Week 11 - Headache - Moodle Version PDF

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Canadian College of Naturopathic Medicine

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headache diagnosis treatment

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This document details learning outcomes for a course on headache, along with introductions and approaches to diagnosis and treatment.

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Headache CMS200 Learning Outcomes 1. Analyze the classification, causes, and types of headaches, including primary and secondary headaches, and their specific characteristics. 2. Evaluate common and rare headache types, including migraines, tension-type headaches, cluster headaches, and life-...

Headache CMS200 Learning Outcomes 1. Analyze the classification, causes, and types of headaches, including primary and secondary headaches, and their specific characteristics. 2. Evaluate common and rare headache types, including migraines, tension-type headaches, cluster headaches, and life-threatening headaches. 3. Diagnose headaches using the International Headache Society's classification system, POUND mnemonic, and other clinical criteria. 4. Conduct a thorough patient history, including physical and neurologic examination, and red flag signs and symptoms in diagnosing headaches. 5. Determine when and how to use neuroimaging, lumbar puncture, and other diagnostic tests appropriately in evaluating headaches. 6. Explain the significance of headache characteristics, such as onset, duration, frequency, pain character, location, and associated features in diagnosis. Learning Outcomes 7. Construct and rank a differential diagnosis based on demographic information, risk factors, symptoms, and signs. 8. Assess the etiology, prevalence, and impact of headache disorders on patients' quality of life, including those in children and adolescents. 9. Correlate modifiable risk factors, medication overuse, and lifestyle factors with headache severity and frequency. 10. Monitor patient progress, reassess the working diagnosis, and address factors driving headache escalation. 11. Demonstrate the importance of providing patient reassurance and addressing concerns when appropriate. Introduction Headaches can occur for many different reasons Proper evaluation can be challenging Headaches burden is thought to be underestimated, perhaps undertreated Unusually among chronic diseases, much morbidity among the otherwise young and healthy, including children and adolescents Prevalence peaks between the ages of 25 - 40 and decreases with age Headache disorders more common in women Introduction < 1% of all headaches are life-threatening. Nevertheless, many patients have anxiety related to their headaches, especially related to disorders such as tumors Understand their agenda and addressed these concerns directly when serious causes have been ruled out Challenge: 1. reassure and appropriately treat patients with benign headaches while 2. finding the rare, life-threatening headache 3. without excessive evaluation ICHD-III The International Classification of Headache Disorders Formal, comprehensive headache classification system Classifies over 200 headache disorders into categories and subcategories We will only be discussing a small fraction of these Main categories: 1. Primary headache 2. Secondary headache 3. Neuropathies & facial pains and other headaches Clinicians should attempt to use their criteria to accurately diagnose each headache Approaches to History-Taking Two general approaches: 1. Focus on serious causes i. Learn the alarm features that should prompt consideration of a serious pathologies ii. Know which aspects of the history predict a higher likelihood of abnormalities on neuroimaging (i.e. which represent a serious cause for headache) 2. Understand the typical features of common benign headache syndromes. Physical Examination Rarely provides clues to the diagnosis Nevertheless, any abnormalities found on physical examination (especially visual, motor, reflex, sensory, speech or cognitive), warrant further investigations, since these abnormalities are one of the best predictors of CNS pathology These findings can be subtle Physical exam choice should be guided by diagnostic hypotheses Among the most commonly performed: Neurological, including cranial nerve exams Head and neck exam Findings particularly suggestive of secondary headache causes include: focal neurological deficits, papilledema, bitemporal hemianopia, homonymous hemianopia, decreased visual acuity, or increased pain with Valsalva method. New or Old? Adding the distinction of new versus old to primary versus secondary provides a clinically useful structure New headaches: Headaches of recent onset, or Chronic headaches that have changed in character/quality. Change in severity is less important than change in quality Though old headaches can be either primary or secondary, they are most often due to migraine or tension-type headache (both of which are primary headaches) Pay particular attention to the presence of alarm features when evaluating new headaches Most new headaches are benign diagnoses, but almost all serious headaches are new headaches Primary or Secondary? Headaches are classified as primary or secondary Primary headaches: conditions themselves E.g. tension-type headache, migraine headache, cluster headache Secondary headaches: symptoms of other illnesses. Primary Secondary E.g., those caused by infection or vascular disease Can be difficult to distinguish clinically, but it is important to do so Primary headaches: no dangerous underlying cause, although can cause significant suffering and be disabling Secondary headaches: may be a sign of significant pathology (life threatening or significant disability) Helps to decide on further work-up Primary headaches: no labs or imaging that act as gold standard; diagnosed clinically Secondary headaches: diagnosis may require further diagnostic testing to identify the underlying disease of