Secondary Headaches

Secondary Headaches•Medication related•Posttraumatic•Disorders of intracranial pressure•Structural•Cranial neuralgias•Vascular•Infectious•Metabolic

SNOOP: review clinical features for organic origin•Systemic symptoms (fever, wt loss) or Secondary risk factors (cancer, HIV)

•Neurological symptoms (confusion, impaired alertness, or consciousness)

•Onset: sudden, abrupt or split-second•Older: new-onset and progressive, esp

middle age (>50 yrs think giant cell, ischemia, cervical disease)

•Previous headache history: first headache or different

Reasons to Consider Neuroimaging in Migraineurs

•Unusual, prolonged or persistent aura• Increasing frequency, severity, or change

in clinical features•First or worst migraine•Basilar •Confusional•Hemiplegic•Late-life migraine accompaniments•Aura without headache•Headaches always on the same side•Posttraumatic

Migraine Aura vs. StrokeFeature Migraine aura Stroke

Onset Slow progression Sudden

Duration 15-60 minutes Often shorter

Distribution Spreads during attack No spread

Visual symptoms Positive—scotoma Negative—visual loss

Sequential symptoms Present (eg. Visual, then sensory)

Absent

History of similar attacks Present Often absent

History of migraine Present Present or absent

Etiologies of HA that can be missed on CT•Vascular disease headaches

▫Saccular aneurysms▫Subarachnoid hemorrhages▫Arteriovenous malformations (esp posterior

fossa)▫Carotid or vertebral artery dissections▫Ischemic stroke▫Cerebral venous sinus thrombosis▫Vasculitis▫Reversible cerebral vasoconstriction syndrome

Etiologies of HA that can be missed on CT•Neoplastic Disease

▫Parenchymal and extra-axial neoplasms▫Meningeal carcinomatosis▫Pituitary tumor and hemorrhage▫Metastatic brain tumors

•Cervicomedullary Lesions▫Chiari malformation▫Foramen magnum meningioma▫Acoustic schwannoma

Etiologies of HA that can be missed on CT• Infections

▫Meningoencephalitis▫Cerebritis and brain abscess

•Other▫CSF leak (intracranial hypotension)▫Intracranial hypertension▫Idiopathic hypertrophic pachymeningitis

Case 1•A 30 yof presents w/ expressive aphasia

and confusion lasting 30 min, after which she developed a severe unilateral HA. H/O migraines w/ sensory aura since age 18, generally premenstrual. Pt is afebrile, neurological examination now normal. MRI/MRA +/- contrast is normal. LP: protein 50 mg/dL, glucose 90 mg/dL, WBC 200 (100 lymphocytes), RBC 0. Cultures, gram stain negative.

•Which of the following diagnoses is most likely?▫A. Cerebral autosomal dominant

arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL)

▫B. Complicated migraine▫C.Headache with neurological deficit and

cerebrospinal fluid lymphocytosis (HaNDL) syndrome

▫D. Myopathy, encephalopathy, lactic acidosis, stroke-like episodes (MELAS)

▫E. Viral meningitis

•C. HaNDL: headache with neurological deficit and CSF lymphocytosis syndrome

•This is an idiopathic benign disorder that may be confused with viral meningitides, by symptoms are transient, afebrile.

•CADASIL and MELAS have MRI abnormalities

•Complicated migraines do not have CSF abnormalities

Severe, acute, “worst headache of my life”

Severe, acute, “worst headache of my life”

Obviously, subarachnoid hemorrhage, but…

Severe, acute, “worst headache of my life”

12-25% of patients with severe, sudden-onset “worst headache of their life” have subarachnoid hemorrhage

Ddx of Acute, severe new-onset headache• Vascular disorders

▫ Ischemic disease▫ Subdural/epidural

hematoma▫ Unruptured/ruptured

aneurysm▫ Systemic lupus

erythematosis▫ Temporal arteritis▫ Arterial dissection▫ Venous thrombosis▫ Hypertension

