Headache & Migraine
mastergp.in — The Practising Physician
Primary Headache vs Secondary Headache
The comparison between primary headache and secondary headache revolves around their underlying causes and relative prevalence:
| Feature | Primary Headache | Secondary Headache |
|---|---|---|
| Nature & Cause | Generally considered benign, recurrent, and non-dangerous. They most often have no organic cause. | Described as “pretty different” and are caused by an underlying organic issue, often considered a dangerous headache. |
| Most Common Cause | The single most common cause of primary headache is tension headache. The second most common cause is migraine. | The single most important cause of secondary headache is systemic infections. This category includes conditions such as meningitis, encephalitis, sinusitis, a plain upper respiratory tract infection, or any fever due to a viral illness. |
| Least Common Cause | The least common cause of primary headache is cluster headache. | The least common cause of secondary headache should be answered as brain tumor. Brain tumor is generally considered a very unimportant cause, accounting for less than 0.1%. |
Primary headaches fall under the category of non-dangerous headaches, whereas dangerous headaches are often related to increased intracranial tension (ICT). Primary headaches mainly encompass tension type headache (TTH), migraine, and cluster headache.
Headache Cause — Diagnosis Points
| Headache Cause | Diagnosis Point(s) |
|---|---|
| Subarachnoid Hemorrhage (SAH) | Worst headache of my life or Thunderclap headache. This condition may also be associated with nucal rigidity. |
| Cluster Headache | Excruciating retroorbital pain described as feeling like someone is drilling a hole in the eye with a drill machine. Patients are typically restless or pace around due to the 10/10 pain intensity. |
| Migraine | Headache is generally pulsatile/throbbing in nature and is disabling. Key associated features include photophobia (aversion to light) and phonophobia (sensitivity to sound). |
| Tension Type Headache (TTH) | Described as a holocranial bandlike sensation or compression around the head. Importantly, it does not affect activities of daily living. |
| Giant Cell Arteritis (GCA) | Seen in senior citizens (e.g., 70 years of age) complaining of temporal headache. A highly characteristic associated symptom is jaw claudication. |
| Acute Angle Closure Glaucoma (AACG) | Retroorbital pain and red eye often triggered by conditions involving darkness (e.g., watching a movie), which causes mydriasis and increases intraocular pressure. |
| Trigeminal Neuralgia (TN) | Characterized by unilateral brief electric shock-like pain in the face. The pain is often initiated by a cutaneous trigger. |
Medications to Avoid in Migraine
Several medications and classes of drugs should be avoided or are contraindicated in the treatment of migraine due to their mechanisms of action and potential adverse effects.
| Medication/Class | Conditions to Avoid (Contraindications) | Rationale for Avoidance |
|---|---|---|
| Triptans (e.g., Sumatriptan, Rizatriptan) | Ischemic Heart Disease (IHD)/Coronary Artery Disease (CAD), Prinzmetal’s angina, Uncontrolled Hypertension, Peripheral Arterial Disease (PAD), Prior Strokes/Cerebrovascular Disease (CVD), Basilar Migraine, Hemiplegic Migraine, Pregnancy | Triptans are 5HT 1B/1D agonists that cause vasoconstriction of blood vessels. This effect is dangerous in vascular conditions: • Vasoconstriction in IHD/CAD further reduces blood supply to the heart, potentially leading to angina or myocardial infarction. • Vasoconstriction in systemic vessels can increase blood pressure, worsening uncontrolled hypertension. • In basilar migraine, they are contraindicated because the disease is caused by vasoconstriction of the basilar artery; giving a triptan would worsen the concurrent cranial palsy. • They are contraindicated in pregnancy as they can induce smooth muscle contraction. |
| Ergot Alkaloids (e.g., Dihydroergotamine, Ergotamine) | Ischemic Heart Disease (IHD)/Coronary Artery Disease (CAD), Prinzmetal’s angina, Uncontrolled Hypertension, Peripheral Arterial Disease (PAD), Pregnancy | Ergots are powerful vasoconstrictors that act on various 5HT receptors and alpha-1 receptors. Similar to triptans, they are avoided in vascular disease and pregnancy. • Excessive use (Ergot Toxicity) can lead to severe vasoconstriction, potentially causing digital ulcers or gangrene. |
| Opioids | General migraine treatment | Opioids should generally be avoided in the treatment of migraine. |
A significant risk to avoid is the combination of certain migraine medications with other drugs that increase serotonin levels, which can lead to Serotonin Syndrome.