which the headache is a symptom Secondary New Due to another illness Recent onset or changed character Serious New Secondary Primary Serious Primary/Secondary and New/Old Tension headaches, Migraine Benign cough headache, Primary headaches, Cluster Benign exertional headache headaches Cervical degenerative joint Infectious: disease, Temporomandibular Upper respiratory tract joint syndrome infection Sinusitis Secondary Vascular: Temporal arteritis Space-occupying lesions: Brain tumors Old New Secondary Headaches Many conditions can cause headache Many conditions that cause headaches are benign, but some can lead to severe disability or mortality. Life-threatening headaches (e.g. meningitis, intracranial hemorrhage, brain tumor, temporal arteritis, and glaucoma) are rare but “must-not-miss” Secondary Headaches Goals regarding secondary headaches: Determine whether the headache is dangerous Although rare, serious must be excluded by the patient history, (including e.g. the SNNOOP10), physical examinations, targeted imaging and other assessments New secondary headaches are particularly worrisome Diagnose and treat of the underlying condition Treatment less likely to be effective if cause is not addressed Red Flags: SNNOOP10 Findings suggested by The American Headache Society to detect secondary (serious) cause of headache When these signs or symptoms are present, rapid diagnosis and management are critical Finding Related Secondary Headaches Systemic symptoms including fever Headache attributed to infection or nonvascular intracranial disorders, carcinoid or pheochromocytoma Neoplasm in history Neoplasms of the brain; metastasis Neurologic deficit or dysfunction (including Headaches attributed to vascular, nonvascular decreased consciousness) intracranial disorders; brain abscess and other infections Onset of headache is sudden or abrupt Subarachnoid hemorrhage and other headaches attributed to cranial or cervical vascular disorders Red Flags: SNNOOP10 Finding Related Secondary Headaches Older age (after 50 years) Giant cell arteritis and other headache attributed to cranial or cervical vascular disorders; neoplasms and other nonvascular intracranial disorders Pattern change or recent onset of headache Neoplasms, headaches attributed to vascular, nonvascular intracranial disorders Positional headache Intracranial hypertension or hypotension Precipitated by sneezing, coughing (Valsalva), or Posterior fossa malformations; Chiari exercise malformation Papilledema Neoplasms and other nonvascular intracranial disorders; intracranial hypertension Progressive headache and atypical presentations Neoplasms and other nonvascular intracranial disorders Red Flags: SNNOOP10 Finding Related Secondary Headaches Pregnancy or puerperium Headaches attributed to cranial or cervical vascular disorders; postdural puncture headache; hypertension-related disorders (e.g., preeclampsia); cerebral sinus thrombosis; hypothyroidism; anemia; diabetes Painful eye with autonomic features Pathology in posterior fossa, pituitary region, or cavernous sinus; Tolosa-Hunt syndrome; ophthalmic causes Posttraumatic onset of headache Acute and chronic posttraumatic headache; subdural hematoma and other headache attributed to vascular disorders Pathology of the immune system such as HIV Opportunistic infections Painkiller overuse or new drug at onset of Medication overuse headache; drug headache incompatibility Red Flags: Accuracy Finding LR+ Dysequilibrium 49 Confusion or lethargy 1.5 New-onset seizure 1.36 History of malignancy 2.02 History of HIV infection 1.8 Thunderclap headache 1.9 Progressive headache over weeks to months 12 Diplopia 3.4 (for temporal arteritis) Hemiparesis 3.69 Headache causing awakening from sleep 1.7-98 Headache worse with Valsalva maneuver 2.3 Neuroimaging For patients with red flags (because of increased risk of intracranial pathology). The 2019 American College of Emergency Physicians (ACEP) clinical policy on headache recommends neuroimaging in the following scenarios: Headaches with associated with any new neurologic deficits New and sudden-onset severe headaches HIV-positive patients with a new type of headache Patients older than 50 with a new headache See also the American College of Radiology Appropriateness Criteria Predicting Neuroimaging Abnormalities Context Pretest probability for detecting severe intracranial pathology Thunderclap headache 43% Patient referred to 1.2% neurologist for chronic headache Predicting Neuroimaging Abnormalities Finding LR+ LR- Cluster-type headache 11 0.95 Abnormal findings on neurological examination 5.3 0.71 Headache difficult to classify and not clearly a primary headache 3.8 0.66 Headache with aura 3.2 0.51 Headache with focal neurologic symptoms 3.1 0.79 Headache aggravated by exertion or Valsalva 2.3 0.7 Headache with vomiting 1.8 0.47 Worsening headache 1.6 1 Male sex 1.3 0.86 Quick onset headache 1.3 0.79 New-onset headache 1.2 0.89 Headache with nausea 1.1 0.86 Increased headache severity 0.83 1.2 Neuroimaging: Specific Choice The American College of Radiology Appropriateness Criteria document and interactive site can help guide specific choice of imaging, including different scenarios For most dangerous causes of headache, MRI or CT is acceptable. In suspected emergent headache, non-contrast head CT is the test of choice, but alternative/additional imaging is done as needed based on differentials Neuroimaging: Specific Choice Institute for Clinical Systems Improvement recommendation MRI with and without contrast for patients with progressively worsening headaches over weeks to months Reason: possibility of subdural hematoma, hydrocephalus, tumor, or another progressive intracranial lesion For acute head trauma and if an intracranial hemorrhage is suspected Head CT without contrast media is recommended (availability, speed, and accuracy) MRI of the brain is more sensitive for detecting subdural hematoma, and is therefore particularly important in identifying smaller lesions Lumbar Puncture Useful for identifying: Infection, red blood cells (suggests bleeding), xanthochromia (yellowing caused by bilirubin in the CSF), abnormal cells associated with some CNS malignancies Therefore, especially considered in patients with headache(s) and the following: Fever with altered mental status Meningeal signs Focal neurological deficits History of HIV or another immunocompromised state Suspicion of idiopathic intracranial hypertension Other Labs Lab(s) May be used to investigate (e.g.) Complete blood count (CBC) Infection Erythrocyte sedimentation rate (ESR) Giant cell (temporal) arteritis and other and/or vasculitides C-reactive protein (CRP) Metabolic panel Metabolic causes of headaches Endocrine testing Pituitary gland abnormalities. Rhinosinusitis Inflammation of the sinuses and nasal mucosa, most often caused by viral infections, but may also be bacterial. Leads to feelings of facial pain, pressure, or fullness Epidemiology Develops in 0.5 - 2% of all upper respiratory tract infections Rhinosinusitis Timing Acute (up to 4 week) Acute bacterial rhinosinusitis diagnosis requires symptoms for longer than 10 days or worsen after five to seven days Subacute (4 - 60 years) children (2 months to 10 years). Viral (3-4 times more common) adults (7.6 per 100,000) children (5.2 per 100,000) Meningitis Epidemiology Risk factors Bacterial Immunocompromised state Functional or anatomical asplenia Cochlear implants Cerebrospinal fluid leaks Recent trauma or instrumentation Alcoholism Smoking Viral Exposure to enterovirus or arbovirus (mosquito-borne) infections (especially in summer and early fall) Meningitis Epidemiology Risk factors General Travel to endemic areas (e.g., tuberculosis, fungal, or parasitic infections), Occupational or hobby-related contact with animals or soil (e.g., Listeria monocytogenes, Cryptococcus neoformans), Sexual activity (e.g., N. meningitidis, HSV), Injection drug use (e.g., S. aureus), Close contact with a person with meningitis (e.g., N. meningitidis, H. influenzae type b) Meningitis Time Course Acute, with rapid onset and progression, particularly for bacterial Median time to seeking medical care: Bacterial meningitis: 24 hours Viral meningitis: 2 days Meningitis Signs and Symptoms Classically: fever, headache, neck stiffness, and altered mental status May not be present in all cases and may vary with age Older patients More likely: altered mental status, focal neurologic deficits Less likely: headache and neck stiffness Children Vague symptoms e.g. irritability, lethargy, poor feeding Others: photophobia, phonophobia, nausea, vomiting, seizures Meningitis Signs and Symptoms Signs of meningeal irritation (meningeal signs) – not particularly good sensitivity or specificity; not reliable to rule out bacterial meningitis Kernig sign (resistance to knee extension with hip flexion) Brudzinski sign (involuntary hip flexion with neck flexion) Nuchal rigidity (resistance to neck flexion) Jolt accentuation of headache (worsening of headache by horizontal rotation of the head). Others: Rash (especially in meningococcal disease), cranial nerve palsies (especially in tuberculous or fungal meningitis), papilledema (in increased intracranial pressure), focal neurologic deficits (in encephalitis or stroke). Meningitis In community-acquired bacterial meningitis Finding Frequency (sensitivity) at least 2 of: headache, fever, stiff neck, mental status changes 95% headache 87% stiff neck 83% temperature > 38.0°C 77% change in mental status 69% focal neurologic findings 33% abnormal CT scan 34% Kernig and Brudzinski sign 60% Meningitis Further Testing Definitive diagnosis: CSF obtained from lumbar puncture (LP) Should be done promptly in all patients with suspected meningitis Exception: contraindications or high-risk features for increased intracranial pressure or cerebral herniation e.g. focal neurologic signs, papilledema, immunocompromised state Head CT should be performed before LP in these patients Meningitis Prognosis and Clinical Course Requires prompt recognition and treatment Bacterial Rapidly fatal if untreated; 25% mortality rate (community-acquired) 7 to 21 days of treatment (i.e. antibiotics, corticosteroids) Complications: hearing loss, neurologic sequelae (e.g., cognitive impairment, seizure disorder, hydrocephalus, cerebral infarction), subdural effusion, empyema, abscess, endocarditis, septic arthritis, disseminated intravascular coagulation (DIC) Viral Generally self-limited with a good prognosis supportive care; may last over 2 wks Complications: hearing loss, developmental delay, seizure disorder Head Trauma Epidemiology 9.3% of headaches in the emergency department More common in Males Children and young adults Elderly Risk factors: Motor vehicle accidents Falls Assaults and abuse Sports Alcohol or drug intoxication Coagulopathy Previous neurosurgical procedures Head Trauma Epidemiology Head trauma is minor in 90% of cases Definition: Glasgow Coma Scale (GCS) score ≥ 13 and appear well on examination But, among those with minor head trauma, 7.1% had severe intracranial injury requiring prompt intervention (i.e. patient’s you’d want to have sent to the ER) Head Trauma Time Course May have acute, subacute, or chronic effects depending on the severity and type of injury Headaches after minor trauma are most likely to be chronic Some symptoms may appear immediately after the traumatic event, while others may appear days or weeks later Mild traumatic brain injury (TBI) may be temporary Moderate to severe TBI: may result in long-term complications or death Head Trauma Signs and Symptoms Finding Frequency (sensitivity) Nausea 19-29% Headaches tend to Vomiting 18% meet criteria for Seizure 3.1% tension-type Any amnesia 50% headaches Loss of consciousness 65% Headache 50% Detectable skull fracture (any) 16% Decline in GCS score 21-31% Focal neurologic deficit 4.8-17% Predicting CT Detecting Severe Injury Among patients with minor head trauma, the following are associated with severe intracranial injury on CT: Finding LR Physical examination