ICL 10.1: Secondary Headache Flashcards
what are the 2 secondary headache disorders?
these are headaches that are caused by something else!
- thunderclap headaches = SAH, RCVS, PRES, CVT, dissection
- stroke
honorable mentions = trigenminal neuralgia, pituitary apoplexy, spontaneous intracranial hypotension, giant cell arteritis
what are the SNOOP4 red flags of secondary headaches**
ⓢystemic symptoms and signs = fever, chills, night sweats, weight loss
Ⓝeurologic symptoms and signs = global or focal, AMS, unilateral weakness
Ønset sudden
Ønset at age older than 50 yo: very unusual for primary headache disorder to present after 50 yo
℗rogression of headache
℗recipitation of headache w/ valsalva: coughing, sneezing, bending over, heavy lifting indicate ICP
℗ostural headache = headache worse with standing
℗apilledema
℗regnancy
what is a thunderclap headache?
acute, severe headache that reaches peak intensity at onset (that’s why it’s called a thunderclap headache!)
this is a neurologic emergency and urgent evaluation for secondary headache is recommended
what do you do if you think someone is having a thunderclap headache?
CT and if negative do a LP, possibly MRI, other neurovascular imaging
management and treatment depend on underlying cause
45 yo woman presents to ED with intense global headache, which she experienced sudden onset of “the worst headache of my life”
Headache features: pancephalic, severe intensity
Asociated sx: photophobia, phonophobia, and neck stiffness
History of migraine
Family history: cerebral hemorrhage in mother
Exam: BP 180/100 mm Hg, no focal weakness
subarachnoid hemorrhage
“worst headache of my life” is like almost pathoneumonic for SAH
what is the clinical presentation for a thunderclap headache caused by a subarachnoid hemorrhage?
Worst Headache Of Life (WHOL): can be acute or several days to weeks
this is the most common cause of TCH (25% of TCH)
SAH carries poor overall outcome w/ significant morbidity and mortality, so initial eval of TCH should focus on ruling out SAH
associated features: nausea, vomiting, photophobia, neck stiffness, AMS, possibly HTN
causes: trauma > aneurysmal bleed
what is a sentinel headache?
a small aneurysmal bleed reported in 20 to 50% of TCH cases
so it’s a SAH but it’s not as big so you don’t pick it up as well
what testing do you do if someone is having a SAH thunderclap headache?
- CT of the head without contrast
can be negative if the CT is done late in the game or it’s a small bleed (will be negative at 3 weeks)
- if that’s negative, do a LP to look for blood, a cell count and visual inspection for xanthochromia (yellowness from blood breakdown)
- mass spectrophotometry has 95% sensitivity at 12 hours
how do you treat a SAH TCH?
Repairing source of bleed and preventing complications like lowering HTN to reduce headaches
when would you do an LP for a suspected SAH TCH and what would the results show?
you would do an LP if:
1. the patient presents with a first unusually severe headache
- TCH w/ negative CTH
- subacute progressive headache that is new and was a TCH at onset
- headache associated w/ fever, confusion, meningism, or seizures
- if you suspect high or low CSF pressure (even if papilledema is absent) if CTH does NOT show a mass lesion or other cause of symptoms
19 year old man comes to ED due to sudden onset of severe occipital headache, and in the waiting room he experiences first generalized tonic-clonic seizure
No past medical history, other than occasional migraines as a preteen
VS BP 165/95 mm Hg, Pulse 110 bpm, temp 99.3 degrees. He was lethargic and somewhat confused
Labs were remarkable for tox screen showing THC. He admits to daily pot smoking
reversible cerebral vasoconstrictive syndrome (RCVS)
what does RCVS stand for?
reversible cerebral vasoconstrictive syndrome
what is RCVS syndrome?
a type of TCH from cerebral vasoconstriction that is typically occipital though not necessarily
varies clinically w/ regard to presence and severity of neurologic deficits and imaging abnormalities
may present with focal deficits and/or seizures because this vasoconstriction can cause strokes, seizures, etc.
affects those 20 - 50 years of age ; women> men
described in peripartum period or in patients exposed to sympathomimetic drugs and SSRIs
what will imaging show for someone with RCVS?
- CSF normal
- imaging w/ diffuse, segmental, reversible cerebral vasoconstriction
- MRI brain with MRA head can show abnormalities but it can also be normal
- cerebral angiography is a gold standard for vessel imaging!! –> will show “string of beads” appearance
how do you treat RCVS?
- fluid bolus
2. calcium channel blockers like verapamil
what things can cause RCVS?
