HEADACHES PBL STEVEN J. SCHEINER, M.D. Board Certified in Pain Medicine Board Certified in Neurology Diplomate, American Academy of Pain Management Senior Physician, Pain Relief Unit Department of Anesthesiology and Critical Care Hadassah Medical Center

IHS Classification ICHD-II The International Classification of Headache Disorders. 2nd edition. Cephalalgia 2004; 24(Suppl 1): 9–160

Migraine without Aura At least 5 attacks At least 5 attacks Headache attacks lasting 4-72 hours if untreated Headache attacks lasting 4-72 hours if untreated Has at least two of the following, but not weakness: Has at least two of the following, but not weakness: 1. unilateral location 2. pulsating quality 3. moderate or severe pain intensity 4. aggravation by or causing avoidance of routine physical activity Has at least one of the following: Has at least one of the following: 1. nausea and/or vomiting 2. photophobia and phonophobia Not attributed to another disorder Not attributed to another disorder The International Classification of Headache Disorders. 2nd edition. Cephalalgia 2004; 24(Suppl 1): 9–160

Migraine with Aura At least two headache attacks that fulfill the characteristics of migraine without aura At least two headache attacks that fulfill the characteristics of migraine without aura Headaches usually follow the aura but may begin with it & last 4-72 hours if untreated Headaches usually follow the aura but may begin with it & last 4-72 hours if untreated Has at least one of the following reversible symptoms (lasting 4-60 min) but no weakness: Has at least one of the following reversible symptoms (lasting 4-60 min) but no weakness: 1. Positive or negative visual symptoms such as scintillating scotomas, blind spot (scotoma), blurred vision, zigzag lines, homonymous hemianopsia 2. Positive or negative sensory symptoms such as tingling or numbness The International Classification of Headache Disorders. 2nd edition. Cephalalgia 2004; 24(Suppl 1): 9–160

Aura Without Headache At least two attacks of symptoms typical of auras, but not weakness, such as visual, sensory or speech disturbances that resolve in 1 hour & are not followed by headache At least two attacks of symptoms typical of auras, but not weakness, such as visual, sensory or speech disturbances that resolve in 1 hour & are not followed by headache The International Classification of Headache Disorders. 2nd edition. Cephalalgia 2004; 24(Suppl 1): 9–160

Tension-Type Headache (TTH) Subclassified by Frequency HA lasting 30 minutes to 7 days HA lasting 30 minutes to 7 days Has at least two of the following: Has at least two of the following: 1. Bilateral location 2. Mild or moderate pain intensity (may inhibit but not prohibit activity) 3. Pressing/tightening (nonpulsating) quality 4. No aggravation while climbing stairs or similar routine physical activity Has both of the following: Has both of the following: 1. No nausea or vomiting (can have anorexia) 2. No more than one of photophobia and phonophobia Not attributed to another disorder Not attributed to another disorder The International Classification of Headache Disorders. 2nd edition. Cephalalgia 2004; 24(Suppl 1): 9–160

Infrequent Episodic TTH At least 10 episodes At least 10 episodes Less than 1 day/month Less than 1 day/month (Average less than 12 days/year) (Average less than 12 days/year)

Frequent Episodic TTH At least 10 episodes At least 10 episodes 1 or more days/month but less than 15 days/month for at least 3 months 1 or more days/month but less than 15 days/month for at least 3 months (12 or more days/year & less than 180 days/year) (12 or more days/year & less than 180 days/year)

Chronic TTH 15 or more days/month for more than 3 months 15 or more days/month for more than 3 months (180 or more days/year) (180 or more days/year) Headache lasts hours or may be continuous Headache lasts hours or may be continuous

Case # 1: Prof. Ben-Hur Female age16 with Migraine History at ER Female age16 with Migraine History at ER No fever No fever Non Focal Neuro Exam Non Focal Neuro Exam No Meningeal Signs Per Neurology No Meningeal Signs Per Neurology Initially Tx with Analgesics & IV Fluid Initially Tx with Analgesics & IV Fluid

Case # 1 Continued Headache Worsened Despite Tx Headache Worsened Despite Tx Patient Became Hyperactive & Agitated with Increasing Pain Patient Became Hyperactive & Agitated with Increasing Pain Otherwise No Change in Exam Otherwise No Change in Exam Tx with Diazepam Tx with Diazepam WBC 16 Attributed to Stress Reaction WBC 16 Attributed to Stress Reaction Final Disposition & Diagnosis Final Disposition & Diagnosis

