Hemiplegic Migraine and MUMS
Posted on June 15 2025,
Hemiplegic Migraine & MUMS
Understanding these rare but serious forms of migraine with motor weakness
What is the Motor Migraine Subtype?
Motor migraines are a clinically important group of migraine disorders characterized by unilateral weakness during attacks. Two distinct conditions fall under this umbrella: hemiplegic migraine and MUMS (Migraine with Unilateral Motor Symptoms). While both involve motor weakness, they have fundamentally different underlying mechanisms and require different approaches to diagnosis and treatment.
Hemiplegic migraine is a rare but distinct form of migraine characterized by an aura that includes true unilateral weakness along with other neurological symptoms. Unlike typical migraine, the defining feature is temporary paralysis or weakness on one side of the body during attacks, caused by underlying genetic ion channel dysfunction.
MUMS is a recently characterized migraine syndrome that presents with distinct motor weakness patterns rather than true hemiplegic weakness. First systematically described in 2007, MUMS challenges traditional migraine classification by presenting with "give-way weakness" that mimics stroke but constitutes a distinct migraine subtype with its own characteristic features.
Both conditions affect young to middle-aged adults, can mimic stroke, and require specialized management approaches. Understanding the differences between these conditions is crucial for proper diagnosis and treatment.
0.01%
Population Prevalence
Hemiplegic migraine affects approximately 1 in 10,000 people, making it one of the rarest migraine subtypes.
12-17
Average Age of Onset
Symptoms typically begin in adolescence, though onset can range from age 1 to 51 years.
2.5:1
Female to Male Ratio
Like other migraine types, hemiplegic migraine affects women more frequently than men.
Understanding MUMS (Migraine with Unilateral Motor Symptoms)
While MUMS is not formally recognized in current headache classification systems, it has significant clinical implications due to high rates of stroke misdiagnosis and substantial disability burden.
What is "Give-Way Weakness"?
Give-way weakness is the pathognomonic (characteristic) feature of MUMS that distinguishes it from true hemiplegic migraine. During strength testing, the patient initially demonstrates normal power but then suddenly "gives way" or releases resistance when the examiner applies pressure.
How it differs from true weakness:
- True weakness (hemiplegic migraine): Consistent reduced strength throughout the test, gradual fatigue, follows neuroanatomical patterns
- Give-way weakness (MUMS): Sudden loss of resistance after initially normal power, characteristic pattern specific to MUMS
Clinical significance: This pattern indicates a distinct migraine subtype with unique pathophysiology rather than hemiplegic migraine, which changes both diagnostic approach and treatment strategies.
MUMS Clinical Characteristics
MUMS affects patients in their mid-30s on average (range 14-59 years) and shows a clear female predominance. The condition demonstrates several key features that distinguish it from hemiplegic migraine:
100%
Give-Way Weakness
All MUMS patients demonstrate this functional weakness pattern on examination
58%
Persistent Weakness
Experience weakness between attacks, more common in chronic migraine patients
38%
Stroke Misdiagnosis
Initially told they had a stroke despite normal imaging
MUMS vs Hemiplegic Migraine: Key Differences
MUMS Features
- Weakness pattern: Give-way weakness (distinct subtype)
- Age of onset: Mid-30s average
- Genetics: No known genetic basis
- Imaging: Always normal
- Recovery: Often incomplete between attacks
- Associated features: High rates of chronic migraine
Hemiplegic Migraine Features
- Weakness pattern: True neurological weakness
- Age of onset: Often childhood/adolescence
- Genetics: Known ion channel mutations
- Imaging: May show transient changes
- Recovery: Usually complete between attacks
- Associated features: May have cerebellar signs
Clinical Recognition of MUMS
MUMS should be suspected when patients present with:
- Subjective arm weakness with objective arm AND leg involvement
- Give-way weakness pattern on examination
- Normal brain imaging despite concerning symptoms
- High healthcare utilization with repeated stroke workups
- "Avalanche" or "tsunami" symptom onset rather than orderly progression
- Symptoms that can occur without headache pain
Why MUMS is Called "Super Migraine"
MUMS has earned the colloquial term "super migraine" for several reasons:
- Symptom intensity: More severe light sensitivity, nausea, and scalp sensitivity than typical migraine
- Additional features: More frequent eyelid drooping, eye redness, and tearing on the headache side
- Duration: Symptoms can last from minutes to months, far exceeding typical aura duration
- Complexity: Multiple neurological symptoms occurring simultaneously rather than in sequence
- Association with chronic migraine: Often occurs in patients with high-frequency migraine patterns
Historical Development of MUMS Understanding
Original research (Dr. William Young, 2007): Study of 24 patients with normal MRIs during severe migraine attacks who developed unilateral weakness. All patients shared similar patterns distinct from traditional hemiplegic migraine.
Key methodology: Compared patients with MUMS to those with equally severe migraine without motor symptoms to identify distinguishing features.