findings suggestive of skull fracture 16 GCS score of 13 4.9 2 or more vomiting episodes 3.6 GCS score < 15 at 2 hrs post injury 3.5 Any decline in GCS score 3.4-16 Pedestrians struck by motor vehicles 3.0-4.3 No features of Canadian Head CT Rule 0.04 No features of New Orleans Criteria 0.08 No features of Nexus II ~0 Physical examination findings suggestive of skull fracture Head Trauma New Orleans Criteria Canadian CT Head Rule Nexus II Older than 60 years 65 years or older Evidence of significant skull Intoxication Dangerous mechanism fracture Headache (pedestrian struck by vehicle, Scalp hematoma Any vomiting occupant ejected from Neurologic deficit Seizure vehicle, fall >1m or 5 stairs) Altered level of alertness Amnesia Vomiting more than 1 Abnormal behavior Visible trauma above the episode Coagulopathy clavicle Amnesia longer than 30 Persistent vomiting minutes Age  65 GCS score less than 15 at 2 hours Suspected open, depressed, or basilar skull fracture Head Trauma Mild TBI Early (minutes to hours) Late (days to weeks) More common Dizziness/poor balance, Anxiety/nervousness fatigue, headache, nausea irritability, difficulty concentrating, difficulty remembering, disorientation, drowsiness, increased sleep, headache, light sensitivity Less common Confusion, vomiting depression, decreased sleep, difficulty initiating sleep, light-headedness, tinnitus Head Trauma Mild TBI Other More common Feeling foggy, feeling slowed down, blurred vision, noise sensitivity Less common Clinginess, emotional lability, personality changes, sadness, amnesia, delayed verbal and other responses, feeling stunned, inability to focus, loss of consciousness (90% women 94% obese Also, rapid weight gain or loss, female sex hormones Medications: tetracyclines, vitamin A derivatives, corticosteroid withdrawal. Idiopathic Intracranial Hypertension Time Course Chronic Daily, can last for months to years Variable course, with periods of remission and exacerbation Some patients experience spontaneous resolution, while others may have persistent or progressive symptoms that require long-term treatment Idiopathic Intracranial Hypertension Signs and Symptoms Two most prominent symptoms: Chronic headache Often the first and most common symptom Frequently migraine-like with nausea, photophobia, and phonophobia Progressive visual deterioration Vision loss, transient visual obscurations (TVOs), diplopia, enlarged blind spot Other features: cranial nerve palsies (especially CN6 – 12% of cases), cognitive deficits, olfactory dysfunction, and pulsatile tinnitus Most valuable findings Papilledema (~90%) TVOs elevated intracranial pressure (ICP) on lumbar puncture (diagnostic) Idiopathic Intracranial Hypertension Further Testing Modified Dandy criteria Signs and symptoms of increased ICP No localizing neurological signs (except for CN6 palsy) Normal neuroimaging studies (except for findings related to increased ICP) MRI or CT Cerebrospinal fluid (CSF) opening pressure of more than 25 cm H2O with normal CSF composition Lumbar puncture to measure CSF opening pressure and composition No other cause of increased ICP identified Visual function tests (e.g. visual acuity, visual field, color vision, contrast sensitivity) used to monitor progression and severity of visual impairment Idiopathic Intracranial Hypertension Prognosis/Clinical Course Untreated, can lead to irreversible visual loss due to optic atrophy Risk factors for poor visual outcome: male sex, black race, severe papilledema at presentation, fulminant disease course, and lack of weight loss Patients may have persistent or recurrent headache despite normalisation of ICP Risk factors for chronic headache: medication overuse, comorbid migraine, psychological factors Temporomandibular Disorder Headache Temporomandibular joint dysfunction causes headache or facial pain that comes on with chewing Epidemiology 10% to 15% of adults, peak ages 20 to 40 years Twice as common in women than in men. Associated with other pain conditions (such as chronic headache), fibromyalgia, autoimmune disorders, sleep apnea, and psychiatric illness Temporomandibular Disorder Headache Time Course Can be acute or chronic Chronic TMD is defined by pain of more than three months’ duration. Signs and Symptoms Often associated with jaw movement (e.g., opening and closing the mouth, chewing) Pain is in the preauricular, masseter, or temple region, with severity ranging from mild to debilitating May be limited of jaw mobility Adventitious jaw sounds (e.g., clicking, popping, grating, crepitus) may be present, but also occur in up to 50% of asymptomatic patients (i.e. low specificity) Temporomandibular Disorder Headache Signs and Symptoms Finding Sensitivity (%) Facial pain 96 Ear discomfort 82 Headache 79 Jaw discomfort or dysfunction 75 Other symptoms: dizziness, neck, eye, arm, or back pain. Temporomandibular Disorder Headache Further Testing None; a clinical diagnosis based on history and physical examination Prognosis/Clinical Course Symptoms resolve within two weeks with treatment Most patients improve with a combination of noninvasive therapies Brain Tumors Epidemiology Primary Rare; 1.4% of all cancers 0.8% of headaches presenting to the emergency department Peak prevalence between 55 and 64 years Slightly more common in men Metastatic ~7 times as common as primary Primary Tumor Frequency Brain metastases are the most common Lung 37% neurologic complication of systemic cancer (20-40% adult patients) Breast 19% May be the first manifestation of Melanoma 16% cancer in some patients Brain Tumors Time Course Classically, progressive headache Primary Variable, depending on tumor type, location, and grade; may be chronic or acute Metastatic Varies depending on the type and aggressiveness of the primary tumor. Brain Tumors Signs and Symptoms About 1/3 of patients with brain tumors present with a primary complaint of headache Classically: severe, worse in the morning, and occurring with nausea and vomiting Actually, patients with brain tumors more often report