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2/16/2018 History Recent Advances 32 y/o female referred to our clinic for an increase in headaches for the past three months in Neurology Case Presentation History of headaches since childhood provoked by hunger or lack of sleep

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2/16/2018 1

Recent Advances in Neurology Case Presentation

Rebecca L. Michael, MD February 16, 2017

History

  • 32 y/o female referred to our clinic for an

increase in headaches for the past three months

  • History of headaches since childhood

– provoked by hunger or lack of sleep

  • In adolescence, frequency increased with onset
  • f menses and then again with onset of using

OCPs

History continued

  • Location was unilateral frontotemporal region,

alternated sides equally

  • Quality is described as pressure and at times

throbbing

  • Associated with nausea, photophobia,

phonophobia and osmophobia.

History continued

  • She endorsed a preceding visual aura

– alternating wavy lines and black spots – evolved into her entire visual field over 30 minutes – occurred 75% of the time

  • She stopped OCP’s at age 28 with

improvement in HA frequency to only 2-3 days/month

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2/16/2018 2

History continued

  • Prior three months, headaches increased
  • 18 headache days per month and 8 days of “headache

hangover”

  • Quality of pain is similar
  • Worse in the morning
  • Worse with valsava and bending over
  • Visual ‘aura’ now however is “blacked out” central vision
  • Only clear precipitant is stress with her upcoming wedding

History continued

  • Past Medical History

– menorrhagia s/p thermal ablation and eventual hysterectomy – hyperthyroidism s/p thyroidectomy – anxiety – obesity

  • Review of Systems

– snoring – insomnia

History continued

  • Headaches are minimally responsive to tylenol

and advil

  • Rizatriptan and intranasal sumatriptan caused

side effects

  • Has never been on a preventive medication

for migraine

Question 1

Based on the history, what is the most likely diagnosis?

  • A. Chronic Migraine
  • B. Sleep Apnoea Headache
  • C. Idiopathic Intracranial

Hypertension, IIH

  • D. Chronic Tension Type Headache
  • E. Cerebral Venous Sinus Thrombosis

C h r

  • n

i c M i g r a i n e S l e e p A p n

  • e

a H e a d a c h e I d i

  • p

a t h i c I n t r a c r a n i a l H . . . C h r

  • n

i c T e n s i

  • n

T y p e H e . . . C e r e b r a l V e n

  • u

s S i n u s T . . .

44% 5% 11% 3% 37%

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Chronification of Migraine

  • usually develops from episodic migraine

(annual conversion rate of about 3%)

  • Risk factors

– Non-modifiable

  • age, female sex, low education status

– Modifiable

  • MOH, ineffective acute treatment, obesity, depression

and stressful life events

May A, Schulte LH. Chronic migraine: risk factors, mechanisms and treatment Nature Reviews Neurology 12, 455–464 (2016)

Neurological Exam

BP: 115/73 HR: 78 BMI 32.94

  • CN intact, visual acuity normal
  • Visual fields: enlarged blind spot bilaterally
  • optic discs blurred bilaterally

– obscuration of all borders

  • remainder of neurological exam normal

Question 2

The most common headache phenotype (according to ICHD-3) described in patients with IIH in the Idiopathic Intracranial Hypertension Treatment Trial, IHTT, is which of the following

  • A. Migraine
  • B. Probable migraine
  • C. Tension-type headache
  • D. Probable tension-type headache

M i g r a i n e P r

  • b

a b l e m i g r a i n e T e n s i

  • n
  • t

y p e h e a d a c h e P r

  • b

a b l e t e n s i

  • n
  • t

y p e . . .

27% 14% 9% 49%

  • Largest randomized controlled trial on IIH ever

conducted

  • 38 neuroophthalmology centers in US
  • 165 subjects

– Bilateral papilledema, mild visual field loss, lumbar puncture with elevated OP

  • acetazolamide w/ diet (70) vs. placebo w/ diet (69)
  • Primary outcome

– Perimetric mean deviation (PMD) at 7 months

  • Numerous secondary outcomes

IIHTT

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Headache Characteristics at Baseline

  • HA present in 84% at baseline
  • Locations of headache

Frontal 68% Global 36% Ocular 47% Posterior 39% Nuchal 47% Unilateral 30%

  • Characteristic of pain

pressure like 47% throbbing 42%

Friedman DI et al. Headache 2017; 57, 1195-1205

Headache Characteristics at Baseline

  • “Migraine” associated symptoms common

photophobia (70%), phonophobia (52%), nausea (47%), vomiting (17%), worsened with physical activity (50%)

