Hemiplegic Migraine

Hemiplegic migraines are a type of migraine that can cause some unusual and scary symptoms, mimicking strokes. “Hemiplegic” means “half of the body is paralyzed,” so people who have this type of migraine might feel weakness or even paralysis on one side of their body, like one arm or leg not working properly.

Here are some of the common symptoms of hemiplegic migraines:
Severe Headache: Like other migraines, there’s usually a really bad headache, often on one side of the head. This can be accompanied by other common migraine symptoms including fatigue, sensitivity to lights, sounds and movements.
Weakness on One Side: You might feel weak or numb on one side of the body—like you can’t move your arm or leg properly.
Vision Changes: Some people see blurry or flashing lights, or even lose sight in one eye for a while.
Speech Problems: It can be hard to speak clearly or understand others because the migraine can affect the brain.
Confusion or Difficulty Thinking: It might feel like your brain is foggy, and you can’t think or concentrate well.
Nausea and Vomiting: Like other migraines, you might feel sick to your stomach.

Hemiplegic migraine is a complex disorder with a clear genetic basis in familial cases, where mutations affect ion channels critical to neuronal excitability and signaling. The motor deficits seen in HM are likely due to the involvement of the motor cortex and possibly other areas like the brainstem, combined with the effects of cortical spreading depression and abnormal neurovascular interactions. The pathophysiology remains an area of active research.

Treating hemiplegic migraines (HM) can be challenging due to their complexity and the potential for severe neurological symptoms. The management of HM typically involves a combination of acute treatment strategies for headache relief and preventive therapies to reduce the frequency and severity of attacks. However, because hemiplegic migraines are a rare and often genetically linked condition, treatment approaches may need to be tailored to individual patients, especially in cases with familial hemiplegic migraine (FHM).

  1. Acute Treatment of Hemiplegic Migraine Attacks

Acute treatment for hemiplegic migraines aims to relieve the headache and associated symptoms, but caution is necessary due to the potential risk of complications such as stroke-like symptoms.
Triptans (e.g., sumatriptan, rizatriptan): These are generally contraindicated in hemiplegic migraine, especially in familial forms. They can potentially worsen symptoms by constricting blood vessels, and there’s concern that they might increase the risk of stroke in these patients.
NSAIDs (e.g., ibuprofen, naproxen): Nonsteroidal anti-inflammatory drugs (NSAIDs) may help alleviate the headache and are often used as first-line agents.
Anti-emetics: Medications like metoclopramide or prochlorperazine can be used to manage nausea and vomiting associated with the migraine.
Ergotamines: These should also generally be avoided due to the risk of vasoconstriction.
Corticosteroids: In some cases, dexamethasone or other corticosteroids might be used, particularly if there’s significant inflammation or if the symptoms do not improve with standard treatments.
Magnesium: Some evidence suggests that magnesium supplementation may help reduce the frequency or severity of attacks, though its use is more common in general migraine management and not specific to HM.
Intravenous (IV) therapy: In severe or prolonged attacks, IV fluids, anti-nausea drugs, and anti-inflammatory medications may be used in a hospital setting to stabilize the patient and provide symptom relief.

  1. Preventive Treatment

Preventive treatments aim to reduce the frequency of hemiplegic migraine attacks. These treatments are crucial for patients who experience frequent or disabling episodes.
Verapamil is often the first-line preventive treatment for familial hemiplegic migraine (FHM), especially in patients with CACNA1A mutations. It is believed to help by stabilizing neuronal excitability.
Other calcium channel blockers, such as diltiazem, may also be used in some cases.
Topiramate and valproate are anticonvulsants that can be effective in reducing the frequency of hemiplegic migraine attacks. They are particularly useful in patients with FHM or those who experience aura with severe neurological manifestations.
Levetiracetam and lamotrigine are sometimes considered, especially in cases where there is an overlap with epilepsy-like symptoms.
Beta-blockers, such as propranolol, may be used as a preventive measure in patients with more typical migraine features (e.g., without motor symptoms) but are generally not first-line for hemiplegic migraine due to concerns over possible worsening of motor deficits in some cases.
Amitriptyline or venlafaxine may be considered for migraine prevention, particularly in cases where patients have concurrent mood disorders, although evidence for their effectiveness specifically in HM is limited. CGRP inhibitors, such as erenumab, galcanezumab, and fremanezumab, have been shown to be effective in treating other forms of migraine. However, their use in hemiplegic migraine is not as well-studied, and caution is advised, particularly in cases with known genetic mutations, as the safety and efficacy in this population have not been fully established.
For patients with familial hemiplegic migraine, genetic counseling is often recommended to help understand the risk of inheritance and implications for family members.
Some cases of hemiplegic migraine may require adjustments to medications based on the specific genetic mutations (e.g., CACNA1A mutations) present, which can affect treatment choices, especially concerning the use of medications that alter neuronal calcium channels.
Preventing triggers is a key part of managing hemiplegic migraine. Lifestyle changes can help reduce the frequency of attacks:
Regular Sleep Patterns: Encourage a consistent sleep schedule to avoid sleep deprivation, a common migraine trigger.
Stress Management: Techniques like yoga, meditation, and relaxation exercises can be helpful in managing stress, which is a known migraine trigger.
Dietary Changes: Certain foods may trigger migraines in susceptible individuals, and a balanced diet free from common triggers like caffeine, chocolate, or processed foods may be beneficial.
Hydration: Maintaining proper hydration is important, as dehydration is a frequent migraine trigger
Research into the pathophysiology and treatment of hemiplegic migraine is ongoing, and new therapies are being explored:
Gene Therapy: Given the genetic nature of familial hemiplegic migraine, gene therapy is an area of active research. This could, in theory, target the specific mutations (e.g., in CACNA1A or ATP1A2) that lead to the dysfunction in neuronal excitability.
Botulinum Toxin: While botulinum toxin (Botox) is commonly used for chronic migraine, its use in hemiplegic migraine is not well-established but may be considered in refractory cases.
Neurostimulation: Techniques such as transcranial magnetic stimulation (TMS) or vagus nerve stimulation (VNS) are being explored as alternative therapies, particularly for refractory cases of migraine.

Stroke Mimic: Because hemiplegic migraine can present with stroke-like symptoms, it is important to rule out other causes, such as ischemic stroke, especially in the acute setting. MRI and CT scans may be required to differentiate HM from more serious conditions.
Co-treatment with Other Specialists: Neurologists, geneticists, , and sometimes cardiologists or other specialists may need to be involved in the management of hemiplegic migraine, particularly in cases where there is a familial history of the condition or other complex features. Physical, occupational and speech therapies will be helpful to improve the impaired functions