a bifrontal, tension-type headache. Relatively common: Headache, altered mental status and focal weakness, seizures Neurologic examination: hemiparesis, impaired cognition, sensory loss, gait abnormalities First symptom of a primary brain tumor Finding Sensitivity (%) headache 23.5 generalized seizures 21.30 unilateral weakness 7.10 unsteadiness 6.10 expressive language disorder 5.80 visual problems 3.20 confusion 4.50 unilateral numbness 2.30 personality problems 1.60 diplopia 0.30 other symptoms: 24.2 anosmia, apraxia, cognitive delay, drowsiness, dysphagia, hallucinations, memory loss, nausea and vomiting, pain, and stiff neck Finding Sensitivity (%) Hemiparesis 55 – 60 Impaired cognition 55 – 60 Headache 25 – 40 Focal weakness 20 – 30 Altered mental status 20 – 25 Sensory loss 20 Frequency of findings Papilledema 20 in metastatic brain Seizure activity 15 – 20 tumors Gait abnormality 15 – 20 Aphasia 15 – 20 Gait disturbance 10 – 20 Speech difficulty 5 – 10 Visual disturbance 5–8 Hemianopsia 5–7 Limb ataxia 5–7 Sensory disturbance 5 Nausea/vomiting 5 Somnolence 5 None 5 to 10 Brain Tumors Presenting Manifestations of Primary Brain Tumors by Location Tumor location Neurologic signs Frontal lobe Dementia, personality changes, gait disturbances, generalized or focal seizures, expressive aphasia Parietal lobe Receptive aphasia, sensory loss, hemianopia, spatial disorientation Temporal lobe Complex partial or generalized seizures, quadrantanopia, behavioral alterations Occipital lobe Contralateral hemianopia Thalamus Contralateral sensory loss, behavioral changes, language disorder Cerebellum Ataxia, dysmetria, nystagmus Brain stem Cranial nerve dysfunction, ataxia, papillary abnormalities, nystagmus, hemiparesis, autonomic dysfunction Brain Tumors Further Testing Primary Brain MRI with gadolinium contrast Biopsy is required for definitive diagnosis Chest and abdomen CT if suspected metastatic disease Secondary Enhanced CT scanning or brain MRI Rounded, well-circumscribed, non-infiltrative masses surrounded by edema and enhanced by contrast Surgical biopsy may be necessary for definitive diagnosis Brain Tumors Prognosis/Clinical Course Primary Depends on tumor type, grade, location, and patient factors; Five-year survival rate is 33.4% Glioblastoma has the worst prognosis (1.2%) Secondary Median survival: 3-6mo Depends on a number of factors, such as the number and location of lesions, the type and extent of systemic disease Medication Overuse Headache (MOH) Also called rebound headache Refractory daily or near-daily headache that can result from overuse of analgesic medication Overuse can increase headache frequency, reduced efficacy of preventive or abortive medications I.e. Patients inadvertently increase headache frequency by overuse of analgesics, and tolerance develops to headache analgesia Headache, no longer responsive to treatment, worsens when analgesic is abruptly stopped Medication Overuse Headache (MOH) Epidemiology One of the most common secondary headache disorders globally Prevalence: ~1% More common in women, lower socioeconomic status Frequent cause of headache in patients referred to specialized headache clinics 30% to 50% of patients who develop chronic headaches have MOH Often associated with chronic migraine or tension-type headache Overuse of acute headache medication to alleviate the original headache pattern Hence, a complication of treating chronic primary headaches Analgesics are broadly available without healthcare professional intervention and people are often unaware of the potential consequences of progressively increased use Medication Overuse Headache (MOH) Medications classes (and their combinations): Non-steroidal anti-inflammatory drugs (NSAIDs) Acetaminophen Triptans Ergotamines Opioids Highest risk: opioids, followed by triptans, ergotamines, and nonopioid analgesics Medication history: include non-prescription analgesics and substances, including opiates, that may be obtained from others Often pre-existing sleep disturbances and psychiatric disorders (particularly depression, anxiety, obsessive-compulsive disorder) Medication Overuse Headache (MOH) Time Course ≥15 headache days per month in patients who have had >3 months of excessive use of abortive medications Intake must be 10 d/mo for ergotamines, triptans, opioids, combinations or multiple drug classes Intake must be 15 d/mo for NSAIDs, ASA, acetaminophen May be episodic or persistent Typically starts in the morning Should be suspected in patients with frequent headache Medication Overuse Headache (MOH) Signs and Symptoms Depends on the type and frequency of medication used No classic signs and symptoms Neck pain, sleep disturbance, autonomic (rhinorrhea, lacrimation) and gastrointestinal (nausea, vomiting, diarrhea) symptoms, comorbid anxiety and depression Often overlap with other acute and chronic headache types Medication Overuse Headache (MOH) Detecting medication overuse headache Context Finding Sensitivity (%) LR+ LR- People with Answer yes to both: 95.2 4.76 0.06 frequent "do you take a treatment for attacks more than 10 migraines days per month?“ And "is this intake on a regular basis?" Family Answer of at least once to: “How many times in 100 3.85 0 medicine the past year have you used an illegal drug or used a prescription medication for nonmedical reasons?” Medication Overuse Headache (MOH) Further Testing None for MOH specifically; diagnosis is based on clinical history and physical examination Medication Overuse Headache (MOH) Prognosis/Clinical Course Initial worsening of headache and withdrawal symptoms are common during medication weaning After appropriate treatment (gradual withdrawal), residual symptoms likely due to underlying primary headache disorder In ~75% of patients, discontinuing the overused medication results in reversion Relapse rate is high (about 30% per year; about 50% at 5 years) Temporal Arteritis Headache essentially exclusive to patients over 50 often accompanied by jaw claudication. More common in patients with