- Triptans or ergots should be avoided when combined with SSRIs (Selective Serotonin Reuptake Inhibitors) or TCAs (Tricyclic Antidepressants) because this combination increases serotonin concentration and hyper-excitable activity in the sympathetic nervous system.
- Serotonin syndrome presents with symptoms like hypertonia, hyperreflexia, agitation, delirium, hyperthermia, and tachycardia.
Oral triptans are generally not recommended in patients under 18 years of age because they have no proven efficacy in the pediatric group.
Opioids : Morphine, Fentanyl.
Symptoms of Migraine
The symptoms of migraine can be categorized into features related to the headache itself, associated symptoms, and pre-headache neurological phenomena known as aura.
Core Headache Features:
- Nature of Pain: The headache is typically pulsatile or throbbing in nature, which lends credibility to the diagnosis. It can also be described as a pounding headache.
- Severity: Migraine headaches are usually moderate to severe in intensity.
- Location: The pain is generally unilateral (involving one side of the head), though it can sometimes become bilateral or holocranial. Unilateral presentation gives more credibility to the diagnosis.
- Disability: A key feature is that the headache is disabling in character, meaning it affects activities of daily living and can lead the patient to miss work or school. The disability is a factor in deciding the need for prophylaxis.
- Aggravating Factors: The pain is generally aggravated by routine physical activity or movement. Patients often find that neck movements worsen the headache, making them avoid movement, as they feel their head will explode.
Associated Symptoms (Non-Aura):
These features are often used in the diagnostic criteria for migraine without aura:
- Nausea and Vomiting: Nausea is a common symptom, though vomiting is generally rare. Some patients may experience a feeling of “pukishness”.
- Sensory Sensitivities: Patients exhibit aversion to light (photophobia) and sensitivity to sound (phonophobia). Patients with migraine often prefer to turn off all lights, draw curtains, and lie down in a dark, quiet room.
- Irritability and Mood Changes (Prodrome): Headache episodes can be preceded by symptoms such as mood disturbance, irritability, or depressive symptoms for up to two days prior to the onset of the headache.
Aura (Classical Migraine):
Approximately 20% of patients experience classical migraine, which is associated with an aura, while 80% have common migraine, which does not include an aura. Aura is generally a fully reversible neurological symptom that precedes the headache.
- Visual Aura (Most Common): The majority of aura experiences are visual. These can manifest as:
- Visual blurring.
- Zigzag lines in the periphery of vision, often called fortification spectra.
- Flickering spots in the visual fields.
- Scotomas (blind spots) or scintillating scotomas.
- Other Aura Types:
- Sensory symptoms such as paresthesias (tingling) or numbness in a limb.
- Speech disturbances.
- Rarely, auditory aura.
The symptoms of migraine, particularly the disabling nature and sensitivities to light and sound, often cause patients to retreat and rest, which distinguishes it from other primary headaches like cluster headache, where the patient is typically restless and pacing due to the excruciating pain. The duration of a migraine attack can range from 4 to 72 hours.
Think of a migraine like a sudden, severe traffic jam on a usually busy highway (the blood vessels and nervous system in the head). The initial phase (aura) might be like brief, confusing warning signs (visual disturbances) due to temporary lane closures (vasoconstriction). The main headache phase, however, is the full-blown, pounding jam (throbbing pain/vasodilation), which causes intense pressure and makes normal activities (like driving or moving your head) impossible, forcing you to seek a dark, quiet rest stop (relief from photophobia and phonophobia).
IHS Criteria for Migraine Diagnosis
The International Headache Society (IHS) criteria for the diagnosis of migraine, as presented in the sources (often aligned with the information found in Harrison’s textbook), emphasize specific characteristics of the headache and associated symptoms.
For the diagnosis of migraine, particularly migraine without aura (the most common type, representing 80% of cases), the criteria require at least five attacks. For migraine with aura, two or more attacks are required.
The criteria are generally broken down into features of the headache itself and associated symptoms.
I. Headache Characteristics
The headache attack must include at least two of the following features:
- Unilateral location: The pain typically involves one side of the head, though it can become bilateral.
- Pulsating/Throbbing quality: The pain has a pulsatile or throbbing nature.
- Moderate to Severe Intensity: The pain is usually moderate to severe in intensity.
- Aggravated by Routine Physical Activity: The pain increases with routine physical activity or movement, which is why patients prefer to sit quietly.
