Case Files Neurology - part 4 docx

Patients should have serial orthostatic blood pressuremeasurements to document a decrease in blood pressure or increase in heartrate with postural changes, which is associated with ortho

[15.3] A 35-year-old man is diagnosed with a seizure disorder There is nohistory of trauma or medical condition What is the most common type

of seizure in adults with epilepsy?

A Absence seizures

B Complex partial seizures

C Grand mal seizures

D Todd paralysis

Answers[15.1] A Lip smacking, chewing, and swallowing are common findings in

complex partial seizures

[15.2] B Absence seizures are typified by staring off episodes without

con-scious awareness

[15.3] B The most common type of seizure with epilepsy in adults is

com-plex partial seizures

CLINICAL PEARLS

❖ Complex partial seizures are the most common form of seizure in

adults

❖ The differential of complex partial seizure includes absence

seizures and also multiple medical disturbances, including sient ischemic attacks

tran-❖ In approximately one-third of women with seizures, there is a

rela-tionship between seizures and the menstrual cycle, and theseizure frequency can double This is often called catamenialseizure exacerbation or catamenial epilepsy

REFERENCES

Bazil CW, Morrell MJ, Pedley TA Epilepsy In: Rowland LP, ed Merritt’s ogy, 11th ed Philadelphia, PA: Lippincott Williams & Wilkins; 2005:990–1014 Murro AM Complex partial seizures Available at: http://www.emedicine.com/ NEURO/topic74.htm Accessed March 20, 2007.

neurol-Schacter SC Epilepsy In: Evans RW, ed Saunders manual of neurologic practice Philadelphia, PA: Saunders/Elsevier; 2003:244–265.

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❖ CASE 16

A 52-year-old healthy white male is brought to the emergency room (ER) after

he has had a car accident in which he hit the dividing rail He apparently didnot suffer any significant injuries and at the time of the examination was fullyawake On further questioning, he reported driving on the highway and thenwithout any warning hit the rail He immediately stopped the car His wife,who was in the car with him, stated that he suddenly stopped responding in themiddle of the sentence, and the car started to go to the left When they hit therail, he woke up and braked the car He denies feeling lightheaded, nausea, orwarning prior to the loss of consciousness He also denied feeling ill or dis-oriented on awakening, and he was immediately aware of his surroundings.There was no evidence of tongue biting or urinary incontinence, or convulsivejerking In the ER, the patient had an unremarkable examination, laboratorywork, and CT scan of his head He was admitted for 24-hour observation, and

a neurologist was consulted The patient admitted to two previous syncopalepisodes, both in his office, and both without provocation On one occasion hewas seated, on the second occasion he was standing and suffered a fall In nei-ther case did he have any warning or postevent confusion After the secondepisode he scheduled an appointment with his family doctor but did not havethe chance to see him prior to the accident On review of systems, the patientcomplained of frequent fatigue and lack of energy over the last year but attrib-uted it to work schedule and lack of adequate exercise His detailed neurologicexamination showed no abnormal findings

◆ What is the most likely diagnosis?

◆ What is the next diagnostic step?

◆ What is the next step in therapy?

ANSWERS TO CASE 16: Cardiogenic Syncope Related

to Bradycardia

Summary: This 64-year-old man presents with an acute and temporary loss of

consciousness and history of two similar episodes in the past These episodeswere not associated with warning signs or symptoms nor followed by persist-ent confusion, weakness, or findings on examination

◆ Most likely diagnosis: Cardiogenic syncope related to bradycardia

◆ Next diagnostic step: Cardiac evaluation and invasive

electrophysiology

◆ Next step in therapy: Pacemaker placement

Analysis Objectives

1 Know common causes of acute loss of consciousness or syncope

2 Describe the workup for syncope

3 Be familiar with the management of syncope

Clinical Considerations

In this case, the patient suffered an acute loss of consciousness that was out any provocation or premonitory symptoms including nausea, sweating, orabdominal discomfort He did not become pale or ashen according to his wife.The event occurred while he was sitting in his car, and he regained conscious-ness quickly These findings are less consistent with a vasovagal or orthostaticsyncope because it was not associated with a change in position from sitting

with-or lying down to standing with-or upright and was not associated with signs andsymptoms suggestive of low blood pressure

His wife denied any convulsions or postictal confusion, and the patientdenied any premonitory symptoms On examination there was no evidence oftongue biting or urinary incontinence, making a good case against an epilep-tic seizure Therefore the most likely diagnosis in this patient is cardiogenicsyncope An evaluation should be performed including a tilt-table testing Thepatient should also have an MRI of the brain with and without contrast andelectroencephalograph (EEG) Routine laboratory tests should be undertaken

to assess for metabolic or endocrine problems; complete blood count (CBC)for evidence of anemia or infection An electrocardiograph (ECG) and 24-hourHolter monitoring are usually obtained After an evaluation and follow-up, thepatient may have repeated bouts of syncope, which requires more extensiveevaluation and therapy This particular patient experienced repeated syncopalepisodes There was a negative workup, an invasive electrophysiological study

was ordered, and the patient was diagnosed with “sick sinus syndrome.” Thetreatment was an implanted dual chamber pacemaker, and the patient was dis-charged home with resolution of syncope and fatigue.

