Headaches Other Than Migraines Less Common Headache Syndromes. Objectives. Headache Overview 2/12/2015

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1 Headaches Other Than Migraines Less Common Headache Syndromes Albert Simon DHSc, MEd., PA C Professor and Chair Department of Physician Assistant Sciences Objectives 1. Identify the key clinical features of the less common headache syndromes and be cognizant of initial workup or referral strategies for these syndromes 2. Recognize the danger signs of headache from the history and physical examination. 3. When confronted with a patient scenario, use key history, physical exam, lab and imaging data to arrive at a correct diagnosis Headache Overview Up to 80% of the population suffers from headache at some time 46% of adults worldwide report an active headache disorder Headache is one of the 10 most disabling conditions experienced by both genders Headache is the 5 th most disabling disorder experienced by females. Many headaches are incorrectly diagnosed 1

2 Common Headaches by Classification Primary Headache Secondary Headache Type % Type % Migraine 16 Systemic infection 63 Tension type 69 Head injury 4 Cluster 0.1 Vascular disorders 1 Idiopathic 2 Subarachnoid <1 stabbing hemorrhage Exertional 1 Brain tumor 0.1 Data from Olesen J, Goadsby PJ, Ramadan N, et al. The Headaches. Philadelphia: Lippincott Williams & Wilkins; Primary headaches 1. Migraine 2. Tension type headache 3. Trigeminal autonomic cephalalgias 4. Other primary headache disorders Cranial neuralgias, central and primary facial pain and other headaches 13. Cranial neuralgias and central causes of facial pain 14. Other headache, cranial neuralgia, central or primary facial pain headache.org/home and news IHS Classification ICHD II Secondary headaches 5. Headache attributed to trauma or injury to the head and/or neck 6. Headache attributed to cranial or cervical vascular disorder 7. Headache attributed to nonvascular intracranial disorder 8. Headache attributed to a substance or its withdrawal 9. Headache attributed to infection 10. Headache attributed to disorder of homoeostasis 11. Headache or facial pain attributed to disorder of the cranium, neck,eyes, ears, nose, sinuses, teeth, mouth or other facial or cervical structure 12. Headache attributed to psychiatric disorder Caveats Concerning Headaches About 90% of headache patients seen in primary care will have a primary headache disorder. The vast majority of these will be either migraine or tension type headaches Thus our job in primary care is to identify those folks who have an uncommon cause of headache from the much larger group of headache patients that we see 2

3 Sorting Through History and Physical Exam are key to appropriate diagnosis Imaging studies are rarely indicated in the primary care setting A good history will supply most of the information needed to make the diagnosis Physical exam primarily to rule out secondary causes of headache Target red flag issues on both history and physical exam Red Flags in Headache Red Flag Differential Diagnosis Possible Workup Headache beginning after 50 y of age Temporal arteritis, mass lesion Erythrocyte sedimentation rate, neuroimaging Very sudden onset of headache Subarachnoid hemorrhage, pituitary Neuroimaging, lumbar puncture if apoplexy, hemorrhage into a mass lesion or computed tomography is negative vascular malformation, mass lesion (especially posterior fossa mass) Headaches increasing in frequency and Mass lesion, subdural hematoma, Neuroimaging, drug screen severity medication overuse New onset headache in patient with risk Meningitis (chronic or carcinomatous), Neuroimaging, lumbar puncture if factors for HIV infection or cancer brain abscess (including toxoplasmosis), neuroimaging is negative metastasis Headache with signs of systemic illness (eg, Meningitis, encephalitis, Lyme disease, Neuroimaging, lumbar puncture, serology fever, still neck, rash) systemic infection, collagen vascular disease Focal neurologic signs or symptoms of Mass lesion, vascular malformation, Neuroimaging, collagen vascular evaluation disease (other than typical aura) stroke, collagen vascular disease (including antiphospholipid antibodies) Papilledema Mass lesion, benign intracranial Neuroimaging, lumbar puncture hypertension (pseudotumor cerebri), meningitis Headache following head trauma Intracranial hemorrhage, subdural hematoma, epidural hematoma, posttraumatic Neuroimaging of brain, skull, and, possibly, cervical spine headache From: CURRENT Diagnosis & Treatment in Family Medicine, 3e Key Points in the History The single most important question is how long have you had the headache? 3