Major findings: MUMS patients had more severe light sensitivity, nausea, scalp sensitivity, eyelid drooping, eye redness, and tearing - essentially "super migraine" characteristics.
Clinical impact: Recognition that many neurological symptoms accompanying migraine may not fit traditional aura definitions but constitute important migraine subtypes requiring different treatment approaches.
Understanding Aura and Brain Mechanisms
To understand motor migraines, it's essential to grasp what happens in the brain during aura. The traditional view that aura is caused by blood vessel constriction followed by dilation has been largely replaced by our understanding of cortical spreading depression.
Cortical Spreading Depression Explained
What it is: A slow-moving electrical wave that travels across the brain's surface, causing temporary loss of normal brain activity followed by recovery.
How it manifests: Creates the classic "marching" pattern of aura symptoms as the wave moves through different brain regions - visual cortex first (causing visual aura), then sensory cortex (numbness/tingling), then motor cortex (weakness).
Time course: The wave moves slowly, which explains why aura symptoms develop gradually over 5-60 minutes rather than appearing suddenly like stroke symptoms.
In animals: This phenomenon can be directly observed in laboratory animals and correlates with migraine preventive medications that reduce the likelihood of triggering these waves.
Visual Aura Patterns
Classic visual auras include the "scintillating scotoma" or "fortification spectrum" - characterized by shimmering zigzag lines that start small near the center of vision and expand outward over 10-30 minutes.
Less Common Aura Types
- Alice in Wonderland syndrome: Distorted perception where objects appear closer/farther than they are, or feeling that one's own body is misshapen
- Olfactory auras: Unusual smells that aren't actually present
- Gustatory auras: Strange tastes
- Déjà vu/jamais vu: Feelings of familiarity or unfamiliarity
- Delirium-like states: Brief periods of confusion or altered consciousness
Late-Life Migraine Accompaniments
Some patients, particularly older adults, experience aura-like symptoms without significant headache. These "migraine equivalents" can be confused with stroke and often prompt emergency room visits. They feature:
- Visual or sensory symptoms that spread like typical aura
- Episodes lasting 15-20 minutes each
- Tendency to cluster over days or weeks, then disappear for months
- Little to no headache pain
- Normal brain imaging and stroke workup
Hemiplegic Migraine: Types and Genetics
Hemiplegic migraine is classified into familial and sporadic forms, with the familial type further subdivided based on genetic mutations. Understanding these genetic foundations helps guide diagnosis and treatment decisions.
Familial Hemiplegic Migraine Type 1 (FHM1)
Gene: CACNA1A (chromosome 19p13)
Function: Encodes alpha-1A subunit of P/Q-type calcium channel
Penetrance: 67-89%
Clinical features: Approximately 50% have cerebellar involvement (gaze-evoked nystagmus, ataxia, vermian atrophy). Some families have severe attacks with coma and prolonged hemiplegia.
Associated conditions: Spinocerebellar ataxia type 6, episodic ataxia type 2, benign paroxysmal torticollis of infancy
Special consideration: S218L variant associated with severe phenotype - mild head trauma can trigger attacks with prolonged hemiplegia, coma, cerebral edema, or death
Familial Hemiplegic Migraine Type 2 (FHM2)
Gene: ATP1A2 (chromosome 1q23)
Function: Encodes catalytic subunit of sodium/potassium ATPase
Penetrance: 63-87%
Clinical features: Less well-defined than FHM1, but different variants linked to frequent long-lasting hemiplegia, recurrent coma, or seizures with intellectual disability. Some have cerebellar involvement.
Associated conditions: Migraine with brainstem aura, various epilepsy syndromes, rarely alternating hemiplegia of childhood
Genetic overlap: Some ATP1A2 variants cause both FHM2 and alternating hemiplegia of childhood
Familial Hemiplegic Migraine Type 3 (FHM3)
Gene: SCN1A (chromosome 2q24)
Function: Encodes transmembrane alpha subunit of brain sodium channel
Penetrance: 100%
Clinical features: Only a few families reported. Phenotype includes epilepsy. Novel feature: "elicited repetitive daily blindness" - stereotyped spells of blindness (usually bilateral) lasting up to 30 seconds, triggered by eye rubbing, light changes, or standing.
Associated conditions: Multiple epilepsy syndromes, febrile seizures
Unique feature: Visual symptoms can be triggered by specific actions, distinguishing it from other FHM types
Emerging Genetic Findings
Recent research has identified PRRT2 variants in 17% of hemiplegic migraine patients who don't have mutations in the three established genes. While not an ion channel gene, PRRT2 encodes a protein that interacts with SNAP25, which may regulate voltage-gated calcium channels.
Sporadic Hemiplegic Migraine
Patients who are the first in their family to develop hemiplegic migraine have sporadic hemiplegic migraine. However, up to 20% of sporadic cases actually have mutations in the known familial genes, due to either de novo mutations or inheritance from an asymptomatic parent.
In severe sporadic cases with additional neurological symptoms, the frequency of identifiable genetic variants may be much higher.
