Difficult Cases

Scottsdale Headache Symposium 2016

• Alex Sinclair• NIHR Clinician Scientist & Honorary

Consultant Neurologist • a.b.sinclair@bham.ac.uk

• No conflicts of interest

Presenting complaint • 21 year old woman referred for an opinion on whether to site a shunt

Headache History• No headaches as child or during most of her adult life

• Continous headaches for 13 months

• Worse in morning and increases on valsalva and bending

• Continous tinnitus over the same time period

• Excerbations of the headache are linked to blurred vision, obscurations, occasional diplopia

• Since summer 2015 increase in weight by 1 stone (8 weeks)

• Asthma• Increased BMI• Hypothyroid• Heartburn• Cholecystectomy 2014• Appendectomy 2012

Social history• Nursery nurse at risk of loosing job due

to time off work• Married no children

Allergies• Dystonic reaction to prochlorperazine

Family history• Grandmother glaucoma

Drug historyPast medical history• Topiramate 50mg OD (6/12)• Acetazolamide 500mg BD• Celebrex 100mg BD• Co-Codamol up to 8 daily• Salbutamol inhaler• Levothyroxine 50mcg• Lansoprazole 30mg

First presentation• No papilloedema

• MRI brain - normal

• CT brain –normal

• CT venogram – normal

• LP – 32cm CSF; normal constituents

MRI imaging

Post contrastPre contrast

Referred to the IIH clinic• Noted one stone weight gain in 8 weeks prior to

presentation, in conjunction with new diagnosis of hypothyroidism

• No antibiotic use• No steroid use• No Vit A analogues• No Vit A supplementation• Not anaemic• No history of sleep apnoea• No venous thrombosis

Summary of LP’s in regional hospital

Relief of tinnitus and headaches after LP

32

45

25

30

MAR APR MAY  JUN JUL AUG SEPT OCT NOV DEC JAN FEB MAR APR MAY  JUN JUL AUG SEPT OCT

3130

25

45

32

2015 2016

1 stone weight gain

ExaminationRight Left

Acuity Un-aided 6/36 6/18

Acuity Corrected 6/18 6/9Ishihara colour vision Full fast Full fast

Pupils Normal, no RAPD

EOM Left VI nerve palsy

BMI 40.4

Full neurological exam otherwise normal

Right eye Left Eye

No fix losses, false pos false neg 5%No correction put upMD ‐5.92 DBPSD 6.66 DB

No fix losses, false pos or false negMD ‐1.97 DB PSD 1.83 DB

Could it be IIHWOP?• IIH With Out Papilloedema• Typically have a phenotype of obesity and

female gender• Debated area

• May have been over diagnosed in some studies

• New Diagnostic criteria*Friedman DI et al. Neurology 2013;81:1159–65

IIHWOP Diagnostic criteria

• Fulfil the criteria B-E above +• Unilateral or bilateral sixth nerve palsy• Or 3 neuro-imaging findings suggestive of raised ICP

• Empty sella, flattening of the posterior aspect of the globe, distention of the perioptic subarachnois space ±a tortuous optic nerve and transverse sinus stenosis

• Venous stenosis.

Diagnostic criteria for adult IIH* (Friedman 2013 Neurology)

A. Papilloedema

B. Normal neurological examination except for cranial nerve abnormalities

C. Neuroimaging: Normal brain parenchyma without evidence of hydrocephalus, mass or structural

lesion and no abnormal meningeal enhancement or venous sinus thrombosis on MRI and magnetic

resonance venography; if MRI is unavailable or contraindicated, contrast-enhanced CT may be

used.

D. Normal cerebrospinal fluid (CSF) composition

E. Elevated lumbar puncture opening pressure (≥ 25cmH2O) in a properly performed lumbar puncture

IIHWOP case 2Chronic daily headache 3 yearsNo papilloedemaNo 6th nerve palsy

LP 52 cmCSFBMI 49

LP 37 cmCSFBMI 49

LP 54 cmCSFBMI 49

Only imaging finding was empty sella

• LP shunt inserted

• Referred to Tier 3 Weight management program

Challenging diagnosisDon’t all have the classic imaging findings or 6th

Nerve palsy

IIHWOP – practical approach•• Chronic headaches• Obese female• No papilloedema

•

Consider • Repeat interval LP • ICP monitoring

• Raised ICP on separate occasions is increasingly supportive of diagnosis• Caution about interpreting improvement post LP

± Any evidence of Raised ICP on MRI? LP

Baseline OCT

IIHWOP increasingly likely

IIH WOP likely INCREASING LIKELIHOOD OF PATHOLOGICALLY RAISED ICP

15 20 25 30 35 40 4510 50 55 60

IIHWOP – Vision

Clover leaf appearance of functional visual fields can occur

• No papilloedema• Don’t develop papilloedema• Do not lose vision• Functional visual loss can occur• Visual phenomenon from migraine aura may occur

IIHWOP management1.Manage

conservatively Facilitate weight loss

2.Ongoing disability

Phenotype headachePhenotype headache

Any evidence of medication overuse?

Any evidence of medication overuse? Withdrawal

Any evidence of migraine

Any evidence of migraine Acute and preventative strategies

Consider shunting