• Nonvascular disorders▫ Intermittent

hydrocephalus ▫ Benign intracranial

hypertension▫ Post LP ▫ Intrathecal injections▫ Intracranial neoplasm▫ Pituitary apoplexy

Ddx of Acute, severe new-onset headache•Crash migraine•Cluster•Benign exertional headache•Benign orgasmic cephalgia•Posttraumatic

Ddx of Acute, severe new-onset headache

• Acute intoxications• Noncephalic infection

▫ Acute febrile illness▫ Acute pyelonephritis

• Cephalic infection▫ Meningoencephalitis▫ Acute sinusitis

• Acute mountain sickness

• Disorders of eyes▫ Acute optic neuritis▫ Acute glaucoma

• Cervicogenic▫ Greater occipital

neuralgia▫ Cervical myositis▫ Cervical facet and root

syndromes• Trigeminal neuralgia

Facts on Subarachnoid Hemorrhage•> 30,000 people/year have SAH; 18,000

deaths•50% patients with SAH have minimal to

moderate headache•10% of patients have no headache at

onset, 8% have mild gradually increasing headache

•Stiff neck is absent in 36% of patients•10-43% patients have sentinel headache

Probability of Recognizing Aneurysmal SAH on CT scan after initial eventTime after Ictus Probability

Day 0 95%

Day 3 74%

1 week 50%

2 weeks 30%

3 weeks Almost 0%

van Gijn and van Dongen, 1982

CSF

•25 % reduction in RBC between 1st and 3rd tubes can be seen in cases of ruptured aneurysms

•RBCOxyhemoglobindegraded by macrophagesbilirubin by day 3-4

•Oxyhemoglobin can be detected at 2 hours, xanthochromia not present in all cases until after 12 hours

Other causes of Xanthochromia

•Jaundice (total plasma bilirubin > 10 mg/dL)

•CSF protein > 150 mg/dL•Dietary hypercarotenemia•Malignant melanomatosis•PO intake rifampin

Cerebral Venous Thrombosis• Typically thunderclap• Moderate to severe,

throbbing• Nausea/vomiting

photophobia, phonophobia in 59%

• CT dense triangle• False positives in children

or hemoconcentration

Case 2

•25 yo chronic HA. She began to experience b/l, pressurelike headaches about 6 months before; initially, relieved by over-the-counter ibuprofen taken in high doses. HA were bitemporal, at base of skull, occ nausea, no vomiting occur 5 days/wk. PMHx amenorrhea. On PE: BP: 140/70, p 80, R 15, T 37, wt 120kg, h 160 cm. Visual fields nl, no papilledema, no spontaneous venous pulsations noted.

•Which studies is most likely to establish a diagnosis in this patient?▫A. Cervical MRA▫B. Cervical MRI▫C. CT of the sinuses▫D. Magnetic resonance venography (MRV)▫E. Serum prolactin level

•D. Magnetic resonance venography (MRV)

•Syndrome of increased intracranial pressure and can be confirmed by LP after CT performed to r/o mass lesion

Reasons to consider LP to evaluate headache

Presentation Possible Cerebrospinal Fluid Diagnosis

First or worst headache Subarachnoid hemorrhage or meningitis

Headache w/fever or other symptoms or signs suggesting infection

Meningitis or encephalitis

Subacute or progressive headache in patients with risk factors

-Cryptococcal meningitis in HIV patients-Leptomeningeal metastasis with h/o primary cancer-Pseudotumor cerebri in obese female without papilledema

Orthostatic headache with diffuse dural enhancement on MRI

Low cerebrospinal fluid pressure syndrome

Spontaneous Intracranial Hypotension Syndrome•Usu presents when a patient is upright,

relieved when lying down•When spontaneous intracranial

hypotension syndrome persists chronic daily headache without orthostatic features