Clinical Features and Symptoms
Hemiplegic migraine attacks are complex events characterized by multiple types of aura symptoms that evolve over time. Understanding the typical progression helps distinguish these attacks from other serious conditions like stroke.
Diagnostic Criteria (ICHD-3)
Required features:
- At least two attacks fulfilling criteria for migraine with aura
- Aura consisting of both fully reversible motor weakness AND fully reversible visual, sensory, and/or speech symptoms
- Not better accounted for by another diagnosis
Additional criteria for familial type: At least one first- or second-degree relative with hemiplegic migraine attacks
Typical Attack Progression
Motor aura is the hallmark of hemiplegic migraine, but it's rarely the only type of aura present. Each attack typically includes two or more different aura types that evolve over 20-30 minutes, usually in this sequence:
Visual Aura
Usually begins first with scintillating scotoma or visual field defects
Sensory Symptoms
Numbness and paresthesia, often starting in the hand
Motor Weakness
Unilateral weakness spreading from hand to arm to face
Speech/Language
Aphasia or speech difficulties may occur
Brainstem Symptoms
Vertigo, dysarthria, ataxia, or consciousness changes
Headache
Usually develops during or after aura symptoms
Attack Characteristics
- Frequency: Mean of 3 attacks per year, but ranges from a few per lifetime to 250 per year
- Duration: 41-58% of patients have auras lasting over 60 minutes; 2-8% last 24 hours or more; rarely up to 4 weeks
- Triggers: Stress, bright light, intense emotions, sleep changes, exertion, mild head trauma, conventional angiography, regadenoson (cardiac stress test agent)
- Laterality: May switch sides between attacks; bilateral motor signs occur in up to one-third of patients
- Age pattern: Attack frequency often decreases after age 50; may evolve into typical migraine without weakness
Ordered vs Avalanche Progression
Hemiplegic migraine (ordered progression): Symptoms follow a predictable sequence as cortical spreading depression moves across brain regions. Typically: visual aura → sensory symptoms → motor weakness → speech/language problems → brainstem symptoms. Each phase lasts 5-45 minutes before transitioning to the next.
MUMS (avalanche progression): Multiple symptoms erupt simultaneously in rapid succession, like a "tsunami" or "avalanche." Numbness, weakness, speech problems, and other symptoms may all begin within 30 seconds of each other, creating overwhelming symptom complexity.
Detailed Symptom Progression
Motor symptoms most often start in the hand and gradually spread up into the arm and then the face. The degree of weakness can vary from mild to severe, and the unilateral features may switch sides between or during attacks.
Visual aura typically begins first and can include scintillating scotoma, visual field defects, or other typical migraine visual symptoms.
Sensory symptoms include numbness and paresthesia, often following the same distribution as the motor weakness.
Speech and language symptoms can include aphasia or dysarthria, which may be particularly concerning and stroke-like.
Brainstem symptoms (similar to migraine with brainstem aura) may include vertigo, dysarthria, ataxia, bilateral visual or sensory symptoms, hyperacusis, tinnitus, and diminished consciousness.
Neurological Examination Findings
During Attacks
- Motor findings: Unilateral weakness affecting upper more than lower limbs
- Reflexes: May find Babinski sign or unilateral hyperreflexia
- Sensory changes: Decreased sensation in same distribution as weakness
- Speech: Possible aphasia or dysarthria
- Consciousness: Can range from alert to comatose in severe cases
Between Attacks (Interictal Period)
- FHM1: Majority have cerebellar findings including gaze-evoked nystagmus, dysarthria, gait or limb ataxia
- FHM2: Minority have cerebellar findings
- FHM3: Limited data, but may have epilepsy-related findings
- Most patients: Otherwise normal neurological examination
Warning: Severe Attacks
Some patients experience severe attacks with encephalopathy, coma, seizures, fever, or prolonged weakness lasting days to months. These occur in 1-33% of patients depending on the genetic subtype and require immediate medical attention. In rare cases, severe attacks can lead to permanent brain injury or death.
Diagnosis and Evaluation
Diagnosing motor migraines requires careful clinical evaluation to distinguish between hemiplegic migraine, MUMS, and more serious conditions like stroke. The key is recognizing the characteristic features of each condition while establishing the presence of migraine-related symptoms.