polymyalgia rheumatica aka giant cell arteritis Epidemiology >50 yoa, women>men Among those over 50: 0.02% Among those with polymyalgia rheumatica (PMR): 15% 30–40% of patients referred for temporal artery biopsy Timing Chronic and of varying duration Usually, headache onset is gradually over a few hours, but can have a rapid onset Temporal Arteritis Signs and Symptoms Finding LR+ Headache may be unilateral or bilateral Scalp tenderness and jaw 17.0 Temporal location in 50% of patients claudication (may involve any location on the head) Headache and jaw claudication 8.0 Pain almost always described as dull Jaw claudication 6.7 and boring Diplopia 3.5 Scalp tenderness Beaded temporal artery 4.6 Jaw claudication Enlarged temporal artery 4.3 Other nonspecific manifestations of a chronic inflammatory disorder: Scalp tenderness 3.0 Fever, fatigue, weight loss Temporal artery tenderness 2.6 Any temporal artery abnormality 2.0 Temporal Arteritis Further Testing Finding LR+ LR- Temporal artery biopsy is the ESR elevated 1.1 0.2 gold standard ESR > 50 mm/h 1.2 0.35 ESR (CRP is another option) ESR > 100 mm/h 1.9 0.8 Anemia and thrombocytopenia Positive temporal  0.15 may be present but are not artery biopsy particularly helpful for diagnosing temporal arteritis Temporal Arteritis Prognosis/Clinical Course Untreated, blindness in 50% Treatment should be initiated prior to the biopsy and then discontinued if necessary Primary Headaches Mostly tension-type headaches, migraines, and cluster headaches (though there are many others) Further categorization: Short versus long Fewer than 4 hours: ‘short headache.’ Cluster headaches, neuralgiform headache attacks, primary stabbing headaches. More than 4 hours: ‘long headache.’ Migraine, tension headaches, persistent daily headaches, hemicrania continua. If there are no red flags, history and physical examination are usually sufficient to diagnose these headaches Some primary headaches, (e.g. benign cough, sexual, or primary exercise headache) strongly resemble serious headaches. Neuroimaging is indicated in work-up Excellent prognosis Patients should be educated about the benign nature of these headaches Can have significant impact on quality of life but no long-term sequelae Short-Lasting Primary Headaches Cluster Headaches Short-lasting Unilateral Neuralgiform Headaches Short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA) Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) Primary Stabbing Headaches Cluster Headaches Severe unilateral headaches that cluster in time. Typically in young men and associated with autonomic symptoms and restlessness. Epidemiology Relatively rare Prevalence ~0.1% 4-6 times more common in men than in women 70% of patients reporting onset before 30 years of age May be precipitated by alcohol Cluster Headaches Time Course Characterized by brief (15 to 180 minutes) episodes of severe head pain with associated autonomic symptoms Several (up to 8) episodes per day Episodic type (80-90%) Periods of weeks to months (average 6-12 wks), followed by remission for at least one month (up to 12 months) Chronic type (10-20%) No remission or remission of less than one month Cluster Headaches Signs and Symptoms Pain is rapid onset and most commonly occurs in the retro- orbital area, followed by the temporal region, upper teeth, jaw, cheek, lower teeth, and neck, and is usually unilateral; most often sharp, but may be pulsating/pressure-like Ipsilateral autonomic symptoms such as eyelid edema, nasal congestion, lacrimation, or forehead sweating usually accompany the pain Restlessness/agitation in 80-90% of cases Cluster Headaches Further Testing Neuroimaging is particularly advisable here, given LR for finding abnormalities on imaging Cluster Headaches Prognosis/Clinical Course Significant socioeconomic impact and associated morbidity ~80% of patients report restricting daily activities Only 25% of patients with cluster headaches are diagnosed correctly within one year of symptom onset >40% report a delay in diagnosis of five years or more Comorbidities: depression (24%), sleep apnea (14%), restless legs syndrome (11%), and asthma (9%). Many with cluster headaches report suicidal thoughts 2% of patients in one study had attempted suicide Other Short-Lasting Primary Headaches Headache Short-lasting Unilateral Neuralgiform Primary Stabbing Headache Headache Epidemiology Estimated 6.6 per 100,000 0.2-2% population prevalence ~0.007% Up to 35% of patients with headaches Age of onset typically is between 35-65 42-45% of people with migraines (mean: 48) In these cases stabs tend to be at the male predominance in SUNCT, and a site typically affected by migraines female predominance in SUNA Mean age of onset: 28 ~1.5:1 F:M Timing Triggered by a stimulus to the trigeminal No clear trigger nerve (simple or a noxious) Shortest-lasting headache Last 1s – 10min (SUNA tends to last 80% of stabs last 3 seconds or less longer than SUNCT) (rarely, stabs last for 10-120 seconds) At least one a day (at least half the times Usually one or a few attacks per day they occur) Rarely, repetitively over days Tend to occur during the day Months-long intervals between attacks Headache with the most frequent attacks: in 76% of cases range from 2 to 600 /d, (average < 100) Other Short-Lasting Primary Headaches Headache Short-lasting Unilateral Neuralgiform Primary Stabbing Headache Headache Syndrome Moderate to severe unilateral pain with orbital, Sharp, sudden stabbing, non- supraorbital, temporal and/or other trigeminal pulsatile. pain of moderate to severe distribution occurring as single stabs, series of intensity in the temporal (42%) or stabs or in a throbbing/saw-tooth pattern peri-orbital regions. Changes locations in ~2/3 of patients At least one of the following (autonomic) findings (same or opposite hemicranium) ipsilateral to the pain: 1. conjunctival injection and/or lacrimation No cranial autonomic symptoms 2. nasal congestion and/or rhinorrhoea 3. eyelid oedema 4. forehead and facial sweating 5. forehead