A 38-Year-Old Man With Double Vision, Numbness, and Headache

Olusegun John Oluwole, MBBS

Disclosures

July 28, 2020

This patient's headache had many features that would be considered typical of migraine; however, it also had features that are not often encountered in routine presentations of migraine.

Migraine has its highest incidence at puberty and between 35-45 years of age.[2] It typically presents with throbbing unilateral/hemicranial headache of moderate to severe intensity, lasting 4-72 hours unless aborted by medications. It may be accompanied by photophobia, phonophobia, osmophobia, nausea, and vomiting and is typically made worse with movement.[3] Many patients also experience associated cranial autonomic symptoms, such as redness of the eyes, ptosis, tearing, nasal congestion, and pupillary changes.[3]

Although migraine headache is typically described as unilateral, it can also be bilateral or holocephalic. Before settling on a diagnosis of migraine, other possible explanations or underlying secondary causes for the headache must be ruled out.[3] Therefore, skillful history-taking and careful physical examination, including optic disc visualization, are imperative. This is not to suggest that every patient with headache should undergo extensive investigations or head imaging, such as head CT or MRI. A useful mnemonic to remember when secondary headaches are highly likely is "SNOOP."[4] This includes the presence of Systemic symptoms/signs; the presence of Neurologic symptoms/signs; sudden Onset of headache; Older age at onset of headache (> 50 y); and Pattern change, Papilledema, aggravation with Posture, and Progressive headache.[1]

As many as 1 out of 5 patients with migraine have additional features called "migraine aura," which usually start within 60 minutes of headache onset.[4] These aura symptoms may have visual, sensory, motor, aphasic, and brain stem components.[3] Visual aura manifests with visual field deficits, scintillating scotomas, shining lights (photopsia), and zigzag visual patterns (teichopsia and fortification spectra). Sensory aura can include tingling, numbness, or outright loss of sensation in one half of the face, with or without involvement of ipsilateral upper and lower extremities. Aphasic aura usually takes the form of expressive aphasia, whereas brain stem aura manifests as lightheadedness, vertigo, loss of consciousness, or even external ophthalmoparesis. Motor aura tends to present with hemiparesis in a migraine syndrome recognized as hemiplegic migraine.

Hemiplegic migraine could be sporadic or familial.[5] Patients presenting with hemiplegic migraine typically have at least two other forms of aura that coexist with weakness during the particular headache presentation. This is often a helpful clue to distinguish hemiplegic migraine from other potential causes of a similar presentation. Patients may present for the first time with hemiplegic migraine after many years of experiencing common migraine (without aura). This appears to true for the patient in this case, whose previous headaches had not been accompanied by any forms of aura. Of note, the patient manifested additional aura forms other than weakness, including visual aura (photopsia), sensory aura (numbness, tingling, and hemisensory deficits), and brain stem aura (external ophthalmoparesis). This is often the case in patients with hemiplegic migraine.

Familial hemiplegic migraine is differentiated from the sporadic form by the presence of a positive family history.[5] The familial form has been linked to mutations in various ion channel genes, including CACNA1A, ATP1A2, and SCN1A.[6] Some cases of sporadic hemiplegic migraine have also been associated with these mutations. This is especially true in patients who have additional neurologic manifestations during clinical examination, such as cerebellar ataxia.[6]

The patient in this case exhibited mild cerebellar ataxia in the right upper extremity, which could be viewed as an additional neurologic manifestation; however, the International Classification of Headache Disorders recognizes cerebellar ataxia as a type of brain stem aura.[3]

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