Distinguishing Physical Exam Findings in MUMS from Hemiplegic Migraine
Critical Examination Findings
MUMS examination:
- Give-way weakness affecting at least two sites on the affected side
- Sudden loss of resistance during strength testing
- Better functional performance than predicted by formal testing
- Characteristic weakness pattern specific to MUMS
Hemiplegic migraine examination:
- Consistent true weakness following neuroanatomical patterns
- May have Babinski sign or hyperreflexia during attacks
- Weakness corresponds to degree of functional impairment
- May have associated cerebellar signs (especially FHM1)
When to Suspect Each Condition
Suspect Hemiplegic Migraine When:
- Episodic attacks of true unilateral weakness with other aura symptoms
- Young patient with stroke-like symptoms that resolve completely
- Family history of similar attacks
- Gradual spread of symptoms over minutes
- Associated typical migraine features
- Consistent weakness pattern on examination
Suspect MUMS When:
- Give-way weakness on examination
- Patient reports arm weakness but exam shows arm AND leg involvement
- High healthcare utilization with normal imaging
- Chronic migraine pattern with motor complaints
- Persistent weakness between migraine attacks
Red Flags Requiring Immediate Evaluation
- Sudden onset of severe symptoms (possible stroke)
- Persistent neurological deficits
- Fever, altered consciousness, or seizures
- First episode in someone over 50
- Progressive worsening rather than gradual resolution
Diagnostic Testing
Brain imaging (MRI preferred over CT) is recommended for:
- First episode or atypical symptoms
- Prolonged attacks or incomplete recovery
- Patients without established diagnosis
Genetic testing may be helpful for:
- Sporadic cases with severe symptoms
- Familial cases where symptoms differ between family members
- Confirming diagnosis when clinical picture is unclear
Distinguishing from Stroke
Hemiplegic migraine: Gradual onset over minutes, multiple aura types, complete recovery, positive symptoms (scintillations, tingling), migrainous features
Stroke: Sudden onset, negative symptoms (numbness, weakness), may have persistent deficits, less likely to have migrainous features
Exception: Cervical artery dissection can mimic hemiplegic migraine with gradual onset and migrainous features
Imaging Findings During Attacks
Brain imaging during hemiplegic migraine attacks usually shows normal findings, but several patterns may be observed:
Possible Imaging Abnormalities During Attacks
- Cortical edema and enhancement: May appear contralateral to the hemiparesis
- Hyperperfusion: Seen on perfusion-weighted MRI or SPECT imaging
- Hypoperfusion: May occur initially, followed by hyperperfusion
- Vasodilation or vasoconstriction: Visible on angiography (avoid conventional angiography as it can trigger attacks)
- Prominent cerebral veins: Transient finding on susceptibility-weighted MRI corresponding to neurologic deficits
Chronic Imaging Changes
FHM1 patients: Frequently show chronic cerebellar atrophy
Severe cases: May develop cortical hemispheric atrophy, cortical laminar necrosis, or global/regional hypometabolism on PET scanning
Important: These chronic changes are uncommon and usually only seen in patients with severe, recurrent attacks
MUMS Imaging Characteristics
Brain imaging in MUMS: Always normal, even during acute attacks
Clinical significance: The contrast between dramatic clinical symptoms and consistently normal imaging is a key diagnostic feature of MUMS
Recommendation: Brain MRI should be performed for first episodes to exclude structural causes, but repeated imaging is usually unnecessary once MUMS is diagnosed
Example Case Study: MUMS Misdiagnosed as Stroke
Patient: 62-year-old woman with history of migraine with aura in teens that resolved in her 40s
New symptoms (5 years duration): Left-sided headache with nausea and light sensitivity, followed by facial numbness spreading to arm and leg, simultaneous left arm and leg weakness, speech difficulties, eyelid drooping, and severe associated migraine symptoms
Attack pattern: Unlike typical hemiplegic migraine, pain preceded numbness and weakness, and numbness/weakness occurred simultaneously rather than sequentially
Clinical course: 27 total episodes, hospitalized 17 times for "stroke," required 2 weeks of rehabilitation each time for weakness to resolve, completely normal brain imaging
Key finding: Give-way weakness on examination rather than true neurological weakness
Treatment success: Once taught to self-administer injectable migraine medication (Compazine), never required hospitalization again and attacks became much milder
Hormonal Considerations and Birth Control
Both hemiplegic migraine and MUMS involve migraine with aura, which creates important considerations for hormonal contraception. The decision about birth control safety requires careful evaluation of individual risk factors.
The Contraception Controversy
The core issue: Migraine with aura is a statistically significant risk factor for vascular events (stroke, heart attack, blood clots). Adding estrogen-containing birth control, which is prothrombotic (increases clotting tendency), may compound this risk.
Why it's controversial: Different medical organizations provide conflicting recommendations, and the actual risk increase is debated among specialists.
Professional Organization Guidelines
American College of Obstetrics and Gynecology (Most Restrictive)
- All hormonal birth control options considered unsafe in migraine with aura
- Recommends avoiding all estrogen-containing contraceptives
- Most conservative approach
American Academy of Neurology/American Headache Society (Moderate)
- Try to limit to lowest estrogen dose possible
- Avoid high-dose estrogen (≥20 micrograms ethinyl estradiol)
- Contraindicated if estrogen provokes or changes aura patterns
- Consider individual risk factors
International Headache Society (Most Liberal)
- Depends on other individual risk factors
- May allow estrogen in patients without additional risks
- Most permissive approach with careful monitoring
Additional Risk Factors to Consider
- Cardiovascular risks: High cholesterol, high blood pressure, diabetes
- Family history: Bleeding or clotting disorders in patient or family
- Smoking: Significantly increases vascular risk
- Age: Risk increases after age 35
- Aura changes: If estrogen provokes or alters aura patterns
Safe Contraceptive Options
Generally considered safe for motor migraine patients:
- Progesterone-only options: Pills, injections, implants
- IUDs: Both hormonal (progesterone) and non-hormonal (copper)
- Barrier methods: Condoms, diaphragms, cervical caps
- Non-hormonal: Fertility awareness methods, sterilization
Key point: Progesterone is not associated with increased clotting risk - only estrogen-containing contraceptives raise concerns.
