Woman With Worsening Headaches and Neurologic Symptoms

THE CASE:

A 37-year-old woman complains of frequent, severe headaches. She describes the pain as a pressure-like feeling that is usually located at the top of her head and occasionally spreads to one of her temples; she rates its intensity as 9 on a 10-point visual analog scale. The pain becomes throbbing when she tries to engage in any kind of physical activity. The headaches typically last 24 to 48 hours and occur 2 or 3 times a week. They are usually associated with severe nausea; occasional vomiting; and increased sensitivity to odors, bright lights, and loud noises.

The patient also reports that occasionally, 40 to 50 minutes before the onset of a headache, she experiences numbness in her right arm and right side of her face. The numbness is sometimes accompanied by the appearance of black spots (usually multiple) that at times obliterate the entire visual field. All these symptoms disappear within an hour. Initially (in her teenage years), she experienced these symptoms 3 or 4 times a year; however, in the past 2 years they have been occurring more frequently, and occasionally they last longer (up to a few days or even a week) and are more severe than formerly. She notes that at times the symptoms are not followed by a headache.

Her headaches began in her early teenage years. Initially, they occurred sporadically and were usually triggered by dietary factors. In the past 10 years, the headaches have occurred regularly--once or twice a month--until 2 to 2.5 years ago, when their frequency increased to 2 or 3 times a week. In addition, over the past 2 years the headaches have become more severe. For headache relief she has been taking 400 mg of ibuprofen orally, once or twice a day up to 3 times a week, with intermittent success.

Both her maternal grandmother and a maternal aunt had severe headaches. Her medical history is otherwise insignificant.

Physical and neurologic examinations reveal no abnormalities. An MRI scan of the brain performed 10 years ago was normal. A recent MRI scan shows multiple lesions in the periventricular white matter on both sides of her brain

THE DIALOGUE:

Primary care doctor: This patient has severe, worsening headaches associated with progressive neurologic symptoms. What would be your diagnostic approach?

Headache specialist: First, we need to find out why her symptoms are worsening. In our practice, we frequently see patients with migraine who report worsening headaches. However, this patient also reports neurologic symptoms that are becoming more intense and prolonged.

Primary care doctor: How would you classify her headaches that started during her teenage years?

Headache specialist: The patient reports having severe headaches that are associated with nausea, vomiting, photophobia, phonophobia, and increased sensitivity to odors; they also have usually been triggered by dietary factors. This clinical picture clearly points to migraine. In light of the normal MRI brain scan performed 10 years ago, the neurologic symptoms she had at the time were most likely part of a classic visual aura.

Primary care doctor: What do you think has caused the aggravation of her symptoms during the past 2 years?

Headache specialist: Migraine tends to worsen over time in certain patients if not properly treated. This worsening is sometimes referred to as a transformation from episodic to chronic migraine. The transformation may be precipitated by a variety of factors. The 2 largest categories of contributing factors are psychological and pharmacologic. However, this patient has neither a history of medication overuse nor any clear history of significant emotional or physical factors that might have contributed to the aggravation of her symptoms.

Primary care doctor: Her neurologic symptoms are not only lasting longer and becoming more severe, but they also sometimes occur without an ensuing headache. What might explain this phenomenon?

Headache specialist: Migraine aura without a headache typically occurs in older patients who have a long-standing history of migraine with aura; thus, the phenomenon might be explained by atherosclerotic or other changes in the blood vessel walls that decrease their flexibility. Usually (although not always), these patients report having auras that are typical for them-that is, of similar presentation and duration. The only difference is that the aura is not followed by a headache. Migraine aura without a headache is a benign condition.

This patient is relatively young and does not have any risk factors (eg, smoking, hyperlipidemia, diabetes mellitus) that could lead to an early onset of atherosclerotic blood vessel changes. In addition, her neurologic symptoms are becoming more severe and they sometimes last up to a week. Even without the recent abnormal brain MRI findings, these developments should prompt continuation of the workup.

Primary care doctor: What further workup is warranted?

Headache specialist: Whenever a patient presents with intermittent, fully reversible neurologic symptoms, multiple sclerosis (MS) should be ruled out. The first step in the workup is to order MRI scans of the brain, with and without contrast-even if a scan performed just a few years earlier was normal.

This patient's most recent MRI scan shows multiple white matter lesions that were not present 10 years earlier. These findings definitely heighten suspicion but still do not nail down the diagnosis. Although it is unlikely, her symptoms might still be explained by a migraine transformation.

Primary care doctor: How often are abnormalities found on MRI brain scans of patients with headache? Headache specialist: We see white matter changes on MRI scans of the brain quite frequently. In the majority of patients, these changes have no clinical significance.

According to the literature, 44% of patients with chronic headaches may have abnormal MRI findings.¹ However, in cases such as this one, in which suspicion is heightened, further workup should be performed. For this patient, lumbar puncture and CSF analysis were ordered, which revealed oligoclonal banding.

Primary care doctor: How frequently do you see patients with coexisting MS and migraine with visual aura?

Headache specialist: We find abnormalities on the MRI brain scans of many patients who have migraine with visual aura, hemiplegic migraine, or vestibular (basilar) migraine. These patients often present a diagnostic challenge. In these cases, the question arises of whether the patient’s symptoms are related to the migraine’s aura or to some other neurologic condition, such as MS.² However, we rarely diagnose MS.

On the other hand, according to recent studies, as many as 64% of patients with MS present with headaches. ^3,4 These patients may have had a history of headaches before the onset of MS, or a headache disorder may develop either at the onset of clinically evident MS or during its course. Interestingly, headaches develop in many patients with MS after interferon-beta therapy is started.⁵

References:

REFERENCES:

1.

Tsushima Y, Endo K. MR imaging in the evaluation of chronic or recurrent headache.

Radiology

. 2005;235:575-579.

2.

Evans RW, Rolak LA. Migraine versus multiple sclerosis.

Headache

. 2001;41:97-98.

3.

D'Amico D, La Mantia L, Rigamonti A, et al. Prevalence of primary headaches in people with multiple sclerosis.

Cephalalgia

. 2004;24:980-984.

4.

Gee JR, Chang J, Dublin AB, Vijayan N. The association of brainstem lesions with migraine-like headache: an imaging study of multiple sclerosis.

Headache

. 2005;45:670-677.

5.

Khromov A, Segal M, Nissinoff J, Fast A. Migraines linked to interferon-beta treatment of multiple sclerosis.

Am J Phys Med Rehabil

. 2005;84:644-647.