63
Chronic Neurologic Problems
Cynthia Amerson
http://evolve.elsevier.com/Lewis/medsurg/
CONCEPTUAL FOCUS
Cognition Intracranial Regulation
Coping Mobility
Functional Ability Safety
Inflammation
LEARNING OUTCOMES
1. Compare the etiology, clinical manifestations, and Parkinson disease, myasthenia gravis, amyotrophic lateral
interprofessional and nursing management for types of sclerosis, and Huntington disease.
primary headaches. 4. Explain the potential impact of chronic neurologic disease
2. Describe the clinical manifestations, diagnostic studies, on well-being.
and interprofessional and nursing management of seizure 5. Outline the major goals of nursing care for the patient with
disorder and restless legs syndrome. a chronic, progressive neurologic disease.
3. Discern the clinical manifestations and nursing and
interprofessional management of multiple sclerosis,
KEY TERMS
absence seizure myasthenia gravis (MG)
amyotrophic lateral sclerosis (ALS) myasthenic crisis
aura Parkinson disease (PD)
cluster headache restless legs syndrome (RLS)
focal-onset seizures seizure
generalized-onset seizures seizure disorder
Huntington disease (HD) status epilepticus
migraine headache tension-type headache (TTH)
multiple sclerosis (MS) tonic-clonic seizure
This chapter discusses headaches, chronic neurologic disorders, these diseases have similar concerns and problems. They must
and degenerative neurologic disorders. Chronic neurologic deal with the disease and its impact on quality of life. Many
disorders include seizure disorder and restless legs syndrome patients have concerns about safety, mobility, self-care, and cop-
(RLS). Degenerative nerve diseases lead to nerve damage that ing. Patients and their caregivers often need psychosocial sup-
worsens as the diseases progress. These neurologic problems port, especially as the disease progresses and disability worsens.
have no cure. Treatment aims to reduce symptoms and help the
patient maintain optimal function. Many have a genetic basis.
Degenerative nerve diseases include multiple sclerosis (MS),
HEADACHES
Parkinson disease (PD), myasthenia gravis (MG), amyotrophic Headache is one of the most common types of pain. Most peo-
lateral sclerosis (ALS), and Huntington disease (HD). They ple have functional headaches, such as migraine or tension-type
affect many activities, including vision, balance, movement, headaches. Others have organic headaches caused by intracra-
speech, swallowing, and heart and lung function. Patients with nial or extracranial disease.
1538
, CHAPTER 63 Chronic Neurologic Problems 1539
Pain-sensitive structures in the head include venous sinuses, They are not caused by a disease or another medical problem.
dura, cranial blood vessels, and some nerves. There are 3 divi- Secondary headaches are caused by another disease or problem,
sions of the trigeminal nerve (cranial nerve [CN] V), facial such as sinus infection, neck injury, or brain tumor.
nerve (CN VII), glossopharyngeal nerve (CN IX), vagus nerve A patient may have more than 1 type of headache. The his-
(CN X), and the first 3 cervical nerves. Pain occurs when these tory and neurologic assessment are key to deciding the type of
structures experience pull, displacement, inflammation, or headache. The assessment is often normal. Unexplained abnor-
swelling. Because these nerves have both motor and sensory mal findings require further diagnostic studies.
functions, increased pain intensity and symptoms can occur
when a person moves.
We classify headaches as primary or secondary. This is based
TENSION-TYPE HEADACHE
on the International Headache Society (IHS) guidelines outlin- Tension-type headache (TTH), or stress headache, is the most
ing characteristics of headaches.1 Primary headaches include common type of headache. It is characterized by its bilateral
tension-type, migraine, and cluster headaches (Table 63.1). location and pressing or tightening quality. TTH is usually of
TABLE 63.1 Interprofessional Care
Comparison of Headaches
Tension-Type Headaches Migraine Headache Cluster Headache
Location
Bilateral, bandlike pressure at base of skull Unilateral in 60%, may switch sides; often anterior location Unilateral, radiating up or down from 1 eye
Quality
Constant, squeezing, tightness Throbbing, synchronous with pulse Severe, bone crushing
Frequency
Cycles for many years Periodic, cycles of several months and years May have months or years between attacks.