APPROACH TO CARDIOGENIC SYNCOPE

Definitions

Syncope: A sudden brief loss of consciousness (LOC).

Orthostatic syncope: Syncope associated with a sudden change in position

from supine to sitting up or sitting to standing up

Electroencephalography: The neurophysiologic measurement of the

elec-trical activity of the brain by recording from electrodes placed on thescalp or, in special cases, subdurally or in the cerebral cortex

Epilepsy: Neurologic condition that makes people susceptible to seizures.

A seizure is a change in sensation, awareness, or behavior brought about

by a brief electrical disturbance in the brain

Tilt-table testing: Test to evaluate how the body regulates blood pressure

in response to some very simple stresses while lying on a special table

It involves cardiac monitoring (ECG), blood pressure monitoring, andintravenous (IV) infusion of drugs to stress the system

Sick sinus syndrome: A type of bradycardia in which the sinoatrial (SA)

or sinus node is not working as it should.

Clinical Approach

Syncope can result from a variety of cardiovascular and noncardiovascularcauses The most common pathophysiologic mechanism for cardiovascularsyncope is decreased cerebral blood flow with resultant cerebral hypoxia,which prompts immediate and forceful rearrangement of posture to ensure anadequate flow of the blood to the central nervous system (CNS) Decreasedcerebral blood flow is most commonly caused by decreased cardiac output(CO) and arrhythmias Heart rate below 35 and above 150 beats/min can causesyncope even without the presence of cardiovascular disease Although brady-cardia can occur at any age, it occurs most frequently in the elderly and is usu-ally caused by ischemia or fibrosis of the conduction system Digitalis,beta-blockers, and calcium channel blockers can also cause bradycardia.However, supraventricular or ventricular tachyarrhythmias that cause syncopecan be related to cardiac ischemia or electrolyte abnormalities

Among the most common non–cardiac-related mechanisms of syncopeare peripheral vasodilation, decreased venous return to the heart, andhypovolemia

History is critical in making the correct diagnosis in the case of syncope Itshould guide the evaluation and not the other way around Syncope of cardiacetiology occurs suddenly and ends abruptly without warning or post-event

confusion The postural changes are often not necessary for the termination ofthe event This presentation is the most common sequela of the arrhythmia andrequires careful electrophysiological study as well as cardiac catheterization torule out ischemia as the cause of the conduction defect.

Exertional syncope suggests cardiac outflow obstruction, mainly caused byaortic stenosis, and therefore warrants echocardiogram as the first step in eval-uation Cough or micturition syncope as well as syncope occurring during anynatural or iatrogenic Valsalva maneuver, implicates decrease in venous returnand can be present even in healthy individuals

Vasovagal syncope is not a serious or life-threatening condition but is an

abnormal reflex This results in a drop in blood pressure leading to decreasedblood flow to the brain resulting in dizziness or fainting The mechanism ofvasovagal syncope is the subject of a great deal of research It is typically pre-cipitated by unpleasant physical or emotional syncope most commonly pain,sight of blood or gastrointestinal discomfort It usually occurs in the uprightposition, and the patient describes a sensation of lightheadedness, dimmedvision and hearing, depersonalization, sweating, nausea, and increased heartrate The patient usually wakes up immediately after the event but if preventedfrom obtaining a supine position, usually by well-wishing observers, syncope

can be prolonged and accompanied by brief convulsions (so-called convulsive syncope) This almost always precipitates neurologic consult for the new onset

seizures

The picture is often complicated by the spontaneous micturition, which is widely believed to be a sign of epileptic activity Contrary to popular belief,

incontinence can be the result of any syncopal episode if the patient happens

to have a full bladder prior to the event Most often, if clearly elucidated, apure vasovagal episode in the patient without any risk factors for cardiovascu-lar disease and a normal post-event physical examination does not require anyfurther evaluation

Syncope caused by epileptic seizure is abrupt in onset and most of the timeassociated with focal or generalized tonic or clonic muscular activity, clearly

described by the witnesses Tongue biting and urinary incontinence are common but by no means required for the diagnosis Most of the time the

patient experiences at least brief postictal confusion, making it the single mostimportant sign for the differentiation from other causes of syncope

