ICL 10.1: Secondary Headache

Question 1

what are the 2 secondary headache disorders?

Answer 1

these are headaches that are caused by something else!

1. thunderclap headaches = SAH, RCVS, PRES, CVT, dissection
2. stroke

honorable mentions = trigenminal neuralgia, pituitary apoplexy, spontaneous intracranial hypotension, giant cell arteritis

Question 2

what are the SNOOP4 red flags of secondary headaches**

Answer 2

ⓢ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

Question 3

what is a thunderclap headache?

Answer 3

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

Question 4

what do you do if you think someone is having a thunderclap headache?

Answer 4

CT and if negative do a LP, possibly MRI, other neurovascular imaging

management and treatment depend on underlying cause

Question 5

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

Answer 5

subarachnoid hemorrhage

“worst headache of my life” is like almost pathoneumonic for SAH

Question 6

what is the clinical presentation for a thunderclap headache caused by a subarachnoid hemorrhage?

Answer 6

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

Question 7

what is a sentinel headache?

Answer 7

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

Question 8

what testing do you do if someone is having a SAH thunderclap headache?

Answer 8

1. 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)

2. if that’s negative, do a LP to look for blood, a cell count and visual inspection for xanthochromia (yellowness from blood breakdown)
3. mass spectrophotometry has 95% sensitivity at 12 hours

Question 9

how do you treat a SAH TCH?

Answer 9

Repairing source of bleed and preventing complications like lowering HTN to reduce headaches

Question 10

when would you do an LP for a suspected SAH TCH and what would the results show?

Answer 10

you would do an LP if:
1. the patient presents with a first unusually severe headache

2. TCH w/ negative CTH
3. subacute progressive headache that is new and was a TCH at onset
4. headache associated w/ fever, confusion, meningism, or seizures
5. 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

Question 11

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

Answer 11

reversible cerebral vasoconstrictive syndrome (RCVS)

Question 12

what does RCVS stand for?

Answer 12

reversible cerebral vasoconstrictive syndrome

Question 13

what is RCVS syndrome?

Answer 13

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

Question 14

what will imaging show for someone with RCVS?

Answer 14

1. CSF normal
2. imaging w/ diffuse, segmental, reversible cerebral vasoconstriction
3. MRI brain with MRA head can show abnormalities but it can also be normal
4. cerebral angiography is a gold standard for vessel imaging!! –> will show “string of beads” appearance

Question 15

how do you treat RCVS?

Answer 15

1. fluid bolus

2. calcium channel blockers like verapamil

Question 16

what things can cause RCVS?

Answer 16

1. cannabis, cocaine, ecstasy, amphetamines, LSD, binge drinking
2. sympathomimetics, nasal decongestants
3. serotonergic drugs: SSRIs, triptans
4. immunosuppressants: tacrolimus, cyclophosphamide
5. nicotine patches
6. herbal medications: ginseng
7. blood products

Question 17

which conditions predispose someone to developing RCVS?

Answer 17

1. pregnancy
2. eclampsia, preeclampsia
3. neoplasia: pheochromocytoma, bronchial carcinoid, glomus tumour
4. neurosurgery, head injury
5. hypercalcaemia
6. porphyria
7. intracerebral hemorrhage, subarachnoid hemorrhage

Question 18

what does PRES stand for?

Answer 18

posterior reversible encephalopathy syndrome

Question 19

what is PRES?

Answer 19

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

Question 20

what imaging do you do for suspected PRES?

Answer 20

1. CTH and LP are usually normal and often miss the diagnosis
2. MRI however will show vasogenic edema most often in the posterior white matter and cortex, though frontal areas can be involved as well

Question 21

how do you treat PRES?

Answer 21

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

Question 22

what is the prognosis of someone with PRES?

Answer 22

it’s favorable!

neurological status of most patients returns to baseline in days to weeks

Question 23

what is the cause of PRES?

Answer 23

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

Question 24

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

Answer 24

cerebral venous thrombosis (CVT)

Question 25

what does CVT stand for?

Answer 25

cerebral venous thrombosis

Question 26

what is a cerebral venous thrombosis?

Answer 26

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

Question 27

what is the clinical presentation of someone with a cerebral venous thrombosis?