Attacks occur in clusters over a period of 2–12 weeks
Duration
30 min–7 days 4–72 hr 5 min–3 hr
Time and Mode of Onset
Not related to time May be preceded by prodromal symptoms or aura Nocturnal, often awakens person from sleep
Onset after awakening
Improves with sleep
Associated Symptoms
Palpable neck and shoulder muscle tension Aura: visual, sensory, aphasic Facial flushing or pallor
Stiff neck Irritability, sweating Unilateral lacrimation, ptosis, rhinitis
Tenderness Nausea, vomiting
Photophobia
Phonophobia
Prodromal symptoms: sensory, motor, or psychic phenomena
Treatment: Abortive and Symptomatic Drugs
Nonopioid analgesics: aspirin, acetaminophen, α-Adrenergic blockers α-Adrenergic blockers
NSAIDs • ergotamine Serotonin receptor agonists
Analgesic combinations • dihydroergotamine • almotriptan
• butalbital/acetaminophen/caffeine NSAIDs • eletriptan
• butalbital/aspirin/caffeine (Fiorinal) Serotonin receptor agonists • frovatriptan
Muscle relaxants • almotriptan • naratriptan
• eletriptan (Relpax) • rizatriptan
• frovatriptan (Frova) • sumatriptan
• naratriptan (Amerge) • zolmitriptan
• rizatriptan (Maxalt) High-flow 100% O2
• sumatriptan (Imitrex)
• zolmitriptan (Zomig)
Combination
• acetaminophen/caffeine/aspirin
• sumatriptan/naproxen (Treximet)
CGRP antagonists
• Lasmiditan (Reyvow)
Continued
,1540 SECTION 12 Problems Related to Movement and Coordination
TABLE 63.1 Interprofessional Care—cont’d
Comparison of Headaches
Tension-Type Headaches Migraine Headache Cluster Headache
Treatment: Preventive
Antiseizure drugs Antiseizure drugs α-Adrenergic blockers
• topiramate (Topamax) β-Adrenergic blockers • ergotamine tartrate
• divalproex (Depakote) Botulinum toxin A Antiseizure drugs
β-Adrenergic blockers CGRP antagonists • gabapentin
Botulinum toxin A Tricyclic antidepressants • topiramate
mirtazapine (Remeron) Corticosteroids
Muscle relaxation training CGRP antagonists
Selective serotonin reuptake inhibitors Lithium
• fluoxetine (Prozac) verapamil
• paroxetine (Paxil)
Tricyclic antidepressants
• amitriptyline
• doxepin
• nortriptyline
TABLE 63.2 Interprofessional Care
Headaches
Diagnostic Assessment
• History and physical assessment
• Neurologic assessment
• Inspect for local infection
• Palpate head for tenderness, bony swellings
A B C • Auscultate for bruits over major arteries, especially neck
Tension Migraine Cluster • Laboratory studies
Most Less Least • CBC
frequent site frequent site frequent site • Electrolytes
Fig. 63.1 Location of pain for common headache syndromes. (A) Ten- • Urinalysis
sion headache is often described as feeling of a weight in or on the • Diagnostic studies
head or a band squeezing the head. (B) Migraine headache is usually • Angiography
unilateral, in the temple on 1 side of the head. The pain can be bilateral. • CT scan, MRA, MRI
(C) Cluster headache pain is focused in and around 1 eye. • EEG
• LP
mild or moderate intensity. It can last from minutes to days.
TTHs are divided by frequency into episodic or chronic. Management
• Drug therapy (Table 63.1)
Chronic TTH occurs more than 15 days per month. It can lead
• Biofeedback
to decreased quality of life and severe disability.1
• Cognitive-behavioral therapy
• Relaxation therapy
Etiology and Pathophysiology • Sleep modification therapy
We do not know the cause of TTH. It may have a neurobio-
logic basis similar to migraine headaches. Triggers may include
stress, anxiety, or depression. They may develop after exposure vomiting. Activity does not make the pain worse. There are no
to caffeine or smoking. Many patients have tight cervical mus- prodromal symptoms (warning symptoms of impending head-
cles and general pain in the back, hip, and knees. For some, epi- ache). Many patients have a combination of migraine headache
sodic headaches evolve into chronic headaches. Headaches may and TTH, with features of both occurring together.
occur intermittently for weeks, months, or even years.
