Transcript Thunderclap Headache

eEdE-57
Thunderclap Headache Beyond
Aneurysms- A Clinically
Integrated Approach
Daniel Strauchler, MD1
Daniel Berlin, MD MSc2
Joseph Platnick3
X Wu4
Ajay Malhotra, MD1
1Yale
New Haven Hospital, New Haven, CT, 2Neurology Group
Bergen, Ridgewood, NJ, 3Radiology Associates of Ridgewood,
Waldwick, NJ, 4Yale University School of Medicine, New
Haven, CT
Purpose/Outline
• Define thunderclap headache (TH)
• Review the differential diagnosis of TH
• Is it possible to clinically distinguish between benign and malignant
causes of TH?
• Review the workup of TH- role of LP and CTA
– Present case series of 119 patients undergoing CTA for headaches
• Case presentations
Definition of Thunderclap headache
(1986)
• Very severe headache which reaches
maximum intensity within 1 minute
Differential diagnosis
•
•
•
•
•
•
•
•
•
•
•
Subarachnoid hemorrhage
Reversible vasoconstrictive syndrome/
Posterior reversible leukoencephalopathy syndrome
Cerebral venous sinus thrombosis/ Cortical vein thrombosis
Arterial dissection
Orgasmic/exertional headache
Spontaneous intracranial hypotension
Pituitary apoplexy
Primary thunderclap headache
Parenchymal hemorrhage
Cerebral infarct
Infection (including bacterial, viral, and aseptic meningitis and sinusitis)
– For comprehensive differential list see Devenney et al. The journal of headache
and pain. 2014;15:49.
Thunderclap headache (TH)
• Landtblom et al. Cephalalgia 2002
• Prospective study of 137 patients presenting
within 10 days of TH to emergency dept in
Sweden over 1.5 year period
• Each patient was examined by a neurologist, had
CT head acutely, and if necessary lumbar puncture
was performed
• Each patient was followed up by telephone on a
regular basis for 1 year
Patients with TH
Women
Men
All
All patients
57%
43%
137
SAH
13
10
23 (17%)
Median age
Non-SAH
Median age
58 (32-79)
65
49
114 (83%)
39 (18-86)
CT and CSF findings
Finding
Patients (n)
SAH
23
Cerebral infarction
5
Intracerebral hemorrhage 3
Aseptic meningitis
4
Cerebral edema
1
Sinus thrombosis
1
Results
•
•
•
•
•
•
•
Incidence of TH was 43/100,000 adult population/yr
17% of patients had SAH (23/137)
5/23 patients with SAH required LP for diagnosis
8% of patients had coital headache (1/5 had SAH)
86% of patients had headache onset over 2 seconds
Majority of patients were resting at time of TH (60%)
There is an increased prevalence of primary headaches
in patients with non-SAH TH
Can clinical characteristics distinguish SAH
from non-SAH TH?
”Headache characteristics
in SAH and benign TH”
• Prospective study of
102 patients who
presented with TH
• Only 2 characteristics
occurred exclusively in
pts with SAH:
seizures and diplopia
Landtblom A et al. Cephalalgia 2002;22:354-360
Linn et al. J. Neuro, Neurosurg, Psychiatry 1998; 65; 791-793
Study Conclusions
• TH is mostly a benign condition
• In most cases, it was not possible to determine the cause of TH
• TH due to SAH versus non-SAH cannot be distinguished based
on clinical grounds- ancillary testing is necessary
Study Limitation
• Vascular imaging was not performed
Case Series
We retrospectively reviewed 119 Cases of patients presenting with headache
who underwent CTA of head and neck in addition to noncontrast CT of head.
•9/119 (8%) were positive including:
•3 - subarachnoid hemorrhage related to aneurysm.
•2 - intraventricular hemorrhage of unknown etiology
•1 – arteriovenous malformation
•1- arteriovenous fistula in scalp
•1 – parenchymal (hypertensive) hemorrhage
•1- parenchymal (metastasis) hemorrhage
•110/119 (92%) were essentially negative or unchanged.
•Of which:
•3- venous thrombosis diagnosed subsequently of MRV
•3- cerebrovascular accident diagnosed subsequently on MRI
•13 – had aneurysms (2 newly diagnosed, 8 previously treated, 3 known)
Case Series
• Small percentage of cases 3/119 (3%) with
subarachnoid hemorrhage compared with previous
studies likely reflects scanning of patients without
true TH (worst at onset).