In patients with known epilepsy, defined as recurrent seizures, betweenwhich there is complete recovery, evaluation should be centered over the

antiepileptic medications Blood levels should be checked for the current

medications and if low, the cause or causes need to be elucidated The mostcommon causes are noncompliance or introduction of the new medication thatinterferes with the absorption or metabolism of the current antiepileptic drug

or drugs Frequently, however, recurrent seizures happen despite adequateblood level of antiepileptic medication It can be a result of concurrent acuteillness, behavioral changes (staying up all night, skipping meals, or drinkingalcohol) or simply inadequate seizure control

Orthostatic syncope has different etiology in elderly (e.g., over 50 years)

and young patients When occurring in the young it is most often confusedwith epilepsy because of age and absence of cardiovascular risk factors.Orthostatic syncope almost always happens with the sudden change of posturefrom lying or sitting to standing or after prolonged standing without moving.The classic example is a young soldier fainting during the military parade on

a hot summer afternoon When this happens in young and otherwise healthyindividuals, it always requires a table-tilt test, because sequential measure-ments of orthostatic blood pressure changes in the clinic may not be enough

In the elderly, however, orthostatic syncope is often caused by olemia, or increased venous pooling as seen after prolonged bed rest Theother contributing factor in this population is polypharmacy that often includescombination of beta-blocker, loop diuretic, and nitrate; combining dehydra-tion, vasodilatation and delayed cardiac response to cause sudden orthostaticchanges in blood pressure without adequate compensatory response

hypov-The other possibility for the orthostatic hypotension leading to the syncope isautonomic nervous system abnormality By far the most common cause of dysau-tonomia is diabetic neuropathy, where interruption of the sympathetic reflex arcinhibits adequate adrenergic response to standing The other less frequent causes

of autonomic failure are amyloidosis, syphilis, spinal cord injury or syringomyelia,alcoholic neuropathy, or acute inflammatory demyelinating polyradiculoneuropa-thy (AIDP) also known as Guillain-Barré syndrome, which can all affect theperipheral or central autonomic pathways Orthostatic hypotension is also one ofthe cardinal features of multiple system atrophy, an atypical Parkinsonian syn-drome, which consists of a variable combination of parkinsonism, cerebellar dys-function, dysautonomia, and pyramidal symptoms However, orthostatichypotension can be present later and often to a milder degree in idiopathicParkinson disease, and often aggravated by the use of dopaminergic agents

Evaluation

Patients suspected of cardiogenic syncope or any nonepileptic syncope shouldundergo an extensive evaluation including 12-lead ECG, two-dimensional-echocardiography, 24-hour Holter monitoring for arrhythmias, and possiblycardiac catheterization Patients should have serial orthostatic blood pressuremeasurements to document a decrease in blood pressure or increase in heartrate with postural changes, which is associated with orthostatic syncope

Discussion

Bradycardias are caused by two problems: disease of the sinus node or disease

of the conduction system The sinus node is the pacemaker of the heart The

electrical impulse that generates the heart beat arises in the sinus node Disease

of the sinus node, therefore, can result in the lack of sufficient electricalimpulses (and thus a lack of sufficient heart beats) to maintain the body’s

needs Sinus node disease that leads to symptoms caused by a slow heart rate

is called sick sinus syndrome Most sinus node disease is related to simple

deterioration in sinus node function caused by aging Likewise, mias caused by Wolff-Parkinson-White syndrome or prolonged-QT syndromecan also lead to insufficient cardiac output and syncope

tachyarrhyth-Treatment

In the case of syncope, diagnosis is the most difficult part The treatment isonly as effective as the diagnosis is correct In the case of vasovagal syncope,treatment often is not required

Orthostatic hypotension can be treated by avoiding hypovolemia, electrolyteimbalance, and excess alcohol intake If this is not enough increased salt intakeand fludrocortisone can be recommended If orthostasis is related to venous pool-ing in the legs, fitted elastic hose can enhance the venous return and cardiac output.Obviously, if seizures were found to be the cause of the syncope, they have to betreated with appropriate antiepileptic medications, and the patient needs to bereferred to as epileptologist for further evaluation Tachyarrhythmias are treatedwith variety of antiarrhythmic drugs, the discussion of which is beyond this case.Sick sinus syndrome, if symptomatic, is often treated with permanent pacing, toavoid an onset of fatal arrhythmia or sinus arrest

Answers[16.1] B This is most likely caused by a vasovagal reflex (drop in blood pres-

sure) in response to painful or emotionally charged stimulus and isusually non–life threatening

[16.2] B Exertional syncope is caused by insufficient cardiac output

neces-sary to meet exertional demands This is usually caused by cardiac flow obstruction often associated with aortic or subaortic stenosis andrequires an echocardiogram

out-[16.3] C Seizure is the most likely diagnosis given a prior history of brain

trauma, which can predispose to a seizure focus Although clinicalsigns such as incontinence and tongue laceration are not specific forseizure, in the context of a possible cerebral focus, seizure is the mostappropriate answer

CLINICAL PEARLS

❖ It is prudent to ask for clarification when the patient complains of

“dizziness.”