Diagnostic Studies
Clinical Manifestations The history may be the most useful tool for diagnosing TTH
Patients often have a bilateral frontal-occipital headache (Fig. (Table 63.2). If TTH is present during physical assessment, we
63.1). They describe the pain as a constant, dull pressure or may see increased resistance to passive movement of the head
bandlike. There may be increased cervical and neck muscle and head and neck tenderness. Electromyography (EMG) may
tone with neck pain. The patient may have sensitivity to light show sustained contraction of neck, scalp, or facial muscles.
(photophobia) or sound (phonophobia). There is no nausea or However, the patient may not have increased muscle tension
, CHAPTER 63 Chronic Neurologic Problems 1541
BOX 63.1 BIOLOGIC SEX CONSIDERATIONS aphasic. Visual symptoms, such as flashes of bright lights, blind
spots, distortions, or zigzag lines, are the most common type of
Headaches aura. Sensory auras may start as tingling or numbness in the fin-
Men gers or in the mouth, voices that do not exist, or unusual odors.
• Cluster headaches are 3 to 7 times more common. Aphasic aura presents as speech problems.7
• Have more exercise-induced headaches. A migraine may last 4 to 72 hours. Patients often describe
the pain as steady, pounding, or throbbing. It is synchronous
Women
with the pulse and worsens with movement. The pain is usually
• Migraine headaches are 3 times more common.
unilateral (Fig. 63.1). During the headache, patients may try to
• Have more tension-type headache.
avoid noise, light, odors, people, and stress.
with an EMG, even during a headache. Imaging is done when SAFETY ALERT
symptoms raise concern about a possible pathologic cause. • A severe headache accompanied by a stiff neck, vomiting, confusion, weak-
ness, double vision, a rash, or difficulty arousing requires emergent evalua-
tion.
MIGRAINE HEADACHE • These signs may indicate life-threatening illness.
Migraine headache is a recurring headache characterized by
unilateral throbbing pain. Migraines are most common between
the ages of 25 and 55. One in 4 US households have someone Diagnostic Studies
with migraines. Migraines account for up to 5 million emer- The diagnosis is usually based on the history. Neurologic and
gency room visits a year.2 The IHS divides migraines into 2 cate- other diagnostic examinations are often normal (Table 63.2).
gories: migraine without aura and migraine with aura. Migraine No specific laboratory or radiologic test can diagnose migraine
without aura is more common.1 headache. Neuroimaging techniques (e.g., head CT scan, MRI)
are not part of the routine evaluation unless the neurologic
Etiology and Pathophysiology assessment is abnormal. If atypical features are present, further
We do not know the exact cause of migraines. They likely result testing is done.
from complex interactions between trigeminal neuron, vascu-
lar, genetic, hormonal, and environment factors causing vasodi-
lation and inflammation in the brain.3 Current theory suggests
CLUSTER HEADACHE
a complex series of neurovascular events starts the headache. Cluster headache is the most severe form of primary headache.
People who have migraines have a state of neuron hyperexcit- It is classified as a trigeminal autonomic cephalalgia (TAC).
ability in the cerebral cortex, especially in the occipital cortex. Cluster headache effects the trigeminal nerve (CN V) and has
Genetics may play a role. 50% of people with migraine have associated autonomic symptoms (e.g., tears, stuffy nose). Age at
first-degree relatives with the disorder.3 onset is often 20 to 50 years, but they can start at any age. They
Migraines are related to many disorders. These include sei- more often affect persons who smoke and men (Box 63.1).8
zure disorder, ischemic stroke, asthma, depression, anxiety,
hypertension, and irritable bowel syndrome.4 In many cases, Etiology and Pathophysiology
migraines have no known precipitating events.5 Other risk fac- We do not know the cause nor how cluster headache occurs.
