)-
Cardiovascular
● B-Blocker- Typically Propranolol is used. Metoprolol likely has some efficacy,
○ Mechanism for B-blocker not clearly understood.
● Verapamil - Can be used for cluster headache prophylaxis
Anticonvulsants
● Topiramate
● Valproate
Calcitonin gene-related peptide (CGRP) Antagonist
● Newer category

31
Q

Analgesic Rebound Headache

A

● Defined as development or build up of tolerance in the presence of chronic pain
medication use.
● Can occur with Opioids, Triptans, NSAIDs, Acetaminophen
● Usually pre-existing Headache syndrome.
● Generally treatment requires withdrawal, often slowly, from the offending agent.

32
Q

Analgesic Rebound Headache overview

A

● Headaches greater than 15 days/ month for greater than 3 months.
● This also applies to other types of chronic pain, when opioids are used for greater than several
months. (Low Back Pain, etc.)
● Slow reduction of pain medicine over time. Various strategies exist.
● May require long-acting substitute, and tapering dose. May require formal drug rehab

33
Q

Red Flags for headaches

A

● Onset- Sudden (Thunderclap) , Trauma, Exertion, Positional
● Altered Mental Status
● Seizure
● Fever or systemic symptoms, Eye pain
● Neurologic Symptoms/ Signs and/or deficit
● Visual Changes
● Medications- Anticoagulant, Antiplatelets
● Immunosuppression - Medications, HIV, etc.
● PMHx– No prior Headache History, or a change in headache quality or progressive
● PMHx– Pregnancy, Lupus, Cancer, Sarcoidosis,
● Pregnancy (MRI no contrast)
● Physical Exam- Neck Stiffness, Papilledema
● Onset of Sx after 50

34
Q

Indications for Neuroimaging to Evaluate Headaches

A

● Headache- change in typical headache characteristics or pattern, intensity, “first or worst headache”
● Abnormal Neuro exam or neurodeficit
● Onset of migraines over 50 years old
● Change in pattern or new onset, or a post traumatic headache
● Fever/ Immunosuppressants or systemic symptoms- Brain Abscess
● Altered Mental Status
● Stiff Neck, Papilledema, painful eye
● Awakening from sleep
● Changing in position, sneezing, coughing, exercise
● Previous Cancer diagnosis or immunosuppresion
● Seizure
● Personality change, intellectual or cognitive deficit. (Space Occupying Lesion)
● Hypertension
● Pregnancy

35
Q

Pregnancy red flags for headaches

A

Preeclampsia, New onset or a changing headache requires evaluation for secondary
headache cause in the setting of pregnancy.

35
Q

Indications for Neuroimaging with headaches

A

Brain MRI typically with contrast is preferred.
● Radiologist can exercise discretion and can change your contrast order.
Noncontrast Head CT is a good initial test in acute settings:
● Trauma
● Thunderclap
● New Headache with neurologic deficit
● Chronic headache with a change in features
● Sensitivity drops over time given resorption of blood.

36
Q

Red Flags for headaches with SLE

A

Cognitive Dysfunction can occur and a “Lupus Headache” which presents similar to migraines. Potential relationship to vasculitis. As it begins to affect the CNS, patients with Lupus can present with altered mental status, encephalopathy, psychosis, seizures. Additionally patients with lupus are on immunosuppressants

37
Q

Cause of Thunderclap Headaches

A

● Migraine
● Subarachnoid hemorrhage
● Cerebral venous thrombosis
● Diffuse Cerebral Vasospasm (Call-Fleming syndrome) - reversible vasoconstriction, idiopathic.
● Accelerated hypertension - Diastolic >120, reducing BP relieves pain- Careful attributing it to BP.
● Pituitary apoplexy - spontaneous hemorrhage of pituitary adenoma
● Cocaine and adrenergically active drugs- vasoconstriction
● Perimesencephalic non-aneursymal subarachnoid hemorrhage - less severe subtype of SAH

38
Q

Subarachnoid hemorrhage

A

● Caused by rupture of a berry aneurysm of arteries at bifurcations of the Circle of
Willis, given the abrupt nature at onset, the term “Thunderclap Headache” was
coined. (10-25% of patients complaining of “worst headache of their life”,
“thunderclap”, presenting to the ED have a SAH)
● High mortality 30 day survival rate is only 50%, and half of those that survive,
will have some type of neurologic impairment.
● 1% of patients presenting to the ED with a headache have a subarachnoid
hemorrhage (SAH)

39
Q

Subarachnoid Hemorrhage diagnosis

A

● Sudden severe headache, with maximum intensity in 10 minutes SAH until proven otherwise
● +/- altered mental status
● Vomiting
● Hypertension
● Stiff neck
● Severe Distress
● Possible more subtle manifestations can occur.
● Mainstay of evaluation is initial noncontrast CT and/or LP

40
Q

Ottawa Subarachnoid Hemorrhage Rule

A

● Age > 40yo
● Neck Pain or Stiffness
● Witnesses Loss of Consciousness
● Onset during exertion
● Thunderclap Headache
● Limited neck flexion on Examination

41
Q

Diagnostic Studies for SAH

A

● Non Contrast Head CT - initial test for suspicion of intracranial bleed. SAH (As well as a Stroke)
○ In the case of Subarachnoid Hemorrhage shows the bleed, but in smaller “warning leaks” CT can be normal. If high suspicion, should next perform a Lumbar Puncture
● Lumbar Puncture
○ This is done looking for blood or xanthochromia (Yellowish, bilirubin present → breakdown of blood)

42
Q

Warning Leaks in SAH

A

● With all this talk about the “thunderclap”, patients can present with more mild headaches, with
normal imaging, and no significant findings. Headache earlier in the day that has now resolved.
● They can have what’s referred to as a Sentinel Bleed, or a Warning Leak.