❖ Vertigo and lightheadedness should be differentiated because their

evaluations are very different

❖ Lightheadedness often includes dimmed vision, nausea,

palpita-tions, and diaphoresis before syncope

❖ Tongue biting and urinary incontinence is not pathognomonic for

seizure activity, nor does the absence of those signs excludeepileptic seizures

patho-Linzer M, Yang EH, Estes NA 3rd, et al Diagnosing syncope Part 2: unexplained syncope Clinical Efficacy Assessment Project of the American College of Physicians Ann Intern Med 1997 Jul 1;127(1):76–86.

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❖ CASE 17

A 23-year-old graduate student was studying late at night for an examination,telling his friends that he was not at all worried about his forthcoming exami-nations Suddenly, he stood from his chair, stared at the wall, and fell to thefloor, his arms and legs uncontrollably shaking He complained during thisepisode that he was hurting He then began mumbling incoherently He wasnever incontinent of urine or stool and did not bite his tongue His friends wereable to escort him to the bed, following which he stared vacuously, continuallyasking where he was and who they were His friends took him to the emer-gency room

◆ What is the most likely diagnosis?

◆ What is the next diagnostic step?

◆ What is the most likely useful consultation?

ANSWERS TO CASE 17: Pseudoseizure

Summary: A 23-year-old man suddenly “seized” in all four extremities, remained

conscious, complaining about pain and querying his surroundings

◆ Most likely diagnosis: Pseudoseizure.

◆ Next diagnostic step: See a physician Have a careful neurologic

assessment and psychiatric assessment as well Blood studies, brainimaging, electroencephalograph (EEG), and EEG monitoring may need

to be obtained

◆ Consultation: Initiate interaction with psychiatrist and recognize that

patients with pseudoseizures may also have associated bona fide seizures

Analysis Objectives

1 Know diagnostic approach to pseudoseizures

2 Understand that pseudoseizures reflect psychodynamic issues and can

be associated with bona fide organic seizures

Considerations

Pseudoseizures are one of the most misunderstood areas in neurology A goodexample that helps clarify this situation is asthma Patients with asthma canbecome upset and develop an asthmatic attack Or, they can make themselves agi-tated and bring on an asthmatic attack And, they can feign asthmatic symptoms.Similarly, patients, with epilepsy can have emotional angst that prompts a seizure,they can feign a seizure, and they can also have psychiatric disturbance in whichthey are not “pretending” to seize but have characteristics of seizure that are non-physiologic The patient in this case is having four-extremity motor activity yet iswide awake and aware of his surroundings This level of consciousness is incon-sistent with bilateral cerebral hemisphere epileptic activity

APPROACH TO PSEUDOSEIZURES

Definitions

Pseudoseizures: An attack resembling an epileptic seizure but having

purely psychological causes; it lacks the electroencephalographic teristics of epilepsy and the patient may be able to stop it by an act of will

charac-Malingering: Intentional production of false or exaggerated symptoms

motivated by external incentives, such as obtaining compensation ordrugs, avoiding work or military duty, or evading criminal prosecution.Malingering is not considered a mental illness

Clinical Approach Etiologies and Clinical Presentation

Hysterical attacks have been described by Briquet (1887), Charcot (1887–1889),and Breuer and Freud (1895) There are multiple theories, spawning the advent

of psychiatry, and recently intertwined with numerous overlapping neurologictheories Originally termed hysterical seizures, these attacks are associatedwith loss of impulse, especially in stressful situations, and were formerly (anderroneously) defined as only involving women Current knowledge reveals thatthese “spells” are more common in young adults and in female adolescents.The attacks often consist of storms of movements that are difficult to defineprecisely Patients may arch their backs, engage in bizarre movements, and can

have pelvic movements as well Rotatory head movements, kicking, and

bicycling movements can also occur Patients can experience episodes of loss

of consciousness, twitching or jerking, and unusual emotional states, such asintense feelings of fear or déjà vu The episodes can last 20 minutes, but are

not associated with electrical abnormalities in the brain as is the case with

epileptic seizures Most investigators agree that falls during these psychogenicattacks never physically traumatize the patient Tongue biting and urinaryincontinence can occur but are not common