•Neck or interscapular pain may precede the onset of headaches “hanger sign”

•MRI may show gadolinium enhancement, cerebellar tonsil descent, or may be normal

•LP: opening pressure 0-70 cm H2O, may be negative (normal 5-20 cm H20 or 3-15 mm Hg)

•CT myelography may be more sensitive for determining site of CSF leak▫Most leaks occur in spine

Case 3

•62 yo f, acute onset of headaches 2 years earlier, at that time she awakened in the morning with severe headache. No h/o headaches. Upon standing up, pain intensified, but would improve upon reclining. Within one week postural component dissipated, but HA persisted 24 hours a day. Examinations reportedly normal. LP no blood or xanthochromia. Pressures not recorded.

•Variety of analgesics and treatments tried without benefit.

•Pmhx: negative except Morton’s neuroma removal 2 weeks prior to onset of headache.

•Neuroimaging normal; labs normal•Pt hospitalized, did not respond to

standardized IV protocols.•LP: opening pressure 10 cm H20.

Following removal of 10 cc CSF, pressure 7 cm H20

•IV caffeine produced modest improvement.•Autologous injection of 20 cc blood injected at

L2-L3 with complete reduction of HA•Three months later, headaches returned,

second administration of autologous blood undertaken

•Complete resolution of headaches 3 years f/u.•Upon further questioning, pt acknowledged

Morton’s neuromal removal had been preceded by spinal anesthesia: speculated CSF leak occurred at this time.

Arterial Dissections

•60-95% cervical artery dissections associated with headache▫25% also have neck pain, usu resolved after

1 week•70% vertebral artery dissections

associated with headache▫50% have neck pain, can last up to 5 weeks

•Ipsilateral pain

Case 4

•35 yof with severe daily headaches for 1 year. H/O poorly controlled Type I diabetes, treated with insulin pump and has early retinal changes. Renal function is normal, but she has had a recent episode of chest pain for which she is undergoing cardiac testing. She has been overusing triptans and has developed medication overuse headache.

•Which of the following bridging therapies is indicated in this patient to treat her medication overuse headaches?▫A. Dihydroergotamine (DHE) 45▫B. Nonsteroidal anti-inflammatory drugs▫C. Steroids▫D. Topiramate▫E. Triptans

•B. Nonsteroidal snti-inflammatory drugs (NSAIDS)

•DHE undesirable for ischemic heart disease

•Steroids: brittle diabetic, however good bridging therapy for non-diabetics

•Topiramate: preventative agent, not bridging agent

Case 5•70 yom, h/o CAD, HTN, DM, experienced an

episode of right amaurosis fugax, found to have 90% stenosis of right internal carotid artery. He undergoes right carotid endarterectomy, uneventful. At 1 week post-op he complains of severe, pulsating, right-sided headache. He also had a brief 1 hour episode of difficulty coordinating movements on the left, which he attributed to fatigue. Sent for head CT: normal. No bruits. Fundi show only arteriolar narrowing.

•What is the most likely source of the patient’s headache?▫A. Carotid intimal tear▫B. Giant cell arteritis▫C. Hyperperfusion syndrome▫D. Ipsilateral ischemia▫E. Small intracranial bleed

•C. Hyperperfusion syndrome

•This is presumed to be due to reperfusion of a hemisphere with an increased volume of blood at higher velocity compared to previously stenosed vessel.

•Although he has focal neurologic symptoms, ipsilateral ischemia is not felt to be the case

•Giant cell arteritis is unrelated to current history

Conclusions

•R/O secondary causes of headache•Obtain more history•SNOOP•Systemic symptoms or Secondary risk

factors•Neurological symptoms•Onset: sudden, abrupt or split-second•Older: new-onset and progressive•Previous headache history: first headache

or different

Headache Education Seminar

•Multidisciplinary seminar•Patients can be referred prior to

neurology evaluation•2nd Monday of every month, America

Building