tors include age, female gender, obesity, low education level, and Imaging studies show activation of the hypothalamus and tri-
stressful life events (Box 63.1). geminal vascular complex at the onset.8 There may be a genetic
For some patients, specific factors may trigger a headache. influence. Many attacks occur at night, waking the person 1 to
Triggers include hormone fluctuations, environment factors hours after falling asleep. Alcohol, caffeine, and tyramine foods
like bright lights or loud noises, barometric pressure changes, can be diet triggers. Pain often starts within 1 hour of a drink.
skipping meals, odors, alcohol, depression, lack of sleep, and Strong odors (e.g., gasoline, paint fumes) or getting overheated
stress. Common food triggers include caffeine, monosodium are other triggers.9 Cluster headaches may occur at high alti-
glutamate, and high tyramine foods (red wine, aged cheese).5 tudes when less O2 is available in the atmosphere.
Clinical Manifestations Clinical Manifestations
Prodromal symptoms and an aura may precede the headache Patients have severe, intense pain lasting from 15 minutes to 3
phase by several hours or days. Prodromal symptoms occur hours. The pain is sharp and stabbing, described as excruciating.
before the onset of aura or headache. Common prodromal It is usually around the eye, radiating to the temple, forehead,
symptoms include a change in activity level, depression, fatigue, cheek, nose, teeth, or jaw along the CN V pathway (Fig. 63.1).
pallor, yawning, or difficulty concentrating.6 Aura occurs in Patients have characteristic autonomic features include swelling
about 25% of patients. An aura is a group of neurologic symp- around the eye, lacrimation (tearing), facial flushing or pallor,
toms that occur before a headache begins but may continue nasal congestion or running, and miosis (pupil constriction).
into the headache. The symptoms of an aura are completely During the headache, the patient may be agitated and restless,
reversible. There are 3 main types of auras: visual, sensory, and unable to sit still or relax.9
Chronic Neurologic Problems
Cynthia Amerson
http://evolve.elsevier.com/Lewis/medsurg/
CONCEPTUAL FOCUS
Cognition Intracranial Regulation
Coping Mobility
Functional Ability Safety
Inflammation
LEARNING OUTCOMES
1. Compare the etiology, clinical manifestations, and Parkinson disease, myasthenia gravis, amyotrophic lateral
interprofessional and nursing management for types of sclerosis, and Huntington disease.
primary headaches. 4. Explain the potential impact of chronic neurologic disease
2. Describe the clinical manifestations, diagnostic studies, on well-being.
and interprofessional and nursing management of seizure 5. Outline the major goals of nursing care for the patient with
disorder and restless legs syndrome. a chronic, progressive neurologic disease.
3. Discern the clinical manifestations and nursing and
interprofessional management of multiple sclerosis,
KEY TERMS
absence seizure myasthenia gravis (MG)
amyotrophic lateral sclerosis (ALS) myasthenic crisis
aura Parkinson disease (PD)
cluster headache restless legs syndrome (RLS)
focal-onset seizures seizure
generalized-onset seizures seizure disorder
Huntington disease (HD) status epilepticus
migraine headache tension-type headache (TTH)
multiple sclerosis (MS) tonic-clonic seizure
This chapter discusses headaches, chronic neurologic disorders, these diseases have similar concerns and problems. They must
and degenerative neurologic disorders. Chronic neurologic deal with the disease and its impact on quality of life. Many
disorders include seizure disorder and restless legs syndrome patients have concerns about safety, mobility, self-care, and cop-
(RLS). Degenerative nerve diseases lead to nerve damage that ing. Patients and their caregivers often need psychosocial sup-
worsens as the diseases progress. These neurologic problems port, especially as the disease progresses and disability worsens.
have no cure. Treatment aims to reduce symptoms and help the
patient maintain optimal function. Many have a genetic basis.