Pseudoseizure has been equated with psychogenic seizures In some series,approximately 1–2% of patients with pseudoseizures also had bona fideorganic seizures Pseudoseizures should not be confused with malingering,which can be very difficult to separate The boundaries between conscious andsubconscious behavior in psychogenic seizures can be difficult to detect Truemalingerers can prove quite resourceful in pretending to have focal abnormal-ities on examination, even producing voluntary Babinski signs Even fortrained medical professionals, the differences between epileptic seizures andpseudoseizures are difficult to recognize Physicians believe pseudoseizuresare psychologic defense mechanisms induced by stress or episodes of severeemotional trauma The seizures happen when patients try to avoid or forget thetrauma It is not at all uncommon that patients referred to an epilepsy center,after monitoring, turn out to have pseudoseizures The diagnosis of nonepilepticseizures has become more prevalent with a better understanding of the psycho-logic issues related to these events and correlation of these changes with normalbrain activity It is important for the physician to recognize that these patients areoften calling for help, and it is inappropriate to view them as “crocks” or feelanger toward them, thinking that they might be trying to “fake out” the doctor.Certainly, a thorough history and neurologic examination are important, asare EEG recordings and brain imaging In difficult cases, ongoing EEG moni-toring might be necessary, to assess brain physiological function and process-

ing during the episode Psychiatric assessment is also very important A good rule is that bilateral seizure activity without confusion or unconscious (i.e.,

able to talk coherently to the examiner while their arms and legs are shaking),

is rarely organic This is because bilateral seizure activity in the brain is

usu-ally associated with altered consciousness because both hemispheres in thebrain are compromised

Pseudoseizures should be queried when a patient with seizures has a normalneurologic evaluation/assessment (often including normal EEG monitoringduring the seizure), and the seizures are not only refractory to treatment but arealso influencing family members and impacting the patient’s life (this can alsooccur in organic seizures) in areas with psychodynamic meaning/ importance

Treatment

The earlier in the syndrome the patient is diagnosed, the better the chances forcomplete recovery, however, diagnosing and treating this disorder is not easy.The diagnosis requires an inpatient admission where the patient is continu-ously monitored by both an EEG and video camera Both the EEG readingsand tapes are scanned by medical professionals On diagnosis, patients receive

an outline for treatment This plan includes a discussion of the illness with thepatient, termination of anticonvulsant medicines, which are sedating andworsen the problem, and counseling services Many patients are also treatedfor depression or anxiety A multidisciplinary approach is the best manage-ment for patients to help resolve both the old and new stresses in their lives,and a significant portion of the patient’s symptoms can be eliminated

Comprehension Questions

[17.1] A 35-year-old man is suspected to have pseudoseizure Which of thefollowing is the best method to confirm the diagnosis?

A Resting EEG monitoring

B Initiation of anti-epileptic therapy and observation

C Psychiatric evaluation

D Video EEG monitoring

[17.2] A 23-year-old man is noted to have tonic-clonic activity while yellingand screaming for a fire extinguisher Which of the following is themost likely etiology?

A Malingering

B Psychological defense mechanism to a significant traumatic event

C Hypertensive encephalopathy

D Complex partial seizure

[17.3] Which of the following is the best evidence of pseudoseizure?

A Cocaine found on urine drug screen

B Oxygen saturation level of 80%

C Alert with generalized (bilateral) convulsions

D History of diabetes mellitus

Answers[17.1] D Up to 1–3% of patients with pseudoseizures have true, organic

epilepsy This is why patients often require invasive and/or noninvasivevideo EEG monitoring to determine whether true epileptic events arepresent For the vast majority of patients with pseudoseizures, termi-nation of anti-epileptic therapy is recommended

[17.2] B Pseudoseizure, like many psychoneurological syndromes, has

psy-chological origin and is often associated with a past history of a nificant emotional or physical traumatic life event(s)

sig-[17.3] C Generalized convulsions or bilateral convulsions are typically

asso-ciated with loss of consciousness or significantly impaired alteration ofconsciousness, which can last several minutes after the ictal event

CLINICAL PEARLS

❖ Pseudoseizures should be considered when patients bilaterally

“seize” but maintain normal consciousness

❖ Sexual abuse and head injury are important risk factors of

pseudo-seizures, reported in approximately one-third of patients

❖ Asthma has been reported in 26.5% of pseudoseizure patients

❖ Pseudoseizures can coexist with organic seizures in up to 3%

of patients

❖ Pseudoneurologic syndromes mimic almost any neurologic disease

Presenting syndromes can include pseudoparalysis, pseudosensorysyndromes, pseudoseizures, pseudocoma, psychogenic movementdisorders, and pseudoneuro-ophthalmologic syndromes

REFERENCES

Bazil CW, Morrell MJ, Pedley TA Epilepsy In: Rowland LP, ed Merritt’s ogy, 11th ed Philadelphia, PA: Lippincott Williams & Wilkins; 2005:990–1014 Neidermeyer E Nonepileptic attacks In: Niedermeyer E, Lopes da Silva F, eds Electroencephalography: basic principles, clinical applications, and related fields, 5th ed Philadelphia: Lippincott Williams & Wilkins; 2005:621–630.

neurol-de Wet CJ, Mellers JD, Gardner WN, Toone BK Pseudoseizures and asthma.

