Secondary Headaches - cdn.ymaws.com fileOutline •Thunderclap headache •Abnormalities of ICP...
Transcript of Secondary Headaches - cdn.ymaws.com fileOutline •Thunderclap headache •Abnormalities of ICP...
Secondary Headaches
Rashmi Halker MD FAHS Mayo Clinic Phoenix, AZ
[email protected] Twitter: @rashmihalker
Disclosures
• No financial disclosures • Many medications used in the treatment of
headache disorders are off label
Outline
• Thunderclap headache • Abnormalities of ICP • Medication overuse headache • Other secondary headaches that may be
tested
Thunderclap Headache
(Primary) Thunderclap Headache: ICHD-3
• A. Severe head pain fulfilling criteria B and C • B. Abrupt onset, reaching maximum intensity
in <1 min • C. Lasting for ≥5 min • (D. Not better accounted for by another ICHD-
3 diagnosis)
Thunderclap Headache Differential Diagnosis
• Subarachnoid hemorrhage • RCVS • CVST • Cervical artery dissection • Acute hypertensive crisis • Ischemic stroke • Pituitary apoplexy • Cardiac Cephalgia • Giant Cell Arteritis
• Idiopathic TCH • Sexual activity • Exertion • Cough
• CSF Leak • Lesion (colloid cyst) • Meningitis
RCVS = reversible cerebral vasoconstriction syndrome CVST = cerebral venous sinus thrombosis
Subarachnoid Hemorrhage (SAH) • 90% present with headache
• In ED: account for 25% of “worst headache” presentations with abnormal examinations
– In 18%, headache is absent, mild, or gradual in onset, or overshadowed by other symptoms
• Aneurysmal SAH = 80% of non-traumatic SAH
• 1 month case-fatality rate = 30-40%
• Accounts for 3% of stroke but 5% of all stroke deaths
• Survivors:
– Functionally dependent = 10-20%
– Reduced quality of life = 60%
Polmear A. Cephalalgia 2003
Linn FH et al. Lancet 1994;344:590-593 Morgenstern LB et al. Ann Emerg Med 1998;32:297-304
Edlow JA, et al. J Emerg Med 2008;34:237–251
Aneurysmal SAH: Risk Factors for Aneurysm Rupture
Patient-specific • Cigarette smoking • Hypertension • Race/Ethnicity
– African race (2.1xbaseline risk)
– Japanese, Finnish descent
• Family history • F > M (1.6:1) • Age > 60
Aneurysm-specific • Size: > 5 mm AC, > 3 mm PC • Posterior circulation • Multiple aneurysms • Multiple lobes (daughter
sacs) • Growth during follow up
imaging
Wermer MJH et al. Stroke. 2007;38:1404-1410 Weibers DO, et al. Lancet 2003, 362:103–110.
Diagnosis of Aneurysmal SAH Sensitivity of CT
Sensitivity Specificity Negative predictive
value
Positive predictive
value CT < 6h 100% 100% 100% 100%
CT overall 93% 100% 99.5% 100%
Perry JJ, et al. BMJ 2011;343:d4277 doi: 10.1136/bmj.d4277
Bottom line: Modern 3rd generation CT is extremely sensitive in identifying subarachnoid hemorrhage when it is carried out within six hours of headache onset and interpreted by a qualified radiologist.
CSF XANTHOCHROMIA AFTER SAH
Perry JJ, et al. Ann Emerg Med. 2008;51:707-713. Vermulen M et al. J Neurol Neurosurg Psychiatry. 1989; 52(7): 826–828.
71 4 WEEKS
90 3 WEEKS
100 2 WEEKS
100 1 WEEK
100 12 HOURS
PROBABILITY (%)
TIME AFTER HEMORRHAGE
A negative CT and negative CSF is sufficient to rule out SAH.
Cerebral Venous Sinus Thrombosis (CVST)
• Thunderclap HA in 10% (Headache 75%)
• CT normal in 25%
• CSF: normal in 70% (lymphocytosis, rbcs, and/or increased protein in 30%)
• Up to 40% may have elevated CSF opening pressure alone
• If CVST suspected, MRI/MRV study of choice
• Tx: Anticoagulation
Filipidis A et al. Neurosurg Focus 2009; 27(5):E3.