Living with MUMS: A Patient Perspective
Understanding the real-world impact of MUMS helps healthcare providers and families appreciate the complexity of this condition. The following insights come from Cannon Hodge (@migrainebabe), a patient advocate living with MUMS, and highlight both challenges and coping strategies.
Symptom Patterns and Progression
Typical attack sequence: "It comes on like an avalanche or tsunami - hand goes numb, 10 seconds later speech begins to slur, 10 seconds later can't hold up head, 10 seconds after that face begins to freeze and lip tugs down. All within about 30 seconds."
Variable duration: Symptoms can last anywhere from a few minutes to several months, with three months being reported as the longest duration for some symptoms.
Persistent baseline symptoms: Many patients experience ongoing paresthesia (tingling/numbness) and weakness between attacks, particularly on the dominant side.
Comprehensive Symptom Profile
Motor Symptoms
Right-sided weakness (arm predominantly), neck weakness causing head drop, leg weakness, full-body paralysis in severe attacks
Facial Involvement
Facial paralysis, facial droop, inability to open mouth during severe attacks, dysphasia (swallowing difficulties)
Speech and Language
Dysarthria, stuttering, aphasia, complete inability to speak during severe episodes
Sensory Features
Persistent paresthesia, blurred vision, visual phenomena (fairy lights, visual snow), vertigo and dizziness
Associated Symptoms
Ataxia and clumsiness, nausea and vomiting, tinnitus, allodynia (a hallmark MUMS symptom)
Severe Episodes
Complete body paralysis except left hand, inability to sit up or support torso, episodes lasting hours to months
Key Distinguishing Features from Patient Experience
-
Proprioception issues:
- "When I lift my arm, it feels like someone's pushing down on it"
- "I can slowly try to coax movement from toe up, but my legs still will feel like sand"
- Inconsistent patterns: "Every attack is different - rarely follows the same formula"
- Allodynia prominence: Severe skin sensitivity as a consistent feature
- Functional preservation: Left hand often remains functional even during severe attacks
- Jazz-like variability: Symptoms change and flow unpredictably, unlike the ordered progression of hemiplegic migraine
Practical Coping Strategies
Communication Tools
- Pre-written phone messages: Bulleted messages like "Can you take me to a seat," "Can you help me get on the bus," with header explaining "I'm having a migraine attack, you don't need to call an ambulance"
- Point-and-communicate system: During speech difficulties, can point to needed message for others to read
- Medical alert devices: Button-activated emergency communication for severe attacks with paralysis
Self-Management Techniques
- Breath work: Progressive muscle relaxation and controlled breathing for maintaining control during overwhelming symptoms
- Education: Learning everything possible about MUMS to reduce fear and improve understanding
- Independence maintenance: Continuing to live independently and make plans despite unpredictable symptoms
- Medication preparedness: Quick access to rescue medications when early symptoms appear
"Once you understand the layers of what's happening to your body, it becomes less scary. When you can't move your body, at least you can control how you breathe, and that gives you better control when everything else is spinning out of control."
Treatment and Management
Treatment for hemiplegic migraine focuses on both acute attack management and prevention. The approach varies based on attack frequency, severity, and whether the patient has familial or sporadic disease.