J Neurol Neurosurg Psychiatry 2003 May;74(5):639–641

University of Michigan Adult health advisor: seizures Available at: http://www med.umich.edu/11ibr/aha/aha_seizure_crs.htm Updated 2005.

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❖ CASE 18

A 24-year-old white female has a 12-year history of headaches Theseheadaches started in grade school, and the patient remembers missing schoolwith her headaches Typically, she gets one of these headaches one to twotimes per month The headache starts over the right eye, and the headache isusually preceded by flashing lights and zigzag lines Once the headacheoccurs, there is extreme nausea and vomiting, and the patient goes into a darkroom to minimize her head pain Generally, the headache lasts 4 to 6 hours,but the patient feels tired and listless for the next 24 hours The patient feelsthat the headache worsens with her menstrual cycle, and certain foods espe-cially red wine can exacerbate her headache Her general and neurologicexaminations are normal

◆ What is the most likely diagnosis?

◆ What is the next diagnostic step?

◆ What is the next step in therapy?

150 CASE FILES: NEUROLOGY

ANSWERS TO CASE 18: Migraine Headache

Summary: A 24-year-old white female has a 12-year history of monthly

headaches that started in grade school The headache starts over the right eyeand is preceded by flashing lights and zigzag lines Once the headache occurs,there is extreme nausea and vomiting, and the patient goes into a dark room tohelp with her head pain Generally, the headache lasts 4 to 6 hours, and wors-ens with menses and certain foods

◆ Most Likely Diagnosis: Migraine headaches

◆ Next Diagnostic Step: MRI of the head

◆ Next Step in Therapy: Consider use of medications such as one of the

triptans to help treat the headaches

Analysis Objectives

1 Know how to recognize a migraine headache and be able to distinguish

it from headaches of other etiologies

2 Understand what medications are available to treat migraineheadaches

3 Be familiar with a workup for headaches and also be aware of otherclinical disorders, which have headache as a prominent feature

Considerations

When evaluating a patient for headache, the clinical history is of criticalimportance The nature (type of pain and associated symptoms or triggers),severity, and duration of the headache is important in determining what type

of headache it is and how to manage it In this case, the patient has a prior tory of headaches characterized as episodic and associated with nausea andvomiting, sensitivity to light (photophobia) and sound (phonophobia) Herexaminations have been normal, and therefore, her clinical history is highlysuggestive of a vascular or migrainous headache

his-APPROACH TO MIGRAINE HEADACHES

Definitions

Migraine with aura: Formally referred to as classic migraine, in which

the migraine begins with visual, auditory, smell, or taste disturbances

5 to 30 minutes before pain onset

Migraine without aura: Formally referred to as common migraine, which

is not typically associated with an aura

Clinical Approach

The prevalence of migraine varies from 0.5–2% in the adult population Thesex distribution of migraine in children is approximately 1:1 but in adults,women predominate in a ratio of 3:1 It is thought that 24 million Americanssuffer from migraine, 18 million females and 6 million males Of migraineurs,25% report greater than four attacks per month; 35% experience two to threeattacks per month; and 40% have a single attack per month Older literaturehas divided migraine into two large subgroups, common and classic migraine.Common migraine is now called migraine without aura, and classic migraine

is now referred as migraine with aura Migraine with aura approximates 25%

of the entire migraine group

Clinical Characteristics

Prodrome The prodrome of migraine consists of nonspecific phenomenon

that can occur days but more often hours before the actual head pain Thesesymptoms can be more mental such as depression, euphoria, and irritability ormore constitutional such as increased urination, defecation, anorexia, or fluidretention Often photophobia, phonophobia, and hyperosmia accompany theprodrome

Aura An aura or a prodrome can precede the actual head pain, but an aura

is differentiated from a prodrome in that an aura is often associated withfrank neurologic dysfunction usually transient in nature Migraine with auraoccurs in approximately 25% of migraine attacks The aura can be seenfrom 5 minutes to 1 hour prior to the head pain It is uncommon to have theaura occur simultaneously with the headache Visual auras are the mostcommon and include scotomas, teichopsias, fortification spectra, photop-sias, and distortion of images Sensory auras such as numbness and tingling

in a limb are second most common, and aphasia and hemiparesis occur lessoften

Headache Pain The headache pain in migraine occurs unilaterally in 65%

of migraineurs It is usually located in the periorbital region and can extend

to the cheek and ear The pain can switch from side to side with differentheadaches Migraine pain can occur in any place of the head and neck, includ-ing the posterior strap muscles of the cervical area Typically, the head painlasts at least 4 to 8 hours but can last for several days although this is rare.The quality of the pain can be mild to severe and usually has a pulsating andthrobbing quality The patient often is troubled by associated symptoms,which can occur with the actual head pain These include nausea and vomit-ing, photophobia, and phonophobia and can be more disabling to the patientthan the actual head pain

