Woman With Severe Headache and Neurologic Deficit

THE CASE:
A 32-year-old woman complains of severe throbbing pain at the top of her head; numbness and weakness on theleft side of her face and in her left arm and leg; and nausea, vomiting, and light and noise sensitivity. She rates theseverity of the pain as 8 on a 10-point scale. The headache started about 36 hours earlier, with pain in the left temporalarea. As the pain increased, it radiated to the opposite side and eventually involved the entire head.

When the pain started, the patient felt light tingling in her lips (more on the left side) that slowly spread downto her left arm and leg. The tingling increased, and the affected areas eventually became numb and weak.

Her blood pressure is 130/90 mm Hg; heart rate, 84 beats per minute; and respiration rate, 16 breaths perminute. A neurologic examination reveals decreased pain sensitivity on the left side of the face, neck, and ipsilateralextremities. Muscle strength is rated as 5 of 5 in her right arm and leg and 3 of 5 on the left side. Deep tendon reflexesare somewhat elevated on the left side. No other neurologic abnormalities are noted.

The patient initially experienced headaches during adolescence. These occurred about once or twice a month,were usually located at the top of her head bilaterally, and occasionally radiated and localized to one side. At thattime, she experienced an aura (flashing lights or dark spots in both visual fields) 20 to 40 minutes before the headachebegan. Over the next few years, the headaches occurred more regularly and episodes became more prolonged, lastingup to 5 or 6 days. Three years ago, an MRI scan of the brain showed no abnormalities.

During the past 3 years, some migraine episodes (usually the prolonged attacks) became associated with numbnessand weakness in her face and extremities on one side or the other that lasted from a few hours up to 2 to 3 days,with gradual but complete resolution. Most of her headaches now are typical migraines (with or without aura), butabout every 1 to 2 months, the neurologic symptoms accompany the headache.

The patient is otherwise healthy and does not smoke. Her mother, sister, and an aunt (her mother's sister) havea history of migraine. The patient said that her mother has migraine attacks with visual aura, but no family memberhas migraine episodes associated with neurologic deficits.

• What is the differential diagnosis of severe headache with accompanying neurologic deficit?
• What tests and clinical signs are most helpful in pinpointing the diagnosis?
• What therapeutic strategy will relieve both the migraine pain and the neurologic symptoms?

THE DIALOGUE:

Primary care doctor: This patient seems to have severaltypes of headache; the most evident is migraine withand without visual aura. What is the significance of theneurologic symptoms that accompany her headaches,and how can I best determine their cause?

Headache specialist: The symptoms are most likely associatedwith her migraines; however, the diagnosis canbe established only after other causes of the neurologic deficits are excluded. I would first consider possiblestroke and transient ischemic attack. When a patientcomplains of the worst headache of his or her life, it isimportant to rule out subarachnoid hemorrhage. In thiscase, the severity of the pain and its gradual onset facilitateruling out the latter diagnosis.

A neurologic deficit caused by a transient ischemicattack should last less than 24 hours. This womanpresented with signs of paresis that had persisted for36 hours, thus ruling out transient ischemic attack.

Primary care doctor: Which imaging studies would youorder to exclude an ischemic event?

Headache specialist: The procedure of choice is a CT orMRI scan of the brain. CT is more valuable when subarachnoidhemorrhage is suspected. In the case of suspectedischemia, brain MRI is preferred because it may take aslong as 48 hours for a CT scan to demonstrate ischemicchanges. When an aneurysm is suspected, consider magneticresonance angiography (MRA) of the brain.

Primary care doctor: What were the results of theseneuroradiologic studies in this patient?

Headache specialist: We decided to repeat brain MRIbecause her symptoms had persisted and worsened overthe past few years. An MRA scan of the brain was alsoperformed. Although the office neurologic examinationshowed hemiparesis, the MRI scan did not demonstrateany ischemic changes of the brain and MRA revealed noblood vessel abnormalities. Thus, we were able to ruleout stroke and cerebral blood vessel malformations.

