Headache Master School Japan-Osaka 2016II. Management of refractory headachesCase Presentation 2. SUNCT/SUNA:concept, management and prognosis

Noboru Imai(Department of Neurology, Japanese Red Cross Shizuoka Hospital)

October 23th, 2016; Osaka International House Foundation

The trigeminal autonomic cephalalgias (TACs)

3.1 Cluster Headache3.2 Paroxysmal Hemicrania3.3 Short-lasting unilateral neuralgiform headache attacks (SUNCT/SUNA)

3.3.1 Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT)3.3.2 Short lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA)

3.4 Hemicrania Continua3.5 Probable TAC

IHS. ICH-3beta. Cephalalgia 2013

The pathogenesis of TACs

May A. Lancet 2005; 366: 843-855

Posterior hypothalamic activation of TACs

Cluster headache(PET)

May et al., Lancet 1998; 351:275-278

May et al., Ann Neurol 1999; 46:791-793

Hemicrania continua(PET)

Paroxysmal hemicrania(PET)

SUNCT(fMRI)

Matharu et al., Ann Neurol 2006;59:535-545

Matharu et al., Headache 2004;44:747-761

SUNCT/SUNAA. At least 20 attacks fulfilling criteria B–DB. Moderate or severe unilateral head pain, with orbital, supraorbital, temporal, and/or other trigeminal painDistribution: lasting for 1–600 seconds and occurring as single stabs, a series of stabs, or in a saw-tooth patternC. At least one of the following cranial autonomic symptoms or signs, ipsilateral to the pain:

1. conjunctival injection and/or lacrimation2. nasal congestion and/or rhinorrhea3. eyelid edema4. forehead and facial sweating5. forehead and facial flushing6. sensation of fullness in the ear7. miosis and/or ptosis

D. Attacks have a frequency of at least one a day for more than half the time when the disorder is activeE. Not better accounted for by another ICHD-3 diagnosis.

3.3.1 SUNCT

• Diagnostic criteria:

• A. Attacks fulfilling criteria for 3.3 Short-lasting unilateral neuralgiform headache attacks

• B. Both of conjunctival injection and lacrimation (tearing).

3.3.2 SUNA

• A. Attacks fulfilling criteria for 3.3 Short-lasting unilateral neuralgiform headache attacks, and criterion B below

• B. Only one or neither of conjunctival injection and lacrimation (tearing).

Case 1: 27-year-old man

• Moderate or severe left-sided headaches lasting 30–120 min with ipsilateral lacrimation, nasal congestion and rhinorrhoea

• First episode: 5 months ago

• The frequency of attacks: 1 or 2 times per day

• Persistent background pain

• One month pain-free period

• Triggered by neck exercise, wasabi

• Past history– Acute treatment:

Oral Triptans, NSAIDs, OTCs ; not effectiveOxygen; moderate pain reduction within 15 to 30 minutes

– Preventive treatment:

Carbamazepine; not effective

• Family history– None

• On examination– General and Neurological examination normal

• Imaging– MRI normal

Treatment

• Acute treatment

– subcutaneous sumatriptan (6 mg) ; not effective

• Preventive treatment

– Oral steroid, verapamil, lithium, lomerizine, indomethacin; not effective

– Topiramate; ,the frequency was reduced to half, but not effective 2 months later

Obtaining a good history

• Headache episodes consist of a series of saw-tooth pattern attacks!

• Lamotrigine: suppressed attacks up to 200mg daily

10

0

Case 2: a 5-year-old girl

• Severe, unilateral (mainly left-sided) temporal headaches lasting 20–90 s with ipsilateral lacrimation, nasal congestion and rhinorrhoea, facial flushing

• First episode at 16 months old

• The frequency of moderate or severe headache attacks gradually increased 84 to 10–20 times per day

• Triggered by hitting her head or body, taking a bath, experiencing a temperature change, or sleeping

• Alleviated by carbamazepine

Genetic analysis of the pedigree

I-1 I-2 (76y)

II-1* (56y)

III-1 (32y)

II-3 (51y)II-2 (50y)

III-3* (41y)III-2* (29y)

IV-4* (1y)IV-3* (5y)

III-4* (24y) III-5* (22y)

IV-2* (6y)IV-1* (12y)

A novel SCN9A mutation in this family suggested that the family suffers from a variant of paroxysmal extreme pain disorder.

Familial pedigree

: Position of the mutation(NM_002977: c.5218G>C, p.Val1740Leu)

Affected individuals Unaffected individuals

Electropherograms of the heterozygous SCN9A mutation. A G/C change is represented as an “S” according to the IUPAC code.

The subtraction ictal SPECT co-registered to MRI (SISCOM)

Functional study

A

A) Transverse, B) sagittal, and C) coronal images.

A

B C

Clinical features of TACsCluster headache Paroxysmal

hemicraniaSUNCT/SUNA Hemicrania

Continua

Sex F<M F=M F=M F>M

Pain Type Stabbing, boringThrobbing, boring,

stabbingBurning, stabbing,

sharpThrobbing, ache, sharp, pressure

Severity Excruciating Excruciating Moderate to severe

Mild to moderate background with severeexacerbations

Duration and frequency

15–180 mins, 1–8/day

2–30 mins, 5–40/day

1–600 s, 1–200/day Continuous

Autonomic features Yes Yes Yes Yes with

exacerbationsIndomethacin effect No Complete resolution No Complete resolution

First choice abortiveagent

Injectable or nasalsumatriptanOxygen

None None None

First choice preventativeagents

VerapamilPrednisone

Indomethacin Lamotrigine Indomethacin

Cohen et al., Brain 2006; 129:2746-2760 ;Cittadini et al., Brain 2008; 131:1142-1155Miller et al., Curr Pain Headache Rep 2014; 18: 438

Thank you for your attention!