4 Access Medicine: I Have a Patient with Headache. How Do I Determine the Cause? Symptom to Diagnosis: An Evidence Based Guide, 2e, 2010 Cases Some cases modified from: Access Medicine: I Have a Patient with Headache. How Do I Determine the Cause? Symptom to Diagnosis: An Evidence Based Guide, 2e, 2010 Case 1 BobM is a 30 year old man who arrives at your primary care office complaining of a headache. He has a long history of mild tension type headaches managed with OTC analgesics. Yesterday he was helping his brother move furniture and while lifting a very heavy dresser he suddenly developed a severe headache. He describes this headache as the worst headache of his life. The headache slowly resolved over about 2 hours. He is now feeling completely well. 4

5 Case 1 What are the red flags in this history? What type of headache is this? Worst headache of my life, what is the main differential? Subarachnoid Hemorrhage Subarachnoid hemorrhage is the leakage of blood into the subarachnoid space. Seventy five percent of subarachnoid hemorrhages are caused by a ruptured aneurysm. Subarachnoid Hemorrhage Most present with thunderclap headache worst headache of my life reaches maximal intensity within minutes May have sentinel headache Watch for vomiting, syncope, altered mental status, focal neurologic signs 5

6 Subarachnoid Hemorrhage True or False? Roughly half of aneurysmal SAH occur during nonstrenuous activity, rest, or sleep? Subarachnoid Hemorrhage Subhyaloid Retinal Hemorrhage Case 1 BobM s past medical history is notable only for hypertension controlled by Lisinopril 5mg qd. On physical exam, he appears well and not in any distress. His vital signs are temperature, 36.9 C; pulse, 82 bpm; BP, 112/82 mm Hg; RR, 14 breaths per minute. His neck is supple and detailed neurologic exam is also normal. 6

7 Case 1 A noncontrast CT scan and lumbar puncture are performed and are WNL. What are the other Diagnostic Considerations? Have to consider the following: Primary Cough Headache Presents as generalized headache that begins suddenly associated with bouts of coughing, laughing or sneezing. Typically lasts only a few minutes Must exclude serious causes of headache before making this diagnosis. Must exclude vascular causes or congenital malformations that may displace cerebral. 7

8 Benign Exertional Headache Can resemble both cough headache and migraine. Key is link to exercise (any form) Generally lasts less than 30 minutes, may throb or pulsate Aggravated by hot weather or at high altitude Benign Thunderclap Headache Severe headache with rapid onset often with no provocation R/O major vascular catastrophes with first episode Headache Associated With Sexual Activity Also called orgasmic headache or sex headache is precipitated by sexual excitement. Three types have been described Dull headache that increases as sexual excitement increase An explosive headache at orgasm A post coital headache Males affected more than females although this type of headache is more common in migraine and exertional headache sufferers The headache typically lasts less than 2 hours 8

9 Case 2 JennyO is a 68 year old woman who comes to an outpatient clinic complaining of headaches x 1 month. She wakes up almost every morning with a moderate to severe headache. She describes the headache as bitemporal. Her past health is negative for having headaches of any consequence in the past. Case 2 JennyO reports otherwise feeling well. She says the headaches occur nearly every morning, irrespective of day of the week or whether she has slept at home or at her weekend house. She denies neurologic symptoms such as focal numbness, weakness, or visual disturbances. She denies snoring or excessive daytime somnolence. She read on an Internet site that new onset, morning headaches are classic for brain tumors, and she is very nervous. Case 2 A good question to ask most every patient 9

10 What is our can t miss R/O? Brain Tumor Classic wakes patient from sleep or morning headache, associated with position, N and V Most likely in patients with other cancers Lung 37%, breast 19%, melanoma 16% Metastatic tumors 7x more likely than primary Can present with seizure or signs of increased IP Exam for focal neuro deficient (about 8%) Papilledema 10

11 Case 2 JennyO s physical exam, including neurologic exam, is normal. What Study Should we Order Now? Case 2 Her medical history reveals noninsulindependent diabetes mellitus, which has been under good control. She reports no recent change in her diet, weight, or medication. She denies use of caffeine, alcohol or other medication. She sleeps well and her husband indicates she does not snore. 11

12 Case 2 Medications are 325 mg/d po aspirin 10 mg/d po of atorvastatin 5 mg of glyburide po bid 1000mg of metformin bid Where are we on Differential? Headache Associated with Substances or Withdrawal Medical Morning Headache (Toxic, metabolic, overuse) Drug Related (Overuse) Headache Various drugs, such as nitrates, monoamine oxidase inhibitors, caffeine and analgesics used for a long period, may cause headache. Most of these present as Chronic Daily Headache Dx usually via history 12