Answer 27

1. papilledema** (sign of increased ICP) 2. seizures 3. 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

Question 28

what population is most often effected by cerebral venous thrombosis?

Answer 28

mean age of onset is 20 – 40 years slight female preponderance, especially in young patients

Question 29

what causes cerebral venous thrombosis?

Answer 29

1. idiopathic 2. infection 3. hemodynamic like dehydration, CHF, fever 4. hypercoagulable states = pregnancy, factor V leiden, 5. hormonal = oral contraceptives, pregnancy 6. inflammation = SLE, sarcoidosis, IBD

Question 30

what imaging do you get for a suspected cerebral venous thrombosis?

Answer 30

MR venogram to see the blood flow through the venous system

Question 31

how do you treat a cerebral venous thrombosis?

Answer 31

depends on the cause 1. anticoagulation/ thrombolytic therapy 2. decrease ICP 3. antibiotics, if septic CVT 4. anti-epileptic medications 5. surgery: thrombectomy, hematoma evacuation (rare)

Question 32

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

Answer 32

carotid dissection carotid dissection is causing sympathetic chain damage which presents as Horner's syndrome

Question 33

what are the risk factors for having a carotid dissection?

Answer 33

1. HTP 2. migraine usually occurs in people over 50

Question 34

what are the symptoms of a carotid dissection?

Answer 34

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

Question 35

what imaging would you use for a suspected carotid dissection?

Answer 35

MRA or angiogram looks like a flame sign from the acute tapering of blood flow

Question 36

how do you treat a carotid dissection?

Answer 36

1. repair 2. NSAIDs 3. rarely anticoagulation

Question 37

what types of stroke present with headaches?

Answer 37

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

Question 38

what conditions cause a thunderclap secondary headache?

Answer 38

1. sucharachnoid hemorrhage 2. RCVS = reversible cerebral vasoconstrictive syndrome 3. PRES = posterior reversible encephalopathy syndrome 4. CVT = cerebral venous thrombosis 5. dissection (usually of the carotid)

Question 39

what is the clinical presentation of trigeminal neuralgia?

Answer 39

1. severe, stabbing, lancinating, electric-shock like pain in the distribution of the trigeminal nerve 2. attacks last from seconds to 2 minutes 3. may be precipitated by mechanical factors such as wind, touch, toothbrushing, eating, drinking 4. 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)

Question 40

how do you treat trigeminal neuralgia?

Answer 40

1. carbamazepine* 2. gabapentin 3. baclofen 4. lamotrigine you can do surgical options sometimes to burn the nerve or relieve compression but not common

Question 41

what is the ICHD-III criteria for trigeminal neuralgia?

Answer 41

at least 3 attacks of unilateral facial pain fulfilling the following: 1. occurring in 1+ divisions of the trigeminal nerve with no radiation beyond the trigeminal nerve distribution 2. 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 3. no clinically evident neurological deficit

Question 42

what is pituitary apoplexy?

Answer 42

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

Question 43

what is the clinical presentation of pituitary apoplexy?

Answer 43

1. holocranial headache w/ an acute or thunderclap onset 2. nausea, vomiting 3. constriction of visual fields 4. ophthalmoplegia

Question 44

how do you treat pituitary apoplexy?

Answer 44

surgical intervention

Question 45

what is spontaneous intracranial hypotension?

Answer 45

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

Question 46

what are the symptoms of spontaneous intracranial hypotension?

Answer 46

1. postural aggravation: better w/ lying down because increase of ICP, worse w/ getting up 2. bilateral, neck stiffness 3. auditory abnormalities or tinnitus, vertigo, diplopia

Question 47

what will you see on imaging of a patient with spontaneous intracranial hypotension?

Answer 47

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

Question 48

how do you treat spontaneous intracranial hypotension?

Answer 48

1. fluids 2. 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

Question 49

what is giant cell arteritis?

Answer 49

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)

Question 50

what is the clinical presentation of giant cell arteritis?

Answer 50

1. scalp tenderness 2. jaw claudication 3. polymyalgia rheymatica 4. constitutional symptoms

Question 51

how do you treat giant cell arteritis?

Answer 51

high dose steroids do a biopsy too