| Treatment Category | Medication | Detailed Dosing & Evidence |
|---|---|---|
| Frequent/Prolonged Aura (First-Line) | Verapamil (sustained-release) |
Dosing: Start 120 mg once daily → 120 mg twice daily → 120 mg three times daily (max 360 mg/day for most patients, 120 mg/day max for small/elderly patients) Evidence: Case series showed complete resolution in 50% within first month Monitoring: Blood pressure, heart rate, constipation, peripheral edema Most commonly used and effective for hemiplegic migraine |
| Headache-Predominant (First-Line) | Flunarizine |
Dosing: 2.5-5 mg once daily (evening), titrate to 10mg/day as needed Evidence: Study of 13 children showed ≥50% reduction in attack frequency in 85% Availability: Unavailable in United States Highly effective when available |
| Alternative Headache Treatment | Topiramate |
Dosing: Start 25 mg daily, increase by 25-50 mg weekly to max 100 mg BID Rationale: Standard migraine preventive, subset of migraine with aura Good alternative when flunarizine unavailable |
| Alternative Headache Treatment | Amitriptyline |
Dosing: Start 10 mg at bedtime, increase weekly up to 50 mg at bedtime Rationale: Standard migraine preventive with additional pain modulation Consider for comorbid depression or sleep issues |
| Familial Hemiplegic Migraine (First-Line) | Acetazolamide |
Dosing: 250 mg twice daily Mechanism: Particularly effective for channelopathies colocalized with FHM1 Evidence: Effective in related conditions (hypokalemic periodic paralysis, episodic ataxia type 2) Side effects: Paresthesias, taste changes, kidney stones (rare) Specifically targets ion channel dysfunction |
| Refractory Aura-Predominant | Lamotrigine |
Dosing: Start 25 mg daily, slow titration in 25 mg steps weekly/biweekly to 100 mg daily (max 300 mg studied) Evidence: Case series of 47 patients with migraine aura, including 2 with hemiplegic migraine; prospective study of 59 patients showed reduced aura intensity Critical Warning: Rash in up to 10%, Stevens-Johnson syndrome risk (~1 in 1000 adults) Contraindication: Age <16 years (dramatically increased Stevens-Johnson risk) Discontinue immediately for any rash - difficult to distinguish benign from life-threatening |
| Acute Aura Management | Intranasal Ketamine |
Evidence: Reproducible decrease in aura intensity and duration in 5 of 11 FHM patients when given at attack onset Administration: Must be given at very early symptom onset Specialized use for acute aura interruption |
| Refractory Prevention | OnabotulinumtoxinA |
Protocol: Injections every 12 weeks Evidence: Case series of 11 patients (4 FHM, 7 sporadic) showed reduction in frequency and severity of pain and aura Consider for patients failing first-line therapies |
| CGRP Inhibition (Limited Data) | Galcanezumab, Fremanezumab |
Evidence: Small series of 6 patients - 4 had reduction in weakness days, 2 had increase Note: CGRP infusion failed to induce aura in FHM patients (different role than typical migraine) Mixed results, may work differently than in typical migraine |
| Acute Supportive (Severe Attacks) | IV Naloxone |
Dosing: 0.4 mg IV Evidence: Case report of aura symptom resolution within 2 minutes (did not help headache) Experimental use for severe aura symptoms |
Acute Management of Severe Attacks
Patients with severe hemiplegic migraine attacks may require hospitalization due to fever, depressed consciousness, seizures, or prolonged weakness. Management includes both supportive care and specific interventions:
Supportive Management
- IV hydration: Maintain adequate fluid balance
- Antiemetics: Prochlorperazine 10 mg with diphenhydramine 12.5 mg pretreatment (prevents akathisia)
- Magnesium sulfate: 1-2 g IV (mixed evidence but good safety profile)
- IV opioids: When necessary with careful monitoring
- Monitor for complications: Seizures, cerebral edema, prolonged neurological deficits
Corticosteroids for Severe Attacks
Evidence base: Limited to pediatric case reports, but may be beneficial for resistant severe symptoms
- Methylprednisolone: 100 mg daily for 5 days (pediatric case report)
- Dexamethasone: 0.5 mg/kg daily divided into 3 doses for 3 days, followed by gradual oral tapering
- Combination therapy: Early treatment with pulse corticosteroids plus hypertonic saline showed benefit in case with CACNA1A-related encephalopathy
Experimental Severe Attack Management
Hypertonic saline (3% solution):
- Dosing: 1.5 mL/kg/hour, adjusted to maintain sodium levels 145-155 mEq/L
- Duration: 2 days in reported case
- Indication: Cases with cerebral edema on MRI
- Evidence: Single pediatric case report with sporadic HM and CACNA1A-related encephalopathy
- Outcome: Reduction in seizures and attack duration
MUMS Treatment Approach
Treatment of MUMS requires aggressive migraine management rather than stroke-focused interventions. As a distinct migraine subtype, MUMS often responds well to specialized migraine therapies tailored to its unique characteristics.
