11/10/2017 Other Primary Headaches · 11/10/2017 2 Other primary headaches may seem rare and...
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11/10/2017 1 Other Primary Headaches Matthew S Robbins, MD Associate Professor of Neurology, Albert Einstein College of Medicine Chief of Neurology, Jack D Weiler Hospital, Montefiore Medical Center Director of Inpatient Services, Montefiore Headache Center Disclosures • Contracted research: eNeura (site PI for study; funds to institution) Off-label uses • All therapies are off-label for other primary headache disorders Objectives 1. To review the classification and diagnostic criteria for unusual or rare primary headache disorders. 2. To understand secondary pathologies that may mimic or underlie such disorders. 3. To recognize recent scientific developments in new daily persistent headache and other primary headache disorders.
Transcript of 11/10/2017 Other Primary Headaches · 11/10/2017 2 Other primary headaches may seem rare and...
11/10/2017
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Other Primary Headaches
Matthew S Robbins, MD
Associate Professor of Neurology, Albert Einstein College of MedicineChief of Neurology, Jack D Weiler Hospital, Montefiore Medical Center
Director of Inpatient Services, Montefiore Headache Center
Disclosures• Contracted research: eNeura (site PI for study;
funds to institution)
Off-label uses• All therapies are off-label for other primary
headache disorders
Objectives
1. To review the classification and diagnostic criteria for unusual or rare primary headache disorders.
2. To understand secondary pathologies that may mimic or underlie such disorders.
3. To recognize recent scientific developments in new daily persistent headache and other primary headache disorders.
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Other primary
headaches
may seem rare
and unusual
ICHD-3 beta. Cephalalgia 2013; 33: 629–808 ©International Headache Society 2013/4
4. Other primary headache
disorders
4.1 Primary cough headache4.2 Primary exercise headache4.3 Primary headache associated with sexual activity4.4 Primary thunderclap headache4.5 Cold-stimulus headache4.6 External pressure headache4.7 Primary stabbing headache4.8 Nummular headache4.9 Hypnic headache4.10 New daily persistent headache (NDPH)
AppendixA4.11 Epicrania fugax
Primary short-duration headachesFeature Cough Exercise Sexual Thunderclap
Trigger Cough or Valsalva Strenuous exercise 1. ↑intensity w/ ↑excitement2. Explosive around orgasm
Spontaneous
Onset Sudden Variable 1. Gradual2. Abrupt
Abrupt
Duration 1s – 2h <48h 1m – 24h if severe<72h if mild
≥5m
Secondary considerations
ChiariLow ICP
DissectionNeoplasm
Aneurysm
SAHDissection
RCVS
SAHDissection
RCVSLow ICP*
SAHRCVS
DissectionApoplexy
ICHCVT
Neoplasm
Others
Treatments IndomethacinLP?
IndomethacinBeta-blockers
Warm-up, avoid heat, altitude
IndomethacinBeta-blockers
Temporary abstinencePassive role
Self-limitedNimodipine?
ICHD-3 beta, Cephalalgia 2013
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Baskin Robbins
Cold stimulus headache
ICHD-3 beta, Cephalalgia 2013
External compression headache
RCVS underlies many cases of 1º
sexual or thunderclap headache
• 30 patients with headache associated with sexual activity and no neurological deficits
– 20/30 (67%) had secondary causes
• SAH (1), basilar artery dissection (1), RCVS (18)
– Demographics, features, drug response, clinical course were similar between primary and secondary sexual headache
• 168 patients with RCVS
– Sexual activities as a trigger predicted recurrent RCVS
• HR 5.68 (95% CI 1.11-29.15, p = 0.038)
Yeh YC et al, Cephalalgia 2010Chen SP et al, Neurology 2015
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Primary stabbing headache
A. Head pain occurring spontaneously as a single stab or series of stabs and fulfilling criteria B-D
B. Each stab lasts for up to a few seconds
C. Stabs recur with irregular frequency, from one to many per day
D. No cranial autonomic symptoms
E. Not better accounted for by another ICHD-3 diagnosis
ICHD-3 Beta. Cephalalgia 2013
Hagler S, Ballaban-Gil K, Robbins MS. Curr Pain Headache Rep 2014
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Primary stabbing headache
• Temporal forms
– monophasic, intermittent, and chronic daily
• Be wary of index disorder – diagnosis and treatment
• Rule out secondary causes
• Overrepresentation of autoimmune diseases?
