Overview
Definition:
Status migrainosus (SM) in adolescents is defined as a severe migraine attack that lasts for more than 72 hours, or recurrent attacks within 72 hours with incomplete recovery between attacks
It represents a disabling neurological event requiring prompt and aggressive management.
Epidemiology:
While precise incidence data for adolescents in ED settings is limited, migraine is common in this age group, with prevalence estimated between 5-15%
A subset of these will experience SM, posing a significant burden on emergency services
Peak onset is typically in adolescence for both episodic and chronic migraine.
Clinical Significance:
Status migrainosus in adolescents is not only a cause of significant suffering and functional impairment but also raises concern for secondary causes of headache
Effective ED management is crucial to break the cycle of pain, prevent dehydration and exhaustion, and reduce the need for hospitalization, thereby improving patient outcomes and optimizing ED resource utilization.
Clinical Presentation
Symptoms:
Prolonged, severe, unilateral or bilateral throbbing headache
Nausea and/or vomiting
Photophobia and phonophobia are usually present
Some adolescents may also exhibit aura symptoms such as visual disturbances, sensory changes, or speech difficulties
Significant functional impairment, leading to inability to attend school or participate in normal activities.
Signs:
Patients may appear distressed, tired, and dehydrated
Vital signs can be normal or show mild tachycardia due to pain and anxiety
Neurological examination is typically non-focal, but can reveal mild photophobia or phonophobia
Signs of dehydration may include dry mucous membranes and decreased skin turgor
Rule out meningeal signs (nuchal rigidity, Kernig's, Brudzinski's) to exclude meningitis.
Diagnostic Criteria:
Diagnosis is primarily clinical, based on the International Classification of Headache Disorders (ICHD-3) criteria for Status Migrainosus
Key components include: A) Headache fulfilling criteria for migraine without aura or migraine with aura
B) Headache lasting >= 72 hours
C) Current attack is debilitating
D) No other diagnosis better explains the symptoms
The ED setting requires a rapid assessment to exclude red flags and initiate appropriate treatment.
Diagnostic Approach
History Taking:
Detailed headache history: onset, duration, frequency, character, location, severity, associated symptoms (nausea, vomiting, photophobia, phonophobia, aura)
Previous headache history and treatment response
Triggers
Family history of migraine
Review of systems to identify potential secondary causes
Red flags: sudden onset (thunderclap), fever, stiff neck, focal neurological deficits, papilledema, altered mental status, new headache in patients <3 years old, or significant change from usual pattern.
Physical Examination:
Complete neurological examination: assess mental status, cranial nerves (especially visual acuity, pupillary response, extraocular movements, facial sensation and symmetry), motor strength, sensation, reflexes, and coordination
Fundoscopy to check for papilledema
Palpate head and neck for tenderness
Assess for meningeal irritation
Thorough general physical examination to identify signs of infection or systemic illness.
Investigations:
Laboratory investigations are generally not indicated for typical SM unless red flags suggest an alternative diagnosis
If infection is suspected, CBC, ESR, CRP, and blood cultures may be considered
Lumbar puncture (LP) for CSF analysis (cell count, differential, protein, glucose, Gram stain, bacterial cultures) is indicated if meningitis or encephalitis is suspected
Neuroimaging (CT head without contrast initially, MRI brain with and without contrast if indicated) is warranted if red flags are present, such as focal neurological deficits, altered mental status, or suspicion of intracranial pathology like hemorrhage, tumor, or venous sinus thrombosis
EEG may be considered in cases of prolonged altered mental status or suspected seizures.
Differential Diagnosis:
Other causes of severe, persistent headache in adolescents include: Tension-type headache (less severe, bilateral, non-pulsating, no nausea/vomiting), Cluster headache (severe, unilateral orbital/supraorbital pain with autonomic features, shorter duration), Meningitis/Encephalitis (fever, nuchal rigidity, altered mental status), Intracranial hemorrhage (sudden onset, focal deficits), Brain tumor (progressive, associated with neurological deficits, papilledema), Cerebral venous sinus thrombosis (variable presentation, often with focal deficits), Carbon monoxide poisoning, Medication overuse headache (often associated with frequent analgesic use), Sinusitis (facial pain, purulent nasal discharge), Ocular pathology (glaucoma, uveitis).
