Mayo Clinic 2 - 53 - Secondary Headache Disorders

Front 1

When a thunderclap headache (TCH) is identified, what is the next step in management?

Back 1

CT of the head, followed by lumbar puncture if the CT is negative; however, depending on the clinical picture, MRI of the brain with or without additional neurovascular imaging may be needed for diagnosis and management

Front 2

Most common cause of thunderclap headache?

Back 2

Subarachnoid hemorrhage (SAH) - 25%; initial evaluation of a TCH should focus on ruling out a subarahnoid hemorrhage

Front 3

What are sentinel headaches?

Back 3

Headaches that are clinically similar to SAH headaches, except they do not have neck stiffness, altered mental status, or focal neurologic symptoms. Likely due to a small undetectable leak instead of a structural change the aneurysm wall resulting in pain

Front 4

Diagnostic value of CT in the setting of TCH

Back 4

100% within the first 12 hours of symptom onset but decreases to 50% within 1 week

Front 5

Most important diagnoses to consider in thunderclap headache? (9)

Back 5

1. Subarachnoid hemorrhage (SAH) (20%)

2. Cervical artery dissection (20%)

3. Reversible cerebral vasoconstriction syndrome

4. Posterior reversible encephalopathy syndrome

5. Cerebral venous sinus thrombosis

6. Spontaneous intracranial hypotension

7. Stroke

8. Pituitary apoplexy

9. Third ventricle colloid cyst

Front 6

Clinically, how do patients with a cervical artery dissection present? (5)

Back 6

1. Headache that is ipsilateral to the dissection

2. Usually located in the jaw, face, ear, and periorbital and frontal regions

3. Localized to the occipital region

4. Nonthrobbing and severe pain

5. Unilateral Horner syndrome can often be seen

Front 7

How is the diagnosis of reversible cerebral vasoconstriction syndrome made?

Back 7

By the presence of diffuse, segmental cerebral vasoconstriction seen on MRI coupled with MRA of the head

Front 8

Imaging and angiographic findings with RCVS may be indistinguishable from its main differential diagnosis, which is?

Back 8

Primary angiitis of the central nervous system (PACNS)

Front 9

Difference between RCVS and PACNS?

Back 9

RCVS - thunderclap onset

PACNS - subacute, gradually progressive onset

Front 10

Mainstay treatment of RCVS

Back 10

Calcium channel blockers

Front 11

Mainstay treatment for PACNS

Back 11

Immunosuppression

Front 12

Prognosis for RCVS

Back 12

favorable with prompt diagnosis and management, although diagnostic delay can result in permanent neurologic sequelea

Front 13

Complications of RCVS

Back 13

SAH

Ischemic and hemorrhagic infarctions

Posterior reversible encephalopathy syndrome (PRES)-like changes on MRI

Front 14

Prognosis of PACNS

Back 14

Poor; even with prompt diagnosis and management, permanent neurologic deficits are common

Front 15

Clinical features of posterior reversible encephalopathy syndrome

Back 15

Global and focal neurologic syndromes and signs, such as altered mental status, visual loss, and seizures. May present with TCH.

Front 16

How is the diagnosis of posterior reversible encephalopathy syndrome made? (3)

Back 16

1. MRI of the brain shows vasogenic edema most often in the posterior white matter and cortex, although more frontal areas can be involved.

2. CT of the head and subsequent lumbar puncture will miss the diagnosis!

3. PRES can occur in patients who present initially with RCVS, and vice versa. Identifying RCVS with PRES is important because its presence will change management

Front 17

Treatment of PRES

Back 17

1. Depends on the underlying cause: acute hypertensive crisis requires a graduated decrease in blood pressure.

2. If PRES is due to eclampsia, magnesium is commonly used

3. RCVS in association with PRES may require CCBs in addition to other measures to lower the BP without causing hypoperfusion in the presence of stenoses

Front 18

Up to ___% of patients with cerebral venous sinus thrombosis (CST) present with a TCH without other neurologic signs

Back 18

10%

Front 19

Clinical features of CVST headaches

Back 19

1. Persistent

2. Progressive

3. Exacerbated by Valsalva maneuvers, such as couging, heavy lifting, or bearing down

4. Lying down also increases ICP and can be prainful for these patients

Front 20

Most striking characteristic of a spontaneous intracranial hypotension headache

Back 20

Postural aggravation

Front 21

Features of spontaneous intracranial hypotension

Back 21

1. Postural aggravation, orthostatic headache resolves with recumbency

2. Young or history of minor trauma or connective tissue disorder

3. Bilateral

4. Additional neurologic symptoms are often present

Front 22

MRI findings in spontaneous intracranial hypotension

Back 22

1. Diffuse pachymeningeal enhancement and extradural fluid collections

2. "Brain sag" with low-lying cerebellar tonsils

3. Crowding of the psoterior fossa

4. Subdural hematomas

5. Effacement of the prepontine cistern and flattening of the pons

6. Descent of the optic chiasm

Front 23

Best test for identifying a leak in spontaneous intracranial hypotension?

Back 23

CT myelogram

Front 24

First-line treatment for spontaneous intracranial hypotension? If that fails?

