It’s not all about Migraine!
ERIC HASTRITER, MD FAHS BANNER PEDIATRIC SPECIALISTS CARDON CHILDREN’S MEDICAL CENTER DECEMBER 6TH, 2016 MY PROCESS THE SAGA CONTINUED Program Disclaimer: The accuracy and utility of the materials presented are based on the International Classification of Headache Disorders, 2nd edition (ICHD- II), statements made will be evidence-based, the limitations being due to the nature of trials in children, most of the evidence comes from adult studies but clinical evidence across the pediatric headache community will be discussed Conflict of Interest: There is no conflict of interest at this time
Objectives
Describe a typical diagnostic path used by neurologists obtaining proper primary headache diagnosis other than migraine Understand the utility of preventative or abortive medications, diagnostic tests, and psychological/bio-behavioral approaches in management of diagnosis Discuss the timing of when to refer to a specialist Migraine Criteria- ICHD-II
At least 5 attacks, lasting 1 to 72 hours Headache has at least 2 of the following: • Unilateral location, may be bilateral, frontotemporal (not occipital) • Pulsating quality (throbbing) • Moderate or severe pain intensity • Aggravated by routine physical activity • at least 1 of the following: Nausea and/or vomiting. Photophobia and phonophobia may be inferred from the child’s behavior • Not attributed to another disorder
17 year old male
Frequent headaches that began in august (with school) 2-3/month, 1 hour to 5 days Global, squeezing, moderately intense, did not interfere with his activity No nausea, vomiting, phonophobia, but had photophobia Worked 4 nights/week at fast food establishment to save money for college PMH, FH, SH and ROS were benign, general and neurologic exam were normal
What was the most likely diagnosis?
What diagnostic studies were indicated?
What management options were appropriate? Tension Type Headache
30 minutes to 7 days, bilateral, pressing/tightening (non-pulsatile) mild to moderate intensity, not aggravated by physical exertion Associated with: . lack of associated features . may have pericranial muscle tenderness . nausea/vomiting do not occur . no more than 1 of photophobia/phonophobia and it is not prominent Tension Type Headache
Most common type worldwide Rarely interferes with function Infrequently present if episodic Most common reasons buying OTC meds Little known about pathogenesis of tension-type headache 1-year prevalence ranges from 40-80% Slightly more common in females
Tension Type Headache
Differential: episodic migraine, cervicogenic headache, and secondary causes of headache If presents with chronic tension type headache: neuroimaging is warranted to rule out space occupying lesions Cervicogenic HA-strictly unilateral headache Tension Type Headache
Treatment-lifestyle changes (stress reduction and management, relaxation therapy, massage, cognitive behavioral therapy Abortives: NSAIDS, combination products with caffeine if caffeine naïve Preventatives if > 10 days/month, amitriptyline or nortriptyline Tension Type Headache
What was the most likely diagnosis?
What diagnostic studies were indicated?
What management options were appropriate?
13 year old female
March 2011: flu-like illness (mom thought), headaches began and have been daily Bilateral, continuous moderate pain, she had difficulty describing quality (feel tightening, throbbing, pulsating, worse with movement) Some nausea, photophobia and phonophobia, but they were not prominent No autonomic features No systemic or neurologic symptoms
13 year old female
Labs from nearly 2 years ago, mono positive, and then a year after the fact West Nile Virus was positive MRI, MRA, MRV all were negative Hematologic workup was negative LP was normal pressure, no infection
13 year old female
What diagnosis could this be?
What needs done in this patient?
What treatment is suggested in this patient?