CVST Risk Factors
Transient • Infections
– CNS (empyema, meningitis) – ENT, Face/neck (otitis,
mastoiditis, sinusitis, etc) • Pregnancy & puerperium • Physical precipitants
– Head trauma – Jugular/subclavian catheters
• Prothrombotic drugs – OCPs, HRT, Lithium, tamoxifen,
etc • Dehydration • Diabetic ketoacidosis
Permanent • Genetic/acquired prothrombotic
conditions – Protein C, S, antithrombin
deficiencies, factor V Leiden, prothrombin mutations
– APLS, nephrotic syndrome, congenital heart disease
• Malignancy • Dural fistula • Inflammatory diseases
– Behcet’s, SLE, Sjogren’s, Wegener’s, GCA, Sarcoidosis, IBD
• Thyroid disease (hyper & hypothyroidism)
Ferro JM & Canhao P. Curr Cardiol Rep 2014;16:523.
Arterial Dissection
• Thunderclap HA 13% (Headache 75%)
• CT/CSF usually normal
• MRI/MRA diagnostic procedures of choice
• Tx: remains controversial, Anticoagulation vs antiplatelet therapy
Jensen MD et al. Neurologist 2008;14(1):5-8. Redecop GJ. Can J Neurol Sci 2008;35(2):146-52.
Pituitary Apoplexy • Acute clinical syndrome: headache, vision loss,
ophthalmoplegia, altered MS, and variable endocrine abnormalities
• Hemorrhage or infarction of pituitary gland • Pre-existing pituitary (macro)adenoma is common • Clinical risk factors:
– trauma, increased ICP, postpartum (Sheehan syndrome), anticoagulation, bromocriptine therapy, diabetic ketoacidosis, conventional angiography, radiation therapy, and open heart surgery
• Treatment: surgical decompression or steroids • Complications: coma, death (over weeks)
Rogga JM. AJNR 2002 23: 1240-1245.
Pituitary Apoplexy MRI • Imaging features:
– If acute, T1 iso or hyperintense
– If acute, T2 hypointense enlarged pituitary gland
– T2 blooming artifact if hemorrhage
– Restricted diffusion may be early sign of hemorrhage
– Rim enhancement common – May see adjacent dural
thickening and mucosal thickening of sphenoid sinus
Images: http://emedicine.medscape.com/article/1198279-overview
Reversible Cerebral Vasoconstriction Syndrome
(RCVS) • Most commonly precipitated
post-partum or with the use of vasoactive substances – Cannabis – Cocaine – SSRIs – Binge drinking – Nasal decongestants
• “Strings & beads” on imaging • May result in:
– PRES – SAH – ICH – Ischemic stroke
• Treated with IV/PO calcium channel blockers
Ducros et al. Brain 2007; 130:3091-3101
Thunderclap Headache Summary
• Ask about mode of onset of headache, especially for patients presenting with “a headache” or patients in the ED
• Know the differential diagnosis of TCH
• All patients should be aggressively evaluated with CT, CSF, and if negative, MRI/MRA/MRV
Pseudotumor Cerebri Syndrome (Idiopathic Intracranial Hypertension)
A. Papilledema B. Normal neurological examination except cranial nerve
abnormalities C. Normal gad-enhanced brain MRI (female/obese) and MRV
(atypical) [ contrast enhanced CT if contraindication for MRI] D. Normal CSF composition E. Elevated opening CSF pressure (>250 mm CSF adults; >280
mm CSF children; >250 mm CSF children if not sedated or obese)
Freidman DI, et al. Neurology 2013;81:1159-1165 Friedman DI, et al. Neurology 2013;81:1159-1165
PTCS without papilledema (B-E plus VI nerve palsy) PTCS without papilledema or VI nerve palsy (B-E plus 3 of the following)
– Empty sella (65%) – Flattening posterior globe – Distention perioptic subarachnoid space + tortuous optic nerve – Transverse venous sinus stenosis
• PTCS without papilledema is uncommon!