13 Toxic or Metabolic Headaches Metabolic conditions hypoxia, hypercapnia, and hypoglycemia, and toxins such as monosodium glutamate and carbon monoxide may cause headache as well. Dx via history or through appropriate laboratory testing. Case 2 Laboratory testing revealed CBC and chem profile WNL HgbA1c of 5.9% (down from 7% from 3 months earlier). Case 2 13

14 Case 3 ReginaV is an 72 year old woman who comes to your office complaining of headaches x3 months. She reports always having had mild headaches that never troubled her enough to see a doctor. This headache has been persistent, bilateral, band like, and throbbing. She denies visual changes, head trauma, or neurologic deficits. She does C/O fatigue and says that she has lost about 15 lbs over the last month. She denies any difficulty or pain with chewing. Case 3 Past medical history reveals Hypertension tx with HCTZ Breast mass noted 2 years before (the mass was thought to be low suspicion for malignancy and the patient declined work up). Case 3 What significant maladies are in our differential at this point? 14

15 Temporal Arteritis Giant cell arteritis (GCA) is a chronic vasculitis of large and medium sized vessels. Diagnostic Criteria of American Rheumatologic Society Age greater than or equal to 50 years at time of disease onset Localized headache of new onset Tenderness or decreased pulse of the temporal artery Erythrocyte sedimentation rate (ESR) greater than 50 mm/hour Biopsy revealing a necrotizing arteritis with a predominance of mononuclear cells or a granulomatous process with multinucleated giant cells Temporal Arteritis Other Findings Systemic symptoms of fever, fatigue, and weight loss Jaw claudication (vs TMJ) Visual Symptoms (amaurosisfugax, diplopia) Polymyalgia rheumatica Cough Arm claudication 15

16 Temporal Arteritis Scalp tenderness Abnormalities of the Temporal Artery Eye findings Optic atrophy Ischemic optic atrophy Synovitis Temporal Arteritis Lab workup ESR Males age/2 Women age + 10/2 Temporal artery biopsy 16

17 Temporal Arteritis CRA Ischemic Atrophy Case 3 Physical exam reveals temperature, 37.1 C; BP, 130/82 mm Hg; pulse, 72 bpm; RR, 10 breaths per minute. Head and neck bilateral cataracts with some prominence of the temporal arteries. Heart, lung, and abdominal exams were normal. Breast exam revealed a 2 3 cm mass in the left breast that was soft and freely mobile, which seemed unchanged from a description in the patient's chart from 2 years earlier. Extremity exam was notable for bruises over her left elbow and shoulder from a fall. Neurologic exam is intact. 17

18 Case 3 Given the physical exam what possibility enters into our differential at this point? Subdural Hematoma Tearing of the bridging veins that drain from the surface of the brain to the dural sinuses Watch for high risk patients (ie anticoagulants) When not massive can present hours to days after the injury as headache. Noncontrast CT Case 3 What investigations should be ordered specifically to R/O out primary differentials? 18

19 Case 3 Results show: CT imaging WNL Sed Rate 125mm/hr What would you order now? What about ultrasound? Case 3 Should you wait for biopsy to begin treatment with prednisone? Case 4 FrankL is A 60 y/o male presents with a terrible right frontal headache and blurred vision OD. The headache began suddenly about an hour ago and is associated with copious vomiting. He has no hx of chronic or frequent headache. 19

20 Case 4 What are the concerning aspects of this patients history? Case 4 Which of the following is the most helpful finding on physical exam to elucidate the diagnosis? A soft left sided carotid bruit B Hx of type 2 DM C Red eye OD D Liver 8cm to percussion in RMCL Case 4 20

21 Case 5 A 30 y/o male presents to the emergency department with a severe right temporal headache that lasted approximately 20 minutes. He describes the headache as stabbing. Although the headache has passed he noticed that his right eyelid was swollen and his pupil looked funny on that side. He is here now because he has had three of these attacks today, each lasting about 20 minutes. Case 5 From the history what diagnosis is suggested? Trigeminal Autonomic Cephalalgias A group of headache syndromes that result in unilateral trigeminal distribution pain and unilateral cranial autonomic features. Includes Cluster headache Paroxysmal hemicrainia Short lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) Short lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA). 21

