ASSOCIATION OF BRITISH NEUROLOGISTSACUTE NEUROLOGY BOOT CAMP 2020

8th June 2020

Dr Anita Krishnan

The Walton Centre NHS Foundation Trust

HEADACHES

Headaches

Dr Anita KrishnanConsultant NeurologistThe Walton Centre NHS Foundation Trust Liverpool

The spectrum of headache(chart shows most frequently occurring only )

Headache

Primary

Migraine Trigeminal Autonomic

Cephalalgia (TAC)

ClusterParoxysmal Hemicrania SUNCT/ SUNA

Neuralgia

Secondary

Global burden of headache

• Worldwide – 43% of the adult population has headache at least once per year – 3- 17% of the adult population suffer with frequent or chronic headaches , usually

migraine , at any given point in time

• England *– 6 million with migraine ( 10% with chronic migraine; 20% have already attended an

emergency department ) – 150,000 with cluster headaches ( 10% with chronic cluster ) – 75,000 with trigeminal neuralgia– Costs the NHS 1 billion/year– the UK economy loses 5-7 billion per year from loss of productivity from headache

disorders

• Neurology outpatients – 33 % of referrals are for headache

• *Headache Services in England- A Report of the All-Party Parliamentary Group on Primary Headache Disorders 2014

Secondary headache

▪ < 1% in a clinic setting or primary care

▪ 3- 13% of headaches presenting to A&E

▪ For NEW ACUTE SEVERE THUNDERCLAP HEADACHE

– 20-43% have a sec cause

– 2-15 % turn out to be SAH

▪ LOOK FOR RED FLAGS

A Bahra ACNR online journal July 2013

Secondary Headache

Thunderclap headache

CNS infectionRaised

intracranial pressure

Elderly

Known malignancy

Immunocompromised

Anticoagulated

New persistent neurol deficit

Low CSF pressure

Temporal arteritis

Glaucoma

Urgent Assessment in hospital

CT BRAIN with contrast----Discuss----Lumbar Puncture --- Discuss----Bloods – ESR, CRPOphthalmology assessmentIF INVESTIGATIONS ARE NORMAL- REASSESS THE PATIENTIF RED FLAGS STILL PERSIST- FURTHER OPINION/ INVESTIGATIONSIf no further red flags- diagnose a primary headache disorder

http://www.thewaltoncentre.nhs.uk/uploadedfiles/leaflets/Migraine%20-%20A%20Comprehensive%20Guide.pdf

Thunderclap headache

Thunderclap headache▪ New sudden onset headache , severe right from onset

– New – Ask about the onset and build up– Asso symptoms like syncope and vomiting at onset are

relevant– Neurological signs – ophthalmoplegia , true neurological

weakness– Location of pain may not be relevant- around the eye,

back of the neck

▪ Following statements may not be relevant– ‘’sudden onset headache’’ Ø– ‘’ worst headache you’ve ever had ‘’ Ø– ‘’ different to your usual migraine ‘’Ø– ‘’like being hit by a cricket bat / base ball bat’’ Ø

▪

▪ Subarachnoid haemorrhage

▪ Subarachnoid haemorrhage

▪ Sub arachnoid haemorrhage

▪ Other intracranial hhge incl pituitary apoplexy

▪ Dissection of cerebral blood vessels

▪ Cerebral Vasulitides

▪ Reversible cerebral vasoconstriction syndromes

▪ Cerebral venous sinus thrombosis

▪ Idiopathic

Thunderclap headache

▪ For NEW ACUTE SEVERE THUNDERCLAP HEADACHE– 20-43% a sec cause

– 2-15 % SAH

▪ Age older than 50, onset during exertion and loss of consciousness at onset were positive predictive factors

▪ A CT scan within 6 hours and interpreted by an expert can be a sensitive test

▪ But as things stand ,a CT and an appropriately timed LP is the recommended tool to look for SAH ( negative patients were followed up – 6 months to 3 year data – and none had SAH detected in that time )

Thunderclap headache

▪ If the story is very good for thunderclap headache and

▪ If the patient is deteriorating neurologically –or-

▪ If there are persistent neurological signs

….EVEN IF INITIAL CT / LP ARE NORMAL –EXAMINE &INVESTIGATE FURTHER

Review initial scans with neuro radiologist

Consider MR angiogram + venogram / CT angiogram + venogram

Carotid and vertebral artery dissection

Young stroke- 25% 100% have headache, 40% describe neck pain

Horner’s syndrome- 40% with a carotid artery dissection, will develop a partial Horner’s syndrome

