Thunderclap Headache

Thunderclap Headache

International Classification of Headache Disorders II diagnostic

criteria (ICHD II), TCHs severe intensity headaches that reach
maximum intensity in less than 1 minute

TCHs are differentiated from other headaches of severe intensity

(eg, migraine, cluster) by the rapidity with which they reach
maximum severity

Patients with a TCH requires emergent evaluation.

Due to the seriousness of the condition, subarachnoid hemorrhage

(SAH) is the first consideration

Causes of TC Headache
More Common
Subarachnoid hemorrhage
Unruptured intracranial aneurysm
(sentinel headache)
Reversible cerebral
vasoconstriction syndromes
Cervical artery dissection
Cerebral venous sinus thrombosis
Spontaneous intracranial
hypotension (CSF leak)
Hypertensive emergency
Intracerebral hemorrhage

Less Common
Subdural hematoma
Retroclival hematoma
Ischemic stroke
Meningitis
Brain tumor
Pituitary apoplexy
Colloid cyst third ventricle
Myocardial infarction
Aqueductal stenosis
Complicated sinusitis
Pheochromocytoma
Primary cough, exertional, and
sexual headache

Clinical Presentation
How long did it take for your headache to reach maximum intensity?
TCH is diagnosed when the patient reports that the headache is
severe and it reached maximum severity very rapidly
Maybe localized to 1 region, or may be holocephalic
Isolated symptom or accompanied by a multitude of different
symptoms, their presence dependent upon the underlying condition
leading to the TCH
More common symptoms include nausea, vomiting, photosensitivity,
phonosensitivity, neck pain, neck stiffness, focal neurologic
symptoms, visual change, altered cognition, and altered level of
consciousness.

Subarachnoid Hemorrhage

Most common cause of TCH, 10-25% of patients presenting with

TCH will have SAH.

SAH must be the first consideration in a patient with TCH

Majority of SAHs (85%) are secondary to rupture of saccular

aneurysms

Less common causes of SAH including non-aneurysmal

perimesencephalic haemorrhage, arterial dissections, arteriovenous
malformations, dural arteriovenous fistula, mycotic aneurysm,
bleeding disorders, vasculitides, spinal cord vascular lesions, and
substance abuse

Subarachnoid Hemorrhage
Headache, typically a TCH, is the most common symptom
Headache may have spontaneous onset, or may follow physical exertion.
In about 1/3 of cases, headache occurs in isolation
Other symptoms, when present, may include: altered consciousness,
cognitive dysfunction, seizures, visual disturbances, nausea, vomiting,
meningismus, photophobia, and dizziness.
2550% of patients with subarachnoid haemorrhage are initially
misdiagnosed. Mortality approximately 50%, including 10-15% of SAH
patients who die prior to obtaining medical services .

Unruptured Intracranial Aneurysm

(Sentinel headache)
sentinel headache or warning headache is a TCH that occurs
days to weeks prior to rupture of an intracranial aneurysm
10 to 43% of patients with SAH. Patients with sentinel headaches
generally do not have meningismus, altered consciousness, or focal
neurological symptoms and signs.
Most commonly occur within 2 weeks of SAH, highest incidence
being within 24 hours of SAH
Theoretically, finding an unruptured aneurysm in a patient with TCH
could lead to treatment of the unruptured aneurysm and avoidance
of the impending SAH.

Reversible Cerebral Vasoconstriction

Syndromes
Most common presenting symptom is TCH (most often repetitive),
approximately 85% of patients
Headaches may occur spontaneously or be provoked by Valsalva
maneuvers
TCH in isolation or accompanied by other neurologic symptoms due to
cerebral edema, ischemic stroke, or transient ischemic attack
Multifocal areas of intracranial vasoconstriction, no evidence of
aneurysmal SAH, normal or nearly normal cerebrospinal fluid (CSF)
studies
Spontaneous reversal of vasoconstriction within several months of
symptom onset without any specific treatment

Cervical Artery Dissection

Headache is the most common symptom in patients with cervical

internal carotid and vertebral dissections, 2/3 of patients

20% report TCH. Can be diffuse in location, the typical headache is

ipsilateral to the dissected artery.