Kim DY et al. J Headache Pain 2017Robbins MS, Evans RW. Headache 2015
Rampello L et al. Clin Neurol Neurosurg 2012
Robbins MS. J Headache Pain 2011
Nummular headache
A. Continuous or intermittent head pain fulfilling criterion B
B. Felt exclusively in an area of the scalp, with all 4:
1. sharply-contoured
2. fixed in size and shape
3. round or elliptical
4. 1-6 cm in diameter
C. Not better accounted for by another ICHD-3 diagnosis
ICHD-3 Beta. Cephalalgia 2013
Nummular headache
Schwartz DP, Robbins MS, Grosberg BM. Curr Pain Headache Rep 2013Cuadrado ML et al. Cephalalgia 2009
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Example case
• 83W with 4 years of frequent headaches
• PMH: DM, HTN, CAD, hyperlipidemia, essential tremor
• Meds: aspirin, metoprolol, simethicone,
pantoprazole
Example case• Nocturnal predominance of headache attacks
– At least 4 nights / week
– Goes to sleep 10PM
– Attacks 12AM, 4AM
• Bilateral, frontal, parietal, occipital location
• Mild phonophobia, rare nausea
• Feels urge to get out of bed
• Duration 3-4 hours
• No aura, autonomic symptoms
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Example case
• Exam normal aside from sensory neuropathy and tremor
• ESR 57, CRP 1.1
• MRI: unremarkable
• Nightly coffee reduced attack frequency
Hypnic headache
A. Recurrent headache attacks fulfilling criteria B-EB. Developing only during sleep, and causing wakeningC. Occurring on ≥10 d/mo for >3 moD. Lasting ≥15 min and for up to 4 h after wakingE. No cranial autonomic symptoms or restlessnessF. Not better accounted for by another ICHD-3 diagnosis
ICHD-3 Beta. Cephalalgia 2013
Hypnic headache: features• Age
– Mean onset age 61– 93.4% onset age >50
• Sleep (“alarm clock” headache)– 2-4AM most common time– 53% NREM (stage 2); attacks NREM or REM– VBM-MRI: ↓gray matter posterior hypothalamus– No clear association with melatonin derangements
• Diagnosis– Clinical– Rule out nocturnal HTN, sleep apnea, mass, cervicogenic
Liang JF et al, Cephalalgia 2014Holle D et al, Ann Neurol 2011
Tariq N et al, Headache 2016Naegel S et al, Headache 2017Gil-Gouveia R, Goadsby PJ, J Neurol 2007
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Hypnic headache acute therapy
Liang JF et al, Cephalalgia 2014
Hypnic headache preventive therapy
Liang JF et al, Cephalalgia 2014
Example case
• 48W presented to a headache center with an unremitting daily headache
• started spontaneously on a particular day in November, 1986
• no background of frequent headache attacks or any clear antecedent symptoms
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Example case
• Pain:– holocephalic, pressure-like or sharp– moderate to severe in intensity– associated with nausea, osmophobia, and poor
concentration
• Medical history: asthma, depression• Family history: maternal grandmother – migraine• Brain MRI normal several years ago
Example case
• Dozens of past prophylactic and acute medication trials were ineffective
• Medications: morphine tablets, ergotamine tartrate/caffeine tablets, prochlorperazine suppositories daily
• Neurologic examination: unremarkable
• Referred for MRI brain with gadolinium:
normal
NDPH: a syndrome identified
• Case series of 45 patients presented in 1986 by
Dr. Vanast from Edmonton seen over 24 months
• Described clinical syndrome
– Daily headache from onset
– No preceding headache history
– Not attributable to other causes
Vanast WJ, Headache 1986
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International Classification of
Headache Disorders, 3rd ed. (beta)
A. Persistent headache fulfilling criteria C and D
B. Distinct and clearly remembered onset, with pain becoming continuous and unremitting within 24 hours
C. Present for >3 months
D. Not better accounted for by another ICHD-III diagnosis
Classification Committee of the IHS, Cephalalgia 2013
What is the cause of NDPH?
