New onset severe headache when should I worry?
Dr Marc Randall
Consultant Neurologist and Acute Stroke Physician Leeds Teaching Hospitals NHS Trust Honorary Senior Lecturer University of Leeds The simple answer from me
Dear Mrs Smith
Further to your attendance at hospital today I can confirm there is really nothing seriously wrong with you.
With your history of migraine headache disorder, and no other signs or symptoms other than severe headache I am 98% certain that you do not have an aneurysm, brain tumor or stroke.
This is why I did not waste too much time seeing you as I am sure you can appreciate I am very busy.
You don’t need a scan and I don’t plan on wasting any of the tax payers money, or exposing you to unnecessary radiation just to stop you worrying.
If I had wasted time scanning you on average we are statistically more likely to have discovered an incidental finding rather than any true causative pathology which would have added to your stresses. I realise your headaches are distressing but with the ridiculous waiting time for a follow up to see a neurologist they will probably have settled down in any case.
If have asked your GP to give you some amitriptyline as you looked a bit fed up with your headaches.
Kind regards Why is the patient worried ?
Cerebral Bleed Aneurysm Tumour • Exclude a secondary headache. • Thunderclap • SAH • Positional • Raised intracranial pressure • Malaise What does serious • Meningitis mean to acute • Weight loss clinicians ? • Cancer
• Not one of those = home ? Problem solved
• 1) Worst most severe headache ever.
So why are you here • 2) Lack of progress through other routes. ?
• 3) Non specific symptoms one of them being headache. Age makes a difference : Frequency of pathological diagnosis by age group.
JN Goldstein et al. Cephalalgia 2006;26:684-690
Copyright © by International Headache Society Features linked with pathology (n=15,062)
Feature OR 95% CI Age > 50 7.26 (4.24 – 12.43) Arrival by Ambulance 3.66 (1.97 – 6.77) Sensory disturbance 6.04 (2.47 – 14.74) Speech disturbance 10.54 (3.92 to 28.30) Vision disturbance 3.02 (1.12 to 8.14) Motor disturbance 11.67 (2.50 – 54.49) Systolic BP > 160 2.34 (1.41 to 3.90) Diastolic BP > 100 1.98 (1.12 to 3.51) Neurological weakness 8.46 (2.29 – 31.23)
John W Gilbert et al. Emerg Med J 2012;29:576-581 Final diagnosis documented for headache in US emergency departments.
JN Goldstein et al. Cephalalgia 2006;26:684-690
Copyright © by International Headache Society P SNOOP4 – (4 P’s added 2010) Systemic
• Unexplained fevers • Unexplained weight loss • Known malignancy • Immunosuppressed or HIV
• Pathology • Tumours, Menningitis, Abscess, Arteritis Neurological
• Motor weakness • Sensory loss • Diplopia • Ataxia
• Pathology • Malignant, Inflammatory or Vascular disorders Onset sudden
• Peak intensity in less then 1 minute
• Pathology • Vascular (SAH, Stroke, Carotid Disection, Cerebral vasoconstriction syndrome, Venous thrombosis) Older
• New onset headache over 50 • Change in pattern over age 50
• Pathology • Neoplastic, Inflammatory disorders, Temporal arteritis Pattern Change
• The 4 P’s • Progressive • Malignant, Inflammatory, Vascular • Precipitated • Chiari, Hydrocephalus, Malignant • Postural • Intracranial Hyper / Hypo tension, Cervicogenic • Papilloedema • SOL , IIH, Venous thrombosis
• Secondary headaches are more common in the emergency setting than GP. • Headache of sudden onset Will the red • Secondary cause in 21 – 43 % (less than flags help ? 10 secs) • SAH : 11.3 – 16.3 Well that % • Stroke : 3.6 % depends on • Intra cerebral bleed : 2.2 – 8 % the study. • Meningitis : 2.9 % • Cerebral Oedema : 0.7 % • Venous thrombosis : 0.7 % Some reassurance if none of the Snoop criteria fulfilled • Individuals with a known primary headache disorder, who present with headache.
• CT scan will show as incidentals ! • Cerebrovascular disease 1.1% • Brain tumour 1 % (meningioma in most cases) • Hydrocephalus 0.3% • AVM 0.2% • Subdural Haematoma 0.2% • Aneurysm 0.1%
Frishberg Neurology 1994 44:1191 - 97 Primary care data The big worry = Thunderclap headache
• History has to be right.
