Hemiplegic Migraine: Symptoms, Treatments and Advice For Managing Attacks

Medically reviewed by: Dr. Michael Marmura

What is Hemiplegic Migraine?

Migraine is a complex neurological disease that affects 1 billion people worldwide. Many people may be familiar with the “typical” symptoms of migraine such as head pain, light sensitivity, sound sensitivity and nausea. However, about 30% of people with migraine experience temporary neurological symptoms called aura. An aura may include visual disturbances (zig-zag lines, blind spots, blurred or partial loss of vision), sensory symptoms such as numbness or tingling as well as speech or language difficulties, and more.

One fairly rare subtype of migraine with aura is called hemiplegic migraine. 

Breaking down the term, hemi means half, and plegic means paralysis. The hallmark symptom of hemiplegic migraine is temporary one-sided weakness or paralysis. The weakness or paralysis is a form of aura. It’s accompanied by at least one other aura symptom, such as visual changes, sensory changes or speech or language problems.¹ 

These symptoms typically occur in a stepwise fashion over several minutes. While symptoms may start to subside within a few hours or more, some patients experience symptoms lasting for several days or even weeks.

A headache may occur with the aura or begin within about 60 minutes after it starts.

Hemiplegic migraine may affect only part of the body, like the face, a hand, or an arm, but weakness of an entire side of the body can occur. Other symptoms can include loss of balance and coordination, difficulty speaking, fever, dizziness, impaired consciousness and confusion. 

Because of these alarming symptoms, a person’s first hemiplegic migraine attack can make them think they are having a stroke or a transient ischemic attack. 

However, even once a person is diagnosed correctly, it can be difficult to explain to bystanders, friends or medical professionals that the symptoms are not the result of a stroke or something life-threatening. Communicating treatment needs can be challenging as well.

Learning about the various symptoms, treatments and how to prepare for and communicate during an attack are important for effective hemiplegic migraine management.

What are the types of Hemiplegic Migraine? 

There are two types of hemiplegic migraine:

• Familial hemiplegic migraine: When one or more first-degree relatives also have the condition. Four different genes have been found to be involved (CACNA1A, ATP1A2, SCN1A, PRRT2). Genetic testing for familial hemiplegic migraine is available but is not always covered by insurance.
• Sporadic hemiplegic migraine: When a person has the condition without a family history. However, gene mutations may still be involved.

An emerging type of hemiplegic migraine that is not currently recognized in the International Classification of Headache Disorders, is Migraine with Unilateral Motor Symptoms or MUMS. To learn more about MUMS, view our podcast with Dr. Thomas Berk and Cannon Hodge. 

Hemiplegic Migraine often starts in childhood or in an individual’s early 20s. People with this migraine type have “stereotyped attacks” which they can usually describe fairly well. They may have 1-2 attacks per month or less. But unlike most patients with migraine with aura, they can have prolonged post-episode fatigue lasting several days.

On the other hand, people with MUMS have an onset of symptoms in their 30s-40s. They usually have a severe headache on most days than not, and several signs of central sensitization or other body pain like allodynia (pain from non-painful stimuli) or hyperalgesia (increased sensitivity to pain).

Treating Hemiplegic Migraine

Treating hemiplegic migraine can be difficult due to the severity of the disease and the limited amount of research conducted on this type of migraine. It is best treated by a healthcare provider who specializes in headache disorders. Since hemiplegic migraine is a subtype of migraine with aura, generally, treatment is similar to migraine with aura. There are no FDA-approved medications specifically for Hemiplegic Migraine.

Although the evidence about migraine triggers is frequently debated, it’s important to be mindful of potential triggers like sleep deprivation, missed meals, changes in routine, stress, bright lights, alcohol use, and menses.

Triptans or No Triptans?

Previously in clinical trials, triptans and ergotamine were contraindicated in the treatment of sporadic and familial hemiplegic migraine.² However, patients with migraine with brainstem aura, familial hemiplegic migraine, or migraine with prolonged aura have used triptans without adverse events, and triptans have been safely and effectively administered as acute treatment for both familial and sporadic hemiplegic migraine.²

Acute treatments generally used:

• NSAIDS (e.g., naproxen, ibuprofen) or acetaminophen are often used first-line.
• Triptans (almotriptan, eletriptan, frovatriptan, naratriptan, rizatriptan, sumatriptan, and zolmitriptan) may be used. While triptans do not treat aura itself, and may not work when taken in the aura phase, they are generally safe to try.
• Neuromodulation devices (Nerivio, Cefaly, Relivion, gammaCore, SAVI Dual): These devices deliver electrical or magnetic impulses to reduce, eliminate or prevent migraine attacks. They are worn or held against different parts of the body to stimulate nerves or areas of the brain and nervous system involved in the migraine process.
• Intranasal ketamine: May reduce the intensity and duration of aura among those with familial hemiplegic migraine. 
• Intravenous verapamil: May resolve symptoms quickly.² Oral verapamil may be used as a preventive option.
• Corticosteroids: For prolonged hemiplegic migraine attacks.²
• Intravenous magnesium
• Intravenous furosemide²
• Greater occipital nerve blocks
• Anti-nausea medications (metoclopramide, prochlorperazine): These can help to relieve nausea, a common symptom during a migraine attack but also may be beneficial in reducing migraine pain.