New Daily Persistant Headache
Subacute onset over 72 hours of daily unremitting headache Resemble CTTH, or CM, but is chronic at onset Diagnosis of exclusion: neuroimaging, hematologic workup required to rule out secondary causes May resolve on own or be refractory to treatment New Daily Persistant Headache
Most common in adolescents/young adults ~10% of patients in tertiary headache clinics Underlying etiology unknown (30% report recent flu-like illness at time of onset) Two clinical subtypes- . benign self limited form . refractory form resistant to aggressive therapy New Daily Persistant Headache
Bilateral, continuous moderate pain(can be mild or severe) Tightening/throbbing/pulsating, and may be aggravated by physical exercise Migraine features (N/V/P/P) but typically not most prominent features No associated trigeminal autonomic features (lacrimation/conjunctival injection) No systemic or neurologic symptoms New Daily Persistant Headache
Differential includes CTTH, CM and secondary causes of headaches Can resemble spontaneous intracranial hypotension (dural tear or leak) if positional component overlooked in history Can resemble pseudotumor cerebri (idiopathic intracranial hypertension) New Daily Persistant Headache
Labs: CBC (rule out chronic anemia or infection), TSH (rule out hypothyroidism), CRP/ESR (rule out temporal arteritis) MRI brain: space occupying lesion, hydrocephalus, MRI w/GAD: spontaneous intracranial hypotension MRV: cerebral venous sinus thrombosis LP: increased/decreased CSF pressure or chronic meningitis
New Daily Persistant Headache
Treatment- no known effective acute or prophylactic therapy Standard acute/preventative for CTTH, CM could be attempted on trial and error basis Doxycycline, Singulair have been used Beware: Medication overuse headache complicating picture New Daily Persistant Headache
What diagnosis could this be?
What needs done in this patient?
What treatment is suggested in this patient?
14 year old male, eye pain
Sharp excruciating right sided orbital and temporal pain Tearing, reddening of eye, ringing in right ear About 20 minutes, 8 times/day, but 1 lasted 3 hours He feels agitated, awakening nightly during baseball season every year Nausea occurs, and so does photophobia Between episodes has lesser pain and takes 800 mg ibuprofen regularly which helps 14 year old male, eye pain
What is his diagnosis?
What investigations need done?
How would you treat this entity? Cluster Headache
Excrutiating strictly unilateral headache, last about 1 hour (15min-3hours), and may recur up to 8/day usually seasonal Associated with cranial autonomic symptoms ipsilateral to pain, can be bilateral 20% have CCH when headaches continue for more than 1 year without remission of > 4 weeks Cluster Headache
Co-Morbid: Tobacco use, OSA MRI brain: intracranial lesions particularly in pituitary and parasellar regions Treatments: . 100% oxygen . Intranasal or parenteral triptans . Corticosteroids . Verapamil Cluster Headache
Prototypical TAC-Trigeminal Autonomic Cephalgias; first division of trigeminal nerve, accompanying ipsilateral autonomic features (lacrimation/ conjuctival injection/rhinorrhea) 80% have cluster 1-3 months, typically 1-2 times per year with remissions in between Nocturnal attacks typical, but daytime attacks occur, usually near same time daily Cluster Headache
Triggers: alcohol and high altitude Strictly unilateral, maximal around or above orbit, may begin or become referred to the temporal, lower facial, or occipital region Extremely severe, piercing, boring, stabbing peaking within 3-5 minutes lasting average 1 hour (15 min- 3 hour) Cluster Headache
> 90% agitation Autonomic features: lacrimation, rhinorrhea, conjunctival injection, ptosis, miosis, facial or periorbital edema Autonomic features may be bilateral, but are prominent ipsilateral to pain Cluster Headache
Migrainous symptoms (N/V/P/P) Can have unilateral Photo/phonophobia Interparoxysmal pain and allodynia (abnormal pain response to normal stimulus) may occur in more than 1/3 of patients with any of the TACs Medication Overuse may be responsible the interparoxysmal pain Cluster Headache
PE may show persistant Horner syndrome if had recurrent attacks for years MRI brain: special attention to pituitary and parasellar regions for mimickers of TACs Overnight polysomnography if features of OSA are present Cluster Headache
Treatment . Acute- 100% oxygen 7-15L/min with closed facemask for 15 minutes . Triptans-sumatriptan 6mg SC, sumatriptan 20 mg NS, zolmitriptan 5 mg NS . Short term prevention- steroid taper 60 mg decrease by 10 mg every 2-3 days . Occipital nerve blockade 2.5 mL bupivacaine with 20 mg methylprednisolone
Cluster Headache
Preventatives . Verapamil 80 mg TID up to 320 mg TID (check ECG after each dose increase). . Long term side effects: gingival hyperplasia, constipation, and peripheral edema) Cluster Headache
What else could be his diagnosis?