II. Associated Symptoms
The attack must include at least one of the following associated symptoms:
- Nausea and/or Vomiting: Nausea is common, although vomiting is described as very rarely occurring.
- Photophobia (Aversion to Light) and Phonophobia (Sensitivity to Sound): Patients often seek a dark, quiet room due to sensitivity to light and sound.
III. Migraine with Aura (Classical Migraine)
For migraine with aura, which occurs in about 20% of cases, the diagnosis includes the general headache criteria but also requires specific characteristics of the aura:
- The aura consists of one or more fully reversible neurological symptoms.
- These are usually visual (e.g., zigzag lines, flickering spots, scotomas) but can also be sensory (e.g., tingling, numbness) or speech disturbances.
- The aura symptoms typically precede the headache.
- The aura symptoms should develop gradually and last between 5 to 60 minutes.
- The headache usually begins within 60 minutes of the aura.
It is important that the headache symptoms are not attributable to another disorder. The disability caused by the headache, which is one of the most important features, is also factored into treatment decisions (prophylaxis).
In summary, for a standard migraine diagnosis, a patient must meet certain frequency requirements and display a combination of specific headache qualities (pulsating, unilateral, moderate-to-severe, worsened by activity) and associated features (nausea/vomiting, and/or photo-/phonophobia).
MIDAS — Migraine Disability Assessment Test
MIDAS stands for the Migraine Disability Assessment Test.
This score is used to grade the severity of migraine. Its primary function is to help evaluate the patient and quantify the disability they experience from their headaches. The score helps determine the level of impact the condition has on the patient’s daily life, such as missing work or school, and thus helps decide the need for prophylaxis.
The MIDAS score rating is crucial for determining the initial therapeutic approach:
- Severe Disability (High Score): If the MIDAS score is more than 20 (or 21 and above), it signifies severe disability. In these cases, the first-line approach for treatment is often triptans.
- Mild to Moderate Disability (Low Score): If the MIDAS score values are less than 20, indicating mild to moderate severity, the first-line approach is typically to start the patient on COX-1 inhibitors, such as indomethacin.
The creation of the MIDAS score provided a standardization tool, ensuring that the decision on whether triptans or simpler analgesics are needed is based on an objective measure rather than personal opinion or bias.
Treatment of Migraine
The treatment for migraine headaches is generally divided into two main categories: acute (abortive) therapy for managing an ongoing attack, and prophylactic therapy for preventing recurrent attacks.
I. Acute Treatment (Abortive Therapy)
The choice of acute treatment depends on the severity of the migraine attack, often graded using the MIDAS score. Anti-emetics should accompany acute treatments due to associated nausea and vomiting.
1. Mild to Moderate Attacks (MIDAS score less than 20):
- Simple Analgesics: These are typically the first-line approach.
- COX-1 Inhibitors: Such as indomethacin, naproxen (550 mg BID), or ibuprofen (400 mg, possibly up to 4-6 times).
- Aspirin: Soluble aspirin (600 to 900 mg per orally).
- Paracetamol (Acetaminophen): Paracetamol (1 gram per oral) can be used, often combined with caffeine. Paracetamol is categorized as a COX 3 inhibitor.
- COX-2 Inhibitors (e.g., Etoricoxib): May be used if COX-1 inhibitors are not tolerated.
2. Moderate to Severe Attacks (MIDAS score 21 and above):
- Triptans: These are the preferred first-line approach for severe disability. Triptans are 5HT 1B/1D agonists. They cause vasoconstriction of the extranasal blood vessels, which reduces the stretching of the dura mater and decreases pain perception.
- Oral Triptans: Commonly used types include Sumatriptan (50 to 100 mg, maximum 200 mg), Rizatriptan (5 to 10 mg, maximum 30 mg), Zolmitriptan, Eletriptan, and Almotriptan. Mouth-dissolving tablets are available if nausea is present.
- Faster Acting Formulations (for rapid relief):
- Subcutaneous Injection: Sumatriptan (6 mg subcutaneous) is highly effective for very severe migraine. Auto injectors are available for patient use.
- Nasal Spray: Zolmitriptan (Zolmet) and Rizatriptan nasal sprays provide quick relief.
- Transdermal Patches: Available for very quick relief.
- Ergot Alkaloids: Dihydroergotamine (DHE) and Ergotamine were used historically and are considered second-line agents after triptans. They also act as powerful vasoconstrictors by binding to various 5HT and alpha-1 receptors.