  • IIH-related symptoms
  • Constant visual loss (34%)
  • Transient visual obscurations (68%)
  • Diplopia (22%)
  • Dizziness (53%)
  • Headache with none of the above (14%)

Friedman DI et al. Headache 2017; 57, 1195-1205

  • Headache phenotype (ICHD-3 beta)

Migraine 52% Probable migraine 16% Tension-type 22% Probable tension-type 4% Not classifiable 7%

  • 50% of migraine or probable migraine subjects (n=94) had a

history of migraine

  • 29% of the other headache types (n=45) had a history of

migraine

Headache Characteristics at Baseline

Friedman DI et al. Headache 2017; 57, 1195-1205

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2/16/2018 5

Back to our case…

  • 32 y/o female w/ increase in headache

frequency and papilledema

  • Referred for urgent evaluation/imaging
  • Ophthalmology exam

MRI brain wwo contrast MR venogram

  • Lumbar Puncture

– initial opening pressure was 25cm

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Criteria for IIH Diagnosis

Initially defined by Dandy and modified by Friedman et. al

  • 1. Required for the diagnosis

A. Papilledema B. Normal neurological exam except for cranial nerve abnormalities C. Neuroimaging (MRI brain wwo contrast for typical patients and MRV for others) D. Normal CSF composition E. Elevated lumbar puncture OP (≥25 mm in adults, ≥28 mm in children)

Friedman DI et al. Neurology 2013

Diagnosis without papilledema

– if B-E are satisfied in addition to a bilateral or unilateral abducens palsy – Diagnosis can be suggested if B-E and three of four neuroimaging are satisfied

i. Empty sella ii. Flattening of posterior aspect of the globe iii. Distention of perioptic subarachnoid space with or without a tortuous optic nerve iv. Transverse venous sinus stenosis

Friedman DI et al. Neurology 2013

Criteria for IIH Diagnosis Neuroimaging Findings Back to our case…

  • Diagnosis of Idiopathic Intracranial

Hypertension was made

  • Initiated on acetazolamide 500mg BID
  • Was seen by nutritionist
  • Referred for sleep medicine evaluation
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2/16/2018 7

Follow Up

  • Lost 12 lbs
  • Headache reduced to prior frequency of 2-3

days/month

  • Denied further TVO’s
  • Repeat ophthalmology exam

– Papilledema no longer present – Optical Coherence Tomography, OCT, normal

  • Repeat LP with opening pressure 30cm

– CSF normal

Optical Coherence Tomography, OCT

  • Retinal Nerve Fiber Layer (RNFL) Thickness

Mollan et al. J Neurol Neurosurg Psychiatry, 2016

Question 3

OCT, optical coherence tomography, is unreliable in which of the following scenarios?

  • A. significant disc edema
  • B. crowded disc
  • C. pseudopapilledema
  • D. answer choices A and B
  • E. answer choices A, B and C

s i g n i f i c a n t d i s c e d e m a c r

  • w

d e d d i s c p s e u d

  • p

a p i l l e d e m a a n s w e r c h

  • i

c e s A a n d B a n s w e r c h

  • i

c e s A , B a n d C

1% 16% 32% 12% 39%

Management

  • Instructed on slow taper diamox
  • Naratriptan given for acute treatment
  • Encouraged continued weight loss
  • Called three weeks later with increase in

headache frequency and severity

  • Initiated on topamax 25 100mg
  • Currently doing well
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2/16/2018 8

Management

  • In the IHTT, acetazolamide + weight loss

program improved visual outcomes

– more so than weight loss program alone

  • acetazolamide is safe up to 4 g/day
  • alternatives

– Topiramate – Zonisamide – Furosemide – Indomethacin, Iron

Friedman DI et al. Headache 2017; 57, 1195-1205

?

Management

Friedman DI et al. Headache 2017; 57, 1195-1205

Conclusion

  • IIH is a condition important to recognize because it may

lead to progressive loss of vision over time, which may be permanent

  • Diagnosis can be challenging

– Overlap with migraine – May present with papilledema or without

  • OCT may help aid in the diagnosis of mild cases or in the

monitoring of response to treatment

  • Additional headache treatments should be undertaken

Thank You

  • UCSF Headache Center Referrals:

– Intractable migraine, cluster headaches, post-traumatic headaches and other unusual or difficult headache disorders – Outpatient treatment – Nerve blocks – Neurostimulation – Telemedicine – Research – Inpatient treatment

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