Hemiplegic Migraine : Symptoms, Types, Causes, Triggers, Treatments

Hemiplegic migraine, constituting less than one percent of all migraine cases, is a rare subtype of migraine with aura.⁽¹⁾ It presents with distinctive visual symptoms, such as flashes of light and zigzag patterns, preceding or accompanying the migraine episode.

Similar to other migraine types, hemiplegic migraines manifest through the following:

• Intense, pulsating headache
• Nausea
• Sensitivity to light and sound

However, what sets hemiplegic migraine apart from other subtypes of migraines is the additional symptoms of temporary weakness, numbness, tingling, and paralysis that affects just one side of the body. These neurological manifestations typically precede the headache. The term “hemiplegia” actually refers to paralysis, which is very much indicative of this condition’s impact.

According to the National Headache Foundation, hemiplegic migraine primarily affects people who are experiencing migraines with aura, encompassing visual disturbances and sensory issues, including speech difficulties.⁽²⁾

Hemiplegic migraine is characterized by various symptoms, including:⁽³⁾

• One-sided Body Weakness: This can affect the face, arm, and leg on one side of the body.
• Numbness or Tingling: Often experienced on the affected side of the face or limb.
• Vision Disturbances (Aura): Flashes of light, double vision, or other visual abnormalities.
• Speech Difficulties: Trouble speaking or slurred speech.
• Drowsiness and Dizziness: Feeling excessively sleepy or experiencing a sense of lightheadedness.
• Loss of Coordination: Difficulty in controlling movements.

In rare instances, people having hemiplegic migraine may experience more severe symptoms such as:

• Confusion
• Loss of movement control
• Decreased consciousness
• Memory loss
• Coma

When to Seek Urgent Medical Attention: If you are encountering severe headaches or migraine, it is advisable that you discuss a treatment plan with your doctor. However, immediate medical attention is warranted if you experience any of the following symptoms:

• Slurred Speech
• Sudden Weakness
• Loss of Consciousness
• Confusion

These symptoms closely resemble those of a stroke and necessitate urgent medical intervention. If you are unsure about the cause of your symptoms, seeking prompt medical care is crucial.

There are two distinct types of hemiplegic migraine, categorized based on your family history of migraine. These are:

1. Familial hemiplegic migraine (FHM): This type affects individuals within a family, involving at least two close relatives. In cases of FHM, there is a 50 percent chance for each child in the family to inherit this condition.⁽⁴⁾
2. Sporadic hemiplegic migraine (SHM): SHM occurs in people who do not have any family history of the condition. It affects people who do not have relatives with a similar history of hemiplegic migraine.⁽⁵⁾

It is important to know that regardless of the type of hemiplegic migraine you have, during an episode of this type of migraine, symptoms such as confusion and difficulty in speaking may occur, resembling those of a stroke. Seeking consultation with a neurologist or headache specialist for appropriate tests is crucial in obtaining an accurate diagnosis and suitable treatment.

Hemiplegic migraine is primarily attributed to certain types of genetic alterations or mutations. Several genes have been identified as contributors to this condition, notably:⁽⁶⁾

• ATP1A2
• CACNA1A
• PRRT2⁽⁷⁾
• SCN1A

These genes are known to play a crucial role in encoding proteins that are essential for allowing easy communication between nerve cells. However, when mutations occur in these genes, it causes a disruption in the release of neurotransmitters in the brain.

The mutations in these genes further disrupt the proper communication channel between specific nerve cells. Consequently, this interruption can lead to the onset of severe headaches and visual disturbances that often associated with hemiplegic migraine.

In Familial Hemiplegic Migraine (FHM), these gene mutations are inherited and run within families, passing from parents to their children. On the other hand, in cases of Sporadic Hemiplegic Migraine (SHM), these gene changes happen spontaneously, without there being any familial history of hemiplegic migraine.⁽⁸⁾

Hemiplegic migraine triggers can vary among individuals, making it difficult to recognize specific factors that may prompt an attack. Keeping a detailed diary noting down your activities and experiences preceding each episode can help in pinpointing the triggers effectively.