Red Flag # 1 Altered Mental Status w Headache Altered Mental Status w Headache Do Not Need Other Findings Do Not Need Other Findings Differential Dxs Include: Bacterial Meningitis Bacterial Meningitis Encephalitis: Herpes is a Neuro Emergency Encephalitis: Herpes is a Neuro Emergency Fungal, Parasitic & Non Infectious Fungal, Parasitic & Non Infectious SAH SAH

#2 AECOM Med Student Husband Jacobi Hospital ER Jacobi Hospital ER Mid 20s with New Moderately Severe Headache Mid 20s with New Moderately Severe Headache Throbbing, No N/V/Photo/Phono Throbbing, No N/V/Photo/Phono No Prior Headache History No Prior Headache History Non Focal Neuro Exam Non Focal Neuro Exam Responded to NSAID Tx Responded to NSAID Tx Discharged Discharged

Case # 2 Continued Two Months Later Returns to ER Two Months Later Returns to ER Headache Recurrence Headache Recurrence Similar to Initial Headache But Less Severe Similar to Initial Headache But Less Severe Non Focal Neuro Exam Non Focal Neuro Exam Responded Well to a Different NSAID Responded Well to a Different NSAID Discharged Discharged

Case # 2 Continued Returns Again to ER in Three Months Returns Again to ER in Three Months Final Disposition & Diagnosis Final Disposition & Diagnosis

Sentinel Headaches Proceed Aneurysmal SAH 20-50% Proceed Aneurysmal SAH 20-50% May be Self Limited or Relieved by Analgesics or Triptans May be Self Limited or Relieved by Analgesics or Triptans Recognition Can Be Lifesaving Recognition Can Be Lifesaving Mechanism: Tiny Bleed or Pressure on Pain Sensitive Intracranial Structures Mechanism: Tiny Bleed or Pressure on Pain Sensitive Intracranial Structures

Case # 3 Hadassah Pain Clinic Male Age 32 with Two Migraines/Year Male Age 32 with Two Migraines/Year 1 st Migraine in Childhood & + FH 1 st Migraine in Childhood & + FH Presents w 1 ½ Weeks of New Daily Headache Presents w 1 ½ Weeks of New Daily Headache Throbbing, Very Mild N/Photo/Phono Throbbing, Very Mild N/Photo/Phono Much Less Severe than Usual Migraine Much Less Severe than Usual Migraine

Case # 3 Continued At Shaare Zedek ER for 3 Days Previous Week At Shaare Zedek ER for 3 Days Previous Week Negative CT of Head Negative CT of Head Unsuccessful LP Unsuccessful LP D/C with Steroid Tapper for Status Migranosis D/C with Steroid Tapper for Status Migranosis Disposition & Diagnosis Disposition & Diagnosis

Diagnosis Case # 3 New Daily Persistent Headache (NDPH) New Daily Persistent Headache (NDPH) Posttraumatic in a very broad way Posttraumatic in a very broad way As a result of viral meningitis As a result of viral meningitis This is a typical presentation of NDPH & more common than after a blow to head This is a typical presentation of NDPH & more common than after a blow to head Also seen after malarial meningitis Also seen after malarial meningitis

IHS Classification ICHD-II The International Classification of Headache Disorders. 2nd edition. Cephalalgia 2004; 24(Suppl 1): 9–160

Cluster Headache At least 5 attacks fulfilling the following: At least 5 attacks fulfilling the following: Severe or very severe unilateral orbital, supraorbital and/or temporal pain lasting minutes if untreated1 Severe or very severe unilateral orbital, supraorbital and/or temporal pain lasting minutes if untreated1 Headache is accompanied by at least one of the following: Headache is accompanied by at least one of the following: 1. ipsilateral conjunctival injection and/or lacrimation 2. ipsilateral nasal congestion and/or rhinorrhoea 3. ipsilateral eyelid edema 4. ipsilateral forehead and facial sweating 5. ipsilateral miosis (pupillary constriction) and/or ptosis 6. a sense of restlessness or agitation Attacks have a frequency from one every other day to 8/day Attacks have a frequency from one every other day to 8/day Not attributed to another disorder Not attributed to another disorder The International Classification of Headache Disorders. 2nd edition. Cephalalgia 2004; 24(Suppl 1): 9–160