What investigations need done?
How would you treat this entity?
13 year old with migraine
Headaches since she was 6, diagnosed with migraine and nothing seems to be helping Pain is all day long, sharp, stabbing, usually around the temples, mostly on left side Both eyes get red, ears ringing bilaterally Sensitive to light and sound out of the left ear and eye Nausea present, no vomiting Ibuprofen helps but it comes back 13 year old with migraine
PMH, FH, SH, ROS and physical exam all normal When asked about all day long headache, she said it came and went throughout the day Further questioning revealed that is came and went around 20-30 minutes, but happened 8-20 times per day In between she was head pain free
13 year old with migraine
What could be her diagnosis?
What investigations need done?
How would you treat this entity?
Paroxysmal Hemicrania
Similar to CH however shorter and more frequent attacks MRI brain with coronal GAD sequences of pituitary for all suspected paroxysmal hemicrania Responds rapidly AND completely to indomethacin Paroxysmal Hemicrania
Slight female predominance Late adolescence to early adulthood mean age 36 (1 year -81 years reported) Typically unilateral (bilateral reported though) Located maximally in V1 region, however may occur in parietal and temporal regions Paroxysmal Hemicrania
Severe intensity, throbbing, pressure, stabbing or boring Attacks 2-30 minutes, 1-40/day, average 5-15/day Paroxysmal Hemicrania
Autonomic features: ipsilateral lacrimation, nasal congestion most common, but can inlude rhinorrhea, conjunctival injection, eyelid edema, ptosis, miosis, forehead or facial sweating (>50% of patients), ear fullness sensation in 30% of patients Migraine features: Photophobia/phonophobia 66% of patients, may be unilateral Paroxysmal Hemicrania
Nausea/vomiting 33% of patients Motion sensitivity 50% Agitation/Restlessness in 80% Spontaneous attacks, no nocturnal predominance Differential: cluster headache, trigeminal neuralgia, SUNCT syndrome, and hemicrania continua Paroxysmal Hemicrania
Dull inter-ictal pain, may resemble hemicrania continua, but hemicrania continua exacerbations are longer lasting with less autonomic features Secondary causes have been reported with lesions in pituitary and posterior fossa Treatment: Indomethacin Paroxysmal Hemicrania
Titrate up to 75 mg TID, protect mucosal lining due to gastric side effects, if poorly tolerated cox-2 inhibitors, ASA, topiramate, or gabapentin may be tried Occipital nerve blockade may help May last years or decades Some have spontaneous remission Paroxysmal Hemicrania
What else could be her diagnosis?
What investigations need done?
How would you treat this entity? 15 year old excruciating pains
4 year history of headaches behind right eye that last all day Severe to excruciating, stabbing on right temple/eye, tearing in right eye, injection in right eye Able to trigger it combing hair occasionally, but not always Nothing over the counter seemed to help Amitriptyline had been tried unsuccessfully 15 year old excruciating pains
Further questioning revealed he had pain that came and went throughout day The longest pain was 3 minutes and the shortest pain was 5 seconds of stabbing He estimated the range of episodes throughout the day were around 60-70 times/day PMH/FH/SH/ ROS and exam were normal 15 year old excruciating pains
What could be his diagnosis?
What investigations need done?
How would you treat this entity?
SUNCT Syndrome
Short lasting Unilateral Neuralgiform Headache attacks with Conjunctival Tearing Strictly unilateral, severe, stabbing, shooting, lancinating, burning, V1 distribution occur with conjunctival injection and tearing 5 - 240 seconds, in between attacks pain free Frequent up to 200/day attacks Slight male predominance Age 35-65 years (10-77 years) Natural history-last years, decades or lifelong
SUNCT Syndrome
Migrainous features are not uncommon especially unilateral photophobia Autonomic symptoms are frequently seen including rhinorrhea, nasal congestion, eyelid edema, ptosis, miosis, facial redness, conjunctival tearing obviously May be triggered by tactile stimuli, most commonly mastication and trigeminal innervated areas
SUNCT Syndrome
Brain MRI looking at pituitary, parasellar region, and brainstem in all patients with suspected SUNCT syndrome Anticonvulsants: lamotrigine, gabapentin, topiramate, and carbamazepine. Parenteral lidocaine: effective in most Surgical procedures- mixed results . Percutaneous trigeminal ganglion rhizolysis . Trigeminal root microvascular decompression . Hypothalamic deep brain stimulation
SUNCT Syndrome
What else could be his diagnosis?