Focal neurology- stuttering pattern Trivial to severe head and neck trauma reported

Can affect -anterior circulation – carotids (extra cranial or intracranial) – can even be bilateral- posterior circulation – vertebrals (usually extracranial). If intracranial can be associated with subarachnoid blood

Cerebral CT angiogram or MR angiogram

Cerebral vasculitis/ primary CNS angiitis

▪ Unwell patients with significant impairment in cognition, worsening consciousness and neurologic deficits with ongoing headache

▪ Abnormal CT/ MR

▪ MRA shows’ beading ‘ constriction and dilatation of vessels

▪ Abnormal CSF - inflammatory cells

▪ Raised inflammatory markers

▪ Rapid deterioration warranting steroids and immunospuppression

▪ A brain biopsy may be required for diagnosis

▪ Vasculitis may also be secondary to other systemic vasculitides

Reversible cerebral vasoconstriction syndromes (Call Fleming syndrome)

▪ Characterised by reversible segmental cerebral vasoconstriction

▪ Drugs esp recreational drugs

▪ Infections

▪ Idiopathic

– Thunderclap headache

– Focal neurological deficits

– Normal CSF ( distinguishing it from cerebral vasculitis )

– CT brain or MR brain May have infarcts, intracerebral hhge or unusually located SAH on scans

– CTa, MRa Multiple, short areas of vasoconstriction on angiogram

CNS infection

CNS infection

▪ Raised temperature

▪ Neck stiffness

▪ Seizures

▪ Alteration in consciousness

▪ Start antibiotics/ antivirals

▪ CT Brain

▪ DO NOT delay an LP if no contraindications

▪ MR brain in encephalitis – temporal lobe changes / hhge

▪ Meningitis- bacterial , viral

▪ Encephalitis- seizures , alteration in consciousness

▪ Cerebral Abscess

▪ Subdural empyema

▪ Paraspinal collections

▪ Unusual organisms

▪ Systemic infection can also cause associated headache

When the patient with ‘encephalitis’ and a lymphocytic CSF is not getting better… think of …

Autoimmune encephalitides

▪ Voltage gated potassium channel antibodies (faciobrachial dystonic seizures and hyponatremia in LGI1 )

▪ NMDA receptor antibodies (Movement disorders )

▪ Anti GAD antibodies

▪ Paraneoplastic antibodies (Subacute – confusion, cognitive disturbance)

▪ CSF – cellular, bands can be positive

▪ Negative for virology

▪ MRI brain – temporal lobe high signal or even normal

▪ Suspect in a patient with encephalitis who is not getting better with aciclovir

▪ Requires steroids and immunotherapy

Cerebral vasculitis Acute disseminated encephalomyelitis

▪ Cerebral Vasculitis

▪ Unwell patients with significant impairment in cognition, consciousness and neurologic deficits with ongoing headache

▪ Abnormal MRI brain + MRA

▪ MRA shows’ beading ‘ constriction and dilatation of vessels

▪ Abnormal CSF - inflammatory cells

▪ Raised inflammatory markers

▪ Rapid deterioration warranting steroids and immunosuppression

▪ ADEM

▪ Acute demyelination of white matter

▪ Brain and spinal cord

▪ Following on from an infection

▪ Cognitive and focal impairment

▪ Large fluffy lesions on MRI brain

Raised CSF pressure –Headaches AND papilloedema / loss of visual acuity /new 6th or 4th nerve palsy

Raised CSF pressure

▪ Headaches waking a patient from sleep

▪ Brought on by Valsalva

▪ Visual obscurations- maybe accompanied by pulsatile tinnitus

▪ Papilloedema

▪ Diplopia from a 4th or 6th nerve palsy

▪ The feeling of tightness / pressure described by patients is not in itself indicative of raised pressure

▪ Tumours

▪ Cerebral venous sinus thrombosis

▪ Malignant meningitis

▪ Significant Chiari malformation

▪ IIH-– diagnosis of exclusion in the

appropriate population of patients– Papilloedema– Ophthalmology assessment with fields – Normal CT or MR brain and venogram– LP - CSF opening pressure measured

in the relaxed decubitus position is > 25 cm

– CSF analysis normal

Papilloedema

▪ Papilloedema is optic disc swelling that is secondary to elevated intracranial pressure

▪ New papilloedema demands urgent investigations

▪ In contrast to other causes of optic disc swelling, vision is usually preserved with acute papilloedema but deteriorates with persistent or worsening swelling

▪ Examine and record findings –If unsure, get a senior review

▪ Covid – 19 restrictions in examining for papilloedema

▪ Please check-– Visual acuity– Colour vision– Optic discs– Eye movements– Pupils– Visual fields– Blood pressure and weight

