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Headaches

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    • Headache or Cephalalgia is pain anywhere in the region of the head and neck.
    • The brain itself is not a pain-sensitive organ since it lacks pain receptors.
    • HA pain - due to pain-sensitive structures located in the head and neck region.

    Pain-Sensitive Pain-Insensitive
    Cranial venous sinuses with afferent veins Brain parenchyma
    Arteries at base of brain and their major branches Ependyma
    Arteries of the dura Choroid
    Dura near base of brain and large arteries Pia
    Dural, Cranial and extracranial nerves Arachnoid
    All extracranial structures Dura over convexity
    Skull

    Primary

    • Migraine
    • Migraine without aura (common migraine)
    • Migraine with aura (classic migraine)
    • Cluster Headache (recurrent severe periorbital attacks)
    • Tension-type headache (e.g., posterior neck muscle contraction)

    Secondary HA

    • Associated with vascular disorders:
      • Subarachnoid hemorrhage (Emergency!)
      • Temporal arteritis (risk of blindness)
      • Venous thrombosis
      • Intracranial hematoma (including epidural, subdural)
      • Severe arterial hypertension
    • Associated with nonvascular intracranial disorders:
      • Infection (meningitis, abscess, sinusitis)
      • Increased CSF pressure (intracranial mass lesion or hydrocephalus)
    • Miscellaneous:
      • Medication: side effect of nitroglycerin or withdrawal (analgesics)
      • Psychological disorders

    • Onset (Acute vs slowly progressive), frequency, duration (hours, days?), quality, intensity (worse ever?), location,
    • Triggers (worse with exertion?, food? menstruation?) and ameliorating factors, associated symptoms (pulsatility, photophobia, phonophobia)
    • Functional impairment (work, IADLs)
    • Red flags (neurological symptoms, severity of disability) for serious disease to differentiate between the causes of headache.
    • Select patients in need of immediate management based on red flags on history and physical signs. Clinical history, diagnostic imaging, laboratory findings
    • Vitals, Level of consciousness, Head and neck, Neurological exam (especially visual, motor, reflex, sensory, speech, or cognitive). If positive finding, investigation is warranted.
    • Identify patients that require referral/brain imaging:
      1. New/explosive onset
      2. Change in pattern
      3. Jaw claudication
      4. Limb girdle pain
      5. Worse with stooping over, straining, coughing
      6. Neurological signs on exam
      7. Temporal artery tenderness
    • X-rays and other tests may also be used if sinusitis is suspected.

    Diagnostic Alarm Signs for Serious Headache (Red Flags)

    • Sudden onset
    • Onset after 50 years
    • Increased frequency and severity
    • New onset in HIV or cancer patients
    • Associated with systemic illness – hypertension, diabetes, neck aches, fever, rash
    • Altered consciousness, focal neurologic deficit
    • Papilledema
    • Trauma

    • Migraine without aura (common migraine)
    • Migraine with aura: Aura are transient focal neurological symptoms that often precede or accompany the headache.
    • Cluster Headache: Involves recurrent severe periorbital attacks.
    • Tension-type headache: Involves posterior neck muscle contraction.

    Migraine

    Migraine is a common disabling primary headache disorder, ranked as the 3rd most common disorder worldwide. Characteristics include:

    • Pulsatile
    • Lasts 2-72 hours
    • Incapacitating photophobia and phonophobia

    Migraine ISH Diagnostic Criteria

    • At least 6 attacks of headache with the following criteria:

    Headache Characteristics:

    • Headache lasting 4-72 hours.
    • Must have 2 of the following:
      1. Unilateral
      2. Pulsating
      3. Moderate-severe intensity.
      4. Aggravated by physical activity

    Associated Symptoms:

    • Nausea
    • Vomiting
    • Photophobia
    • Phonophobia

    Migraine with Aura

    • Aura:
    • Visual aura is the most common. Subtypes:
      • Photopsia - Flashes of white and/or black or rarely of multicolored lights
      • Scintillating Scotoma - Dazzling zigzag lines. It could be tunnel vision, hemianopsia
    • Auditory or olfactory hallucinations,
    • Somatosensory - Paresthesia e.g. pins-and-needles can migrate up the arm → face, lips, and tongue (ipsilateral). Or temporary dysphasia, vertigo, and hypersensitivity to touch. Differential diagnosis: TIA

    Epidemiology

    • More common in individuals with family history.
    • More common in women, especially women with a mother with migraine.
    • Prevalence: 19% of women in the general population.

    Common Migraine Triggers

    • Food - Cheese and chocolates
    • Alcohol/red wine
    • Menstruation
    • Stress and worry
    • Lack of sleep/oversleep
    • Fatigue
    • Hunger

    Pathophysiology of Migraine

    • Vasodilation - no longer tenable
    • Neurogenic - Cortical spreading depression activates the trigeminal and parasympathetic systems, which causes vasodilation and release of neuropeptides that cause inflammation.
    • Serotonin 5-HT receptors modulate the release of neurogenic peptides.

    Tension Type Headache

    IHS Criteria

    • Tension-type headaches occur < 15 times per month.
    • Lasts from 30 minutes to 7 days.
    • No nausea or vomiting.
    • No photophobia and phonophobia (1 ok).
    • Headache has at least 2 of the following criteria:
      1. Pressing/tightening
      2. Bilateral
      3. Mild-moderate
      4. Not aggravated by physical activity.