43
Q

Space Occupying lesion

A

● A tumor, mass lesion either malignant, infectious (Abscess), hematoma, or otherwise, that is causing
displacement of normal brain tissue, being that space is limited within the enclosed skull.
● Suspect with changes in Neurologic status, Behavior, Cognition, Seizures.

44
Q

Headache in the setting of a brain tumor is caused by ______

A

CSF obstruction and intracranial
hypertension.

45
Q

Space Occupying lesion
Diagnostic Studies

A

● CT
● MRI with contrast
● Labs- CBC, CMP
● LP should be delayed due to increased risk of herniation, can be done once space occupying lesion
is ruled out.
● Comprehensive Neurologic Exam
● Oncology Referral
● Neurosurgical consultation

46
Q

Giant Cell Arteritis

A

● A form of Vasculitis. Giant Cell Arteritis, named also temporal arteritis because it is considered a
systemic panarteritis, which commonly affects the temporal artery. Subacute inflammatory change
seen with lymphocytes, mononuclear cells and giant cells. Leads to thrombosis of the artery. Can
also result in a stroke when effecting the cerebral arteries

47
Q

Giant Cell Arteritis
Diagnosis

A

● Age > 50 yo
● New Headache
● Tenderness when palpating over the artery, decreased pulsations
● ESR > 50mm/h
● Abnormal Artery Biopsy - Vasculitis, Mononuclear cell infiltration, Multinucleated Giant Cells
○ Will also see myalgias, fatigue, fever, anorexia, pain worse at night. Labs - elevated CRP

48
Q

Physical Exam for Giant Cell Arteritis

A

● Palpate over the artery for tenderness, throbbing unilateral, +/- bilateral headache, with pain
localized to the temporal scalp
● Asymmetry of pulses
● Ultrasound, MRA, CTA have been used to demonstrate narrowing of the arteries.

49
Q

Giant Cell Arteritis management

A

IV steroids, followed by oral steroids. These must be started promptly to avoid
co-morbidities.
● Steroid taper given over several weeks.
● Diagnosis should be revisited if their is poor response to corticosteroids

50
Q

Approach to CNS Infection

A

● Can be caused by numerous pathogens.
○ Bacteria
○ Viral
○ Fungi
○ Protozoa
○ Spirochetes
● Conditions include
○ Meningitis - Involving the meninges
○ Encephalitis - Involving the parenchyma
○ Brain Abscess - Consider a space occupying lesion of the brain
○ Epidural Abscess - Involving space between Dura and Bone, can be spinal or intracranial.

51
Q

Approach to CNS Infection
Diagnostic Studies

A

● CBC, CMP
● Blood cultures and gram stain (Ideally prior to antibiotics)
● Neuroimaging
○ CT
○ MRI
○ Chest X-ray
● LP (If mass lesion is not detected, given risk of herniation) with CSF Analysis and gram stain
○ Don’t delay antibiotics for the LP
● Viral Antibody testing
● Sepsis protocol

52
Q

SOFA Sepsis criteria

A

● Fever > 100.9 or <96.8
● Heart Rate >90 bpm
● Tachypnea > 20 breaths/min or PaCO2 < 32 mmHg
● WBC >12,000 or <4,000

53
Q

Diagnosis of Meningitis

A

Diagnosis made with a gram stain smear of the CSF obtained from LP.
● CBC, CMP, Blood Cultures (Should be obtained before starting antibiotics), CSF analysis and culture,
and Chest X-ray

54
Q

Encephalitis

A

● Infection to the brain parenchyma, as opposed to the meninges. Worse with extremes of age.
● Viral Prodrome of Fever, Headache, Neck Pain, or Back Pain, Nausea, Vomiting, Lethargy, May have
Viral Rash
● Can be present concurrently with Meningitis. Called Meningoencephalitis

55
Q

Diagnosis of Encephalitis

A

● CBC, CMP, Blood Cultures (Should be obtained before starting antibiotics), and Chest X-ray, CT, MRI, Viral Antibody testing, LP with viral and bacteria cultures of CSF.

56
Q

Diagnosis of Brain Abscess

A

● Neuroimaging w/ contrast. MRI is better, CT is faster ( Repeated to insure resolution)
● Risk for herniation if LP is performed, and LP may not be helpful
● CBC, CMP, Blood Cultures (Should be obtained before starting antibiotics)
● Consider sepsis workup

57
Q

Brain Abscess

A

● Also considered a space occupying lesion
● Presentation similar to other space occupying lesions, with likely fever or infectious suspicion.
● Caused by any number of bacteria, fungi, protozoa
● Higher risk in HIV patients

58
Q

Brain Abscess treatment

A

○ Surgical excision and drainage with Abx (6-8 wks)
○ CT guided needle aspiration