Cluster Headache During part (but less than half) of the time-course of cluster headache, attacks may be less severe and/or of shorter or longer duration & may also be may be less frequent During part (but less than half) of the time-course of cluster headache, attacks may be less severe and/or of shorter or longer duration & may also be may be less frequent Acute attacks involve activation of the posterior hypothalamic grey matter Acute attacks involve activation of the posterior hypothalamic grey matter Attacks usually occur in series (cluster periods) lasting for weeks or months separated by remission periods usually lasting months or years. However, about % of patients have chronic symptoms without remissions Attacks usually occur in series (cluster periods) lasting for weeks or months separated by remission periods usually lasting months or years. However, about % of patients have chronic symptoms without remissions

Cluster Headache In a large series with good follow-up, 27% of patients had only a single cluster period In a large series with good follow-up, 27% of patients had only a single cluster period During a cluster period, and in the chronic subtype, attacks occur regularly and may be provoked by alcohol, histamine or nitroglycerine During a cluster period, and in the chronic subtype, attacks occur regularly and may be provoked by alcohol, histamine or nitroglycerine During the worst attacks, the intensity of pain is excruciating & patients characteristically pace the floor During the worst attacks, the intensity of pain is excruciating & patients characteristically pace the floor Age at onset is usually years Age at onset is usually years For unknown reasons prevalence is 3-4 times higher in men than in women For unknown reasons prevalence is 3-4 times higher in men than in women

Cluster Headache Plus Some patients have been described who have both cluster headache and trigeminal neuralgia Some patients have been described who have both cluster headache and trigeminal neuralgia They should receive both diagnoses They should receive both diagnoses The importance of this observation is that both conditions must be treated for the patient to be headache free The importance of this observation is that both conditions must be treated for the patient to be headache free

Cluster Headache & Other Trigeminal Autonomic Cephalalgias (TAC) 3.1 G44.0 Cluster headache ( min. & 1-8/day) 3.1 G44.0 Cluster headache ( min. & 1-8/day) G44.01 Episodic cluster headache G44.01 Episodic cluster headache G44.02 Chronic cluster headache G44.02 Chronic cluster headache 3.2 G44.03 Paroxysmal hemicrania (1-45 min. & 1-40/day) 3.2 G44.03 Paroxysmal hemicrania (1-45 min. & 1-40/day) G44.03 Episodic paroxysmal hemicrania G44.03 Episodic paroxysmal hemicrania G44.03 Chronic paroxysmal hemicrania G44.03 Chronic paroxysmal hemicrania 3.3 G44.08 Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) ( sec. & 1/day-30/hour) 3.3 G44.08 Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) ( sec. & 1/day-30/hour) 3.4 G44.08 Probable TAC 3.4 G44.08 Probable TAC G44.08 Probable cluster headache G44.08 Probable cluster headache G44.08 Probable paroxysmal hemicrania G44.08 Probable paroxysmal hemicrania G44.08 Probable SUNCT G44.08 Probable SUNCT

Other Primary Headaches 4.1 G Primary stabbing headache 4.1 G Primary stabbing headache (secs-3 min. & any frequency) 4.2 G Primary cough headache 4.2 G Primary cough headache 4.3 G Primary exertional headache 4.3 G Primary exertional headache 4.4 G Primary headache associated with sexual activity 4.4 G Primary headache associated with sexual activity G Preorgasmic headache G Preorgasmic headache G Orgasmic headache G Orgasmic headache 4.5 G44.80 Hypnic headache (15-30 min. & 1-3/night) 4.5 G44.80 Hypnic headache (15-30 min. & 1-3/night) 4.6 G44.80 Primary thunderclap headache 4.6 G44.80 Primary thunderclap headache 4.7 G44.80 Hemicrania continua 4.7 G44.80 Hemicrania continua 4.8 G44.2 New daily-persistent headache 4.8 G44.2 New daily-persistent headache

Primary Thunderclap Headache The International Classification of Headache Disorders. 2nd edition. Cephalalgia 2004; 24(Suppl 1): 9–160

Secondary Causes of Thunderclap Headache The International Classification of Headache Disorders. 2nd edition. Cephalalgia 2004; 24(Suppl 1): 9–160

SAH “The Worst HA of My Life” Usually Sudden Onset with Exertion Sentinel HAs proceed aneurysmal SAH 20-50% Sentinel HAs proceed aneurysmal SAH 20-50% Recognition of these Has can be lifesaving Recognition of these Has can be lifesaving Thunderclap onset lasting hours-days Thunderclap onset lasting hours-days Meningismus tends to be absent Meningismus tends to be absent

Summary of Crucial Red Flags Altered Mental Status Altered Mental Status Sudden Onset Sudden Onset Focal Neurological Signs Focal Neurological Signs Seizures Seizures