    Cluster Headache

    ISH Diagnostic Criteria

    • Duration: 15 to 180 minutes if untreated.
    • Characteristics: Severe periorbital, supraorbital, or temporal pain.
    • Associated Symptoms:
      • Conjunctival injection
      • Lacrimation
      • Nasal congestion
      • Rhinorrhea
      • Forehead and facial swelling
      • Miosis
      • Ptosis
      • Eyelid edema
    • Frequency: Between one every other day to 8/day.

    Rebound Headaches

    • Headache for 15 days/month with at least one of the following characteristics and 2, 3, and 4:
      1. Bilateral
      2. Pressing/tight non-pulsating quality
      3. Mild/moderate intensity
    • Simple analgesic use >15 days a month for 3 months.
    • Headache has increased during analgesic use.
    • Headache resolves or reverts to previous pattern within 2 months after discontinuation of analgesia.

    • Intracranial hemorrhage
    • Subdural hemorrhage
    • Subarachnoid hemorrhage
    • Meningitis
    • Hypertensive encephalopathy

    Subarachnoid Hemorrhage (SAH)

    • Extravasated blood in the subarachnoid space. The blood activates meningeal nociceptors, leading to diffuse occipital pain along with signs of meningismus.
    • Severe and sudden headache
    • SAH accounts for up to 10% of all strokes and is the most common cause of sudden death from a stroke.
    • Of the hemorrhagic strokes, half are due to a subarachnoid hemorrhage and half due to intracerebral hemorrhage.
    • Intracerebral hemorrhage is associated with HTN and AVM.

    Risk Factors for SAH

    • Estimated that 5% of the population have a berry aneurysm.
    • HTN
    • Smoking and alcohol
    • Sympathomimetic drugs
    • Polycystic kidney disease
    • Coarctation of the aorta
    • Marfan's syndrome

    Hypertensive Encephalopathy

    • Associated with high blood pressure, nausea, vomiting, and blurred vision.
    • Usually associated with blood pressures of 200/130.
    • Headache is diffuse and worse in the morning, subsiding during the day.

    Bacterial Meningitis

    • Fever and severe headaches
    • Neck pain and stiffness (+Kernig's sign)
    • Altered level of consciousness
    • The absence of fever, neck stiffness, and altered mental status in a patient with a headache virtually eliminates the diagnosis of meningitis.
    • In multiple studies, the presence of neck stiffness on examination has a pooled sensitivity of 70%.


    Management of Headache

    Clinical, Diagnostic Imaging, Laboratory Findings

    • Lumbar puncture if subarachnoid hemorrhage, encephalitis, high- or low-pressure headache symptoms, or meningitis is suspected.
    • Laboratory tests (on an individual basis)
    • ESR for suspected temporal arteritis
    • Endocrine, biochemical, infection work-up
    • Search for malignancy if indicated
    • Facial pain may need a thorough assessment by a dental specialist familiar with headaches and facial pain and/or an ENT specialist if sinus or other ENT disorders are suspected.

    • Symptomatic treatment- Analgesics:
      • Ibuprofen, naproxen, and ASA or acetaminophen with or without codeine and/or butalbital, are used for mild to moderate h/a pain.
      • Non-opioid meds should be used less than 15 days per month (to prevent rebound h/a).
      • Medication over-use h/a can result from overuse of analgesics/rebound/dependency, which limits their long-term potential.
    • Ergot derivatives:
      • Ergotamine acts on serotonin receptors and is classically used for migraines and cluster h/a but use is limited by side effects. They may cause rebound h/a if used 10 days per month or more.
    • Triptans:
      • They act on the serotonin (HT-5) subclass 1B and 1D receptor, on extracerebral blood vessels and neurons, and the mechanism of action is prevention of neurologically sterile inflammatory responses around vessels and vasoconstriction.
      • They are contraindicated in patients with cardiac disorders, sustained hypertension, basilar and hemiplegic migraine.
      • Other classes of drugs:
        • Corticosteroids can be useful in many h/a disorders, including status migraine, cluster h/a, and cerebral neoplasms with edema (especially metastatic lesions, temporal arteritis).
        • Other drugs include metoclopramide (maxeran), phenothiazines, Ketorolac, meperidine, indomethacin, dimenhydrinate, and domperidone.

    Indication for Migraine Prophylaxis

    • Interferes with patient's daily routine/QOL.
    • >2 attacks/month
    • Acute medications ineffective or contraindicated.
    • Presence of uncommon migraine conditions:
      • Hemiplegic migraine
      • Basilar migraine
      • Migraine with prolonged aura.
    • Beta blockers
    • Tricyclics
    • Calcium channel blockers
    • AED-valproate, topiramate, and gabapentin
    • Methylsergide

    Patient Education and Counseling for Management of Benign Headache Syndrome:

    • A calendar or diary of headaches is useful for follow-up assessment.
    • A record of medications (usefulness, dosage, side effects) should be kept.
    • Reassurance and explanation are most important to the patient in the long term.
    • Always offer hope to the patient with chronic headaches even if no cure is available; most primary headaches can be controlled.
    • For tension headaches, attempt to modify or eliminate the stressor with behavior modification, biofeedback, relaxation therapy, yoga, exercise, and so on.

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