Evaluation of the Migraine Patient

The evaluation of a migraine headache begins with a complete history andphysical examination If the history is consistent with the typical characteris-tics of migraine and the neurologic examination is normal then appropriatemedication can be prescribed before any testing is undertaken Caution is exer-cised if either the history is atypical (i.e., migraines in a male beginning afterage 50) or if the neurologic examination is abnormal If the physician feels that

a workup is necessary then diagnostic studies can include: (1) routine bloodstudies, (2) sedimentation rate, (3) spinal fluid examination, and (4) an imag-ing study

Routine Blood Studies

There are several systemic illnesses, which are associated with headaches.These include vasculitis, toxic exposures, metabolic diseases, severe hyperten-sion, and infectious diseases Routine blood chemistries (chemistry panel andcomplete blood count [CBC]), HIV testing, vasculitis screen, thyroid functionstudies, and serum protein electrophoresis can be ordered as part of a routineblood study screening

Imaging Study

If the history and neurologic examination do not suggest any focal findingsthen it would be unusual to find an abnormality on an imaging study If thephysician feels that an imaging study is indicated, MRI of the brain is usuallyordered although, a CT scan is often adequate to identify any space occupyinglesion, shift in midline structures, brain herniation, or presence of subarach-noid blood If a lumbar puncture (LP) is indicated in the workup of headache,then head imaging should be done prior to the lumbar puncture

Headaches in Special Clinical Settings

There are several clinical settings where headaches play a prominent feature:

Postspinal Headaches

Approximately 25% of patients will have a headache after lumbar puncture.These headaches are often better when lying down and worsen with sitting orstanding up and can be associated with nausea and vomiting These can occurwith either a traumatic or an atraumatic tap They generally improve over timewith bedrest and fluids but for those post LP headaches that do not improve,

an epidural blood patch (small injection of the patient’s blood injected into theepidural space) at the site of the original spinal tap can be helpful

Postcoital Cephalgia

Postcoital cephalgia occurs both before and after orgasm It is seen equally inmen and women The head pain is usually sudden, often pulsatile, and caninvolve the entire head Fewer than 2% of patients who are seen with sub-arachnoid hemorrhage have the hemorrhage occur during intercourse.Therefore, a benign etiology of postcoital cephalgia is usually the case, andpatients should be pretreated prior to sexual relations with medication, usually

a simple analgesic

Pseudotumor Cerebri

Pseudotumor cerebri (benign intracranial hypertension) is manifested byincreased intercranial pressure without evidence of a CNS malignancy.Patients with benign intracranial hypertension complain primarily of headachesoften associated with visual disturbances Pseudotumor cerebri is usually seen

in female patients who are obese and often have menstrual irregularities

Acute Glaucoma

Acute glaucoma is often characterized by sudden orbital or eye pain in the face

of nausea and vomiting The pain can begin after the use of anticholinergic

drugs An elevated intraocular pressure is the hallmark of acute angle-closureglaucoma.

Carotid Dissection

Patients with carotid dissection, often present with orbital or neck pain ated with neurologic findings suggestive of carotid disease A Horner syn-drome (a constellation of signs produced when sympathetic innervation to theeye is interrupted) on the ipsilateral dissected carotid side, can accompanythese symptoms Trauma to the neck or vigorous movements to the neck willoften trigger the dissection

associ-Brain Tumor

Headaches associated with brain tumors often present as typical tension ormigraine headache The headaches can be quite frequent and can occur on adaily basis, often awakening the patient from sleep Neurologic examinationcan be normal but can reveal focal abnormalities as well as papilledema onfunduscopic examination Headaches are the presenting feature in approxi-mately 40% of brain tumor patients

Sinusitis

The issue of whether chronic sinusitis can contribute to headaches is oftenunclear Patients incorrectly assume that head pain in and above the eye isfrom sinus disease, and in truth of fact the majority of these patients actuallyhave migraine headaches

Subarachnoid Hemorrhage

Subarachnoid hemorrhage occurs from the following: (1) leakage of an ovenous malformation, (2) leakage of a ruptured aneurysm, or (3) trauma.Patients with subarachnoid hemorrhage often present with a debilitatingheadache described as the worst headache of their lives It is of sudden onsetand can be associated with nausea, vomiting, and stiff neck A subarachnoidhemorrhage can look like a migraine attack especially if there is extreme nau-sea and vomiting Subarachnoid hemorrhage is associated with blood in thesubarachnoid space, which is usually documented on MRI or CT scanning Alumbar puncture will confirm subarachnoid hemorrhage, where frank blood orxanthochromic staining of the cerebrospinal fluid (CSF) is noted Patients withsubarachnoid hemorrhage can decompensate quickly, and 50% of patients donot survive their subarachnoid bleed