3. Status Migrainosis (Attack lasting more than 72 hours):
Status migrainosis is often described as a perceived, psychotic state where pain persists despite standard treatment (including painkillers, opioids, and triptans).
- Main Drug: Prochlorperazine is the main treatment for status migrainosis.
- Supportive Care: The patient should be admitted, and dehydration and hypoglycemia should be corrected with IV fluids, possibly including 10% dextrose.
- Opioids/Opiates: Should generally be avoided in migraine treatment.
- Triptans and Ergots are contraindicated in patients with underlying vascular conditions due to their vasoconstricting effects:
- Ischemic Heart Disease (IHD) or Coronary Artery Disease (CAD).
- Prinzmetal’s angina.
- Uncontrolled Hypertension.
- Peripheral Arterial Disease (PAD).
- History of Stroke/Cerebrovascular Disease (CVD).
- Pregnancy: They are contraindicated as they can induce smooth muscle contraction.
- Basilar Migraine: Triptans are contraindicated because the disease is caused by vasoconstriction of the basilar artery, and giving a triptan would worsen the concurrent cranial palsy.
- Serotonin Syndrome Risk: Combining Triptans or Ergots with Selective Serotonin Reuptake Inhibitors (SSRIs) or Tricyclic Antidepressants (TCAs) is risky and can lead to Serotonin Syndrome due to excessive serotonin concentration.
- Pediatric Use: Oral triptans are generally not recommended for patients under 18 years of age due to unproven efficacy.
II. Prophylactic Treatment
Prophylaxis is recommended if the headache is severe and disabling, often measured by a high MIDAS score. Prophylactic medications are aimed at reducing the frequency and severity of attacks.
1. First-Line Agents:
- Beta Blockers: Non-selective beta blockers like Propranolol are considered a primary answer for prevention. Metoprolol is also mentioned.
- Anticonvulsants:
- Topiramate.
- Valproate/Valproic acid.
2. Second- and Third-Line Agents:
- Calcium Channel Blockers: Flunarizine.
- Antidepressants: Tricyclic Antidepressants (TCAs) like Amitriptyline.
- CGRP Antagonists: Drugs like Arenomab (a monoclonal antibody) target the Calcitonin Gene Related Peptide (CGRP), which is considered the most important pathogenic molecule in migraine.
- Other options: Gabapentin and Pregabalin (often used for nerve pain), Candesartan, and Pizotifen.
- Botulinum Toxin (Botox A): Used to block pain sensations carried by the trigeminal nerve, serving as a prophylactic therapy.
Subarachnoid Hemorrhage (SAH)
Subarachnoid hemorrhage (SAH) is a serious condition primarily characterized by severe and distinct headache symptoms, resulting from bleeding into the space surrounding the brain.
Key Clinical Presentation:
- Headache: The defining features of the headache are the “worst headache of my life” or a “Thunderclap headache”. A Thunderclap headache is characterized by the pain reaching its peak onset as quickly as within one minute of starting.
- Associated Findings: SAH is often associated with nucal rigidity. The presence of headache combined with nucal rigidity should alert a doctor to the presence of subarachnoid hemorrhage.
Pathophysiology of Symptoms:
The nucal rigidity seen in SAH develops because there is blood in the meninges. This blood causes meningeal irritation, similar to how pus causes irritation in meningitis, triggering rigidity. The presence of blood in the meninges can also trigger signs such as a Kernig sign and a positive Brudzinski sign in the patient.
Causes:
- Primary SAH: One of the most important causes of primary subarachnoid hemorrhage is the rupture of a berry aneurysm.
- Secondary SAH: Secondary causes often arise from outside factors, such as trauma.
DOC: Nimodipine (to prevent vasospasm) 60 mg every 4 hours for 21 days, started as early as possible
Choice of Investigation: NCCT Head
Giant Cell Arteritis (GCA)
A case of Giant Cell Arteritis (GCA), also known as temporal arteritis, typically presents in senior citizens (geriatric population), such as a patient who is 70 years of age.
The presentation focuses on a few characteristic symptoms and findings:
Core Symptoms and History
- Temporal Headache: The patient complains of a continuous headache on the side of their head. It is often described as a temporal headache.
- Jaw Claudication: A highly characteristic associated symptom is jaw claudication. This symptom is considered to have a very high positive predictive value for GCA.
