Rehan Pradhan Internal Medicine Resident PAHS Headache is among the most common medical complaints and accounts for 1% to 2% of emergency department evaluations and up to 4% of medical office visits. Although most often a benign condition (especially when chronic and recurrent), headache of new onset may be the earliest or principal manifestation of serious systemic or intracranial disease. Headache is caused by traction, displacement, inflammation, or distention of the pain-sensitive structures in the head or neck. A. Within Cranial Vault include venous sinuses; anterior and middle meningeal arteries; dura at skull base; falx cerebri; CN V, IX & X; proximal portions of ICA and its branches near circle of Willis; periaqueductal gray matter and sensory nuclei of thalamus. The ventricular ependyma, choroid plexus, pial veins, and much of brain parenchyma are not pain producing. B. Extracranial Structures include periosteum of skull; skin; subcutaneous tissues, muscles, and arteries; neck muscles; C2 & C3 cervical nerves; eyes, ears, teeth, sinuses, and oropharynx; and mucous membranes of nasal cavity. The key structures involved in primary headache are: ◦ Large intracranial vessels and dura mater, and peripheral terminals of trigeminal nerve that innervate these structures ◦ Caudal portion of trigeminal nucleus, which extends into dorsal horns of upper cervical spinal cord and receives input from first and second cervical nerve roots (Trigeminocervical complex) ◦ Rostral pain processing regions, such as ventroposteromedial thalamus and cortex ◦ Pain modulatory systems such as hypothalamus and brainstem Trigeminovascular system innervates large intracranial vessels and duramater via CN V and synapses in the trigeminocervical complex. Trigeminal activation can mediate the cranial parasympathetic responses via pterygopalatine ganglion, which are secondary events in headache cascade. Cranial autonomic symptoms, such as lacrimation, conjunctival injection, nasal congestion, rhinorrhea, periorbital swelling, aural fullness, and ptosis, are prominent in trigeminal autonomic cephalalgias, including cluster headache and paroxysmal hemicrania, and may also be seen in migraine. Site Headache Unilateral Invariable feature of cluster headache and majority of migraine Bilateral Most tension-type headache Ocular or retro orbital pain Acute iritis, glaucoma, optic neuritis, or retro-orbital inflammation (eg, Tolosa–Hunt syndrome), also common in migraine or cluster headache Paranasal pain Sinusitis Bandlike Tension-type headache Pain within V1 Postherpetic neuralgia (Burning in quality) V2 or V3 Trigeminal neuralgia or tic douloureux (Lancinating pain) Pharynx, EAM Glossopharyngeal neuralgia Onset of pain Character Headache Pulsating or throbbing pain Migraine Steady tightness or pressure Tension-type headache Dull and steady Intracranial mass lesions Sharp, lancinating, stabbing Neuritic cause such as trigeminal neuralgia Icepick-like pain Migraine, cluster headache, or giant cell arteritis Burning pain Postherpetic neuralgia Trigeminal nerve carries sensation from intracranial structures in anterior and middle fossae of the skull (above the cerebellar tentorium). Discrete intracranial lesions in these locations can produce pain that radiates in trigeminal nerve distribution. Glossopharyngeal (IX) and vagus (X) nerves convey sensation from part of posterior fossa; pain originating in this area may also be referred to ear or throat, as in glossopharyngeal neuralgia. Upper cervical (C2-C3) nerves transmit stimuli from the infra tentorial and cervical structures; therefore, pain from posterior fossa lesions often projects to C2-C3 dermatomes. Association Headache Recent wt loss Cancer, giant cell arteritis, or depression Fever or chills Systemic infection or meningitis Visual symptoms Involving optic nerve or tract or central visual pathways Nausea/Vomiting Migraine, posttraumatic headache and intracranial mass Photophobia Migraine, acute meningitis, or subarachnoid hemorrhage Myalgias Tension-type headache, systemic viral infns, giant cell arteritis Rhinorrhea Cluster headache (with lacrimation), Sinusitis Transient loss of consciousness Basilar migraine and glossopharyngeal neuralgia (due to cardiac syncope); Subdural hematoma. Episodic and may be worse during menses and may occur at varying intervals. May be present every day, esp stressful situations and are often maximal at the end of a workday Frequently awaken patients from sleep; often recur at same time each day or night; in bouts separated by symptom-free periods. Severe with time Precipitant Headache Stress or fatigue, menses, hunger, fasting, consumption of ice cream or foods with nitrite (hot dogs, salami, ham, and most sausage), phenylethylamine (chocolate) or tyramine (cheddar cheese), exposure to bright lights, OCP, Nitrates Migraine Coughing or sneezing Structural lesion in posterior fossa Chewing and eating Glossopharyngeal neuralgia and tic douloureux Alcohol Cluster headache Acute URTI or hay fever Chronic sinusitis Exacerbating Headache Relieving factors Anger, excitement, irritation Migraine and TTH Rapid changes in head position such as bending over or by events that transiently raise ICP, such as coughing and sneezing Migraine Darkness, sleep, vomiting, or I/L temporal artery pressure, may diminish during pregnancy Coughing and sneezing Intracranial SOL Less severe on standing Postural headache (maximal when upright, nearly absent