Degenerative nerve diseases include multiple sclerosis (MS),
HEADACHES
Parkinson disease (PD), myasthenia gravis (MG), amyotrophic Headache is one of the most common types of pain. Most peo-
lateral sclerosis (ALS), and Huntington disease (HD). They ple have functional headaches, such as migraine or tension-type
affect many activities, including vision, balance, movement, headaches. Others have organic headaches caused by intracra-
speech, swallowing, and heart and lung function. Patients with nial or extracranial disease.
1538
, CHAPTER 63 Chronic Neurologic Problems 1539
Pain-sensitive structures in the head include venous sinuses, They are not caused by a disease or another medical problem.
dura, cranial blood vessels, and some nerves. There are 3 divi- Secondary headaches are caused by another disease or problem,
sions of the trigeminal nerve (cranial nerve [CN] V), facial such as sinus infection, neck injury, or brain tumor.
nerve (CN VII), glossopharyngeal nerve (CN IX), vagus nerve A patient may have more than 1 type of headache. The his-
(CN X), and the first 3 cervical nerves. Pain occurs when these tory and neurologic assessment are key to deciding the type of
structures experience pull, displacement, inflammation, or headache. The assessment is often normal. Unexplained abnor-
swelling. Because these nerves have both motor and sensory mal findings require further diagnostic studies.
functions, increased pain intensity and symptoms can occur
when a person moves.
We classify headaches as primary or secondary. This is based
TENSION-TYPE HEADACHE
on the International Headache Society (IHS) guidelines outlin- Tension-type headache (TTH), or stress headache, is the most
ing characteristics of headaches.1 Primary headaches include common type of headache. It is characterized by its bilateral
tension-type, migraine, and cluster headaches (Table 63.1). location and pressing or tightening quality. TTH is usually of
TABLE 63.1 Interprofessional Care
Comparison of Headaches
Tension-Type Headaches Migraine Headache Cluster Headache
Location
Bilateral, bandlike pressure at base of skull Unilateral in 60%, may switch sides; often anterior location Unilateral, radiating up or down from 1 eye
Quality
Constant, squeezing, tightness Throbbing, synchronous with pulse Severe, bone crushing
Frequency
Cycles for many years Periodic, cycles of several months and years May have months or years between attacks.
Attacks occur in clusters over a period of 2–12 weeks
Duration
30 min–7 days 4–72 hr 5 min–3 hr
Time and Mode of Onset
Not related to time May be preceded by prodromal symptoms or aura Nocturnal, often awakens person from sleep
Onset after awakening
Improves with sleep
Associated Symptoms
Palpable neck and shoulder muscle tension Aura: visual, sensory, aphasic Facial flushing or pallor
Stiff neck Irritability, sweating Unilateral lacrimation, ptosis, rhinitis
Tenderness Nausea, vomiting
Photophobia
Phonophobia
Prodromal symptoms: sensory, motor, or psychic phenomena
Treatment: Abortive and Symptomatic Drugs
Nonopioid analgesics: aspirin, acetaminophen, α-Adrenergic blockers α-Adrenergic blockers
NSAIDs • ergotamine Serotonin receptor agonists
Analgesic combinations • dihydroergotamine • almotriptan
• butalbital/acetaminophen/caffeine NSAIDs • eletriptan
• butalbital/aspirin/caffeine (Fiorinal) Serotonin receptor agonists • frovatriptan
Muscle relaxants • almotriptan • naratriptan
• eletriptan (Relpax) • rizatriptan
• frovatriptan (Frova) • sumatriptan
• naratriptan (Amerge) • zolmitriptan
• rizatriptan (Maxalt) High-flow 100% O2
• sumatriptan (Imitrex)
• zolmitriptan (Zomig)
Combination
• acetaminophen/caffeine/aspirin
• sumatriptan/naproxen (Treximet)
CGRP antagonists
• Lasmiditan (Reyvow)
Continued
,1540 SECTION 12 Problems Related to Movement and Coordination
TABLE 63.1 Interprofessional Care—cont’d
Comparison of Headaches
Tension-Type Headaches Migraine Headache Cluster Headache
Treatment: Preventive
Antiseizure drugs Antiseizure drugs α-Adrenergic blockers
• topiramate (Topamax) β-Adrenergic blockers • ergotamine tartrate
• divalproex (Depakote) Botulinum toxin A Antiseizure drugs