Preventive treatments generally used:

• Blood pressure medications (verapamil, flunarizine): Oral verapamil may also be used preventively.² Flunarizine may be more effective in children with hemiplegic migraine than in people with migraine with or without aura.² However, it is not available in the USA.
• OnabotulinumtoxinA
• Diuretics (acetazolamide)
• Anti-seizure medications (sodium valproate, lamotrigine)
• CGRP Monoclonal Antibodies (eptinezumab, fremanezumab, galcanezumab, erenumab): Injectable or intravenous treatment that binds to CGRP or its receptors.
• Intravenous ketamine²
• Naloxone
• Beta blockers (ex. propranolol, metoprolol) remain controversial as a treatment for hemiplegic migraine.

Preparing for and Communicating During Hemiplegic Migraine Attacks

Experiencing a hemiplegic migraine attack can be extremely frightening not only for the person experiencing it but also for those witnessing it. During an attack, some individuals are fully aware of their surroundings but may be unable to communicate or move a limb. To help others living with this condition, Maria, a patient advocate, shared her tips on how she prepares for an attack and ways she effectively communicates her needs during an attack.

1. Carry an emergency care statement. Include your name, migraine type, very basic care instructions, and three emergency contact names and numbers. Keep it simple! Make sure to use large, bold letters to be attention-grabbing. 
   • Once the prodrome starts, keep your Emergency Care Statement and phone on the side of your body that is mobile (not paralyzed) so that you can still get to it when the hemiplegia starts.
   • “I feel empowered to leave the house during the three days each month that I have an aura, knowing I have my emergency care statement with me.” 
2. Develop a non-verbal communication system. Using a thumbs-up for ‘yes’ and a thumbs-down for ‘no’ can be a way to communicate during attacks. Make sure to include your non-verbal communication method on your emergency care statement.
3. Choose a designated caregiver. Find someone with the emotional and intellectual capability to advocate for you, not just someone who can be your transportation to the emergency room. 
4. Make a video of you with your primary caregiver. In the video, state your names and explain your migraine type and normal symptoms. This can be another tool to use if a migraine attack happens unexpectedly in public or if you’re with emergency healthcare providers who don’t believe what your caregiver is saying.
5. Carry a detailed information note. On this note, list all of your normal symptoms, care instructions and daily medications if any. This is more detailed than the emergency care statement. The information can be on the same document. For example: I have a slightly painful tingling right arm (and sometimes leg) when I have a migraine attack. Sometimes I am not able to speak or hold my head up. 
6. Always have a plan. Migraine can cause you to miss out on life, but the fear of an attack shouldn’t. Whether you are having dinner at a restaurant, attending your child’s recital, or visiting a friend, develop a plan for where to go or what to do if you have a strong aura that may develop into a full-blown attack. 
7. If you recognize symptoms, stay within close range of your caregiver. Stay close to a caregiver if you start to experience symptoms of an aura, so they can assist you if necessary.
8. Keep track of medications taken during an attack. If you have to go to the emergency room, it is important to have a record of the medications you’ve used within the past 24 hours. If possible, write them down in an electronic app or inform your caregiver when the first sign of an attack occurs.
9. Get involved. Shades for Migraine provides a platform to share your experience. Living with hemiplegic migraine and its symptoms may be difficult, overwhelming and frightening. Opening up and telling others about your experience may bring a sense of community. Shades for Migraine provides a platform for those living with migraine to share their experiences and help educate others about the true impact of migraine disease. Post a photo with #ShadesForMigraine on or around June 21st and share your story if you feel comfortable doing so. 

Overall, hemiplegic migraine is an extremely debilitating condition that can significantly impact a person’s life. Learning strategies to prepare for and communicate during an attack is extremely important. This knowledge can help people living with this condition avoid unnecessary tests and medical procedures, manage their condition more effectively, and reduce the fear and anxiety that can occur with an attack.

References

1. ichd-3.org/1-migraine/1-2-migraine-with-aura/1-2-3-hemiplegic-migraine/
2. www.medlink.com/articles/hemiplegic-migraine
3. pubmed.ncbi.nlm.nih.gov/21784774/

Author

Maria Mormile lives in southern RI with her husband and daughter where she works as a pilates instructor, small-business owner and former architectural drafter and permit coordinator. She had undiagnosed migraine attacks of various subtypes throughout childhood before being diagnosed with hemiplegic migraine at age 23. Maria found physical and mental strength to navigate living with migraine disease and its comorbidities through her practice of pilates and the joy of sharing its benefits with her clients.

Molly O’Brien is a freelance Journalist and Communications Specialist originally hailing from the Seattle area. After graduating summa cum laude from Arizona State University with a degree in Broadcast Journalism, Molly traveled coast-to-coast working in local news. Molly was diagnosed with episodic migraine before the age of four, then turned chronic in her mid-twenties. Disease progression has severely impacted every facet of her life as she tries to balance migraine disease, a career, parenthood, and everyday life.