• Pathology can be missed if the pre test probability is low.
• Incorrect / Over imaging may be a problem.
• Being able to give a positive diagnosis decreases risks of missing pathology.
• Some primary headache disorders will present as thunderclap if not treated appropriately can be serious. • Follow up is essential • Atypical migraine should not really be diagnosed in A & E R L What presents as a “thunderclap?”
• Vascular disorders • Subarachnoid haemorrhage 10% • Subdural, extradural and intracerebral haemorrhage • Cerebral venous sinus thrombosis • Carotid and vertebral artery dissection • Acute cerebral ischaemia Don’t forget primary • Arterial hypertension headaches can • Pituitary apoplexy “thunderclap” • Reversible cerebral vasoconstriction syndrome • Non-vascular intracranial disorders • Spontaneous intracranial hypotension • Acute obstructive hydrocephalus It may just be primary thunderclap headache
• A. Severe head pain fulfilling criteria B and C
• B. Both of the following characteristics: • sudden onset, reaching maximum intensity in <1 min • lasting from 1 h to 10 days
• C. Does not recur regularly over subsequent weeks or months
• D. Not attributed to another disorder (normal cerebrospinal fluid and normal brain imaging are needed Thunderclap - +ve diagnosis
CT CT + con CT and LP Further SAH CTA / MRA CVT / CTV / MRV Dissection MRA Acute hypertension (PRES) MRI Intra cranial hypo MRI + C Stroke MRI Pituitary Apoplexy MRI 3rd Ventricular Cyst MRI Intra cranial infection RVCS CTA/MRA/Ang Cough / Orgasmic / MRI Peri orbital face pain “not headache.”
39 yr old male neck pain, left eye pain, transient right arm weakness and left sided pupil abnormality 2 weeks later presents chronic headache. But onset was hyperacute. Dissection
• Carotid/vertebral dissection in 50-100% • Headache and facial/neck pain • Usually ipsilateral to dissection. • May have thunderclap onset • Headache is sole presenting feature in 15% • Carotid dissection often associated with ipsilateral Horner’s syndrome with or without signs of cerebral ischaemia. • Vertebral dissection may be accompanied by signs of brainstem or cerebellar ischaemia. Acute onset headache language disturbance.
• 55 yr old male sudden onset dysphasia with head ache no other neurological symptoms, dysphasia recovered within 24 hours. Thunderclap headache – 1st scan normal Then chronic headache “migraines” Angiogram showing vasospasm RVCS (Reversible vasoconstriction syndrome)
• Includes a group of disorders ? up to 9% of thunderclap headache. • Recognized as separate from vasculitis. • Inc. Drug induced vasospasm, cannabis, cocaine, nicotine, nasal decongestant
• 55 % initial plain CT or MRI no signs. • Thunderclap headache usually focal signs
• 81% may develop later lesions. • Ischaemic stroke 39% • SAH 34% • Lobar bleeds 20% • Oedema 38%
Intracranial hypotension
• Up to 14 % of patients with intracranial hypotension present with acute severe headache
• CSF Volume depletion as a result of leakage
• Leak may be • Iatrogenic (post dural puncture, surgery. shunt) • Traumatic (basal skull fracture) • “Spontaneous” spinal dural tear (root sleeve) • - initial trauma may not be recalled Presentation (very variable)
• Classic • Orthostatic • Throbbing or not • Frontal or occipito-nuchal (pulling sensation) • Usually bilateral (although unilateral known) • Can be preceded by hours or days by back , neck or intrascapular pain. • Orthostatic headache initially becomes chronic daily (loss positional element • Exertional headache • Thunderclap headache • Second half of day headaches • Paradoxical othostatic (usually chronic)
Mokri 2004 Neurology Clinics Non headache symptoms have all been described
• Tinnitus / muffled hearing • Neck pain • Positional vomiting • Diplopia (6th) • Facial numbness / weakness • Variable episodes of consciousness • Bulbar / extrapyramidal • Bladder disturbance Acute headache and confusion
69 year old lady recent fall, fractured pubic rami, headache and no comprehension. PRES Papilloedema (elevated pressure) Idiopathic intracranial hypertension
• Not benign • Commoner in young women with high BMI (DDx sleep apnoea) • Presents with: • Headache (92-94%) of raised ICP • Papilloedema • Pulsatile tinnitus (64-87%) • Visual obscuration (may be absent) • Can be precipitated by: Vitamin A, Tetracyclines, OCP • Imaging - exclude sinus thrombosis IIH treatments
CSF examination Treatment: (only for diagnosis) • pressure reduction by LP • Weight loss reduces pain • Acetazolamide / Topiramate • CSF Shunting may be necessary if vision threatened. Shunting no place to treat headache. • Venous stenting !!! 35 year old previous headaches on exertion and CT normal.