The Scan when suspecting raised pressure

▪ CT brain+ contrast and venogram

OR

▪ MRI brain and venogram

▪ Accepted findings in IIH– Venous sinus stenosis

– Empty sella

– Slit like ventricles

– Flattened posterior globe

– Optic nerve sheath distension

Radiological findings in IIH

PT ATTENDING OPTICIAN/ MEDICAL UNIT WITH

HEADACHE AND DETECTED TO HAVE PAPILLOEDEMA

OPHTHALMOLOGY ASSESSMENT TO CONFIRM PAPILLOEDEMA + ACUITY AND FIELDS

CT BRAIN with CONTRAST OR CT VENOGRAM TO BE DONE AT ASSESSING HOSPITAL

ABNORMAL

URGENT DISCUSSION/ TRANSFER TO

NEUROSCIENCE CENTRE

NORMAL/ VENOUS STENOSES

LP- PRESSURE AND ANALYSIS

RAISED PRESSURE

IIH(young patients, normal

CSF analysis)

NOT RAISED

Normal analysis

GO THROUGH HISTORY AND

OPHTHAL AGAIN. IF VISION

AFFECTED , THINK OF OPTIC

NEUROPATHIES

Within 24 hours,

sooner if vision is

deteriorating

Within 24 hours,

sooner if vision is

deteriorating

INFECTIVEIINFILTRATIVEINFLAMMTORY( abnormal CSF)

IIH

VISION STABLE IIH

eg: ENLARGED BLIND SPOT with preserved

acuity

ADVICE ON WEIGHT LOSS

ACETAZOLAMIDE,/ TOPIRAMATE

REVIEW OPHTHAL IN 4 Wks,MONITOR

WEIGHT LOSS, ADD OTHER MEDICATION

REFER TO NEUROLOGY TO BE SEEN IN 2-3 WEEKS

FULMINANT IIH

REPEAT LP AND VISION

URGENT DISCUSSION AND TRANSFER TO

NEUROSCIENCE CENTRE

REPEAT LP- DAILY ACUITY AND

FIELDS

MDT DISCUSSION

REPEATED LP / LUMBAR DRAIN

STENT/SHUNT/ ONF/

Consensus Guideline in Adult Idiopathic Intracranial Hypertension: an infographic summary. .

Susan P Mollan et al. Pract Neurol 2018;18:485-488

©2018 by BMJ Publishing Group Ltd

Cerebral venous sinus thrombosis

▪ Headache is the most common symptom:Headache 82% Reduced GCS 31%Papilloedema 50% Cranial nerve palsies 11% (II- VII)Seizure(s) 42% Bilateral cortical signs 4%Focal deficit 41% Cerebellar signs 3%

▪ Precipitating causes include: – Oestrogen containing OCP, trauma, intracranial tumour, infection (face, ear, nose,

sinuses), abscess, meningitis, post partum, polycythaemia, systemic infection , severe dehydration

▪ Headache: progresses over days or weeks – can be thunderclap

▪ CTV or MRI/MRV

Low CSF pressure

▪ Post Lumbar puncture or after a dural puncture / spontaneous

▪ NO Headaches on lying down

▪ Within 5- 15 minutes of sitting up or standing up severe ( very often throbbing ) bilateral severe headache which is incapacitating- ‘Orthostatic headaches’

▪ Headache is a reliable feature in acute cases

▪ The positional element of headache is lost in chronic cases / chronic low pressure has varied presentations that may not include headache at all

▪ Lie flat

▪ IV fluids 8 hourly or 2-3 litres of oral fluids/ 24 hours

▪ Takes a good 1-2 weeks to recover fully

▪ In the spontaneous type- MR brain (with contrast)

▪ If patients are worsening despite medical treatment for 72 hours –consider epidural blood patch

Other red flags

▪ New headaches along with– New neurological deficit

– Immunocompromised

– Known malignancy

– Elderly

– Anticoagulated

– Pregnancy

▪ Temporal arteritis – Jaw claudication

– Stroke like features

– Raised infl markers

– Along with starting steroids a temporal artery biopsy has to be arranged to happen as early as possible ( preferably within 48 hours- 7 days )

▪ GlaucomaLoss of vision and halo effect

Urgent ophthalmology assessment

Headache management after acute severe headache

▪ Persistent headaches are common after acute sudden severe headache or after head trauma ( the New Daily Peristent Headache syndrome )

▪ Behaves like chronic migraine

▪ Advise patients and institute early management along principles of chronic migraine

A Bahra ACNR online journal July 2013

Headaches and incidental findings on scans

Primary headaches

▪ Headaches which happen without an underlying sinister abnormality

▪ These can be – episodic

– chronic

Primary headaches

▪ Migraine- 6-18/100 (episodic) 5-8/100 (chronic ) - many associated with medication overuse

▪ Trigeminal autonomic cephalalgias- 1-2/ 1000

▪ Neuralgias and other headache disorders - ,,

▪ Tension type headache ?

cf: Subarachnoid haemorrhage – 7-10/100,000

So what makes diagnosis and management of primary headaches difficult ?