β-Adrenergic blockers CGRP antagonists • gabapentin
Botulinum toxin A Tricyclic antidepressants • topiramate
mirtazapine (Remeron) Corticosteroids
Muscle relaxation training CGRP antagonists
Selective serotonin reuptake inhibitors Lithium
• fluoxetine (Prozac) verapamil
• paroxetine (Paxil)
Tricyclic antidepressants
• amitriptyline
• doxepin
• nortriptyline
TABLE 63.2 Interprofessional Care
Headaches
Diagnostic Assessment
• History and physical assessment
• Neurologic assessment
• Inspect for local infection
• Palpate head for tenderness, bony swellings
A B C • Auscultate for bruits over major arteries, especially neck
Tension Migraine Cluster • Laboratory studies
Most Less Least • CBC
frequent site frequent site frequent site • Electrolytes
Fig. 63.1 Location of pain for common headache syndromes. (A) Ten- • Urinalysis
sion headache is often described as feeling of a weight in or on the • Diagnostic studies
head or a band squeezing the head. (B) Migraine headache is usually • Angiography
unilateral, in the temple on 1 side of the head. The pain can be bilateral. • CT scan, MRA, MRI
(C) Cluster headache pain is focused in and around 1 eye. • EEG
• LP
mild or moderate intensity. It can last from minutes to days.
TTHs are divided by frequency into episodic or chronic. Management
• Drug therapy (Table 63.1)
Chronic TTH occurs more than 15 days per month. It can lead
• Biofeedback
to decreased quality of life and severe disability.1
• Cognitive-behavioral therapy
• Relaxation therapy
Etiology and Pathophysiology • Sleep modification therapy
We do not know the cause of TTH. It may have a neurobio-
logic basis similar to migraine headaches. Triggers may include
stress, anxiety, or depression. They may develop after exposure vomiting. Activity does not make the pain worse. There are no
to caffeine or smoking. Many patients have tight cervical mus- prodromal symptoms (warning symptoms of impending head-
cles and general pain in the back, hip, and knees. For some, epi- ache). Many patients have a combination of migraine headache
sodic headaches evolve into chronic headaches. Headaches may and TTH, with features of both occurring together.
occur intermittently for weeks, months, or even years.
Diagnostic Studies
Clinical Manifestations The history may be the most useful tool for diagnosing TTH
Patients often have a bilateral frontal-occipital headache (Fig. (Table 63.2). If TTH is present during physical assessment, we
63.1). They describe the pain as a constant, dull pressure or may see increased resistance to passive movement of the head
bandlike. There may be increased cervical and neck muscle and head and neck tenderness. Electromyography (EMG) may
tone with neck pain. The patient may have sensitivity to light show sustained contraction of neck, scalp, or facial muscles.
(photophobia) or sound (phonophobia). There is no nausea or However, the patient may not have increased muscle tension
, CHAPTER 63 Chronic Neurologic Problems 1541
BOX 63.1 BIOLOGIC SEX CONSIDERATIONS aphasic. Visual symptoms, such as flashes of bright lights, blind
spots, distortions, or zigzag lines, are the most common type of
Headaches aura. Sensory auras may start as tingling or numbness in the fin-
Men gers or in the mouth, voices that do not exist, or unusual odors.
• Cluster headaches are 3 to 7 times more common. Aphasic aura presents as speech problems.7
• Have more exercise-induced headaches. A migraine may last 4 to 72 hours. Patients often describe
the pain as steady, pounding, or throbbing. It is synchronous
Women
with the pulse and worsens with movement. The pain is usually
• Migraine headaches are 3 times more common.
unilateral (Fig. 63.1). During the headache, patients may try to
• Have more tension-type headache.
avoid noise, light, odors, people, and stress.
with an EMG, even during a headache. Imaging is done when SAFETY ALERT
symptoms raise concern about a possible pathologic cause. • A severe headache accompanied by a stiff neck, vomiting, confusion, weak-
ness, double vision, a rash, or difficulty arousing requires emergent evalua-
tion.