and facial flushing 6. sensation of fullness in the ear 7. miosis and/or ptosis Other Short-Lasting Primary Headaches Headache Short-lasting Unilateral Neuralgiform Primary Stabbing Headache Headache Syndrome SUNA Occasional extracephalic jabs in the either lacrimation or conjunctival injection but not facial area or randomly distributed both throughout the body (including e.g. SUNCT “jabs in the heart”) both lacrimation and conjunctival injection Conjunctival hemorrhage and monocular vision loss have occurred Other Short-Lasting Primary Headaches Headache Short-lasting Unilateral Neuralgiform Primary Stabbing Headache Headache Further Diagnosis is mostly clinical Diagnosis is mostly clinical Testing However, because these symptoms can However, because these symptoms can suggest an underlying structural pathology suggest an underlying structural pathology a brain (MRI) with pituitary views and imaging may be recommended blood tests for pituitary function are suggested work-up Prognosis/ Has been known to last for up to 46yrs Because of the mildness of the attacks Course No known complications or increased and the benign course, treatment is rarely mortality necessary and reassurance is usually Prognosis is improving as more sufficient (and in any case, the erratic treatments become available pattern would make assessing treatment effectiveness challenging) Long-Lasting Primary Headaches Migraine Tension-Type Headache Migraine Chronic, often disabling, unilateral pulsating headaches accompanied by symptoms such as nausea, photophobia, phonophobia. Can be associated with aura. Epidemiology Second-most-common primary headache disorder Prevalence: approximately 15% among women and 6% among men In 90% of initial visits for headaches in the primary care setting, headaches meet criteria for migraines Tend to start in adolescence (but may begin at other times as well) In children, prevalence increases with age and is more common in children above the age of 14 Affects approximately 8% of children above the age of 14 Migraine Time Course 4-72 hours < 4 hrs In children and adolescents: 2–72 4-24 hrs hours 24-72 hrs Aura: appear gradually, last no longer than 60 minutes Migraine Signs and Symptoms Pain often described as pulsating or throbbing; bilateral in 60-70% of cases Common associations: nausea, photophobia (sensitivity to light), and phonophobia (sensitivity to sound). Physical activity often exacerbates migraine headache. Aura may be present Aura: visual, sensory, or speech symptoms that are completely reversible In children and adolescents: pain that is moderate to severe, more often bilateral until late adolescence, throbbing (can also present as non-throbbing), frontotemporal and aggravated by activity. Photophobia and phonophobia, nausea, and/or vomiting are often present. May be exacerbated by emotional stress, fatigue, menstrual period (though this is not unique to migraine), foods containing nitrite or tyramine Migraine Type Prevalence Auras Zigzags 56% Occur in about 1/3 of patients Stars or flashes 83% Typically visual, precede the Scotoma 40% headache, and last for about 20 Hemianopsia 7% minutes Sensory aura 20% Common: blind spot that is later Aphasia 11% accompanied by flashing lights, Motor aura 4% spots of light, zigzag lines, or Duration Prevalence squiggles (“scintillating scotoma”) < 30 minutes 70% Usually involve 1 portion of the 30–60 minutes 18% visual field. > 60 minutes 7% Migraine: ID Migraine Screening Tool 1. Nausea: “Did you ever feel nauseous Score LR when you had headache pain?” 0-1 0.25 2. Photophobia: “Did light trouble you when you had headache pain (much 2-3 3.25 more than when there was no headache)?” 3. Disabling Intensity: “Did your headache ever limit your ability to work, study or do something you needed to, for at least 1 day?” Migraine: ID Migraine Screening Tool Notable individual items Finding Sensitivity LR+ LR- You feel nauseated or sick to your stomach 60% 3.2 0.49 Functional impairment due to headache any 87% 1.81 0.25 day in last 3 months Sound bothers you (a lot more than when you 83% 1.89 0.30 don’t have headaches) Migraine Further Testing Patients with features of migraine do not typically require imaging unless there are neurological abnormalities, worsening severity or unusual presentations Decisions about imaging in patients with increasingly frequent migraine can be challenging Migraine Prognosis/Clinical Course 3-4%/year probability of escalation from episodic to chronic form Pulsating quality, severe pain, photophobia, phonophobia, and attacks longer than 72 hours predict this escalation Migraine Category Factor Odds Ratio Risk factors for Frequency 0-4/mo 1 escalation from episodic 5-9/mo 7.6 to chronic migraine 10-15/mo 25.4 Medication overuse Opioids 4.4 Triptans 3.7 Ergotamines 2.9 Analgesics 2.7 Obesity 5.53 Diabetes 3.34 Arthritis 3.29 Head or neck Injury Male 3.3 Female 2.4 Tension-Type Headache (TTH) Epidemiology Most common headache disorder globally Estimates on prevalence vary significantly, but are universally relatively high (e.g. 26% here, 38% here, 42% here) At least as common in women as in men Affects 10–25% of school-aged children and adolescents (global prevalence) Tension-Type Headache (TTH) Time Course Duration of 30 minutes to 7 days Classically, worse with stress or at the end of the day Pain tends to wax and wane Tension-Type Headache (TTH) Signs and Symptoms Characterized by bilateral mild to moderate pressure without other associated symptoms Pain is mild to moderate, bilateral and pressing/non-pulsing Tension-Type Headache (TTH) Further Testing Individuals who meet the criteria for tension-type headache but who have normal neurologic examination results require no additional laboratory testing or neuroimaging Decisions about imaging in patients with increasingly frequent TTH can be challenging Tension-Type Headache (TTH) Prognosis/Clinical Course 3-4%/year probability of escalation from episodic to chronic form Long attack duration and nausea are predictive