• 2/106 (2%) with new aneurysm (after excluding
those with known or treated aneurysms, which likely
was factor in repeat imaging) is within range of
previously reported prevalence on unruptured
aneurysms suggesting that these are incidental.
Workup of TH: Importance of LP
• CT head within 12 hours of TH has 98% sensitivity for SAH detection
• CT head at 1 day post SAH is 95% sensitive
Van der Wee et al. J. of Neuro, Neurosurg, Psychiatry 1995; 58: 357-359
• CT head at 5 days post SAH is about 60% sensitive
• A LP can reliably distinguish true SAH from traumatic tap only after 12 hours have elapsed
from symptom onset (the time it takes for xanthochromia to develop)
• Xanthrochromia is best detected by spectrophotometry over visual inspection
• Evidence from a prospective study using multidetector scanners with at least four
detectors suggests that negative scans can reliably exclude subarachnoid hemorrhage if
obtained within the first six hours after onset of the headache
Perry et al. BMJ 2011;343:d4277
Suggests LP may not be needed if patient is scanned within 6 hours.
Controversy: Can CTA replace LP?
•
Some have argued that combining noncontrast CT and CTA can replace LP in
excluding aneurysmal subarachnoid hemorrhage, arguing that combining CT
sensitivity for subarachnoid hemorrhage and CTA sensitivity for aneurysm yields
greater than 99% posttest probability to exclude aneurysmal subarachnoid
hemorrhage.1
• However, others have countered based on utility analysis that frequent use of CTA
in patients with thunderclap headache with limited utility and arguing that a
negative CTA does not exclude the presence of subarachnoid blood, and the
detection of aneurysm does not confirm it as being the cause of SAH.2
– CT with no follow-up was shown to be the best strategy when the pre-test
probability of SAH is low (<1.6%) or the sensitivity of initial non-contrast CT for
blood is high (>99.6%). Otherwise, LP should be the preferred strategy for followup.
1McCormack
et al Acad Emerg Med 2010;17:444–51
2Wu et al EMJ. Jun 4 2015.
Controversy: TH with normal CT and
LP:
Is further investigation necessary?
YES
• Conditions such as arterial
dissection, cerebral sinus
thrombosis, and
vasoconstrictive
syndromes require further
diagnostic imaging
• Angiography can detect a
symptomatic unruptured
intracranial aneurysm
NO
• Out of more than 200
patients with TH who had
normal CT and LP, none
developed SAH in
followup
• Aneurysm detected on
angiography does not
necessarily mean it is
symptomatic
Case 1
• 25-year-old man with chronic history of migraine presents with thunderclap
headache- different from his usual headaches- while having a bowel movement.
• Social history: 1 pack/day smoker, habitual marijuana use
• CT head at that time was normal
• He returned to ED 1 week later for multiple recurrent thunderclap headache.
• Repeat head CT shows new right occipital subcortical hypodensity. MRI shows
corresponding FLAIR signal hyperintensity.
Case 1 continued
• Differential diagnosis includes: Migrainous infarction,
Subarachnoid hemorrhage with subsequent vasospasm,
Posterior reversible leukoencephalopathy
syndrome/Reversible vasoconstrictive syndrome, Stroke
• While consent for LP was being obtained the patient
developed severe visual field abnormalities bilaterally.
• CTA followed by catheter angiogrpahy demontrates focal
areas of vasoconstriction in various artery distributions
Case 1 - Reversible Cerebral Vasoconstriction Syndrome
•
•
•
•
•
Definition
• A group of disorders characterized by prolonged but reversible vasoconstriction of cerebral
arteries associated with recurrent thunderclap which can be associated with other neurologic
symptoms.