1. Daily onset, similar pathophysiology to chronic
migraine or chronic tension-type headache
2. Post-infectious, inflammatory disorder
3. Other mechanisms
4. Recognized entity, misdiagnosed as NDPH
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NDPH triggers at onset
– Infection or flu-like illness (3-30%)– Stressful life events (migraine over-represented)
– Extracranial surgery (intubation)
– Athletic activity
– Childbirth
– Menarche
– Vaccination
– Medications
– Tapering of antidepressants
Li D and Rozen TD, Cephalalgia 2002Mack K, Pediatr Neurol 2004
Meineri P et al, Neurol Sci 2004
Rozen T, Swidan S, Headache 2007
Robbins MS et al, Neurology 2010Peng KP et al, Cephalalgia 2011
Rozen T, Headache 2015
NDPH seasonal variation
Robbins MS et al, Neurology 2010
Li N et al, Headache 2012
Grengs LR and Mack KJ, J Child Neurol 2016
Kedia S et al, Cephalalgia 2013
NDPH etiology• Epstein-Barr virus
– 84% NDPH vs 25% controls had evidence of an active infection (n=32)
• 47% reported atypical symptoms• MRI and CSF not well described
– 71% past infection (n=7)
• Other viral etiologies (n=18)– HSV 42%
– CMV 11%
Diaz-Mitoma F et al, Lancet 1987Li D and Rozen TD, Cephalalgia 2002
Meineri P et al, Neurol Sci 2004
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Other reported associations• Higher rates of hypothyroidism
– N = 69
– OR 16.0 (3.6-72.0) compared to migraine (p<0.0001)
• Cervical spine joint hypermobility
– N = 12, 92%
• Defective internal jugular venous drainage
– N = 10, 80%
• Elevated CSF TNF-α levels (also in CM)
• No clear white matter lesions or infarcts
Bigal ME et al, Headache 2002Rozen T et al, Cephalalgia 2006
Donnet A et al, Neurology 2009
Rozen T et al, Headache 2007Rozen T, Cephalalgia 2015
Chronic migraine also may have an
abrupt onset
Mack KJ, Pediatr Neurol 2004
Epidemiology
• Prevalence
– Population-based• 0.03 to 0.1%
– Clinic-based
• 1.7 to 10.8% adults• 13 to 36.1% teens
• F:M 1.1 to 2.6:1
Castillo J et al 1999, Grande R et al 2009, Bigal et al 2004, Bigal et al 2002, Gladstein and Holden 1996, Koenig et al 2002, Kung et al 2009, Mack 2004,
Meineri et al 2004, Li and Rozen 2002, Li et al 2012, Monzillo and Nemoto 2011,
Peng et al. 2011, Prakash et al 2012, Robbins et al 2010, Siow 2006, Vanast
1986, Grande et al 2009, Takase et al 2004, Robbins 2011, Peres et al, 2011
• Onset
– teens to 50 years
– mean 30-40 years
• Described in most regions
• Comorbidities
– Depression 62.2%
– Anxiety 45.9%
– Panic disorder at onset (n=9)
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Robbins MS. New daily persistent headache. In: Robbins MS, Grosberg BM, Lipton RB, eds. Neurology In Practice: Headache. 2013.
Differential diagnosis: 1°disorders
Potential misdiagnosis
Robbins MS, Evans R, Headache 2012
Treatment• Symptomatic
• Prophylactic medications – mainstay
– Treat according to headache phenotype
• Acute therapies
– Many triptan responsive (32.4%)
• Intravenous medications– (DHE, especially if migraine features)
– Earlier the better?
• Peripheral nerve blocks (up to 60.9%)
• Avoid medication overuse
• Nonpharmacological therapies
• Anecdotal doxycycline + montelukast
Robbins MS et al, Neurology 2010Nagy AJ et al, Neurology 2011
Afridi SK et al, Pain 2006
Gelfand AA et al, Pediatr Neurol 2014Prakash S et al J Headache Pain 2010
Rozen T, AAN 2008
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Prognosis
• Vanast’s initial described excellent prognosis:– 68% at 6 months
– 80% at 12 months
– 86% at 24 months
• 3 subforms
– Persisting (younger, psychiatric comorbidity)
– Remitting– Relapsing-remitting
• Subsequent studies: resolution in 15.5% to 28.6%
Vanast WJ, Headache 1986Robbins MS et al, Neurology 2010
Peng KP et al, Cephalalgia 2011
Epicrania fugax
Pareja JA et al, Cephalalgia 2008Cuadrado ML et al, J Headache Pain 2010
Cuadrado ML et al, Headache 2015Cuadrado ML et al, Cephalalgia 2015
Casas-Limon J et al, Headache 2016
Summary1. Other primary headache disorders are defined by singular clinical
features including tempo, topography, and provoking factors.
2. Many, such as primary thunderclap headache, may actually be secondary disorders where diagnostic testing is elusive.
3. NDPH may be the most common and burdensome other headache syndrome, which starts acutely, is diagnosed after 3 months have elapsed and other secondary and primary headache diagnoses have been excluded.
4. The prognosis of NDPH is variable but often poor, and the treatment approach is largely extrapolated from the management of other forms of chronic daily headache.