Abortive Treatment of Migraine

The treatment of an individual migraine attack once it has occurred isreferred to as abortive therapy Currently there are four medications used inabortive therapy: (1) the triptans; (2) ergotamine; (3) dihydroergotamine;and (4) isometheptene mucate, dichloralphenazone, acetaminophen (Midrin)(Table 18–1)

alcohol); sunlight; hormonal change (menstruation)

Frequency of attack 2–4 per month or sporadically; can be cyclic

with menstruation Sex distribution 70% female

30% male Duration of attack Head pain 4 hours, aura to postdrome

24–36 hours Pain type and severity Begins as dull ache, progress to stabbing

pain; intense Associated symptoms Nausea and vomiting; photophobia, visual

obscuration

Each individual drug has different formulations; thus, these drugs are available

in oral, nasal spray, and intramuscular formulations The drugs work as 1D serotonin receptor agonists These drugs are 80% effective in the treatment

5HT-of an individual migraine attack They should be used early in the headacheoften during the prodrome but can also be used once the headache has started.The drugs can be repeated as early as 4 hours in headache reoccurrence, but

no more than three doses per 24 hours should be used The side effect profile

of the group of seven triptans is similar and include occasional nausea, iting, and numbness and tingling of the fingers and toes Clearcut con- traindications to the use of triptans include a history of coronary artery disease or hypertension If the patient has hemiplegia or blindness as an aura

vom-in a migravom-ine attack, then triptans should not be used

Ergotamine Derivatives

Ergotamines are no longer the cornerstone of the abortive treatment ofmigraine When patients fail triptans then ergotamines should be considered.Usually a 2 mg sublingual tablet is prescribed and repeated for two doses eachseparated by 30 minutes if necessary This dose can be repeated three times perday Ergotamines should not be prescribed on an ongoing daily basis forchronic usage

Dihydroergotamine

An episodic migraine, which can become more chronic and intractable, canrespond to intramuscular or intravenous dihydroergotamine (DHE) The initialdose of DHE is 0.5 mg intravenously with 10 mg of metoclopramide or 5 mgprochlorperazine if nausea is present If the headache improves, the dosage ofDHE plus metoclopramide is repeated for two more doses separated by

8 hours and then one more dose of DHE alone Nasal DHE is now availableand can be used in the abortive treatment of migraine In some patients, thenasal spray can replace intramuscular or intravenous use

Midrin

Midrin is a medication that spans the gap between abortive and prophylactic

therapy The drug consists of three components; acetaminophen (a simple analgesic), dichloralphenazone (a muscle relaxant), and isometheptene mucate (a vasoconstrictor) If Midrin is used in an abortive fashion, patients

are instructed to take two tablets at the onset of a headache or aura, then onetablet hourly for three additional doses (five tablets total) The drug can also

be used as a prophylactic agent for muscle tension headaches taking one tablettwice a day scheduled, and then a third or even a fourth pill for a breakthroughheadache during that day

Prophylactic Treatment of Migraine

Prophylactic therapy is used when there are at least three attacks per month, or

if acute therapy is not effective There are currently several classes of tion, used in the prophylactic treatment of migraine These include anticonvul-sants (topiramate [Topamax], divalproex [Depakote], and gabapentin[Neurontin]) beta-blockers (propranolol [Inderal]), calcium channel blockers(verapamil) and antidepressants (duloxetine [Cymbalta], amitriptyline [Elavil],and nortriptyline [Pamelor]) Other medications used in migraine prevention, butclearly now second-line agents include methysergide maleate (Sansert), lithiumcarbonate, clonidine, captopril, and monoamine oxidase inhibitors Currentlyanticonvulsants are prescribed most frequently in the prophylactic treatment ofmigraine The most common drug now prescribed is topiramate (Topamax) Sideeffects include sleepiness, numbness and tingling in the fingers and toes, andrarely blindness in one eye secondary to increased intraocular pressure.Divalproex (Depakote) has also been used successfully to treat migraine, start-ing out at a dose of 250 mg extended release (ER) at night for 1 week, followed

medica-by sequential increase in dosage each week if headaches do not come under trol Side effects include alopecia and tremor

con-Beta-Blockers

Beta-blockers have been used in the prophylactic treatment of migraine since

1972 The most commonly prescribed beta-blocker is propranolol (Inderal)

Table 18 – 2

COMMON TRIGGERS OF MIGRAINE HEADACHES

Chocolate, cheese, red wine, citrus fruits, coffee, tea, tomatoes, potatoes,

irregular meals

Excessive or insufficient sleep

Changes in hormone balance in women (such as menses, the pill,

Flashing lights or noise

Weather—high pressure conditions, changes in pressure, hot dry winds,

change of season, exposure to sun and glare

Sexual arousal

Smells—paint, fumes from car heaters or perfume