Back 24

Large-volume (20 mL) blind epidural autologous blood patch, which can be repeated 2 to 3 times if there is a partial response; if this fails, the site of the leak can be localized with a myelogram and terated with targeted epidural blood patches, fibrin glue injections, or, in refractory cases, neurosurgical intervention

Front 25

Features of pituitary apoplexy (5)

Back 25

1. Holocranial headache with an acute or thunderclap onset

2. Nausea and vomiting

3. Constriction of visual fields

4. Ophthalmoplegia

5. Systemic symptoms vary wildly (e.g., adrenal crisis and coma)

Front 26

Trigeminal neuralgia is _______

Back 26

the most common cranial neuralgia, with an incidence of 8 per 100,000 persos

Front 27

Average age of onset of trigeminal neuralgia

Back 27

50 years

Front 28

Clinical features of trigeminal neuralgia

Back 28

1. Unilateral, severe, stabbing, lancinating pain in the distribution of the trigeminal nerve

2. Lasting from seconds to 2 minutes

3. May be precipitated by mechanical factors

Front 29

Underlying pathophysiology of trigeminal neuralgia?

Back 29

Irritation of the trigeminal nerve, which is usually idiopathic; however, structural causes should be investigated

Front 30

Surgical options to treat trigeminal neuralgia can be considered when medical options have been ineffective. What are the surgical options? (2)

Back 30

1. Neuroablative procedures that include alcohol block, balloon compression, radiofrequency ablation, and Gamma Knife radiosurgery

2. Microvascular decompression (90% immediate relief; 3.5% recurrence rate annually)

Front 31

Complications of microvascular decompression? (4)

Back 31

1. Cranial nerve deficits

2. CSF leak

3. Hemorrhage

4. Anesthesia dolorosa (numbess with pain in the numbed area)

Front 32

Clinical presentation of patients with geniculate neuralgia

Back 32

1. Unilateral, lancinating pain within the ear that lasts for seconds to minutes

2. May occur several times a day

3. Can develop into a chronic, dull pain within the ear

Front 33

Pathophysiology of geniculate neuralgia

Back 33

Facial nerve irritation that is idiopathic or secondary to a structural lesion and MRI with and without contrast should be performed

Front 34

Characteristics of glossopharyngeal neuralgia

Back 34

Severe, lancinating pain the throat and at the base of the tongue that can radiate to the ear; treated and diagnosed with nerve blocks

Front 35

Incidence of idiopathic intracrnial hypertension (pseudotumor cerebri)

Back 35

1-2 per 100,000 worldwide

Front 36

Underlying pathomechanism of IIH?

Back 36

Controversial: the 2 leading hypotehses are increased brain water content or increased resistance to CSF outflow (or both). The mechanism for headache is either cerebral venous distention or meningeal irritation from increased CSF pressure

Front 37

Clinical presentation of IIH (3)

Back 37

1. Acute to subacute onset

2. Constant pain, worse with recumbency and exacerbated by Valsalva maneuvers

3. Commonly retro-ocular pain is reported and transient visual obscurations provoked by postural changes occur in 50% to 75% of patients

Front 38

Criteria for diagnosis of IIH (3)

Back 38

1. Normal MRI

2. Normal CSF findings with pressure of 250 mm H2O or more

3. Typically, bilateral papilledema is considered necessary for diagnosis

Front 39

Treatment of IIH? (4)

Back 39

1. First-line treatment is acetazolamide in doses of up to 4 g daily

2. Therapeutic lumbar punctures can lower the ICP, but repeated taps are not ideal from a patient perspective and are not recommended because patient avoidance may result in visual loss from a lack of treatment

3. Neurosurgical intervention is indicated in rapidly progressive visual loss refractory to medical treatment

4. VP- or LP shunts are effective for reducing CSF pressures, and optic nerve sheath fenestration is used for relief of papilledema and stabilization of visual loss

Front 40

Clinical presentation of giant cell arteritis (5)

Back 40

1. Scalp tenderness

2. Jaw claudication

3. Polymyalgia rheumatica

4. Constitutional symptoms

5. Headache

Front 41

Describe the headache pain in giant cell arteritis (2)

Back 41

1. Usually constant and throbbing with associated scalp and temporal artery tenderness

2. Amaurosis fugax can occur and is a waning of permanent visual loss

Front 42

Complications of giant cell arteritis?

Back 42

1. Visual loss is end stage

2. Ischemic stroke due to involvement of the extracranial cervical vessels

Front 43

How is the diagnosis of GCA made?

Back 43

1. Clinical features, with determination of ESR or CRP (or both), and these should be considered for every elderly patient with new-onset headache

2. Biopsy documenting granulomatous arteritis

Front 44

Treatment of GCA?

Back 44

Corticosteroids, which can be tapered over 6 to 12 months

Front 45

Occurrence of headache with neoplasms?

Back 45

50% of patients with brain tumors have a headache

Front 46

Pathophysiology of headache with brain tumors?

Back 46

Likely increased ICP, impingement of dural structures, hydrocephalus, or a combination of all of these

Front 47

Define Chiari Type I malformation (4)

Back 47

Age dependent tonsillar descent below the level of the foramen magnum:

1. First decade of life, 6 mm below the foramen magnum

2. Second and third decades, 5 mm

3. Fourth through eight decades, 4 mm

4. Ninth decade, 3 mm

Front 48

Underlying pathophysiology leading to head pain in Chiari Type I malformation?

Back 48

Compression of neural tissues and the alteration in CSF dynamics

Front 49

Clinical presentation of Chiari Type I malformation? (3)

Back 49

1. Headache precipitated by Valsalva maneuver or cough

2. Localized occipitally

3. Brainstem compression may cause posterior fossa symptoms, including vertigo, ataxia, nystagmus, or hearing loss

Front 50

Number of patients with OSAP that complain of headache?

Back 50

50%; present upon wakening, and gets better through the day. Polysomnogram aids in the diagnosis of OSAP

Front 51

Most common patient scenario for IIH?

Back 51

Obese woman of childbearing age