MUMS Treatment Principles
- Treat as migraine, not stroke: Focus on migraine preventive and acute therapies
- Patient education: Explain MUMS as a distinct migraine subtype to reduce anxiety
- Avoid repeated testing: Once diagnosed, resist urge for repeated stroke workups
- Address disability: 58% have persistent weakness requiring comprehensive care
- Super-aggressive approach: MUMS requires more intensive treatment than typical migraine
- Quick intervention: Rapid treatment at symptom onset prevents severe progression
Injectable Medications for MUMS
Injectable formulations appear more effective for MUMS than oral medications, possibly due to absorption issues during severe attacks. Options include:
- Metoclopramide (Reglan): Injectable anti-nausea medication with migraine benefits
- Injectable anti-inflammatories: Non-oral NSAIDs for better absorption
- Compazine (prochlorperazine): Can be self-administered by injection during attacks
- Rationale: Bypass potential absorption problems and provide faster relief
| MUMS Treatment Category | Recommended Options | Dosing & Special Considerations |
|---|---|---|
| First-Line Preventive | Verapamil (sustained-release) | 240-480 mg daily. Most commonly used and effective option for MUMS |
| Aura-Predominant Cases | Lamotrigine | 25-400 mg daily, titrated slowly. Particularly effective for severe aura symptoms |
| Refractory Cases | Ketamine Infusions | 0.1 mg/kg/hr titrated over 3-5 days. 75% show significant improvement in specialized protocols |
| Acute Treatment | NSAIDs, Triptans, Ergotamines, Antiemetics, Corticosteroids | Triptans and ergotamines may be used with MUMS as opposed to HM. Intranasal ketamine may reduce aura intensity |
| Alternative Options | Topiramate, Valproic Acid, Amitriptyline | Standard migraine preventive dosing. Used for patients who fail first-line therapy |
Medications Generally Avoided for Hemiplegic Migraine
Triptans and Ergotamines - Complex Considerations
Traditional avoidance of triptans is based on theoretical concerns rather than evidence of harm:
- Historical exclusion: Patients with hemiplegic migraine were excluded from original triptan clinical trials due to theoretical vasoconstriction concerns
- Outdated vascular theory: Avoidance based on old theory that migraine is primarily vascular, now known to be incorrect
- Limited safety evidence exists: Retrospective study of 76 hemiplegic migraine patients found triptans beneficial and safe for headache phase treatment
- No ischemic strokes reported: In the 76-patient series, no permanent neurologic complications occurred
- One prolonged attack: Single patient had prolonged hemiplegic attack persisting months after triptan use
- Current practice varies: Some headache specialists now use triptans selectively for headache phase; others maintain strict avoidance
Ergotamines have stronger contraindication evidence:
- Case reports of serious complications: Cardiac arrest, cerebral infarcts, spinal cord infarcts
- Increased ischemic risk: Netherlands case-control study showed ergotamine overuse as risk factor for ischemic complications (OR 2.55, 95% CI 1.22-5.36)
- Stroke concern: Some patients with hemiplegic migraine symptoms may actually have acute ischemic stroke; vasoconstrictive medications could worsen cerebral ischemia
Beta Blockers and IV Dihydropyridine Calcium Channel Blockers
Avoided by some specialists due to theoretical concerns:
- Beta blocker concerns: May potentially prolong symptoms or limit compensatory cerebral vasodilatory capacity
- IV dihydropyridine blockers: May lead to cerebral hypoperfusion
- Case report: IV nimodipine possibly provoked seizure during prolonged FHM2 attack
- Evidence limitation: Theoretical concerns not well-supported by clinical evidence
MUMS-Specific Considerations
Theoretical triptan and ergot concerns for hemiplegic migraine do not apply to MUMS. However, clinical experience with triptans in MUMS is extremely limited.
Detailed Treatment Monitoring and Decision-Making
Verapamil Monitoring Protocol
Titration schedule:
- Week 1-2: 120 mg sustained-release once daily
- Week 3-4: 120 mg twice daily (total 240 mg daily)
- Week 5+: 120 mg three times daily (total 360 mg daily) if needed
Maximum dosing:
- Most patients: 360 mg daily maximum
- Small/elderly patients: 120 mg daily maximum
Required monitoring:
- Blood pressure: Check before each dose increase
- Heart rate: Monitor for bradycardia
- Side effects: Constipation (most common), peripheral edema
Expected response: Some patients achieve complete resolution within first month
Lamotrigine Safety Monitoring
Critical safety protocol:
- Starting dose: 25 mg daily only
- Titration: Increase by 25 mg steps weekly or biweekly (slow titration essential)
- Target dose: 100 mg daily if needed (studied up to 300 mg daily)
- Age restriction: Contraindicated under age 16 due to dramatically increased Stevens-Johnson syndrome risk
Rash monitoring:
- Frequency: Up to 10% develop rash in first 1-2 months
- Action required: Immediate discontinuation for ANY rash
- Clinical challenge: Difficult to distinguish benign from life-threatening rash initially
- Stevens-Johnson risk: ~1 in 1000 adults, much higher in children
Treatment Decision Algorithm
Choose initial therapy based on:
- Frequent/prolonged aura predominant: Start verapamil
- Headache pain predominant: Start flunarizine (if available), topiramate, or amitriptyline
- Familial hemiplegic migraine: Consider acetazolamide first
- Attack frequency: Higher frequency favors daily preventive therapy
- Attack severity: More severe attacks warrant more aggressive prevention
- Patient preferences: Regarding daily medication for intermittent symptoms
- Comorbid conditions: Choose agents that may benefit other conditions
Prognosis and Long-term Outlook
The prognosis for hemiplegic migraine is generally favorable, but varies significantly based on genetic subtype, attack severity, and presence of complications. Understanding prognostic factors helps guide both treatment decisions and patient counseling.