What investigations need done?
How would you treat this entity?
14 year old girl with stabbing pain
4 month headache history Occasional stabbing in random locations, most in temple and frontal area that lasted 1-10 seconds, and up to 10 a day, then some days without any pain No migraine features No autonomic features Occasionally would have a true migraine, lasting hours, worse with movement, associated with N/V/P/P responds to naproxen
14 year old girl with stabbing pain
What could be her diagnosis?
What investigations need done?
How would you treat this entity?
Primary Stabbing Headache
Alias: ophthalmodynia periodica, ice pick headache, jabs and jolts syndrome, idiopathic stabbing headache Ultra short paroxysms of stabbing pain (1-10s) unilateral ophthalmic division most common, however anywhere on head may occur, 1-50 times throughout day and evening Onset 12-70 (mean 47 years), female predominance Primary Stabbing Headache
Differential includes SUNCT (attacks longer) or Trigeminal Neuralgia (more V2/V3) or secondary causes Frequent attacks: indomethacin is treatment of choice as preventative Frequently associated with migraine (40%), tension type headache and the TACs Secondary causes: meningiomas, pituitary tumors, giant cell arteritis, cranial and ocular trauma, herpes zoster, and elevated intraocular pressure
Primary Stabbing Headache
Frequently associated with migraine (40%), tension type headache and the TACs Secondary causes: meningiomas, pituitary tumors, giant cell arteritis, cranial and ocular trauma, herpes zoster, and elevated intraocular pressure Primary Stabbing Headache
Usually unilateral Attacks from 1-50 attacks/day Most attacks throughout day and evening Differential includes SUNCT (attacks longer) or Trigeminal Neuralgia (more V2/V3) or secondary causes Primary Stabbing Headache
Investigations usually not necessary unless suspicious for secondary causes Prophylaxis rarely required . Indomethacin 25-75mg TID . Melatonin 3-12 mg/day . Gabapentin 400 mg BID Primary Stabbing Headache
What else could be her diagnosis?
What investigations need done?
How or would you treat this entity?
Tests used
Used DSM-IV to make diagnosis CBCL-child behavior checklist age weighted YSR-youth self report SCARED-the screen for Child Anxiety Related Disorders CDI-Children’s Depression Inventory CPRS-R; Conner’s Parent Rating Scale-Revised CBCL-child behavior checklist
Filled out by parents to assess emotional/behavioral problems of children and adolescents Identifies two types of problems- internalizing and externalizing Internalizing- anxiety, depression, social withdrawal, and somatic complaints Externalizing- aggression and antisocial behavior SCARED-screen for childhood anxiety related disorders Self administered 41 questions over last three months of feelings of anxiety Score >39 indicates clinical impairment CDI-children’s depression inventory
Derived from Beck’s depression Scale Assesses severity of depression symptoms in prior two weeks Age 7-17 19 points or higher discriminates risk for depression CPSR-Conner’s Parent Rating Scale
Self administered 80 questions about behavior during last month Ages 3-17 Scores> 65 indicate clinical impairment Results
Children with headaches had significant internalizing and externalizing problems compared with control 63% and 27% No difference between migraine and tension type found 26% had positive comorbidity with the headache of anxiety and mood disorders. One out of three headache patients needed particular therapy with emotional and behavioral problems Headache Toolbox
Proven Behavioral Therapies Relaxation training Temperature biofeedback (hand warming) combined with relaxation training Electromyographic (EMG) biofeedback (for muscle tension reduction Cognitive Behavior Therapy Complementary Therapies Accupuncture/accupressure Chiropractic therapy Hypnosis and physical therapy
Cognitive Behavioral Therapy
Highly effective Average rate of reduction in headaches compared to controls 68% vs. 20% Improvement was seen in baseline coping skills, social support, physiologic measures at rest and in response to stress. Noncompliance
Prevalent in headache patients Simplifying medication strategies Screening and management of psychiatric co-morbidities Enabling patient to have self-efficacy and take ownership of their headaches is key Website for relaxation: www.dawnbuse.com Natural Remedies for headaches
LipiGesic- sublingual feverfew/ginger appears safe and effective as first line abortive in migraine patients at the onset of a pre-severe migraine (most common side effect is nausea of gagging) Exercise-systematic review on the literature concluded it was promising but adherence to headache research guidelines in a study needed.