Now acute headache and Papilloedema A good history, followed by review of the history, and retaking the history mean you will probably be safe 99% of the time. The main point being Also most of your patients will have a form of migraine so consider longer term support and referral. Prevalence of migraine = 12% in unselected population.
The IHS criteria are very strict in defining of migraine. • 6 classifications A reminder on • 1 Without aura migraine if • 2 With aura • 3 Childhood periodic needed • 4 Retinal migraine • 5 Complicated • 6 Probable • Most of the acute patients we see and label their first severe headache as migraine should really have the diagnosis of probable migraine. To diagnose migraine
• > 5 attacks • At least 2 of the attacks • At least 2 of the following features • Fully reversible • Unilateral • Unilateral • Pulsating • +ve or –ve phenomena • Moderate or severe • Last longer than 5 minutes • Aggravated by action or stimulation • Less than 60 mins (although most of • Nausea the experts accept up to 1.5hrs) • Vomiting • Photophobia • 90 % of all migraine auras are visual • Homonomous • Positive visual phenomena • Zig –zag lines • Bright spots • Scintillations Visual aura in • Hemifield or quadrantic migraine • Can expand to whole field • Central vision usually impaired • Visual aura can in rare cases last longer than hour • Probable migraine with aura • More than a week (extremely rare) • Persistant aura without infarction Migraine aura without headache
• 13% of patients may have visual aura without headache. (Mattsson 1999) • Over 50’s concern recurrent ischaemia (red flag)
• IHS classification • Retinal migraine • ? vasospastic amaurosis fugax • Only really responsive to calcium channel blockers as preventative medications. Early acute therapy almost always beneficial.
Triptans only work for mild and moderate Evidence pain not severe pain. based acute Triptan trials show only early treatment therapy for effective. (only 30% therapeutic response) migraine Your patients are aware of this !
Combined therapy may have best outcome. It’s what the patients know !
n=249 Never (%) Sometimes(%) Always(%)
Take triptan 1st 14 45 41
1st sign of pain 8 51 85
Only bad migraine 97 70 30
Triptan best 50 79 97
Confident works 54 69 82
Opioid needed 31 13 2
Smith et al Headache 2007:755 Abstract Hemiplegic Migraine – A reminder
IHS 1.2.5
• Sporadic • Migraine with aura including motor weakness but no first- or second- degree relative has aura including motor weakness.
IHS 1.2.4
• Familial • Migraine with aura including motor weakness and at least one first- or second-degree relative has migraine aura including motor weakness Diagnostic criteria:
• At least 2 attacks fulfilling criteria B and C • Criteria B • Aura consisting of fully reversible motor weakness and at least one of the following: • fully reversible visual symptoms including positive features (eg, flickering lights, spots or lines) and/or negative features (ie, loss of vision) • fully reversible sensory symptoms including positive features (ie, pins and needles) and/or negative features (ie, numbness) • fully reversible dysphasic speech disturbance
• Criteria C • At least two of the following: • at least one aura symptom develops gradually over ≥5 minutes and/or different aura symptoms occur in succession over ≥5 minutes • each aura symptom lasts ≥5 minutes and <24 hours • headache fulfilling criteria B-D for 1.1 Migraine without aura begins during the aura or follows onset of aura within 60 minutes
• (No / Has a) first- or second-degree relative has attacks fulfilling these criteria A-E
• Not attributed to another disorder
Sporadic cases consideration
• Sporadic cases always • require neuroimaging and other tests to rule out other cause. A lumbar puncture is also necessary to rule out pseudo migraine with temporary neurological symptoms and lymphocytic pleocytosis.
• This condition is more prevalent in males and often associated with transient hemiparesis and aphasia. • 44 year lady
• Referral for second opinion.
Finally a rare but • Seen several colleagues previously serious condition • Recurrent admissions headache • Migraine visual aura • MRI imaging 2008 Brain, Spine MRA all normal
Review history
• Attacks started age 16 • Severe unilateral headaches labeled as “menstrual migraine” • Extreme photophobia • Vomiting • Washed out unable to work 48 hours
• Married at 18 • 2 children (adopted) • Polycystic ovarian syndrome • Multiple ? miscarriage's or phantom pregnancies linked to above.
