EMReports | Hemiplegia and Headache

Hemiplegia and Headache

Larry Mellick, MD, MS, FAAP, FACEP
Professor of Emergency Medicine
Medical College of Georgia

Dixie Griffin, MD
Pediatric Resident
Medical College of Georgia

Pearl: In patients presenting with unilateral weakness, vision changes, aphasia, and headache, consider hemiplegic migraine.

Presentation: A 25-year-old female presented to the emergency department complaining of flashing lights in her right eye that started one hour earlier while watching a home movie. The patient reported that while trying to read, she was unable to discern the words but could understand words spoken to her. She also felt disoriented and thought that she was slurring her words. Following the onset of numbness in her lips, she noted weakness in her right leg. A bilateral parietal headache occurred shortly after the symptoms began. She denied any history of headaches and had no other medical problems. She was not pregnant and was not taking any medications. She denied use of illicit drugs or alcohol. Her family medical history was negative for migraine headaches. She was afebrile, with blood pressure 152/76, heart rate 96, respiratory rate 24, and oxygen saturation 100%. On physical examination, she had ptosis of her right eye and decreased sensation around the right side of her mouth. A right homonymous hemianopsia was present. Her right lower extremity muscle strength was 4/5 compared to 5/5 on left lower extremity. She ambulated well but had difficulty turning around to return to bed, stating she felt disoriented. Her headache was persistent during the examination but she had no photophobia. Complete blood count and electrolytes were within normal limits. Her urine drug screen was negative. There were no acute findings on head CT without contrast. Within 2 hours, her symptoms and headache completely resolved. In a young female with no prior medical history, what could this be?

Diagnosis: Sporadic Hemiplegic Migraine

Discussion: Sporadic hemiplegic migraine (SHM) is a distinct diagnostic subgroup of migraine with aura. Unlike familial hemiplegic migraine (FHM), patients with SHM have no family members with hemiplegic migraines.^1,2,3 During attacks of SHM, patients will have at least two aura symptoms (visual, sensory, aphasic, and motor). Some may have all four symptoms. All patients will have motor symptoms plus at least one other symptom. The motor symptoms are most often unilateral and should not occur consistently on one side during recurring episodes of SHM.^1,2,4 If patients diagnosed with SHM present with same-side motor symptoms, other etiologies such as a brain tumor should be investigated.⁴ Upper limbs are two times more likely to be affected than lower limbs. Visual symptoms are almost always present and may include flashing lights, scotoma, and central vision disturbances. Symptoms usually start with visual, then sensory, and later motor and aphasic symptoms. The symptoms evolve over minutes and may last 1 to 24 hours. Most will resolve in one hour.² Some patients will have basilar-type migraine symptoms – dysarthria, unsteadiness, tinnitus, decreased hearing, vertigo, or diplopia.^1,2 The aura is followed by headache.5

Transient ischemic attack, stroke, and epilepsy (post-ictal state, or Todds phenomenon) should be included in the differential diagnosis of new onset hemiplegic migraine. Imaging should be done if no visual aura is associated with the symptoms, age of onset is greater than 45 years, or the aura lasts longer than 60 minutes.^1,6 Metabolic abnormalities can be easily ruled out by checking electrolytes and liver function tests.

There are only case studies supporting specific drug treatments for SHM including the use of verapamil.¹ Otherwise, the headache associated with SHM can be managed according to local guidelines or practices.

References:

Thomsen LL, Olesen J. Sporadic hemiplegic migraine. Cephalalgia 2004;24:1016-1023.
Thomsen LL, Ostergaard E, Olesen J, et al. Evidence for a separate type of migraine with aura: Sporadic hemiplegic migraine. Neurology 2003;60:595-601.
Thomsen LL, Ostergaard E, Romer SF, et al. Sporadic hemiplegic migraine is an aetiologically heterogeneous disorder. Cephalalgia 2003;23:921-928.
Vetvik KR, Dahl M, Russell MB. Symptomatic sporadic hemiplegic migraine. Cephalalgia 2005; 25:1093-1095.
Thomsen L, Eriksen M, Roemer S, et al.A population-based study of familial hemiplegic migraine suggests revised diagnostic criteria. Brain 2002;125:1379-1391.
Goadsby PJ. Sporadic hemiplegic migraine: Stamp collecting or food for thought? Neurology 2003:60:536-537.

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