Freidman DI, et al. Neurology 2013;81:1159-1165
Friedman DI, et al. Neurology 2013;81:1159-1165
Flattening posterior globe
Distention perioptic subarachnoid space, tortuous optic nerve
Empty sella Transverse venous sinus stenosis
PCTS: Symptoms
• Headache: 94% – Usually daily, bilateral, retro-ocular, may be most
severe in AM – Often migraine phenotype
• Transient visual obscurations: 75% • Intracranial noises: 60% • Diplopia: 38% • Sustained visual loss: 30%
Wall et al. JAMA Neurol 2014.
PTCS: MRV for All?
• Yes, when possible • Definitely for:
– “Atypical” patients, including non-obese women, men, children
– Patients not responding to treatment should be thoroughly evaluated for a possible underlying cause • Contrast-enhanced MR imaging and MRV • CSF cytology (repeat CSF analysis if needed)
Friedman DI, et al. Neurology 2013;81:1159-1165
Secondary PTCS: Risk Factors • Cerebral venous
abnormalities, CVST • Hypercoaguable states • Anemia • Vitamin A and retinoids
– Hypervitaminosis A, isotretinion, excessive liver ingestion, all trans retinoic acid for polymyelocytic leukemia
• Antibiotics – Tetracycline, minocycline,
doxycycline, sulfa drugs, nalidixic acid
• Withdrawal from chronic corticosteroids
• Hormones – HGH, thyroxine (in children),
anabolic steroids, leuprorelin acetate, levonorgestrel
• Lithium • Chlordecone • Encocrine disorders
– Addison, hyperPTH • Hypercapnea
– OSA, Pickwickian syndrome • Renal failure • Turner Syndrome • Down Syndrome
Friedman DI, et al. Neurology 2013;81:1159-1165
General Principles of Management
• Not all patients need to be treated • Goal of treatment: preserve vision • Decisions dictated by status of vision • Document field & disc appearance • Team approach - neurologist and ophthalmologist
Medical Treatment
• Eliminate contributing/causative factors – Tetracycline, vitamin A
• Low sodium diet – 1,500-2,400mg/day; 1tsp=2,300 mg
• Weight loss > 10% body weight
– Bariatric surgery (morbid obesity)
• ICP management
• Headache management
Headache Management: Similar to Chronic Primary Headache Disorder
Symptomatic • Triptans • DHE-45 • NSAIDs (ie – Indomethacin)
Preventive • Topiramate • ARBs • Protriptyline • SNRI • Indomethacin • OnabotulinumtoxinA
Avoid: Valproate, steroids, and TCAs can cause weight gain; opioids; β and Ca++ CBs may reduce perfusion to optic nerve head
Role of Steroids
• Work initially, but not recommended for chronic treatment – Weight gain, fluid retention – Withdrawal associated with PTCS – Raise intraocular pressure – Serious side effects with long-term use
• One role: to “buy some time” while arranging for surgical procedure, etc.
Diuretics
• Acetazolamide (0.5-2g bid with meals) – CSF flow not affected until 99.5% of CA inhibited (may take
up to 4g); Serum HCO3 measure of compliance – Renal stones ~3% – Aplastic anemia 1/15000 patient treatment years and
almost always within first 6 months of therapy • Furosemide 20mg bid; up to 40mg tid (K+ supplementation
required) • Triamterene • Spironolactone: Aldosterone antagonist • Be careful if combining diuretics!