Most often felt in the periorbital, frontal, and temporal regions

Headache associated with vertebral artery dissection is more

commonly felt in posterior head regions

Neck pain is present with 1/3 of carotid artery and 2/3 of vertebral
artery dissections

Cervical Artery Dissection

Headache most common symptom ; 6095% with carotid artery
dissections, 70% with vertebral artery dissections.
Most commonly gradual onset. 20% of patients present with TCH.
Typically cervical artery dissections manifest with symptoms in addition to
headache and neck pain. Isolated headache seen only in 2% to 8% of
patients.
Headache can precede other symptoms by several hours to several days
Other symptoms of cervical artery dissection include:
Horners syndrome, pulsatile tinnitus, amaurosis fugax, diplopia, and
other symptoms that occur due to transient ischemic attacks or strokes
related to the dissected artery

Spontaneous Intracranial Hypotension

Syndrome of low CSF volume and/or pressure secondary to a CSF
leak. Typically preceded by minor trauma such as trivial falls, lifting,
coughing, and sports activities.
TCH is the presenting symptom of SIH approximately 15% of cases
Orthostatic headache is pathognomonic for SIH (worsen substantially
when upright)
Headache may worsen with Valsalva, coughing, sneezing, and bending.
Other accompanying symptoms may include auditory muffling, nausea,
vomiting, neck stiffness, tinnitus, dizziness, visual changes ,
interscapular and upper extremity radicular pain

Cerebral Venous Sinus Thrombosis

Typical headache associated with CVST is subacute in onset,
however 2-10% of patients with CVST present with TCH and 25%
have isolated headache
Continuous with worsening pain associated with increases in
intracranial pressure (Valsalva)
Symptoms that commonly occur include focal neurological symptoms
(eg, hemiplegia, visual changes including diplopia), altered level of
consciousness, altered mental status, seizures, and tinnitus.
Risk factors include hypercoagulable conditions such as
thrombophilia, pregnancy, dehydration, use of oral contraceptives,
cancer, and head trauma

Cerebral Venous Sinus Thrombosis

Initial investigations for SAH are commonly inadequate for the

diagnosis of CVST

In patients with normal neurological examination, brain CT is normal

in about 25%. In those with focal neurological deficits, there are CT
abnormalities in about 90%. Subtle findings on CT can be easily
misinterpreted.

So if the clinical suspicion for CVST is high MRV or CTV must be

considered

Primary Thunderclap Headache

When no cause for TCH is identified despite a comprehensive

diagnostic evaluation, primary TCH is the appropriate diagnosis

Debatable whether primary TCH actually exists or is just

inappropriately diagnosed. ICHD-II classification goes on to say that
evidence that thunderclap HA exists as a primary condition is poor.

Increased use of MRI and non-invasive vascular imaging leading to

higher diagnostic rates of disorders likely to be missed by CT and
CSF evaluation (eg, RCVS)

Physical Examination
Body temperature, blood pressure, heart rate, and other vital signs
Temperature

Suspicion for an underlying infectious etiology

BP

Suggestive of hypertensive emergency/urgency as the etiology of TCH, may initially

be difficult to distinguish from elevated BP as part of a generalized pain and anxiety
response
Elevated blood pressure and bradycardia in a patient with reduced level of
consciousness could suggest elevated intracranial pressure such as can be seen
with a large SAH, intracerebral hemorrhage, and with severe cerebral edema

Neurologic examinations of TCH patients vary widely depending

upon the underlying etiology

More common findings include the presence of meningismus, papilledema, visual

deficits, altered consciousness, and focal neurologic deficits.

Brain CT Without Contrast

Following collection of the patients history and completion of the
physical and neurologic examinations, brain CT without contrast is
indicated
Irrespective of the interval between symptom onset and CT
scanning, the sensitivity of brain CT for SAH was 92.9%.
However, sensitivity was 100% for those patients who underwent
brain CT within 6 hours of symptom onset
The sensitivity of brain CT for detecting SAH decreases as the
interval between symptom onset and image acquisition lengthens.
Sensitivity approached 100% when CT <12 hours, 86% on day 2,
76% after 2 days, and to 58% after 5 days

Brain CT Without Contrast

Because the sensitivity of CT is not 100% in the vast majority of

cases, lumbar puncture is required if a diagnosis is not reached and
not contraindicated following brain CT

Brain CT without contrast may show evidence for other etiologies of

TCH including:
Intracranial hemorrhage, ischemic stroke, cerebral edema, CVST
(eg,
hyperdensity
within
the
occluded
sinus,
venous
infarcts/hemorrhages, edema), intracranial mass lesions, and SIH
(eg, subdural fluid collections)