• Put on fertility drugs PMH • One pregnancy lasted to 24 weeks.
• Unable to tolerate triptans • Extreme vomiting and allergic reaction • When working at well known supermarket • Transiently unable to read screen • Pain in left side of head • Pain in left hand • Numb episodes of left hand lasting Work life for whole days • Left side facial weakness • Random loss of vision • Problems with photophobia (wearing tinted specs) • Diagnosed with hypothyroidism 2008
• Extreme hemiplegic episode • Prolonged sensory loss • Several days • MRI normal • Genetics, Auto immune screen only non specific speckled ANA.
• Gradually tried on various medication • Topiramate some benefit low dose 25mg BD • Still severe headaches despite multiple changes medication. • Using lots of tramadol July 2012
• My first review • History as well documented • Some unusual features for diagnosis hemiplegic migraine • Benefit from Topiramate • Dose increased to 50mg BD • Wean off tramadol Hair loss Oct 2012
• Increasing hair loss • Off tramadol • Possibly related to Topiramate increase. • Wean off and replace with valproate.
• Review again history • Also had DVT age 18 ?
• Anti cardiolipin antibodies repeat and normal Dec 2012 Valproate improvement June 2013
• Headaches stabilized, but SE. • Fewer episodes of transient neurological deficit.
• Hair loss continues • Scalp biopsy by dermatology. • Mild joint ache • ANA remains non specific speckled positive • One positive Ro How do you treat ? Diagnosis ? • Positive Ro • Hemiplegic episodes • Headaches • Joint aches • Hair Loss • Borderline positive repeat anti B2GP1, Clearly Lupus ? negative anticardiolipin
• Rheumatology review • ?? SLE – not complete diagnostic criteria • More fibromyalgic syndrome / chronic connective tissue disease • Membrane stabilising properties • Fewer headaches • Fewer neurological episodes • Well tolerated Acetazolemide • Still having disabling headaches • New episode prolonged hemiparesis • ? Novel treatments
• Scored against baseline headache diary • Daily self injection • Measure response Heparin trial • Continue current meds • Hydroxychloroquine
• Outstanding success • No headache or hemiplegic episodes for 25 of 28 days trial. • Mild symptoms only at period time. • No more mind “fog” • More energy May 2014 • Back to old self
• 4 days after stopping injections back to severe daily headaches, recurrent hemiplegic episodes.
• ?sero negative antiphospholipid British Haem Society 2012
• Clinical criteria • 1. Vascular thrombosis • One or more clinical episodes of arterial, venous or small vessel thrombosis • 2. Pregnancy morbidity • (a) One or more unexplained deaths of a morphologically normal fetus at or beyond the 10th week of gestation • (b) One or more pre-term births of a morphologically normal neonate before the 34th week of gestation because of: (i) eclampsia or severe pre-eclampsia or (ii) recognized features of placental insufficiency • (c) Three or more unexplained consecutive spontaneous miscarriages before the 10th week of gestation, with maternal anatomic or hormonal abnormalities and paternal and maternal chromosomal causes excluded • Laboratory criteria • 1. Lupus anticoagulant (LA) present in plasma, on two or more occasions at least 12 weeks apart • 2. Anticardiolipin (aCL) antibody of immunoglobulin (Ig)G and/or IgM isotype in serum or plasma, present in medium or high titre • (i.e. >40GPL units or MPL units, or > the 99th centile), on two or more occasions, at least 12 weeks apart • 3. Anti-b2–glycoprotein I antibody of IgG and/or IgM isotype in serum or plasma (in titre >the 99th centile), present on two or more occasions at least 12 weeks apart
• Antiphospholipid antibody syndrome (APS) is present if at least one of the clinical criteria and one of the laboratory criteria are me Hughes - Anticoagulation
• Anticoagulation for thrombosis improves headache. Observational study • Hughes G. Antiphospholipid syndrome, migraine and stroke. Lupus. 2010;19:555-556.
APS links Epilepsy Nonvascular dementia Cognitive dysfunction Depression Guillain–Barré syndrome (GBS) Chorea Migraine Transverse myelitis
Don’t forget medication overuse headache
It is a secondary headache But my patient only has short lasting pain
Well that is another talk entirely. Thank you for listening
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