- cannabis, cocaine, ecstasy, amphetamines, LSD, binge drinking
- sympathomimetics, nasal decongestants
- serotonergic drugs: SSRIs, triptans
- immunosuppressants: tacrolimus, cyclophosphamide
- nicotine patches
- herbal medications: ginseng
- blood products
which conditions predispose someone to developing RCVS?
- pregnancy
- eclampsia, preeclampsia
- neoplasia: pheochromocytoma, bronchial carcinoid, glomus tumour
- neurosurgery, head injury
- hypercalcaemia
- porphyria
- intracerebral hemorrhage, subarachnoid hemorrhage
what does PRES stand for?
posterior reversible encephalopathy syndrome
what is PRES?
a type of THC or severe acute headache that’s basically edema that’s reversible –> if it’s misdiagnosed or delay of diagnosis, it can cause infarct and permanent damage
patients will present with global and focal neurological signs and sx, AMS, visual loss, seizures
usually occurs with HTN crisis but can also be from eclampsia, specific drugs (immunosuppressants most commonly)
can occur in patients w/ RCVS initially, and vice versa
what imaging do you do for suspected PRES?
- CTH and LP are usually normal and often miss the diagnosis
- MRI however will show vasogenic edema most often in the posterior white matter and cortex, though frontal areas can be involved as well
how do you treat PRES?
treat underlying cause which is usually HTN
or you might have to discontinue medication if someone’s on immunosuppressants or magnesium if someone was on it for eclampsia
what is the prognosis of someone with PRES?
it’s favorable!
neurological status of most patients returns to baseline in days to weeks
what is the cause of PRES?
the cause of PRES remains controversial, but the most popular theory is that severe hypertension causes interruption to brain autoregulation
cerebral blood flow is usually regulated by dilatation and constriction of vessels to maintain adequate tissue perfusion and to simultaneously avoid excessive intracerebral hypertension
breakdown in autoregulation occurs above a mean arterial blood pressure of 150 - 160 mmHg; in chronic hypertension, it occurs at higher pressures
so uncontrolled hypertension leads to hyperperfusion and cerebral vessel damage, resulting in interstitial extravasation of proteins and fluids, causing vasogenic edema
31 yo woman comes to ED 48 hours after developing an instant sharp, shooting, severe pain behind the right eye for 5 minutes with nausea, photo, phonophobia. Since then, she has constant pain with fluctuating intensity in the right head, face, neck and shoulder
She has monthly migraine. These are unilateral (right or left), frontotemporal throbbing headaches associated with nausea, photo- and phonophobia
PMH: hypothyroidism, menstrual migraine, endometriosis
Medications include daily levothyroxine, oral contraceptives, and prn sumatriptan tabs (none recently)
Exam: normal except for fundus w/ mild BL papilledema
cerebral venous thrombosis (CVT)
what does CVT stand for?
cerebral venous thrombosis
what is a cerebral venous thrombosis?
it is the presence of a blood clot in the dural venous sinuses that usually causes a TCH
you can have a clot anywhere along the venous sinuses so in the superior sagittal sinus, straight sinus, sigmoid sinus, etc.
10% of CVT present w/ TCH w/o any other neurological signs or symptoms however most DO have neurological signs
what is the clinical presentation of someone with a cerebral venous thrombosis?
- papilledema** (sign of increased ICP)
- seizures
- focal deficits
CVT headaches are persistent, progressive, exacerbated by Valsalva because of increased ICP
lying down increases ICP and can be painful
progressive obtundation where the distended cerebral veins will rupture into subarachnoid space
what population is most often effected by cerebral venous thrombosis?
mean age of onset is 20 – 40 years
slight female preponderance, especially in young patients
what causes cerebral venous thrombosis?
- idiopathic
- infection
- hemodynamic like dehydration, CHF, fever
- hypercoagulable states = pregnancy, factor V leiden,
- hormonal = oral contraceptives, pregnancy
- inflammation = SLE, sarcoidosis, IBD
what imaging do you get for a suspected cerebral venous thrombosis?
MR venogram to see the blood flow through the venous system
how do you treat a cerebral venous thrombosis?
depends on the cause
- anticoagulation/ thrombolytic therapy
- decrease ICP
- antibiotics, if septic CVT
- anti-epileptic medications
- surgery: thrombectomy, hematoma evacuation (rare)
45 yo woman with sudden onset of left neck pain and left-sided headache while placing dishes on top shelf of cupboard
She came to ED for the following when she experienced transient left monocular blindness, and noted unequal pupils and left ptosis
PMH: migraine and hypertension
carotid dissection
carotid dissection is causing sympathetic chain damage which presents as Horner’s syndrome
what are the risk factors for having a carotid dissection?
