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When To Refer…..
JAN LEWIS BRANDES, M. D.
ASSISTANT CLINICAL PROFESSOR, VANDERBILT UNIVERSITY
NEUROSCIENCE/CARDIOLOGY UPDATE ST DOMINIC’S
NEW ORLEANS, LOUISIANA FEB 1 2014
When to refer….

REFER - when diagnosis is uncertain

when appropriate diagnostic studies don’t help

when pattern does not meet diagnostic criteria

when patient is not improving

when co-morbidities interfere

when reasonable medication trials are ineffective
The Tasks of Differential Diagnosis in
Headache
What’s
wrong?
Is it serious?
Is it treatable?
User-Friendly IHS Classification

Two Major Categories

Primary headaches (benign headache disorders)


Migraine (with or without aura)

Tension type headache (episodic or chronic)

Cluster headache

Other benign headache

Post traumatic headache

Drug rebound headache
Secondary headache (headaches that are symptoms of organic disease)
Diagnostic Algorithm for Headache
The Headache History





Age, circumstance,
suddenness of onset
Intensity/character of pain
Duration/frequency
Location(s) and radiation
of pain
Preceding and associated
symptoms








Course
Provoking/aggravating factors
Hormonal influences
Ameliorating factors
History (medical and
medication)
Family history
Social/emotional
Impairment/impact
Physical, Laboratory, and Other
Diagnostic Evaluations




Physical exam
Lab testing*
Neurodiagnostic
tests*

Key Elements

General and neurologic
exam

Sed rate, TSH, hematocrit,
glucose

CT, MRI/MRA, EEG, LP,
arteriogram

Dental, ENT, allergy
evaluation
Other*
*Appropriate for variant or atypical forms
Diagnostic Alarms in Evaluation of
Headache

Headache begins
after age 50
Temporal arteritis,
mass lesion

Sudden-onset
headache
SAH, pituitary
apoplexy, bleed into
mass or AVM, mass
lesion (post. fossa)

Accelerating or
changing pattern
of headache
Mass lesion, subdural
hematoma,
medication overuse
Diagnostic Alarms in Evaluation of
Headache

New-onset headache in
patient with cancer or HIV
Meningitis (chronic or carcinomatous),
brain abscess, metastasis

Headache with systemic
illness (fever, stiff neck,
rash)
Meningitis, encephalitis, Lyme,
systemic infection, collagen vascular
disease

Focal neuro symptoms or
signs of disease (other
than aura)
Mass lesion, AVM, stroke, collagen
vascular (inc. antiphospholipid Ab)

Papilledema
Mass lesion, pseudotumor, meningitis
Sixty-seven Year Old Man with
Cough Headache Benign or Symptomatic?
Ten week history of headache
HA occurs within seconds to minutes of
coughing
Bilateral dull pain, progresses to sharp
within minutes
Normal exam, recent bronchitis Rx with
Abx, now clear CXR
Twenty-seven Year Old Man with
Cough Headache Benign or Symptomatic?
Ten week history of headache
HA occurs within seconds to minutes of
coughing or after weight lifting or
running
Bilateral dull pain, progresses to sharp
within minutes
Normal exam, recent bronchitis Rx with
Abx, now clear CXR
Cough Headache: Benign or
Symptomatic ?

Benign

Mean onset age 55

4x more common in men

Immediate onset within
seconds of coughing, or ^’ed
pressure

Lasts seconds to 30 minutes

Neuro exam - normal

Symptomatic

Mean onset age is lower,
about 39

Multiple precipitants, besides
coughing

Lasts seconds to days

Posterior fossa abnormalities
often causal

Neuro exam – posterior fossa
signs
Sixty-seven Year Old Man with Cough
Headache WHEN to Refer ?
First, treated with indomethacin
 No significant improvement = Rx indomethacin
50mg TID
 Reduced the pain severity, but otherwise no
change in attacks. Next?
 Prophylaxis with nortriptyline

Differential Diagnosis for Benign
Cough Headache










Arnold Chiari Malformation
Basilar Impression from Paget’s Disease
Posterior fossa meningioma
Midbrain cyst
Acoustic neuroma
Neoplasm - malignant
Subdural hematoma
Cerebral aneurysm, carotid stenosis, VB disease
Other – Sarcoid
Exertional HA, effort migraine, coital headache
Sixty-seven Year Old Man with
Cough Headache - (cont)

MR imaging of brain with and
without contrast was normal

Nortriptyline was added to Rx when
chest CT read as normal

Decision to proceed with
bronchoscopy.
Sixty-seven Year Old Man with Cough
Headache