Management
Initial Management:
Immediate assessment for airway, breathing, and circulation (ABCs)
Establish intravenous (IV) access for hydration and medication administration
Pain control is paramount
Sedation may be required for severely agitated or distressed patients
Provide a quiet, dark environment.
Medical Management:
First-line: IV fluids to correct dehydration
Antiemetics: IV ondansetron or metoclopramide are often effective for nausea and vomiting, and may also have some antimigraine effects
Analgesics: IV NSAIDs (e.g., ketorolac 30 mg or ibuprofen 400-800 mg) or IV acetaminophen (paracetamol) 15-20 mg/kg
Opioids (e.g., IV morphine or hydromorphone) should be used cautiously and sparingly as second-line agents due to risk of exacerbating nausea/vomiting and potential for medication overuse headache
Triptans: Subcutaneous sumatriptan may be considered in severe cases refractory to initial treatment, but caution is advised due to potential for rebound headache
Dihydroergotamine (DHE) can be effective but is less commonly used in adolescents in the ED due to potential side effects and route of administration
Corticosteroids: IV methylprednisolone (1-2 mg/kg, max 125 mg) or dexamethasone may be considered for refractory cases, though evidence is mixed
Anticonvulsants: IV valproic acid (20-30 mg/kg over 30-60 min, max 1500 mg) or IV levetiracetam (20 mg/kg, max 1500 mg) can be effective in breaking the SM cycle, especially if there are associated neurological symptoms or a history of refractory migraine.
Surgical Management:
Surgical management is not indicated for status migrainosus
The focus is on medical management and addressing underlying causes if present.
Supportive Care:
Continuous monitoring of vital signs, pain levels, hydration status, and neurological examination
Encourage rest in a quiet, dark room
Dietary modifications may be necessary if vomiting persists
Patient and family education on migraine triggers, abortive and prophylactic treatments, and importance of adherence to follow-up.
Complications
Early Complications:
Refractory pain despite treatment
Severe dehydration and electrolyte imbalance
Exhaustion and sleep deprivation
Functional impairment leading to prolonged absence from school and social activities
Risk of medication side effects
Anxiety and depression related to chronic pain.
Late Complications:
Transition to chronic migraine
Development of medication overuse headache if analgesic use is frequent
Increased risk of psychiatric comorbidities (anxiety, depression)
Long-term functional disability
School failure or underachievement.
Prevention Strategies:
Identification and avoidance of migraine triggers
Regular sleep schedule
Stress management techniques
Regular physical activity
Adherence to prescribed prophylactic medications if indicated
Prompt treatment of episodic migraines to prevent progression to SM
Patient and family education on recognizing early signs of worsening migraine.
Prognosis
Factors Affecting Prognosis:
Severity and duration of the attack
Promptness and adequacy of ED treatment
Presence of comorbid conditions (e.g., psychiatric disorders)
Response to initial medical therapy
Adherence to long-term management plan.
Outcomes:
With effective ED management, most adolescents with status migrainosus will experience significant pain relief and recovery within 24-48 hours
However, recurrence is common
Long-term prognosis depends on effective migraine management strategies and prevention of chronic migraine
Many adolescents can achieve functional recovery and return to their normal activities.
Follow Up:
Close follow-up with a pediatrician, neurologist, or headache specialist is essential after ED discharge
This should include evaluation of migraine triggers, optimization of abortive and prophylactic therapies, and management of any comorbidities
A structured follow-up plan can help prevent future episodes and improve long-term outcomes.
Key Points
Exam Focus:
Recognize SM criteria (>= 72 hrs)
Prioritize IV hydration and antiemetics
Consider IV NSAIDs, valproic acid, or levetiracetam for refractory pain
Always rule out secondary causes with careful history and examination
neuroimaging and LP are indicated for red flags
Opioids are last resort.
Clinical Pearls:
Aggressive hydration and prompt antiemetic use are critical
Valproic acid or levetiracetam are often more effective than simple analgesics in breaking the SM cycle
Document neurological exam findings meticulously
Educate family on trigger avoidance and prompt treatment of escalating headaches.
Common Mistakes:
Underestimating the severity of SM
Relying solely on oral medications or opioids
Failing to adequately address nausea and vomiting
Delaying neuroimaging or LP when red flags are present
Inadequate follow-up planning post-ED visit.