▪ Perceptions – outdated theories- ( arises from the neck/ sinuses/ teeth; blood vessel constriction; all

related to hormones )

– Unfamiliar with the concept of severe frequent / chronic headaches

– Treat it exactly like other pain syndromes

– Worry about the ‘brain tumour’

– Expect migraines to be stereotyped and with aura

▪ Expectations– patients and doctors seek quick and complete relief of pain

MigraineThe commonest headache disorder you will ever encounter

▪ Migraine is a form of sensory processing disturbance with manifestations within and outside the central nervous system function

▪ The Trigemino Cervical Complex TCC is currently the accepted pathway of migraine genesis

▪ Pain is only one of the symptoms

▪ Some genetic predisposition –clustering within families/ twins. Genetic types with aura like FHM and CADASIL

Episodic Migraine

▪ Diagnostic criteria:

▪ A. At least 5 attacks fulfilling criteria B–D

▪ B. Headache attacks lasting 4–72 hours (untreated or unsuccessfully treated)

▪ C. Headache has at least two of the following characteristics:– 1. unilateral location – 2. pulsating quality– 3. moderate or severe pain intensity– 4. aggravation by or causing avoidance of

routine physical activity (eg, walking or climbing stairs)

▪ D. During headache at least one of the following:– 1. nausea and/or vomiting– 2. photophobia and phonophobia8

▪ E. Not attributed to another disorder

▪ Episodes of headaches- unilateral or bilateral- lasting for 1-4 days

▪ Moderate to severe in intensity

▪ Aching / throbbing / ‘pressure ‘ like/ stabbing

▪ Involving face, scalp, neck

▪ Can have nausea, light, noise, smell and movement sensitivity

▪ Affects the patient’s capacity to function

▪ Prefers to lie down, sleep, keep away from people/noise

▪ In between episodes is headache free and feels and functions well

Episodic migraine – acute severe attack

Episodic Migraine treatment

▪ Pain control is to be used judiciously and should be less than 10-15 doses in a month

▪ Aspirin 300- 900 mg

▪ Ibuprofen 400- 600 mg

▪ Naproxen 250- 500 mg

▪ Paracetamol 1000 mg

▪ Triptans- Sumatriptan/ Zolmitriptan/ Rizatriptan

▪ In acute severe migraine -Combine Triptan + NSAID+ Antiemetic

▪ If Triptan or NSAID contraindicated – substitute eith paracetamol

▪ Taken right at the beginning of the severe attack

▪ Do not prescribe codeine or opiates

▪ Patients with frequent episodes should be evaluated for a preventative

Simple analgesia (aspirin, paracetamol, NSAID) – if not fully effective

Simple analgesia + triptan if not fully effective

Simple analgesia + triptan + prokinetic antiemetic

Triptan options – oral, orodispersible, nasal, injection Oral absorption can be unreliable in acute migraine

Avoid triptan DURING aura especially prolonged or complex aura

Avoid codeine and opiates

Chronic migraine – acute severe attacks on top of a background of daily dull headaches -for 3 months or longer

Chronic migraine

▪ Part of the ‘ migraine cycle’

▪ Triggers- stress, fatigue, sleep disturbance, dehydration, hormones, missing meals

▪ Medication overuse co exists often ( > 2- 3 doses /week )

Management of chronic migraine

▪ Complex- Education is the key

▪ Getting patients off analgesics and starting them on a preventative is the key

▪ Short courses of NSAIDs like naproxen while they come off codeine; nerve blocks are interim treatments

▪ Knowledge is the key support- along with rest, hydration , avoiding triggers

▪ Lifestyle modifications – regular sleep, food, water / avoid caffeine alcohol painkillers and antidizziness pills

▪ You are unlikely to make it completely better in an acute unit Don’t make it worse by sending them home on strong pain killers

▪ It will get worse before it gets better

▪ Improvement happens over weeks to months not days

▪ Most preventatives are effective only to a certain degree

▪ Refer to neurology if not responding to ADEQUATE Trials of 2 or more preventatives