Bronchoscopy revealed non-small
cell lung carcinoma

Chemotherapy initiated, and
cough eventually suppressed with
resolution of headache

Survived ten months after
differential diagnosis of cough
headache pursued
Twenty seven year old man with
cough/exertional headache

Increased hydration before exercise

Indomethacin 50mg thirty minutes before exercise

MRI of brain was normal

Indomethacin withdrawn after 6 months with no return
of symptoms
“Real” Reasons for Testing
To
aid in diagnosis (rule out
comorbid disease)
To establish diagnosis
To eliminate fear
Often Cited Reasons for Testing
Clinical
shortcut
Patient expectations
Financial incentives
Medicolegal issues
Methods of Investigation

Electroencephalography (EEG)

Computerized tomography (CT)

Magnetic resonance imaging (MRI)

Magnetic resonance angiography (MRA)

Cerebral angiography

Lumbar puncture

Thermography

Electromyography (EMG)
Indications for Neuroimaging

The first or worst headache of the patient’s life

A change in frequency, severity or clinical
features

An abnormal neurologic examination

A progressive or new daily persistent headache

An atypical headache that does not meet the
criteria for an established primary headache

Head pain which is always on the same side

Headache unresponsive to routine therapy
Imaging in Headache
In adult patients with recurrent headache,
defined as migraine, including aura,
no recent change in pattern
no history of seizures
no focal neuro signs/symp.
routine imaging studies are not warranted.
American Academy of Neurology guidelines, 1996
Indications for
Electroencephalography

Alteration or loss of consciousness

Suspected encephalopathy

Persistent residual neurologic deficits

Baseline prior to institution of medicines or procedures which
could induce seizure

Transient neurologic symptoms without ensuing headache
Indications for Lumbar Puncture

The first or worst headache of the patient’s life

A severe, rapid onset, recurrent headache

A progressive headache

An atypical chronic intractable headache

Headache with alteration in mental status, fever, meningeal signs
Investigation of Specific Headache
Types
 Migrainous
infarction
 Migraine
with prolonged or
atypical aura
 Basilar
type migraine
 Migraine
aura without headache
WHEN to refer: Repeated attacks

62 year old woman

No hx of migraine – childhood history of severe motion sickness

Feb 2013 : sudden onset of right arm heaviness, slowed speech and arm and
face numbness and tingling – SBP 190

CT/MRI negative

Repeat episodes Late Feb 2013, August 2013, December 2013

All with repeat CT/repeat MRIs/Cerebral arteriogram

Terrified ---- CLUE ---- sequence ---- timing of episodes, all lasting 30 minutes
with right arm spread and resolution, +/- headache

Migrainous infarction with recurrent attacks

Tx stroke and migraine prevention
Migrainous Infarction
vs. “Complicated migraine
Timing is Everything…..
“Clues” - Focal weakness, numbness, visual or speech difficulty.

Magnetic resonance angiography or arteriography

Cardiac echocardiography (esp. TEE)

Carotid duplex scanning, transcranial doppler

EKG or Holter monitoring

Anti-phospholipid Ab, protein S, protein C, other
hypercoagulable labs, factor V/Leiden

Interpret in setting of stroke risk factors
Migraine Aura Without Headache
-
Migraine equivalent
-
Late life migraine accompaniment
-
“Mimics” cerebral thrombosis, embolism, dissection, epilepsy,
coagulopathies, blood dyscrasias
-
“Clues” - Gradual appearance of symptoms
-
over minutes not seconds
- Positive visual symptoms
- Serial progression
- Duration of 15-25 minutes
Headache of Sudden Onset
Primary headache disorders

Crash migraine

Cluster

Benign exertional headache

Benign orgasmic cephalgia
Headache of Sudden Onset
Secondary headache disorders
 Associated with vascular disorders
- Unruptured saccular aneurysm
- Subarachnoid hemorrhage
- Internal carotid artery dissection
- Cerebral venous thrombosis
- Acute hypertension
Headache of Sudden Onset
Secondary headache disorders

Intermittent hydrocephalus

Benign intracranial hypertension

Pituitary apoplexy

Cephalic infection

Acute mountain sickness

Ophthalmic - optic neuritis, glaucoma
In Headache in Clinical Practice, 2009
Epidural Hematoma
Epidural Hematoma
Diagnostic Problem Solving
 Where
is the historical clue?
 Where are the exam clues?
 What study/studies are critical to
management?
Key Clinical Features of Migraine:
Prodrome and Aura