Natural Remedies for headaches
Thirty studies reviewed on 6 nutraceuticals Butterbur-showed reduction and liver toxicity Riboflavin-400 mg showed marked reduction Ginkgolide B-60-80 mg showed marked reduction Magnesium- 300 mg showed marked reduction Coenzyme Q10- 100 mg showed marked reduction Polyunsaturated fatty acids-Marine ester concentrate- showed marked reduction Studies showed strong evidence but level of evidence is still low in all-more studies are needed. When to Refer to Headache Specialist
When not responding to OTC medications, preventatives or abortives Frequency of headache increasing or disability increasing When something other than migraine suspected, but uncomfortable treating it When you would like help with the time-consuming patient When patient has chronic migraine or chronic daily headache When to Refer to Headache Specialist
Headache specialist requested by parent/patient Role of Pediatric Headache specialist- . Taking time to establish proper diagnosis . Get patient on a good preventative . Get patient on good rescue medication . Decreasing headache disability . Returning improved pediatric patient to their primary care provider References
Baillie LE, Gabriele JM, Penzien DB. A systematic review of behavioral headache interventions with an aerobic exercise component. Headache 2014;54(1): 40-53. Penzien DB. Stress management for migraine: recent research and commentary. Headaches 2009;49(9):1395-1398. Rains JC, Penzien DB, Lipchick GL. Behavioral facilitation of medical treatment of headache: implications of noncompliance and strategies for improving adherence. Headache 2006;46 (suppl 3):S142-S143. Seng EK, Holroyd KA. Behavioral migraine management modifies behavioral and cognitive coping in people with migraine. Headache 2014;54(9):1470-1483. Cady RK, Goldstein J, Nett R, et al. A double blind placebo controlled pilot study of sublingual feverfew and ginger (LipiGesic M) in the treatment of migraine. Headache 2011;51(7):1078-1086. Oelkers-Ax R, L eins A, Parzer P, et al. Butterbur root extract and music therapy in the prevention of childhood migraine: and explorative study. Eur J Pain 2008;12(3):301-313. Brujin J, Duivenvoorden H, Passchier J, et al. Medium-dose riboflavin as a prophylactic agent in children with migraine: a preliminary placebo controlled randomized, double-blind, cross-over trial. Cephalalgia 2010;30(12):1426-1434. Headache Classification Committee of the International Headache Society (IHS). The International Classification of Headache Disorders, 3rd edition (beta version). Cephalalgia 2013;33(9):665-671. Orr SL, Ventkateswaran S. Nutraceuticals in the prophylaxis of pediatric migraine: evidence-based review and recommendations. Cephalalgia 2014:34(8):568-683 Werder DS, Sargent JD. A study of childhodd headache using biofeedback as a treatment alternative. Headache 1984;24(3):122-126. Arruda MA, Guidetti V, Galli F, et al. Primary headaches in childhood—a population-based study. Cephalalgia 2010;30(9):1056-1064. Wojaczynska-Stanek K, Koprowski R, Wrobel Z, Gola M. Headache in children’s drawings. J. Child Neurol 2008;23(2):184-191. Lewis D, Ashwal S, Hershey A, et al. Practice parameter: pharmacological treatment of migraine headache in children and adolescent: report of the American Academy of Neurology Quality Standards Subcommittee and the Practice Committee of the Child Neurology Society. Neurology 2004;63(12):2215-2224. Lewis DW, Ashwal S, Dahl G, et al. Practice parameter: evaluation of children and adolescents with recurrent headaches: report of the Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society. Neurology 2002;59(4):490-498.
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