Lumbar Puncture
Lumbar puncture is performed in the evaluation of patients with TCH and
nondiagnostic brain CTs
Yield of CSF analysis is higher when CSF is collected at least 12 hours
after the onset of symptoms, however a delay in the diagnostic evaluation
of the TCH patient is not recommended
Recommended for all patients: opening pressure, cell count with
differential in tube #1 and tube #4, protein, glucose, Gram stain and
culture, and visual inspection for xanthochromia. If available, CSF
spectrophotometry high sensitivity for the diagnosis of SAH.
In CT-negative SAH to differentiate SAH from a traumatic (bloody) tap,
cell counts in tube #1 and tube #4 are measured and visual inspection for
xanthochromia

Lumbar Puncture
SAH is the likely when:
RBC count in tube #1 and tube #4 remains constant or is higher in tube #4
Visual inspection for xanthochromia has sensitivity of 93%, specificity of 95%,
positive predictive value of 72%, and negative predictive value of 99% for the
presence of cerebral aneurysm
Spectrophotometry had very high sensitivity and negative predictive value (both
100%), but low specificity (75%) and very low positive predictive value (3.3%) for
detecting cerebral aneurysms in patients with suspected SAH and normal brain CTs

Opening pressure maybe in CVST, intracranial infections and space

occupying lesions, and pressure is low with SIH
Protein and WBC count elevated in CNS inflammation and/or infection.
CVST may have a combination of RBC count, protein, lymphocytic
pleocytosis, opening pressure, although only elevated opening
pressure or maybe completely normal results possible

Additional Testing

Conflicting opinions regarding the necessity for additional testing

when the cause of TCH is not determined following brain CT and
lumbar puncture

Additional testing often includes brain MRI with and without

gadolinium and imaging of the intracranial and cervical arteries via
MRA or CTA. Depending upon clinical suspicion, MRV or CTV
maybe considered

MRI with gadolinium is particularly helpful for the diagnosis of SIH,

ischemic stroke, tumors, pituitary apoplexy, colloid cyst, and edema
associated with hypertensive emergencies and reversible posterior
leukoencephalopathy (seen in the setting of hypertensive
emergencies and RCVS).

Additional Testing
Conflicting opinions regarding the necessity for additional testing when
the cause of TCH is not determined following brain CT and lumbar
puncture
American College of Emergency Physicians clinical policy guideline
that states: patients with a sudden-onset, severe headache who have
negative findings on a head CT, normal opening pressure, and
negative findings in CSF analysis do not need emergent angiography
and can be discharged from the ED with follow-up recommended.
Additional testing often includes brain MRI with and without gadolinium
and imaging of the intracranial and cervical arteries via MRA or CTA.
Depending upon clinical suspicion, MRV or CTV maybe considered

Additional Testing
MRI with gadolinium is particularly helpful for the diagnosis of SIH,
ischemic stroke, tumors, pituitary apoplexy, colloid cyst, and edema
associated with hypertensive emergencies and reversible posterior
leukoencephalopathy (+/- hypertensive emergencies and RCVS).
Angiography is performed in the evaluation of the SAH patient in order to
identify an underlying intracranial aneurysm.
Angiography is also useful in evaluating for other causes of TCH,
including unruptured intracranial aneurysm, RCVS, CVST, and arterial
dissections.
However, the risks of catheter angiography need to be considered. Noninvasive angiography via CTA or MRA may be considered in place of
catheter angiography

Initial assessment of TCH

Summary
TCHs are severe headaches that reach maximum severity quickly
TCH can be the only presenting symptom of SAH, the patient with TCH
must be evaluated emergently
Brain CT without contrast and lumbar puncture with basic CSF studies
and inspection for xanthochromia are probably sufficient for the emergent
evaluation of the patient with TCH
Know the changing sensitivity of brain CT and CSF analysis in relation to
the interval between symptom onset
If SAH is detected via CT or lumbar puncture, angiography is performed
in search of an underlying aneurysm
When head CT and lumbar puncture are normal, further testing should
be considered in order to evaluate for causes of TCH other than SAH

References

Schwedt, T. et al. Review: Thunderclap headache. Lancet Neurol

2006; 5: 62131

Dilli, E. Thunderclap Headache. Curr Neurol Neurosci Rep. 2014;

14: 437

Purdy, AR. et al. Dangerous and Thunderclap Headaches.

Headache. 2012; 52; S2:56-59

Schwedt, TJ. Clinical Review: Thunderclap Headaches A Focus on

Etiology and Diagnostic Evaluation. Headache 2013; 563-569