Prodrome

Premonitory phenomena

Occurs hours to days before headache onset

Affects 60% of migraineurs

Symptoms vary widely, but may be consistent per individual

Nonfocal, constitutional symptoms
Key Clinical Features of Migraine:
Prodrome and Aura

Aura

Most often visual(scintillations, scotomas)

Often has hemianoptic distribution

Usually develop and fades within 30 minutes

May reflect wide range of neural deficits

Affects 20% of migraineurs
Key Clinical Features of Migraine:
Headache and Postdrome

Headache

Postdrome/Postict
us
Usually unilateral with throbbing pain
Aggravated by physical activity,
bright light, loud sounds
Onset is gradual, peaks, and then
subsides over a course of 4 to 72
hours
Nausea occurs in up to 90%,
vomiting in ~33%
Pain wanes, leaving tiredness,
irritability, listlessness
48 year woman

8-12 days of headache per month

Sleep

Using > 18 triptans per month

Increase water

Trials of botulinum toxin, topiramate,
nortriptyine, propranolol, amitriptyline,
verapamil, magnesium, gabapentin,
fluoxetine, paroxetine, lamotragine,
metoprolol, valproate, among others

Decrease caffeine

Increase exercise

Longer, higher

Bridging drug for moderate headache

No treatment for mild headache

Injection for severe without narcotics

Longer, higher prevention dosing

Behavioral factors
GENERAL PRINCIPLES OF PREVENTIVE
TREATMENT
Start low and increase dose slowly

Use long-acting formulation if compliance an issue
Adequate trial (2 to 3 months) at an appropriate
dosage
Avoid interfering, overused, and contraindicated
medications
Evaluate therapy
Use headache calendar (diary)
 Attempt to taper and discontinue treatment when
headaches well controlled

When to refer …..when not enough
time or patient needs more support
than you have to give

Set expectations

Keep a DIARY !!

At least 2-3 months, of adequate dose

Encourage behavioral treatments

To reduce, perhaps not completely
stop attacks

Avoid medication overuse

Address hormonal issues

Help with comorbid diagnoses

Support

To make acute medications more
effective

To increase function

Keep out of ER, urgent care
IHS Criteria for Tension Type Headache

Headache lasting from 30 min - 7 days

Headache pain accompanied by two of the following
symptoms:



Pressing/tightening (nonpulsating) quality

Bilateral location

Not aggravated by routine physical activity

Mild or moderate intensity (may inhibit, but not prohibit activities)
Headache pain accompanied by both of the following
symptoms:

No nausea or vomiting

Photophobia and phonophobia absent or only one present
Fewer than 15 day per month with headache
Medication Overuse/rebound
Headache






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Diffuse, bilateral headache every day or nearly
every day
Aggravated by mild physical or mental exertion
Waking with early morning headache
Restlessness, nausea, forgetfulness, asthenia,
depression
Medication withdrawal symptoms when
ergotamine, a barbiturate, or codeine is involved
Tolerance to acute/abortive migraine medication
No response to preventive migraine medication
IHS Criteria for Migraine with Aura
At least 2 attacks, fulfilling…
At least 3 of the following 4 characteristics:

At least one fully reversible aura symptom indicating focal cerebral
cortical and/or brainstem dysfunction

At least one aura symptom developing gradually over 4 minutes, or
at least two symptoms occurring in succession

No aura symptom lasting more than 60 minutes; if more than one
aura symptom is present, expected duration is proportionally
increased

Migraine headache follows aura within 60 minutes; it may also begin
before or simultaneously with aura
Challenges Associated with Migraine
Diagnosis

Clinical diagnosis based on symptoms and “story” as reported
by the patient

No blood test or X-ray can confirm

Symptoms may vary from one attack to another

Migraine may be comorbid with other disorders

Incomplete/lack of information on family history

Patients with migraine may also have tension-type or druginduced headache

Patient evaluation can be time-consuming and laborintensive
Successful Diagnostic Aids

Unstructured account

Onset

Location/duration

Frequency, timing

Quality/severity

Associated features

Precipitating/aggravating/ameliorating factors

Social/Family history

Impact of headache
Barriers to Receiving Effective Treatment
39
46
54
59
Summary

REFER - when diagnosis is uncertain

when appropriate diagnostic studies don’t help

when patterns does not meet diagnostic criteria

when patient is not improving

when co-morbidities interfere

when reasonable medication trials are ineffective
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