Surgical Treatment
Indications – Progressive visual loss despite maximally
tolerated medical therapy – Severe visual loss at presentation – Severe visual loss and unreliable follow-up
Procedures: Optic nerve sheath fenestration Lumbo-peritoneal shunt Ventriculo-peritoneal shunt
Intracranial Hypotension
Spontaneous Intracranial Hypotension (SIH): ICHD-3
• 7.2.3 Headache attributed to SIH A. Any headache fulfilling criterion C B. Low CSF pressure (<60 mm CSF) and/or evidence
of CSF leakage on imaging C. Headache has developed in temporal relation to
the low CSF pressure or CSF leakage, or has led to its discovery
D. Not better accounted for by another ICHD-3 diagnosis
Intracranial Hypotension: MRI findings
Intracranial Hypotension: MRI findings
radiopaedia.org Venous distention sign
Intracranial Hypotension: Imaging Findings
• CT head – Subdural collection – Cerebellar tonsillar herniation
into foramen magnum (acquired Chiari I malformation)
– Dural venous sinus distention
• MRI brain with contrast – Venous sinus engorgement – Pachymeningeal
enhancement (infra- and supra tentorial)
– Pituitary gland enlargement
• MRI brain without contrast – Diffuse brain swelling – Sagging brainstem – Subdural effusions – Increased fluid around optic
nerves – Bulbous dilatation of the
sheath behind globes – Rounding of cross section of
venous sinuses
Medication Overuse Headache
8.2 Medication-overuse headache (MOH)
A. Headache occurring on ≥15 d/mo in a patient with a pre-
existing headache disorder B. Regular overuse for >3 mo of one or more drugs that can be
taken for acute and/or symptomatic treatment of headache C. Not better accounted for by another ICHD-3 diagnosis
8.2 Medication-overuse headache (MOH)
8.2.1 Ergotamine-overuse headache (>10 days/month) 8.2.2 Triptan-overuse headache (>10 days/month) 8.2.3 Simple analgesic-overuse headache (>15 days/month) 8.2.4 Opioid-overuse headache (>10 days/month) 8.2.5 Combination -analgesic-overuse headache (>10 days/month)
Note: “Combination-analgesic” is used specifically for formulations combining drugs of two or more classes, each with analgesic effect or acting as adjuvants
8.2.6 MOH attributed to multiple drug classes not individually overused (>10 days/month)
8.2.7 MOH headache attributed to unverified overuse of multiple drug classes (>10 days/month)
8.2.8 MOH attributed to other medication (>10 days/month)
Other Testable Tidbits
8.1.2 Phosphodiesterase (PDE) inhibitor-induced headache
A. Any headache fulfilling criterion C B. A single dose of a phosphodiesterase (PDE) inhibitor has been
taken C. Evidence of causation demonstrated by all of the D. following:
1. Headache has developed within 5 hours of intake of the PDE inhibitor
2. Headache has resolved within 72 hours of onset 3. Headache has at least one of the following four characteristics:
a. Bilateral b. Mild to moderate intensity c. Pulsating quality d. Aggravated by physical activity
E. Not better accounted for by another ICHD-3 diagnosis.
11.3.1 Headache attributed to acute glaucoma
A. Any headache fulfilling criterion C B. Acute narrow-angle glaucoma diagnosed C. Evidence of causation demonstrated by ≥2 of: 1. headache has developed in temporal relation to onset of
glaucoma 2. headache has significantly worsened in parallel with
progression of glaucoma 3. headache has significantly improved or resolved in
parallel with improvement in or resolution of glaucoma 4. pain location includes affected eye D. Not better accounted for by another ICHD-3 diagnosis
Headache due to subacute angle-closure glaucoma
• Intermittent episodes of transient elevations of intraocular pressure
• Headache often the only presenting complaint – Blurred vision, eye pain, redness – 1/3 describe seeing halos
• 2/3rds of patients misdiagnosed with migraine – Later age of onset (per one study, avg age 50 years) – Most attacks last <1 hour
• Diagnosed by gonioscopy (narrow, occludable angles) • Treatment: laser peripheral iridotomy (LPI)
Schindler et al, Neurology 2005; 65(5):757-758.
Secondary Headaches SNOOP4 Red Flags
• Systemic symptoms (fever, weight loss) or Systemic disease (malignancy)
• Neurologic symptoms or signs • Onset sudden (acute or thunderclap
headache) • Onset after age 40 years • Previous headache history (new or different) • Progressive • Precipitation by Valsalva (cough, bend) • Postural • Pregnancy
Dodick DW. Adv. Stud Med 2003;3:550-555
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