Some of the common triggers associated with hemiplegic migraine include:

• Stress: Emotional or psychological stressors can act as significant triggers for migraine attacks.
• Bright lights: Exposure to intense or flickering lights, such as fluorescent lighting or bright screens, may precipitate an episode.
• Intense emotions: Strong emotions, whether positive or negative, could contribute to triggering hemiplegic migraines.
• Disruption in sleep patterns: Both inadequate and excessive sleep can disrupt regular body rhythms, potentially triggering migraines.
• Certain foods: Processed foods, aged cheeses, salty items, and the additive MSG are known to provoke migraines in some individuals.
• Alcohol and caffeine: Excessive consumption or sudden withdrawal from alcohol or caffeine can trigger migraines.
• Skipping meals: Irregular meal patterns or prolonged fasting may serve as a trigger for some individuals.
• Weather changes: Shifts in weather conditions, especially rapid changes in temperature, humidity, or barometric pressure, can precipitate migraine episodes.

Recognizing and managing these triggers is an important step in alleviating the frequency and severity of hemiplegic migraine attacks.

A diagnosis of hemiplegic migraine primarily relies on recognizing its characteristic symptoms, usually involving a series of attacks with distinct aura, weakness, and neurological manifestations. These symptoms typically reduce once the headache phase resolves.

However, distinguishing hemiplegic migraine from conditions such as stroke, transient ischemic attack (TIA), multiple sclerosis, or epilepsy can be challenging due to overlapping symptoms.

To ascertain a diagnosis and rule out other potential conditions, doctors perform various tests, including:

• CT Scan
• MRI Scan
• Electroencephalogram (EEG)
• Echocardiogram

In cases where there is a family history of this type of migraine, genetic testing might be considered. However, it is important to note that a significant number of individuals with Familial Hemiplegic Migraine (FHM) may not test positive for identified genes associated with the condition. Research is still ongoing to identify all genes linked to hemiplegic migraine.⁽⁹⁾

Managing hemiplegic migraine involves using a combination of preventive measures to reduce the frequency and severity of attacks, as well as using abortive medications aimed at alleviating symptoms during acute episodes.

1. Preventive Medications

   • Beta-Blockers: These medications, such as propranolol or metoprolol, are commonly used to reduce the frequency and intensity of migraines.
   • OnabotulinumtoxinA (Botox): Injections of Botox every few months may be recommended as a preventive measure for chronic migraines, including hemiplegic migraine.⁽¹⁰⁾
   • CGRP Antagonists: Drugs targeting calcitonin gene-related peptide (CGRP), such as erenumab or fremanezumab, have shown efficacy in preventing migraines.⁽¹¹⁾
   • Anticonvulsants: Certain antiepileptic drugs like topiramate or valproate may be prescribed as preventive therapy for hemiplegic migraine.
   • Calcium Channel Blockers: Verapamil, a calcium channel blocker, is specifically used for preventing hemiplegic migraines due to its effect on controlling blood vessel constriction.
   • Antidepressants: Some antidepressants, such as amitriptyline or venlafaxine, might be prescribed for their preventive properties in managing migraines.

2. Abortive Medications for Acute Attacks

   • Intravenous (IV) Medications: During acute migraine episodes, IV medications like magnesium, Toradol (ketorolac), and antiemetics (e.g., Reglan) may be administered to relieve symptoms swiftly.
   • Oral Medications: Nonsteroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen, specific migraine medications like Ubrelvy (ubrogepant), and Nurtec ODT (rimegepant) are used to halt ongoing migraine attacks.

   It is important to realize, though, that every patient’s response to these medications vary.

Hemiplegic migraine presents with more than just severe headache pain, often inducing weakness or paralysis on one side of the body. Although it is not directly associated with a stroke, the resemblance in symptoms necessitates prompt medical attention for sudden weakness or slurred speech.

For individuals with a history of this migraine variant, working together with a healthcare professional to come up with a personalized treatment plan is important. A treatment plan for managing hemiplegic migraine focuses on relieving the current symptoms and also implement preventive measures to manage and minimize future episodes effectively.

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