MIGRAINE HEADACHE • These signs may indicate life-threatening illness.
Migraine headache is a recurring headache characterized by
unilateral throbbing pain. Migraines are most common between
the ages of 25 and 55. One in 4 US households have someone Diagnostic Studies
with migraines. Migraines account for up to 5 million emer- The diagnosis is usually based on the history. Neurologic and
gency room visits a year.2 The IHS divides migraines into 2 cate- other diagnostic examinations are often normal (Table 63.2).
gories: migraine without aura and migraine with aura. Migraine No specific laboratory or radiologic test can diagnose migraine
without aura is more common.1 headache. Neuroimaging techniques (e.g., head CT scan, MRI)
are not part of the routine evaluation unless the neurologic
Etiology and Pathophysiology assessment is abnormal. If atypical features are present, further
We do not know the exact cause of migraines. They likely result testing is done.
from complex interactions between trigeminal neuron, vascu-
lar, genetic, hormonal, and environment factors causing vasodi-
lation and inflammation in the brain.3 Current theory suggests
CLUSTER HEADACHE
a complex series of neurovascular events starts the headache. Cluster headache is the most severe form of primary headache.
People who have migraines have a state of neuron hyperexcit- It is classified as a trigeminal autonomic cephalalgia (TAC).
ability in the cerebral cortex, especially in the occipital cortex. Cluster headache effects the trigeminal nerve (CN V) and has
Genetics may play a role. 50% of people with migraine have associated autonomic symptoms (e.g., tears, stuffy nose). Age at
first-degree relatives with the disorder.3 onset is often 20 to 50 years, but they can start at any age. They
Migraines are related to many disorders. These include sei- more often affect persons who smoke and men (Box 63.1).8
zure disorder, ischemic stroke, asthma, depression, anxiety,
hypertension, and irritable bowel syndrome.4 In many cases, Etiology and Pathophysiology
migraines have no known precipitating events.5 Other risk fac- We do not know the cause nor how cluster headache occurs.
tors include age, female gender, obesity, low education level, and Imaging studies show activation of the hypothalamus and tri-
stressful life events (Box 63.1). geminal vascular complex at the onset.8 There may be a genetic
For some patients, specific factors may trigger a headache. influence. Many attacks occur at night, waking the person 1 to
Triggers include hormone fluctuations, environment factors hours after falling asleep. Alcohol, caffeine, and tyramine foods
like bright lights or loud noises, barometric pressure changes, can be diet triggers. Pain often starts within 1 hour of a drink.
skipping meals, odors, alcohol, depression, lack of sleep, and Strong odors (e.g., gasoline, paint fumes) or getting overheated
stress. Common food triggers include caffeine, monosodium are other triggers.9 Cluster headaches may occur at high alti-
glutamate, and high tyramine foods (red wine, aged cheese).5 tudes when less O2 is available in the atmosphere.
Clinical Manifestations Clinical Manifestations
Prodromal symptoms and an aura may precede the headache Patients have severe, intense pain lasting from 15 minutes to 3
phase by several hours or days. Prodromal symptoms occur hours. The pain is sharp and stabbing, described as excruciating.
before the onset of aura or headache. Common prodromal It is usually around the eye, radiating to the temple, forehead,
symptoms include a change in activity level, depression, fatigue, cheek, nose, teeth, or jaw along the CN V pathway (Fig. 63.1).
pallor, yawning, or difficulty concentrating.6 Aura occurs in Patients have characteristic autonomic features include swelling
about 25% of patients. An aura is a group of neurologic symp- around the eye, lacrimation (tearing), facial flushing or pallor,
toms that occur before a headache begins but may continue nasal congestion or running, and miosis (pupil constriction).
into the headache. The symptoms of an aura are completely During the headache, the patient may be agitated and restless,
reversible. There are 3 main types of auras: visual, sensory, and unable to sit still or relax.9