▪ Give patients information so that they understand the condition, have realistic expectations and follow the management strategy

Prophylaxis- dosage and duration▪ Amitryptiline/ Nortryptiline (10- 100 mg)

▪ Propranalol (20-240 mg )

▪ Topiramate (50-300 mg)

▪ Pregabalin ( 75-600 mg )

▪ Candesartan ( 4- 16 mg )

START SMALL AND BUILD UP THE DOSE -REASONABLE DOSES HAVE TO BE USED FOR 3 -4 MONTHS EACH. THERE IS NO POINT IN SHORT’TRIALS’ FOR 1 MONTH ETC

Flunarizine [5- 10 mg] – not marketed or licensed in the UK

▪ Calcium antagonists, SSRIs,

▪ Occipital nerve injections

▪ Acupuncture

▪ Botulinum Toxin –if they try and fail at least 3 adequate oral preventative therapies

▪ Cefaly

▪ Monoclonal antibodies ( recent NICE approved )

The Trigeminal autonomic cephalalgias

The spectrum of headache(chart shows most frequently occurring only )

Headache

Primary

Migraine Trigeminal Autonomic

Cephalalgia (TAC)

ClusterParoxysmal Hemicrania SUNCT/ SUNA

Neuralgia

Secondary

Cluster headaches

▪ Unilateral, Side locked headaches. Severe pain,

▪ Autonomic symptoms (streaming of the eye and nostril, conjunctival congestion, swelling of face eyelids )

▪ Night time attacks waking pt up from sleep usually 2-6 am

▪ Severe agitation and restlessness

▪ Periodicity

▪ The term ‘cluster’ should not be used to describe migraines or other headaches that occur one after another

Cluster headaches ▪ Treat as an emergency

▪ Acute treatment – Sumatriptan –injection / nasal spray – can use upto 2 doses /day ( different to migraine

recommendations )– High flow oxygen – 15litres/ min through a tight fitting non- rebreathable mask – can be

prescribed for home use via a HOOF form

▪ Short term prevention – Greater occipital nerve blocks

– Prednisolone 60- 80 mg / day 3-5 days and taper off ( counsel on side effects + Lansoprazole )– Should not be used more than twice a year

▪ Long term prevention– Verapamil – likely to need high doses between 480 mg - 960 mg/day -ECG monitoring– Topiramate – Lithium– Gammacore device

▪ Refer to neurology

Trigeminal neuralgia

▪ Usually 2nd and 3rd trigeminal divisions

▪ Paroxysms+ triggers + refractory phase+ remissions

▪ Persistent dull ache of the face is not neuralgia

▪ Compression by vascular loop / tumours/ MS plaques

▪ MRI – 3-5 % will pick up a secondary cause

▪ Vascular compression at the root entry zone of the nerve can be demonstrated in upto 50% of idiopathic trigeminal neuralgias

Management of neuralgia

▪ Carbamazepine (oxcarbazepine) – usually very responsive

▪ Phenytoin- IV in severe situations

▪ Gabapentin

▪ Pregabalin

▪ Lamotrigine

▪ Baclofen

▪ Levetiracetam

▪ Surgery- decompressive / ablative

▪ Refer to neurosciences

Conclusion▪ History is crucial

▪ Look clinically for red flags- if not present consider primary headache ( patients should not go down a ‘ routine ‘ route of scans and LPs unless sufficient reason)

▪ Scans should be based on clinical presentation – every severe headache does not require contrast/ venogram/ angiogram

▪ Primary headaches are a common pain syndrome that have a unique basis and origin from a normal brain - and therefore management is different to other pain syndromes

▪ Opiates have very little/ no role in longterm headache management

▪ Pain medication overuse is a significant problem with chronic headache disorders

▪ Patient education is crucial – Explain and give information leaflet

Primary headaches that should be referred to neurology

New daily persistent headache Trigeminal neuralgia; SUNCT/SUNA Cluster headache Hemicrania Refractory / chronic migraine Unclassifiable, atypical headache or failure to respond to standard migraine therapies.

▪ Severe primary headaches & headaches with red flags where a secondary cause has been ruled out – should be given information on primary headaches and managed P as per local and national guidelines – Local headache pathway and

guidance

– NICE CG 150

– BASH guidelines

ASSOCIATION OF BRITISH NEUROLOGISTSACUTE NEUROLOGY BOOT CAMP 2020

Association of British Neurologists, Ormond House, 27 Boswell Street, London WC1N 3JZ

Email: info@abn.org.uk. Telephone: 02074054060