when lying down) occurs with low CSF pressure Post LP and low Recumbency pressure headache Stooping, bending forward, sneezing or blowing nose Sinusitis General condition of patient Vital signs: Temperature, Pulse, Blood pressure, Respiratory rate Head to toe examination: Built, Cyanosis, skin lesions, Scalp tenderness, Nodularity, erythema, or tenderness over temporal artery, Sinus tenderness, Arterial bruit over orbit or skull, Tongue laceration, Carotid bruits, cervical muscle spasm, Papilledema Neurological examination: Higher mental functions, CNs, motor, sensory, signs of meningeal irritations Cardiovascular system: Murmur Respiratory examination: Chronic lung disease Primary headaches are those in which headache and its associated features are the disorder itself, whereas secondary headaches are caused by exogenous disorders. Primary headache often results in considerable disability and decrease in quality of life. It is a chronic head-pain syndrome with bilateral tight, band like discomfort that builds slowly, fluctuates in severity, and may persist continuously for many days. It may be episodic or chronic without nausea, vomiting, photophobia, phonophobia. Simple analgesics such as acetaminophen, aspirin, or NSAIDs. Behavioral approaches including relaxation can also be effective. For chronic TTH, amitriptyline is the proven treatment. Group of primary headaches that includes ◦ Cluster headache ◦ Paroxysmal hemicrania ◦ SUNCT (Short-lasting Unilateral Neuralgiform headache attacks with Conjunctival injection and Tearing) Short lasting attacks associated with cranial autonomic symptoms, such as lacrimation, conjunctival injection or nasal congestion. Pain is usually severe and may occur more than once a day. Misdiagnosed with "sinus headache” CH is relatively uncommon affecting fewer than 1 in 1000 adults, affecting six men to each woman. Most people developing CH are in their 20s or older. It is characterized by frequently recurring (up to several times a day), brief but extremely severe headache, usually focused in or around one eye, with tearing and redness of the eye, the nose runs or is blocked on the affected side and the eyelid may droop. CH has episodic and chronic forms. Migraine is one of the common causes of recurrent headaches According to IHS, migraine constitutes 16% of primary headaches Migraine afflicts 10-20% of the general population. 15-20% of women and 10-15% of men suffer from migraine. Adults – Female: Male ratio is 2 : 1. In childhood migraine, boys and girls are affected equally until puberty. Migraine is underdiagnosed and undertreated. WHO ranks migraine among the world’s most disabling medical illnesses According to World Federation of Neurology; “Migraine is familial disorder characterized by recurrent attacks of headache widely variable in intensity, frequency and duration. Attacks are commonly unilateral and are usually associated with anorexia, nausea and vomiting”. Triggers: Food, Disturbed sleep pattern, Hormonal changes, Drugs, Physical exertion, Visual stimuli, Auditory stimuli, Olfactory stimuli, Weather changes, Hunger, Psychological factors Diagnosis: Medical History, Headache diary, Migraine triggers, Investigations (only to exclude secondary causes): EEG, CT Brain, MRI MIDAS: Migraine Disability Assessment score According to Headache Classification Committee of International Headache Society, Migraine has been classified as: • Migraine without aura (common migraine) • Migraine with aura (classic migraine) • Complicated migraine Migraine Without Aura No aura or Prodrome Migraine With Aura Aura or prodrome is present Unilateral throbbing headache may Unilateral throbbing headache and be accompanied by N/V later becomes generalised Patient complains of phonophobia and photophobia Patient complains of visual disturbances and may have mood variations • Non-pharmacological treatment – Identification of triggers – Meditation – Relaxation training – Psychotherapy • Pharmacotherapy – Abortive therapy Non-specific Specific – Preventive therapy Non specific treatment: Acetaminophen, Aspirin, Diclofenac, Ibuprofen, Naproxen Specific treatment Drug Dose Route Ergot alkaloids Ergotamine 1-2 mg/d; max-6 g/d Oral Dihydroergotamine 0.75-1 mg SC 5-HT receptor agonists (5HT1B & 5HT1D and some 5HT1F agonism) Sumatriptan 25-300 mg 6 mg Orally SC Rizatriptan 10 mg Orally cGRP receptor antagonist, Gepants have been shown to be effective in acute treatment of migraine and monoclonal antibodies to cGRP have been shown to be effective in 2 early phase clinical trials. Ditans: 5HT1F agonists • • • • • • • • • Patients who have very frequent headaches (>2 per week) Attack duration is > 48 hours Headache severity is extreme Migraine attacks are accompanied by prolonged aura Unacceptable adverse effects occur with acute migraine treatment Contraindication to acute treatment Migraine substantially interferes with the patient’s daily routine, despite acute treatment Special circumstances such as hemiplegic migraine or attacks with a risk of permanent neurologic injury Patient preference Drugs Dose (mg/d) Betablockers Propranolol 40-320 Calcium Channel Blockers Flunarizine Verapamil 10-20 120-480 TCAs: Amitriptyline 10-20 SSRIs: Fluoxetine 20-60 Anticonvulsant: Sodium valproate Antihistaminic: Cyproheptadine 600-1200 4-8 Clinical Neurology 10th edition, Roger P. Simon, Michael J. Aminoff, David A. Greenberg Harrison’s Principles of Internal Medicine – 21st edition Uptodate