Headaches

Question 1

Primary vs secondary headaches

Answer 1

Primary Headaches: No obvious pathological cause, but a well-known syndrome of headaches, such as migraine, tension-type, cluster, etc.

Secondary Headache, or Headaches: A pathological cause can be found, such as tumor, hemorrhage, infection, etc.

Question 2

PHYSICAL EXAMINATION

Answer 2

Concentrate on:

General appearance

Fever or other abnormal vital signs,

supple neck?

Mental status,

level of consciousness,

speech

Vision and the retinal discs: pupils, EOMI? Papilledema?

Asymmetry of strengths or reflexes in extremities,

Babinski sign

Question 3

Secondary Headache Warning Signs and Signals

Answer 3

A single headache (versus recurrent headaches)

Sudden onset (thunderclap headache)

Onset of headaches after age 50 years

Recent onset of headaches, especially < 6 months

Systemic disease (malignancy, AIDS)

Change in headache pattern from a prior pattern

• Progressive headache with loss of headache-free periods
• Change in frequency or severity

Neurologic symptoms or abnormal neurologic exam

Question 4

Lumbar Puncture

Answer 4

Necessary to diagnose meningitis or encephalitis, or possible carcinomatous meningitis

Can be used if there remains suspicion of subarachnoid hemorrhage but no blood is seen on a head CT or an MRI

When opening pressure is elevated, it can help diagnose pseudotumor cerebri, or Idiopathic Intracranial Hypertension,

Question 5

Summary

Answer 5

Primary headache syndromes are diagnosed by defining the clinical features of an individual’s attacks and applying them to established definitions

The majority of headaches seen in primary care will be the primary headache disorders

If care is taken to identify warning signs and symptoms, and appropriate diagnostic tests are negative, the chance of missing a secondary headache is greatly diminished

Question 6

Neuroimaging in Headache Patients

Answer 6

Recurrent migraine: neither CT nor MRI is warranted except

• Recent change in headache pattern
• Focal neurologic signs or symptoms

Nonmigraine headache: Role of CT or MRI is unclear

Role of CT versus MRI in headache patients is unclear, but MRI is more likely to show a cause for headaches;

CT is sensitive for > 90% of subarachnoid hemorrhages, and is faster than MRI

MRI without contrast might be indicated in pregnancy

Question 7

Sometimes, You Can Feel It Coming: The Aura

Answer 7

Develops gradually right before attack and lasts less than an hour, often 20 minutes, in 10 – 15% of patients with migraine

Visual changes:

• Blurred vision or blind spots
• Seeing flashing lights
• Seeing jagged lines
• Difficulty in focusing

Sensory or motor changes:

• Numbness or tingling of the lips, face, or hands on one side of the body
• Weakness in arms or legs, usually on one side of the body

Speech or language changes:

• Inability to understand words
• Loss of speech or inability to speak normally

Question 8

Aura symptoms frequently associated with migraine

Answer 8

Scotoma Teichopsia (bright, wavy lines)

fortification (zigzag patterns)

Photopsia (flashing lights)

Visual and auditory hallucinations

Paresthesias

Metamorphopsia (distorted size of objects)

Question 9

MIGRAINE EPIDEMIOLOGY

Answer 9

It is true that nearly everyone gets an occasional tension type headache, but only a minority of patients with this type of headache seek medical attention for the headache

Migraine is the MOST COMMON HEADACHE THAT IS SEEN IN MEDICAL PRACTICE, since most migraine patients DO seek medical attention

About 15% of adult women and 6% of adult men have migraines in the United States, and 5 % of children

Migraines are more common in whites, less common in Africans, and least common in Asians

Question 10

Professor Harold Wolff

Answer 10

After medical school and a neurology residency in the United States, Wolff dedicated his entire academic and clinical career to proving that there was indeed a pathology of migraine

He had migraine headaches himself

Determined to prove there must be a true pathological basis of migraines

Question 11

Wolff: Where does headache originate?

