Headache-evaluation

Case Discussion:
44 year old woman presents with 5 day history of new-
onset severe headaches culminating in the “most
severe headache” whilst straining on the toilet

Adapted from source

 History of Presenting Complaint
• Sudden onset whilst straining
•Tight, pulsating, diffuse headache
• “Worst headache ever” – Pain 10/10 at onset, currently 8/10
• 10 hours duration so far
•Other symptoms: Nausea and vomiting , Photophobia
• Aggravated by coughing
• Panadol, tramal do little to relieve
•Not affected by movement/posture
•Had 2 similar headaches within the last 5 days (8/10 pain). Both came on at night,
lasted 4 hours before being “slept off”
• No prior history of headaches/migraines
• Has not been able to go to work
• Negatives:
• No sensory, motor or visual symptoms
• No neck stiffness, rash, fever
• No recent infection
• No history of malignancy
• No mental status changes/loss of consciousness
 Other history
Medical: None
•No history of headaches/migraines, malignancy, recent infection
Medications: Mirena (levonorgestrel) (IUD)
Allergies: None known
Surgical: Cholecystectomy (7 yrs ago), C-section (12yrs ago), Mirena implanted (7wks)
Family history:
•Sister – migraines
•Grandmother died of stroke (67yrs)
•Father -leukaemia
Social history;
•Smoker: 25 pack yr history (currently 10/day)
•Alcohol: Drinks 2x/week (8-10 standard drinks/week)
•Divorced, lives in own home with two teenage children – good relationships and
happy
•Works as a medical receptionist
Systems review: NAD
 Physical Examination
Generally:
• Overweight woman , uncomfortable but able to give history without much
distress

Observations:
•BP 145/65, Pulse 98, RR: 17, O2 sats 99% RA
• Afebrile

ENT: NAD - no signs of infection, no facial tenderness

Neurological:
• GCS 15, PEARL (no photophobia)
• Fundoscopy: Bilateral papilloedema
• Remainder of CNS/PNS exam normal
Signs of Meningeal irritation: No neck stiffness, Kernig’s negative
Respiratory: Normal
CV: Normal
GIT: Normal
•No rash
 Headache is either First severe headache or different/severe enough to alarm the patient

History and neurological exam. Beware RED FLAGS:

History
•Sudden, rapid onset Examination:
• Altered mental status • Meningismus
• Occipitonuchal radiation • Toxic appearance or fever
• First severe headache >35 years • Papilloedema
• Prior or co-existent infectious disease • Localising or lateralising abnormalities
• Onset during exertion • Decreased mental status
• Immunosuppression
RED FLAGS PRESENT?
Infection more likely? Intracranial lesion/haemorrhage
more likely?
Possibility of mass lesion ?
No Yes Non-contrast CT head

LP Urgent CT Normal Structural lesion or

bleed
If still concerned - LP

Normal Lesion/bleed found Neurosurgical consult

CSF wbc, no rbc = Opening pressure normal <5wbc, no rbc, normal

probable infection Rbc tube 1,  tube 3 Rbc in all tubes or
glucose & protein xanthochromia
Antibiotics/anti- No xanthochromia = manage as acute
virals = Probable traumatic tap =probable SAH
migraine
Overnight monitoring if
concerned
 Impression?
RED FLAGS PRESENT...
• New headache
•Papilloedema Big Concern?
• Sudden onset SAH
• “Worst headache ever”

Differentials
Severe headache, onset when straining, possible “warning leaks”, risk
• SAH/sentinel headache factors present (smoker, moderate alcohol, age, ? Family history?)
Usually subacute onset, no fever, no recent infection, no signs of
• Meningitis/encephalitis meningeal irritation, no rash
•Raised intracranial pressure headache secondary to:
Prior headaches woke from sleep, but acute
• Space occupying lesion (Tumour, abscess) onset not characteristic, no focal neurology
Onset can be acute, diffuse pounding headache, papilloedema,
•Idiopathic overweight woman BUT must rule out other causes first
• Central venous thrombosis Usually subacute & often accompanied by a combination of
seizures, bilateral neurological findings & altered mental status
Lasts over 4 hours, pulsating, severe enough to disrupt activities,
• Atypical migraine but no prior history

• Medication overuse headache No history of analgesic use

 Investigations
1. CT scan (done already – normal)
2. LP if CT normal
3. Pathology: FBC, U &E, CRP

Results
• Non-contrast CT: NORMAL
- Does this rule out SAH? (Sensitivity within 12 hours approaches 100%, but
cannot rule out minor bleeds)
• Bloods: Normal

• Lumbar Puncture
• Opening pressure 33cmH2 O
• CSF clear in appearance
• Headache relieved
• Three tubes sent for: rbc, wcc, blood, protein, culture, glucose &
xanthochromic index
• Results: All normal (except elevated opening pressure)
 So what does this all mean?
• Negative CT and LP can confidently rule out SAH
• Most likely diagnosis?