factors for this progression Migraine versus TTH Old headaches are most often either migraine or tension-type headache Note: patients often describe more than one type of headache. More than 80% of those with confirmed migraine also have TTH Feature LR+ (Migraine vs TTH) LR– (Migraine vs TTH) Nausea 19.2 0.19 Photophobia 5.8 0.25 Phonophobia 5.2 0.38 Exacerbated by physical activity 3.7 0.24 Unilateral headache 3.7 0.43 Throbbing headache 2.9 0.36 Chocolate as headache trigger 7.1 0.70 Cheese as headache trigger 4.9 0.68 Migraine versus TTH Other precipitants Precipitant LR+ (Migraine vs TTH) LR– (Migraine vs TTH) Any food 3.6 0.59 Stress 1.4 0.7 Alcohol 1.3 0.92 Weather change 1.4 0.87 Menses 1.2 0.82 Missing a meal 1.1 0.83 Lack of sleep 0.83 1.1 Perfume or odors 0.58 1.5 Migraine versus TTH POUND Number of LR+ Pulsatile quality Features (Migraine Present Compared to Duration of 4 - 72 hours Tension Type Unilateral location Headache) Nausea or vomiting 1–2 0.41 Disabling intensity 3 3.5 4–5 24 Chronic Headaches Not a specific headache type and not an official class in the ICHD per se 15 of the headache episodes per month for 3 months Epidemiology 1 - 4% of U.S. adults >30% of these report daily symptoms 40% of patients seen in specialized headache clinics 3 to 5 times more common in women than in men Most chronic primary headaches are either migraines or tension-type headaches; Nevertheless, about 30% to 50% of patients who develop chronic headaches have medication overuse headache (MOH) – medication review is important Serious pathologies don’t often cause frequent headaches, but they must be considered, even in patients with confirmed primary headaches Chronic Headaches Risk factors for progression from episodic to chronic headaches Strongest predictive factors for headaches progressing from episodic to chronic: Frequent headache episodes at baseline Medication overuse Others: Chronic pain, especially musculoskeletal pain Cutaneous allodynia Sleep disorders Obesity High caffeine consumption Stressful life events, especially in middle age Poor prognosis in patients with chronic headache is associated with psychosocial factors, anxiety, mood disorders, poor sleep, stress, and low headache management self-efficacy Chronic Headaches Chronic migraine May evolve from episodic migraine Patient typically report progressively frequent bilateral frontotemporal TTH-type symptoms with superimposed full- blown migraine attacks Comorbidities: obesity, obstructive sleep apnea, depression, chronic pain disorders, cardiovascular disease, sleep and emotional disturbances Chronic Headaches Chronic Tension-type headache Long attack duration and nausea are predictive of development of chronic TTH Bilateral, non-pulsatile, absence of associated symptoms Pericranial tenderness is often found on palpation Assessing Impact of Frequent Headache Headache impact test “This questionnaire was designed to help you describe and communicate the way you feel and what you cannot do because of headaches” Headache diary Document date, duration, symptoms, treatment, and outcome, suspected triggers or other observations PHQ-9, CAGE questionnaire (as indicated) Additional Diagnostic Tips In pediatric patients, history should be obtained from both parent and child Reconsider your working diagnosis over time I.e. consider whether the patient’s progress matches the natural history of your illness script Diagnosis may be inaccurate, incomplete, or changing Patients with primary headache may develop superimposed secondary headaches Referral and Follow-up Neurologist is recommended if: Not clearly a primary headache Red flag symptoms are detected Headaches do not improve with appropriate treatment Problems are too complex or require a multidisciplinary approach Monitor the headache pattern with regularly scheduled follow-up Patients should be educated to report the following: Signs of re-escalation of primary headaches Development of medication overuse headaches (MOH) Red flags related to serious secondary headaches Learning Outcomes Review 1. Analyze the classification, causes, and types of headaches, including primary and secondary headaches, and their specific characteristics. 2. Evaluate common and rare headache types, including migraines, tension-type headaches, cluster headaches, and life-threatening headaches. 3. Diagnose headaches using the International Headache Society's classification system, POUND mnemonic, and other clinical criteria. 4. Conduct a thorough patient history, including physical and neurologic examination, and red flag signs and symptoms in diagnosing headaches. 5. Determine when and how to use neuroimaging, lumbar puncture, and other diagnostic tests appropriately in evaluating headaches. 6. Explain the significance of headache characteristics, such as onset, duration, frequency, pain character, location, and associated features in diagnosis. Learning Outcomes Review 7. Construct and rank a differential diagnosis based on demographic information, risk factors, symptoms, and signs. 8. Assess the etiology, prevalence, and impact of headache disorders on patients' quality of life, including those in children and adolescents. 9. Correlate modifiable risk factors, medication overuse, and lifestyle factors with headache severity and frequency. 10. Monitor patient progress, reassess the working diagnosis, and address factors driving headache escalation. 11. Demonstrate the importance of providing patient reassurance and addressing concerns when appropriate. References 1. Douglas VC, Aminoff MJ. Headache. In: Papadakis MA, McPhee SJ, Rabow MW, McQuaid KR. eds. Current Medical Diagnosis & Treatment 2023. McGraw Hill; 2023. Accessed May 06, 2023. https://accessmedicine-mhmedical- com.ccnm.idm.oclc.org/content.aspx?bookid=3212&sectionid=269151281 2. Rusiecki J. Approach to the Patient with Headache - Case 1. In: Stern SC, Cifu AS, Altkorn D. eds. Symptom to Diagnosis: An Evidence-Based Guide, 4e. McGraw Hill; 2020. 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