22 Cluster Headache Prevalence of cluster headache is <1 percent The overall male to female ratio was 4.3:1 but increasing female rate Some genetic role Cluster Headache Unilateral symptoms that occur with the attack Ptosis, miosis Lacrimation, Conjunctival injection Rhinorrhea Nasal congestion, occur only during the pain attack and are ipsilateral to the pain. Sweating and cutaneous blood flow may also increase Attacks tend to be about 15 to 180 minutes 50% occur nocturnally Cluster Headache The syndrome is characterized by periods of attacks (clusters or bouts) and periods of remission. When in a cluster, patients may experience from one to eight attacks per day, and clusters last from seven days to 12 months Patients are most often asymptomatic between clusters 22

23 Clinical Features of the Trigeminal Autonomic Cephalalgias Cluster Headache Paroxysmal Hemicrania SUNCT Gender M > F F = M F M Pain type Stabbing, boring Throbbing, boring, stabbing Burning, stabbing, sharp Severity Excruciating Excruciating Severe to excruciating Site Orbit, temple Orbit, temple Periorbital Attack frequency 1/alternate day to 8/day 1 40/day (>5/day for more than half 3 200/day the time) Duration of attack min 2 30 min s Autonomic features Yes Yes Yes (prominent conjunctival injection and lacrimation) a Migrainous features b Yes Yes Yes Alcohol trigger Yes No No Cutaneous triggers No No Yes Modified from: Resident Readiness : Internal Medicine > Headache Take Home Points Most headaches seen in primary care office are primary headaches and do not require imaging or lab investigation Maintain a low threshold of suspicion to pursue serious disorders (sub arachnoid hemorrhage) when suspected Specialty referral to a headache clinic may be helpful in difficult cases Headache Patient: Questions to ask H: How severe is your headache on a scale of 1 10 (1 = minimal pain, 10 = severe pain)? How did this headache start (gradually, suddenly, other)? How long have you had this headache? E: Ever had headaches before? Ever had a headache this bad before (first or worst headache)? Ever have headaches just like this one in the past? A: Any other symptoms noted before or during your headache? Any symptoms right now? D: Describe the quality of your pain (throbbing, stabbing, dull, other). Describe the location of your pain. Describe where your pain radiates. Describe any other medical problems you may have. Describe your use of medications (prescription and over the counter products). Describe any history of recent trauma or any medical From: CURRENT Diagnosis & Treatment in Family Medicine, 3e or dental procedures. 23

24 References Bajwa, Z. (n.d.). Headache Syndromes other than Migraine. Retrieved January 19, 2015, from syndromes other thanmigraine?source=search_result&search=headache+syndromes+other+than+migraine&selectedtitle=1~150 Clinch C (2011). Chapter 28. Evaluation & Management of Headache. In South Paul J.E., Matheny S.C., Lewis E.L. (Eds), CURRENT Diagnosis & Treatment in Family Medicine, 3e. Retrieved January 19, 2015 fromhttp://accessmedicine.mhmedical.com/content.aspx?bookid=377&sectionid= Denny C.J., Schull M.J. (2011). Chapter 159. Headache and Facial Pain. InTintinalli J.E., Stapczynski J, Ma O, Cline D.M., Cydulka R.K., Meckler G.D., T(Eds), Tintinalli's Emergency Medicine: A Comprehensive Study Guide, 7e.Retrieved January 19, 2015 fromhttp://accessmedicine.mhmedical.com/content.aspx?bookid=348&sectionid= Goadsby P.J., Raskin N.H. (2012). Chapter 14. Headache. In Longo D.L., Fauci A.S., Kasper D.L., Hauser S.L., Jameson J, Loscalzo J (Eds), Harrison's Principles of Internal Medicine, 18e. Retrieved January 19, 2015 fromhttp://accessmedicine.mhmedical.com/content.aspx?bookid=331&sectionid= Laine, C., & Goldmann, D. (2009). Migraine. In,In the Clinic: Practical Information about Common Health Problems. ACP Press. Stern S.C., Cifu A.S., Altkorn D (2014). Headache. In Stern S.C., Cifu A.S., Altkorn D (Eds), Symptom to Diagnosis: An Evidence Based Guide, 3e.Retrieved January 19, 2015 fromhttp://accessmedicine.mhmedical.com/content.aspx?bookid=1088&sectionid= Smetana G.W. (2012). Chapter 9. Headache. In Henderson M.C., Tierney L.M., Jr., Smetana G.W. (Eds), The Patient History: An Evidence Based Approach to Differential Diagnosis. Retrieved January 19, 2015 fromhttp://accessmedicine.mhmedical.com/content.aspx?bookid=500&sectionid= Stone C (2011). Chapter 20. Headache. In Stone C, Humphries R.L. (Eds),CURRENT Diagnosis & Treatment Emergency Medicine, 7e. Retrieved January 19, 2015 from 24