IDIOPATHIC INTRACRANIAL HYPERTENSION (aka. Pseudotumour

cerebri, benign intracranial hypertension)
 Idiopathic intracranial hypertension
(Pseudotumour cerebri)
Refers to increased ICP with normal CSF
content, normal neuroimaging, no focal
neurological signs (except CN IV palsy)
and no known cause ? Impaired reabsorption

Epidemiology: X
•Incidence: 0.9/100 000
?Overproduction
• Up to 20/100 000 in overweight X
women of childbearing age
• Most common in overweight women
aged 25-45

Pathopysiology:
IDIOPATHIC...But bottom line = the raised
intracranial pressure is transmitted to
structures within the intracranial cavity
including the optic nerves, which results
in the associated symptoms and
complications
 Presentation and diagnosis
• Severe headache is the most common symptom
• Tends to be throbbing, often diffuse, chronic daily headache, often not
relieved by analgesia, may have N/V associated
• Visual symptoms: Diplopia (CN VI palsy), tunnel vision, blurred vision, reduced
visual acuity, blindness
• Pulsatile tinnitus
• MSK symptoms : Neck/back pain, arthralgia
• Depression, impaired concentration or memory

Examination:
• Bilateral disc oedema
• May detect increased blind spot, sixth nerve palsy
reduced visual fields, increased blind spot
 Diagnosis
1. An awake & alert person
2. Signs & symptoms of raised ICP
3. Normal CSF fluid except for raised ICP (>250mmH2 O)
4. Absence of deformity, displacement & obstruction of ventricular system on
imaging
5. No other cause of increased ICP found

BUT, if this condition presents in people who are not overweight, it is recommended
that secondary causes be ruled out:
• Drugs: Tetracycline, penicillin, retinoic acid, lithium, corticosteroids, levodopa,
cyclosporin, GH, oral contraceptives, levonorgestrel implants , phenytoin
• Systemic conditions: SLE, MS, hypertension, psittacosis, renal disease, prolonged
respiratory disease,
• Disordered cerebral drainage: Central venous compression, cerebral sinus
thrombosis
• Endocrine: Pregnancy
 Management
MEDICAL:
• Weight loss
• Acetazolamide (Diamox) – carbonic anhydrase inhibitor that reduces production
of CSF (500mg bd)
• Referral to ophthalmologist for assessment of visual acuity, colour vision,
appearance of the optic nerve – very important as the main concern with this
condition is progressive optic nerve neuropathy due to oedema which can
ultimately result in blindness if not managed

SURGICAL:
• Optic nerve sheath fenestration (prevents pressure from being transmitted to the
optic nerve)
• CSF diversion – lumboperitoneal or ventriculo-peritoneal shunts (reduces ICP)

Follow Up:
• Regular ophthalmology follow up is required as determined by severity of visual
symptoms and response to therapy – ranges from weekly to 4 monthly.
• This tends to be a chronic condition so follow-up is recommended long term
 SUMMARY
“Thunderclap” headaches – ALWAYS rule out SAH with CT then LP if this is
negative and history is suggestive

Negative CT+LP = confidently rule out SAH

(If CT within 12 hours & LP no earlier than 2 hours)

Remember: IIH is suggested by

An awake & alert person
Signs & symptoms of raised ICP
Normal CSF fluid except for raised ICP (>250mmH2 O)
No mass on neuroimaging
No other cause of increased ICP found

Biggest concern = visual disturbances and ultimately loss of vision

Treatment = weight loss + acetazolamide + regular ophthalmology reviews

 References

Brazis PW, Lee AG: Elevated intracranial pressure and pseudotumor cerebri. Curr Opin
Ophthalmol 1998 Dec; 9(6): 27-32

Corbett JJ: Increased intracranial pressure: idiopathic and otherwise. J

Neuroophthalmol 2004 Jun; 24(2): 103-5

Friedman DI, Jacobson DM: Idiopathic intracranial hypertension. J Neuroophthalmol

2004 Jun; 24(2): 138-45

Miller NR, Newman NJ: Pseudotumor cerebri (benign intracranial hypertension).

In: Walsh and Hoyt's Clinical Neuro-Ophthalmology. Vol 1. 5th ed. 1999: 523-38

International intracranial hypertension foundation: www.ihrfoundation.org

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