Answer 11

1. Not from the cerebral hemispheres; these neurons are completely insensitive, even to fire

Pain may come from:

1. Arteries (Carotids, Middle Meningeal), and veins, including sinuses
2. Meningeal distension or irritation of all 3 layers
3. Periosteum of the skull, and inside the sinuses
4. Cranial nerves V, IX

. Other scientists discovered: gray matter of pons is sensitive to pain

Question 12

Wolff’s pathology of migraine

Answer 12

After decades of extremely invasive research on animals and many human volunteers, Wolff concluded:

1. The aura of migraine results from a REDUCTION of blood flow to the occipital cortex in a visual aura, and the frontal or parietal cortices with other auras
2. The actual pain of migraine results from an INCREASE in blood to the brain
3. Wolff also discovered some kind of small molecule is secreted into the blood at the start of the headache

Question 13

Migraine pathology

Answer 13

Released peptides can cause:

1. Inflammation of the meninges and blood vessels, with cytokines and other inflammatory molecules
2. Some vasodilation

Question 14

Could Harold Wolff be wrong in his theory?

Answer 14

Yes, it looks like Wolff went too far! His ability to study decreases or increases in blood flow at that time was greatly limited by current technology

During a migraine aura, there is only a very slight reduction in blood flow

During the painful period of a migraine, the increased blood flow starts AFTER the pain begins

Question 15

Blood flow during the aura

Answer 15

The reduction in blood flow to the cerebral cortex during an aura is a delayed, secondary event, due to these neurons lowering their electrical and metabolic activity

Essentially, there is a slow reduction in blood flow after the aura starts, which is a result of, not the cause of the aura

The actual reduction in perfusion is small

Question 16

The neurogenic theory of migraine pathology,

Answer 16

as for the actual PAIN, of migraine, PET studies repeatedly show that regions of the pons, in the brainstem, become very active electrically up to 30 minutes BEFORE there is an increase in blood flow to the brain

At the beginning of the pain phase of migraine, the pons sends this increased frequency of depolarization and synaptic transmission to one or more adjacent trigeminal nerves, cranial nerve V

Question 17

Migraine research disputes the vascular theory of migraine

Answer 17

THE NEURONAL THEORY OF MIGRAINE

Beginning in the 1980s, new technologies such as functional MRI and PET scans showed that there is only a trivial reduction in blood flow to the cerebral cortex during the AURA, and THIS CHANGE IN PERFUSION OCCURS AFTER a reduction of neuronal electrical activity This reduction in neuronal activity is probably the CORTICAL SPEADING DEPOLARIZATION that Leao had discovered many years before in animals with epilepsy

Question 18

MIGRAINE PATHOLOGY

Answer 18

1. This activation of the pons leads to increased activity in the trigeminal nerve which originates in the pons
2. TRIGEMINOVASCULAR ACTIVATION is the name for this theory, as the cause of the actual pain of migraine
3. The trigeminal nerve is both motor and sensory, with innervation of the face, arteries and veins, and meninges
4. The trigeminal nerve releases multiple transmitters, including serotonin, Calcitonin Gene Related Peptide (CGRP), Substance P, and nitric oxide

Question 19

Serotonin (5HT) and migraine

Answer 19

When large amounts of 5HT are released eventually its reuptake stops further release of this transmitter. Perhaps this is what ends the migraine attack.

Question 20

Serotonin

Answer 20

As the migraine pain worsens, patients not only release serotonin from the pons and the trigeminal nerve, but from their own platelets

The enormous concentrations of platelet serotonin may go to these neurons and the meninges and blood vessels they innervate, and also help in preventing more transmitter release

Question 21

5HT-1f RECEPTOR AGONISTS and migraine

Answer 21

Sumatriptan, and other triptans, are selective 5HT-1b and 5HT-1d and sometimes 5HT-1f RECEPTOR AGONISTS

They are very effective in ending the pain of migraine, but not the aura

Ironically, it was originally thought that serotonin agonists would stop migraine by reversing vasodilation

Question 22

Migraine: Clinical Features

Answer 22

Nauseating “sick” headaches which are accompanied by light and often sound sensitivity, and are worse with activity

Build-up in intensity over @30 to 60 minutes

On average last one day, with a general range of 4 hours to 72 hours

About 15 – 20% of patients get an AURA of visual or sensory or motor deficits, for 20 or 30 minutes before the pain begins

Question 23

Migraine Diagnostic Criteria

Answer 23

Migraine Criteria

>/= 5 attacks lasting 4–72 hours (30 minutes–7 days)

>/= 2 of the following

— Unilateral (bilateral)