Diagnostic Criteria (2007)
• Acute and severe headache (usually thunderclap) with or without focal deficits or seizures,
Uniphasic course (without new symptoms more than 1 month after onset), Segmental
vasoconstriction of cerebral arteries, No evidence of aneurysmal SAH, Normal or near-normal
CSF profile, Complete or substantial normalization of arteries on follow-up angiography (3
months from onset)
Treatment (No randomized trials exist, only observational data and expert opinion)
• Verapamil IA was injected into bilateral ACA, MCA, and PCA with moderate improvement in
vessel caliber
• Nimodipine 60mg q4 hours x 3 weeks, Induced hypertension 160-180 SBP, Daily transcranial
doppler evaluation, Abstain from marijuana
Clinical Course
• Transcranial doppler showed fluctuating vasospasm for about 10 days before normalizing
• CTA 2 weeks after admission showed some overall improvement but persistent irregularities
Precipitating factors:
• 60% due to vasoactive drug: Cannabis most common, SSRI, Nasal decongestants
• 38% spontaneous
• 12% peripartum state
RCVS
Vasculitis
Presentation
Acute
Subacute progressive
headache
CSF
Normal
Pleocytosis
MRI
Usually normal, can
see infarcts, SAH
Abnormal-multifocal
infarctions
Angiography
Medium-large vessel Small Vessel
Treatment
Ca channel blocker
Immunosuppressants
Case 1
Reversible Cerebral Vasoconstriction Syndrome
Clinical course:
•
•
•
•
•
•
•
Mean of 4 thunderclap headaches over 1-4 weeks
Triggers: Valsalva maneuver (sex, exertion, sneezing, bending)
Moderate headache persists between exacerbations
1/3 of patients deteriorate after initial diagnosis
Intracranial hemorrhage, SAH, seizures occurred in first 3 days
Ischemic events occurred around day 12
90-95% of patients have a benign syndrome despite severe
vasoconstriction
Case 2
•
•
•
•
•
•
•
•
•
21-year-old woman presents to ED (for 3rd time) with 1 month of severe right side-locked head pain. She has had 1 day
of left hemiparesis.
Daily and continuous headache characterized as severe pressure (as if “head will explode”) with episodes of sudden
severe worsening
No fever, no meningismus
2 weeks of worsening pulsatile tinnitus
PMH: “Similar headache” 5 years ago with normal MRI
Medications: OCP
Prior imaging: normal CT head and MRA head
Exam: Right eye ptosis, left papilledema, left arm and left drift
Clinical Differential Diagnosis
• Hemicrania continua
• Intracranial mass
• Idiopathic intracranial hypertension
• Cerebral sinus thrombosis
Dense Clot in Superior
Normal CT 3 weeks prior
Sagital Sinus
TI hyperintensity in right
transverse sinus
MRV at time of diagnosis
MRV 9 months post treatment
Case 2
MRI
DWI
Mild diffusion restriction in right frontoparietal region
on Diffusion Weighted Imaging (right image) and ADC
map (left image)
ADC
Case 2- Dural Veinous Thrombosis
•
Hypercoagulable workup positive for lupus
anticoagualant
Incidence of involved Sinuses and Veins
Pathogenesis:
•
•
•
Cerebral vein thrombosis causes local edema/venous
infarction - Cytotoxic edema - Vasogenic edema
Cerebral sinus thrombosis causes intracranial
hypertension
A prothrombotic risk factor found in 85% of patients
with CST
Epidemiology:
• Incidence: 5 people/ 1 million
Cortical veins 17%
Superior sagittal sinus 62%
Vein of Galen and internal
cerebral veins 11%
Diagnosis
• D-dimer: Very good negative predictive value in patients
with abnormal neurological exams but not helpful in
patients with isolated headache
• Imaging gold standard: MRI and MRV with and without
contrast
Acute treatment:
• For children or adults, with or without hemorrhagic
venous infarction, anticoagulation therapy with either
LMWH or unfractionated heparin is recommended (level
1B).
Straight Sinus 18%
Transverse sinuses 86%
NEJM 2005
Jugular veins 12%
Case 2
Evaluation and Treatment
•
•
•
•
•
•
•
OCP stopped
Hypercoagulable panel sent
Lovenox 1mg/kg BID->Coumadin
Hydration 150cc/hr NS
Monitored in ICU
Headaches controlled
Hypercoagulable workup
– + lupus anticoagulant
– Heterozygous prothrombin gene mutation
Dural Veinous Thrombosis
• Most data regarding demographics, clinical features, natural
history, and treatment is derived from the International Study
on Cerebral Vein and Dural Sinus Thrombosis (624 pts)
Ferro et al. Stroke 2004; 35: 664-670
- Epidemiology:
Incidence: 5 people/ 1 million
Dural Veinous Thrombosis
Dural Veinous Thrombosis: Risk factors
A prothrombotic risk factor found in
85% of patients with CST
Case 3-Primary thunderclap headache
• 42-year-old woman with lifelong history of migraine presents to ED (3rd time in 1
week) with thunderclap headache which began during a bowel movement
• 4 years prior: Admitted for status migranosis and CT head, LP, and CTA were
normal.