- Systemic Symptoms: The condition may initially be misdiagnosed as fever of unknown origin (FUO). The patient might present with a fever and be taking paracetamol on and off for a couple of weeks.
Physical and Lab Findings
- Vessel Changes: The doctor may feel a cord-like structure at the temporal mandibular (TM) joint, just anterior to the tragus, which is the thrombosed superficial temporal artery. The vessels may appear thick and nodular.
- Scalp Tenderness: The condition can be associated with scalp tenderness.
- Elevated ESR: Laboratory findings are typically dramatic, with the erythrocyte sedimentation rate (ESR) grossly elevated. The ESR may be a shocking 100 mm fall in the first hour. While ESR is generally a prognostic feature, a combination of temporal headache, jaw claudication, and elevated ESR lends credibility to the GCA diagnosis.
Complications and Sequelae
- Visual Loss: A major concern in GCA is the potential for visual loss (unilateral, bilateral, temporary, or permanent). If the ophthalmic artery, a branch of the internal carotid artery, is involved, the patient may experience monocular blindness.
GCA is an example of an extracranial cause of headache and involves a granulomatous vasculitis typically seen in older patients (over 55 years of age).
Cluster Headache — Clinical Features
The clinical features of cluster headache, the least common cause of primary headache, are characterized by severe, recurrent, unilateral pain and associated autonomic symptoms. Cluster headache is considered an example of a trigeminal autonomic cephalalgia (TAC).
| Feature | Description and Details |
|---|---|
| Pain Intensity and Quality | The pain is excruciating. It is often described as feeling as if someone is drilling a hole in the eye with a drill machine. The pain intensity is rated as 10 on 10. The pain is described as being stabbing or boring in nature. The pain is unilateral. |
| Location | The pain is typically retroorbital or perorbital. |
| Associated Autonomic Symptoms (Ipsilateral) | Cluster headache is associated with ipsilateral (same-side) autonomic nervous system (ANS) symptoms. These frequently include lacrimation (watering/tears from the eyes, referred to as epiphora), conjunctival congestion (red eye), and nasal stuffiness or rhinorrhea (nasal congestion). Eyelid edema can also be seen. |
| Patient Behavior during Attack | The patient is typically very restless, very anxious, and not comfortable sitting down. Instead of sitting quietly like a migraine patient, a person with cluster headache is often running around with headache or pacing to and fro. The pain is so severe that it can awaken the patient from sleep. |
| Gender Predilection | Cluster headache is relatively more common in male patients. It is seen in young males. |
| Attack Frequency and Duration | The attacks typically occur 1 to 8 times per day. The duration of pain in a cluster headache attack is generally short, lasting between 15 minutes to 3 hours. On average, the attack may last about half an hour. |
| Periodicity | The attacks occur in clusters, hence the name. The attacks are not all year around, but usually occur for only few months in a year. These clusters of symptoms may last 8 to 10 weeks. There is sometimes a seasonal predilection for spring (March/April) or autumn (August/September). Nocturnal headache is also a feature. |
| Triggers | Alcohol may be a trigger for cluster headache. |
| Psychotic Features | The intensity of pain can be so severe that some patients may exhibit psychotic manifestations or self-mutilation features (e.g., trying to hurt their eye with a pointed object to relieve perceived pressure). |
Unlike migraine, cluster headache pain is typically non-throbbing. While some migrainous features may sometimes be present, cluster headache is often differentiated from migraine by the high intensity of pain, restlessness during the attack, and the ipsilateral autonomic symptoms.
Yes, the attacks of cluster headache are known to exhibit periodicity and a tendency for nocturnal occurrence, which suggests they can occur at specific times.
Here are the details from the sources:
- Nocturnal Headaches: Cluster headaches can be so severe that they awaken the patient from sleep. This nocturnal occurrence suggests a cyclical timing.
- Cluster Periodicity: The attacks occur in clusters, which means they are not present all year round, but rather concentrated over a period of time, such as 8 to 10 weeks. This suggests a concentration of attacks during specific periods rather than consistently random timing.
- Frequency: During a cluster period, attacks are frequent, occurring 1 to 8 attacks per day.
While the sources do not explicitly state that cluster headaches occur at the exact same time every single day, they do emphasize nocturnal headache and periodicity, which is consistent with a strong tendency for attacks to be concentrated during certain parts of the 24-hour cycle. The pain is intense enough to wake the patient from sleep. The number of attacks daily can be up to eight.