— Pulsating (not pulsating)

— Moderate or severe intensity (mild or moderate) Aggravation by routine physical activity (not)

>/= 1 of the following

— Nausea and/or vomiting (no nausea/vomiting)

— Photophobia and phonophobia (one or neither)

No evidence on history or examination of disease that might cause headaches

Question 24

migraine genetics

Answer 24

There must be a genetic component, since over 80% of patients have a close family member with migraines

Transmission is often mother to daughter

Genes have been found for Familial Hemiplegic Migraine, in which patients may have one sided weakness for days, along with the headache

Question 25

Triggers of migraines

Answer 25

Environmental factors, often called “TRIGGERS,” are also important, since some patients get migraines only after head trauma Many of the migraine patients have learned that certain foods can cause migraines, such as cheese, chocolate, diet drinks, red wine Hormones matter, since about 75% of patients are women, and migraine often starts just after puberty, can end with menopause, and is very common during menstrual periods

Question 26

More triggers and aggravating factors for migraines

Answer 26

Fasting- skipping meals/ eating specific foods/ caffeine intake medication- analgesic overuse Circadian Rhythms- changes in sleep/wake cycles Environment- weather, - lighting, - fragrances/ odors Hormones- PMS, oral contraceptives, pregnancy, menopause, menses Stress/ Overexertion

Question 27

Is migraine psychosomatic or pathological?

Answer 27

No pathology has ever been found in centuries of autopsies done on migraine patients Migraine patients have much higher rates of psychiatric diseases than other people: 1. Depression and bipolar disorder 2. Anxiety disorders 3. Personality disorders, especially borderline and narcissistic

Question 28

Migraine center

Answer 28

dorsal raphe nucleus Locus coeruleus

Question 29

Tension Headache Clinical Features

Answer 29

Dull, bilateral, squeezing, “tight,” nonpulsating pain Routine physical activity does not aggravate pain No vomiting and no more than one of –Nausea –Photophobia –Phonophobia Moderate or severe pain is less common Musculoskeletal component, cervicogenic ŸMedication is seldom necessary for occasional tension type headaches

Question 30

Tension type headaches

Answer 30

If these headaches are infrequent, and not too severe, few patients seek the help of their physicians However, some patients may minimize their symptoms when they do describe their headaches, and they actually have migraines Occasional use of over the counter analgesics is probably safe for tension type headaches

Question 31

Chronic Tension-type Headaches

Answer 31

Average frequency \> 15 days/month, with average duration \> 4 hours/day if untreated and a history of \> 6 months History of less frequent headaches, when younger, is common Gradual increase (evolution) over \> 3 months First of all, these patients must not take ANY analgesics more than once a week

Question 32

Chronic tension type headache co-morbidities

Answer 32

1. Co-morbidities…hypertension, depression, anxiety, insomnia, diabetes/hypoglycemia, other sources of pain, including the neck at levels C2 and C3 2. Is there analgesic abuse, or prescription drugs for pain?

Question 33

most common cause of daily headache

Answer 33

daily analgesics

Question 34

Medication Overuse Headache

Answer 34

This is an enormous problem, in patients with BOTH migraine headaches and also tension type headaches, AFFECTING PERHAPS 2% OF ALL AMERICANS Taking ANY PAIN PILL, either prescription or over the counter, from acetaminophen to narcotics, more than once per week causes INCREASED FREQUENCY OF HEADACHES TO EVERY DAY OR EVERY OTHER DAY Formerly called REBOUND HEADACHE The only true treatment is to limit or stop these drugs

Question 35

Case Scenario : ## Footnote 36-year-old man Has sudden, severe, stabbing pain behind his right eye, spreading to the right temple Headaches are accompanied by lacrimation and nasal congestion Pain 1 hour; attacks occur daily for several weeks, then stop for months at a time

Answer 35

cluster headache

Question 36

Cluster headache

Answer 36

The pain of a cluster headache is always in or close to one eye, the same eye, in every attack The pain does not build up, it is IMMEDIATELY INTENSE The pain may migraine a little toward the temple, but mostly stays around the orbit So intense they are sometimes called SUICIDE HEADACHES More common in men than women

Question 37

Cluster Headache Autonomic features

Answer 37

conjunctival injection lacrimation congestion rhinorrhea swelling miosis ptosis eyelid edema