Scatter white matter FLAIR
signal abnormalities are
unchanged from 5 years prior
Normal MRV
Normal MRA
•
Differential Diagnosis Status Migranosis, Cluster headache, Primary cough headache
/Valsalva-maneuver headache, Primary thunderclap headache, Idiopathic intracranial hypertension
Primary thunderclap headache
•
Diagnostic criteria: Severe head pain, Abrupt onset, reaching maximum
intensity within 1 minute, Lasting for >5 minutes, Not better accounted for by another diagnosis
•
46% of patients with primary thunderclap headache have baseline primary headache disorders
Primary cough headache
• Diagnostic criteria: At least 2 headaches episodes, Occur in association with cough, strain, or other valsalva maneuver,
Sudden onset, Lasting 1 sec- 2 hours
Case 3
• Treatment
- Indomethacin 50mg TID
- Inderal 40mg BID
- Tapered off these medications over 2 months without
headache recurrence
Primary Thunderclap Headache
• 46% of patients with primary thunderclap headache have
baseline primary headache disorders
Linn et al. J. Neuro 1999; 246: 946-948
Number of patients with recurrent TH
•103 patients followed for 1 year
•25% had recurrent events
•No SAH in followup
Landtblom A et al. Cephalalgia 2002;22:354-360
“Long term follow-up of 71
patients with TH mimicking SAH”
• 3.3 year average followup
• 17% had recurrent event
• No SAH in followup
Wijdicks et al. The Lancet 1988; 68-69
Summary/Conclusion
• Majority of TH will have a benign etiology
• Comprehensive testing should be considered to exclude secondary
causes- CT head without contrast is recommended first test. If
continued clinical suspicion for subarachnoid hemorrhage despite
negative CT (and CT performed after 6 hours of headache onset),
LP should be performed.
• There is no reliable way to clinically distinguish primary from
secondary TH.
• In patients with recurrent thunderclap headache, consider
angiographic imaging to evaluate for RVCS.
• In women with progressively worsening headache who are on oral
contraceptive pills, consider cerebral sinus thrombosis
• Primary thunderclap headache is a diagnosis of exclusion.
References
•
•
•
•
•
•
•
•
•
•
•
1. Wu X, Kalra VB, Durand D, Malhotra A. Utility analysis of management strategies for suspected subarachnoid
haemorrhage in patients with thunderclap headache with negative CT result. Emergency medicine journal : EMJ
2016;33:30-6.
2. van der Wee N, Rinkel GJ, Hasan D, van Gijn J. Detection of subarachnoid haemorrhage on early CT: is lumbar
puncture still needed after a negative scan? Journal of neurology, neurosurgery, and psychiatry 1995;58:357-9.
3. Schwedt TJ. Thunderclap headaches: a focus on etiology and diagnostic evaluation. Headache 2013;53:563-9.
4. Schwedt TJ. Clinical spectrum of thunderclap headache. Expert review of neurotherapeutics 2007;7:1135-44.
5. Savitz SI, Edlow J. Thunderclap headache with normal CT and lumbar puncture: further investigations are
unnecessary: for. Stroke; a journal of cerebral circulation 2008;39:1392-3.
6. Perry JJ, Stiell IG, Sivilotti ML, et al. Sensitivity of computed tomography performed within six hours of onset of
headache for diagnosis of subarachnoid haemorrhage: prospective cohort study. BMJ (Clinical research ed)
2011;343:d4277.
7. Linn FH, Rinkel GJ, Algra A, van Gijn J. Headache characteristics in subarachnoid haemorrhage and benign
thunderclap headache. Journal of neurology, neurosurgery, and psychiatry 1998;65:791-3.
8. Landtblom AM, Fridriksson S, Boivie J, Hillman J, Johansson G, Johansson I. Sudden onset headache: a prospective
study of features, incidence and causes. Cephalalgia : an international journal of headache 2002;22:354-60.
9. Fine B, Singh N, Aviv R, Macdonald RL. Decisions: does a patient with a thunderclap headache need a lumbar
puncture? CMAJ : Canadian Medical Association journal = journal de l'Association medicale canadienne
2012;184:555-6.
10. Ducros A, Bousser MG. Thunderclap headache. BMJ (Clinical research ed) 2013;346:e8557.
11. Devenney E, Neale H, Forbes RB. A systematic review of causes of sudden and severe headache (Thunderclap
Headache): should lists be evidence based? The journal of headache and pain 2014;15:49.