- HTP
- migraine
usually occurs in people over 50
what are the symptoms of a carotid dissection?
can present with headache on side of dissection with or without neck pain can be the only manifestation of cervical artery dissection –> can present with TCH in isolation without associated signs of symptoms 20% of the time
headache is the most frequent symptom (55-100%), and the most frequent initial symptom (33-86% of cases)
associated signs : painful Horner’s syndrome, painful tinnitus, or painful hypoglossal nerve palsy
ischemia may occur in ipsilateral eye (transient of permanent blindness) or to brain depending on the progression of the dissection
what imaging would you use for a suspected carotid dissection?
MRA or angiogram
looks like a flame sign from the acute tapering of blood flow
how do you treat a carotid dissection?
- repair
- NSAIDs
- rarely anticoagulation
what types of stroke present with headaches?
headache in 25% of ischemic strokes and 75% hemorrhagic strokes!!
CTH diagnostic in hemorrhagic stroke
but CT will be normal in recent, small, or posterior circulation ischemic stroke
what conditions cause a thunderclap secondary headache?
- sucharachnoid hemorrhage
- RCVS = reversible cerebral vasoconstrictive syndrome
- PRES = posterior reversible encephalopathy syndrome
- CVT = cerebral venous thrombosis
- dissection (usually of the carotid)
what is the clinical presentation of trigeminal neuralgia?
- severe, stabbing, lancinating, electric-shock like pain in the distribution of the trigeminal nerve
- attacks last from seconds to 2 minutes
- may be precipitated by mechanical factors such as wind, touch, toothbrushing, eating, drinking
- usually idiopathic irritation of trigeminal nerve, but do MRI to look for structural causes as well –> can be primary, or secondary to vascular causes, tumors, young people w/ BL sx- MS (2-4% of TGN)
how do you treat trigeminal neuralgia?
- carbamazepine*
- gabapentin
- baclofen
- lamotrigine
you can do surgical options sometimes to burn the nerve or relieve compression but not common
what is the ICHD-III criteria for trigeminal neuralgia?
at least 3 attacks of unilateral facial pain fulfilling the following:
- occurring in 1+ divisions of the trigeminal nerve with no radiation beyond the trigeminal nerve distribution
- pain has 3+ of the following characteristics: recurring in paroxysmal attacks lasting from a fraction of a second to 2 minutes, severe intensity, electric shock-like shooting, stabbing or sharp in quality, or precipitated by innocuous stimuli to the affected side of the face
- no clinically evident neurological deficit
what is pituitary apoplexy?
acute, severe headache that can have a thunderclap onset due to acute hemorrhage or infarction of the pituitary gland
can be caused by pituitary adenomas, pregnancy, general anesthesia, bromocriptine therapy, or pituitary irradiation
what is the clinical presentation of pituitary apoplexy?
- holocranial headache w/ an acute or thunderclap onset
- nausea, vomiting
- constriction of visual fields
- ophthalmoplegia
how do you treat pituitary apoplexy?
surgical intervention
what is spontaneous intracranial hypotension?
spontaneous CSF leak –> reduced ICP –> sagging of the brain –> traction on innervated meninges –> headache
can be caused by minor trauma, connective tissue disorder, or post epidural
what are the symptoms of spontaneous intracranial hypotension?
- postural aggravation: better w/ lying down because increase of ICP, worse w/ getting up
- bilateral, neck stiffness
- auditory abnormalities or tinnitus, vertigo, diplopia
what will you see on imaging of a patient with spontaneous intracranial hypotension?
CTH: low lying cerebellar tonsils
LP: low opening pressure
MRI w/ and w/o contrast, MRI spine: diffuse dural enhancement* and extradural fluid collections
Brain sag: low lying cerebellar tonsils, crowding of posterior fossa, subdural hematomas, effacement of prepontine cistern
CT Myelogram best for identifying site of leak
how do you treat spontaneous intracranial hypotension?
- fluids
- caffeine
if those don’t help you can do a blood patch which is 20 mL of the patients own blood injected into the area of the leak
then if that fails, fibrin glucose injections
what is giant cell arteritis?
a headache syndrome caused by inflammation of blood vessels
most often seen in elderly females over 70 years old
high concern for amaurosis fugax which is a warning sign of permanent visual loss due to block of temporal artery flow (this is the end stage of GCA)
what is the clinical presentation of giant cell arteritis?
- scalp tenderness
- jaw claudication
- polymyalgia rheymatica
- constitutional symptoms
how do you treat giant cell arteritis?
high dose steroids
do a biopsy too