Complete Recovery
Nearly all patients recover completely from individual attacks, though symptoms may be prolonged for hours to days
Age-Related Changes
Attack frequency often decreases after age 50; attacks may evolve into typical migraine without motor symptoms
Severe Complications
Permanent deficits, cognitive decline, or death occur rarely, mostly in patients with severe attacks involving coma or seizures
Prognostic Factors by Genetic Subtype
FHM1 Prognosis
- Severe attacks: 33% in some studies, often with coma and prolonged hemiplegia
- Cerebellar signs: Majority develop interictal cerebellar findings
- S218L variant: Particularly severe - mild trauma can trigger life-threatening attacks
- Recovery: Usually complete, but may take longer than other subtypes
FHM2 Prognosis
- Variable phenotype: Different variants cause different patterns
- Some variants: Associated with frequent, long-lasting hemiplegia
- Intellectual disability: Rare, but reported with seizures in some families
- Cerebellar involvement: Less common than FHM1
FHM3 Prognosis
- Limited data: Only few families reported
- Epilepsy risk: Higher than other subtypes
- Unique features: Elicited repetitive daily blindness
- Overall prognosis: Appears similar to other subtypes
Factors Associated with Better Prognosis
Positive Prognostic Indicators
- Typical attack pattern without severe features (coma, seizures, fever)
- Good response to preventive medications
- Absence of frequent prolonged attacks (>24 hours)
- No history of encephalopathy during attacks
- Normal interictal neurological examination
- Attacks that completely resolve without residual deficits
Risk Factors for Complications
- Early onset: Patients with severe attacks beginning in childhood
- Recurrent severe attacks: Multiple episodes with coma, seizures, or prolonged weakness
- Specific genetic variants: Particularly CACNA1A S218L variant
- Head trauma sensitivity: Patients whose attacks are triggered by minor head trauma
- Incomplete recovery: Attacks that don't resolve completely between episodes
Long-term Outcomes and Complications
Rare but Serious Complications
Permanent brain injury: Reported in patients with severe, recurrent attacks
Cerebral infarction: Very rare, usually in context of extremely severe attacks
Cognitive decline: May occur with repeated severe episodes
Death: Extremely rare, associated with severe attacks with cerebral edema
Chronic cerebellar atrophy: Common in FHM1, usually not progressive
MUMS Prognosis and Long-term Outcomes
The prognosis for MUMS differs significantly from hemiplegic migraine due to its distinct characteristics as a migraine subtype, but patients face substantial ongoing challenges that require specialized care.
Persistent Symptoms
58% experience ongoing weakness between attacks, creating substantial functional impairment
Treatment Response
71% believe migraine treatment improves their weakness symptoms
Healthcare Impact
Higher emergency visits and job loss rates compared to typical migraine patients
MUMS Prognostic Factors
Better prognosis associated with:
- Early recognition and appropriate migraine treatment
- Patient understanding of MUMS as a migraine subtype
- Episodic rather than chronic migraine pattern
- Good response to preventive medications
Challenges include:
- High rate of stroke misdiagnosis creating anxiety
- Persistent interictal weakness affecting daily function
- Need for long-term specialized migraine care
- Employment and disability issues
Important Takeaways
Essential Points for Patients and Providers
Hemiplegic Migraine:
- Rare but serious genetic condition - affects 1 in 10,000 people with known ion channel mutations
- True motor weakness is the defining feature - always accompanied by other aura symptoms
- Genetic testing can guide treatment - especially helpful for familial cases and severe sporadic cases
- Treatment is individualized - based on attack pattern, genetics, and patient factors
- Prognosis is generally good - most patients recover completely with appropriate management
MUMS (Migraine with Unilateral Motor Symptoms):
- Give-Way Weakness - This distinguishes from true weakness
- Often misdiagnosed as stroke - despite consistently normal brain imaging
- Aggressive migraine treatment works - 71% improve with proper migraine management
- Persistent symptoms common - 58% have ongoing weakness between attacks
- Patient education crucial - understanding functional nature reduces anxiety and healthcare utilization
When to Seek Immediate Medical Care
- First episode of weakness and neurological symptoms (either condition)
- Symptoms lasting longer than usual or not resolving
- Fever, seizures, or altered consciousness during an attack
- New or different symptoms compared to previous attacks
- Sudden onset weakness (possible stroke - requires emergency evaluation)
Both hemiplegic migraine and MUMS require specialized care and ongoing monitoring. If you suspect you or a family member may have either condition, work with a neurologist or headache specialist experienced in managing complex migraine disorders. The key is accurate diagnosis to ensure appropriate treatment and avoid unnecessary testing or inappropriate stroke treatments.
The Need for Subspecialty Care
Both conditions are complex enough to require care beyond general neurology:
- Primary care limitations: Most primary care physicians are not familiar with hemiplegic migraine or MUMS
- General neurology gaps: Even neurologists may not be familiar with the detailed literature on these conditions
- Headache specialist expertise: Subspecialty headache care is often necessary for optimal diagnosis and treatment
- Importance of experience: Choose providers who have diagnosed and treated these conditions before
- Adequate communication: Ensure your doctor can answer questions appropriately and understands the nuances
Important Disclaimer
This information is for educational purposes only and should not replace professional medical advice.
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