Question 38

Cluster Headache- epidemiology

Answer 38

Less common than migraines or tension type headache, perhaps 0.1% of people them Brief, 15 min – 2 hour attacks, one-sided, in or around the eye; often 1 hour after falling asleep Occur daily and/or multiple times a day for weeks or months at a time (season) and then disappear in most patients for a year or more Intense pain which peaks rapidly, patients may walk about and even slam their heads into the wall Pathology unknown: hypothalamic, and parasympathetic influences are possible

Question 39

Some common secondary headaches

Answer 39

The pathology is not entirely known for these headaches or “facial pains,” but is thought to exist It is important to consider these especially in patients who have abnormal vision or sensations, or systemic signs such as weight loss or fatigue

Question 40

IDIOPATHIC INTRACRANIAL HYPERTENSION

Answer 40

ALSO CALLED: PSEUDOTUMOR CEREBRI 1. Progressive diffuse headaches with intermittent loss of vision in one or both eyes, especially with eye movements 2. Almost all patients are OBESE YOUNG WOMEN, rarely found in men; some association with estrogen and possibly progesterone supplements, Acutane for acne 3. Increased intracranial pressure: due to overproduction of CSF? Brain swelling?

Question 41

IDIOPATHIC INTRACANIAL HYPERTENSION- signs

Answer 41

Nearly all have papilledema, sen with the ophthalmoscope Gradual, sometimes irreversible loss of visual acuity if not diagnosed early; patients may complain much more of a visual loss than headache when seeking medical help, so ophthalmologists often make the diagnosis Often have an extraocular palsy Diagnosis is supported by a significantly elevated opening pressure during lumbar puncture (well above 25 cm of water)

Question 42

Idiopathic Intracranial Hypertension: treatment

Answer 42

The first or subsequent LPs may be therapeutic by removing some CSF Successfully treated in most patients with WEIGHT LOSS, carbonic anhydrase inhibitors (which help synthesize CSF) Severe cases may require surgery by an ophthalmologist (removal of the sheath around the optic nerves), or a shunt, removing cerebrospinal fluid continuously from the brain or the lumbar cistern

Question 43

Trigeminal neuralgia, tic douloureux

Answer 43

A very brief, shooting pain lasting only seconds, or usually less than two minutes Pain in one of the branches of CN V, usually maxillary or mandibular, rare in ophthalmic branch; many attacks per day, even in sleep Pain is often “triggered” by touching the face, eating, shaving, brushing the teeth, applying lipstick or makeup; seldom occurs during sleep Named by the French, as a repetitive spasm or “wince” of facial pain

Question 44

Trigeminal neuraliga- who gets it, what to do

Answer 44

Most patients are over the age of 60, slightly more common in women The majority of patients have no observable pathology, although there are numerous cases due to an enlarged artery or rarely, a tumor compressing the pons or the trigeminal nerve itself, or multiple sclerosis, a demyelinating disease Consider a brain MRI in patients, especially if they have other symptoms and signs

Question 45

Giant Cell Arteritis

Answer 45

Also known as Temporal arteritis An example of vasculitis, or a non-infectious inflammation of arteries, leading to gradual occlusion at some locations Involves the superficial temporal artery, a branch of the external carotid artery, on one and rarely both sides May spread to the adjacent internal carotid artery, reaching the ophthalmic artery and causing COMPLETE VISUAL LOSS via ischemia

Question 46

Giant Cell Arteritis- who gets it

Answer 46

Almost unheard of in patients under the age of 50, so a concern for elderly patients getting their first headaches Accompanied by fatigue, difficulty chewing, and pain in the neck and shoulders Probably part of the spectrum of Polymyalgia Rheumatica Diagnosis suggested by elevated Erythrocyte Sedimentation Rate (ESR) and C-Reactive Protein, and confirmed by superficial temporary artery biopsy

Question 47

Giant Cell Arteritis- diagnosis, what to do

Answer 47

Curable if the patient is given prednisone within the first weeks of the onset, typically 60mg per day or more, gradually and slowly decreased over many months It is crucial to diagnose this disease before there is any visual loss, which may be permanent If the diagnosis is strongly suggested, begin prednisone immediately, or right after confirmatory blood tests and, before a biopsy can be scheduled