Primary care doctor: What diagnosis did you considerafter you excluded cerebrovascular disease?

Headache specialist: The diagnosis was hemiplegic migraine.This is a variant of migraine with aura, althoughit represents a separate clinical entity. Hemiplegic migraineconsists of typical migraine features, such as unilaterallocation, throbbing quality, and duration of pain,and migraine-associated symptoms (nausea, vomiting,photophobia, and phonophobia). In addition, this type ofmigraine is associated with reversible weakness of oneor both extremities on one side of the body.¹

Two major types of hemiplegic migraine have beenidentified: sporadic and familial. They have similar clinicalpresentations. The familial form is a genetic disorder inwhich mutations in CACNA1A(for hemiplegic migrainetype 1) and ATP1A2(for hemiplegic migraine type 2)genes have been identified.² For familial hemiplegic migraineto be diagnosed, the patient must have at least onefirst- or second-degree relative with the same disorder.This patient denies any family history of similar symptoms;thus, her hemiplegic migraine is the sporadic type.

Primary care doctor: How long do the neurologic symptomstypically persist?

Headache specialist: According to the classification ofthe International Headache Society, neurologic symptomsshould continue for more than 5 minutes but lessthan 24 hours. However, patients with hemiplegic migrainefrequently have neurologic deficits that persistmuch longer. In some patients, symptoms do not completelyresolve for several weeks. In such patients, thenature of the symptoms must be thoroughly investigatedto prevent misdiagnosis of a catastrophic vascularevent. Consider the results of CT, MRI, and MRA, aswell as whether there is a history of similar episodesthat completely resolved. In this patient, her relativelyyoung age, lack of risk factors, and insignificant familyhistory of major vascular diseases also help rule out acerebrovascular event.

Primary care doctor: What is the prevalence of hemiplegicmigraine?

Headache specialist: A survey of the Danish population(5.2 million) showed that the prevalence of hemiplegicmigraine was 0.01%.³ It occurs more frequently in womenthan in men (ratio, 3:1), and there is equal distribution offamilial and sporadic forms.

Primary care doctor: How would you treat a patient withhemiplegic migraine?

Headache specialist: The list of safe, effective medicationsfor hemiplegic migraine includes analgesics, corticosteroids,and NSAIDs. Some reports have describedthe effectiveness of the calcium channel blocker verapamil(either oral or parenteral) in the treatment of bothfamilial and sporadic forms of hemiplegic migraine. Aswith basilar artery migraine, triptans and ergotaminecontainingmedications are not recommended in themanagement of hemiplegic migraine.

In another patient who had the sporadic form ofhemiplegic migraine, both analgesics and corticosteroidsproved ineffective. However, the patient obtainedrapid relief from the migraine episode and the neurologicsymptoms after intravenous administration of 1 g ofvalproic acid. In this patient, the headache diminishedwithin 30 minutes after the injection. The headacheand neurologic symptoms completely resolved within 2hours.⁴

The problem of hemiplegic migraine is not yet wellunderstood. We continue to wait for results of newerclinical studies and newer methods of treatment.

References:

REFERENCES:

1.

Headache Classification Committee of the International Headache Society.International Classification of Headache Disorders. 2nd ed. Cephalalgia.2004;24(suppl 1):8-160.

2.

Sanchez-del-Rio M, Reuter U. Migraine aura: new information on underlyingmechanisms. Curr Opin Neurol. 2004;17:289-293.

3.

Lykke Thomsen L, Kirchmann Eriksen M, Faerch Romer S, et al. An epidemiologicalsurvey of hemiplegic migraine. Cephalalgia. 2002;22:361-375.

4.

Freitag F, Feoktistov A. A woman with rebound headache and intermittentparalysis. In: Purdy RA, Rapaport AM, Sheftell FD, Tepper S, eds. AdvancedTherapy of Headache. 2nd ed. Hamilton, Ontario: BC Decker; 2005:259-265.