34 Headache Medicine, v.1, n.1, p. 34-35, jan./fev./mar 2010

Successful treatment of SUNCT syndrome with

divalproex sodium extended-release

Síndrome SUNCT tratada com sucesso com divalproato de sódio de

liberação prolongada

ABSTRACTABSTRACT

ABSTRACT

Introduction:Introduction:

Introduction: Short-lasting unilateral neuralgiform headache

attacks with conjunctival injection and tearing (SUNCT)

syndrome is a primary rare headache. This syndrome is known

to be refractory to pharmacotherapy. Various treatments have

been tried for SUNCT syndrome, generally of little benefit.

Objective:Objective:

Objective: To report a case of SUNCT syndrome who was

successfully treated with divalproex sodium extended-release

(DVPX ER) and to review the literature about of drugs used in

this treatment.

P P

atient and Method:atient and Method:

atient and Method: The authors report a

case of a 61 year-old man, suffering from SUNCT syndrome,

who was successfully treated with DVPX ER. We review the

literature about several categories of drugs that are used in

the treatment to this syndrome.

Conclusion:Conclusion:

Conclusion: SUNCT syndrome

is clinically well identified, but it is poorly treated. This is first

report of SUNCT syndrome responsive to DVPX ER.

Key words: Key words:

Key words: SUNCT syndrome; headache; divalproex sodium.

RESUMORESUMO

RESUMO

Introdução:Introdução:

Introdução: A síndrome SUNCT (cefaleia de curta dura-

ção, unilateral, neuralgiforme com hiperemia conjuntival e

lacrimejamento) é um tipo raro de cefaleia e refratária ao

tratamento farmacológico. Diversos tratamentos são tenta-

dos e, geralmente, de pouco benefício.

ObjetivoObjetivo

Objetivo: Relatar

um caso de síndrome SUNCT tratada com divalproato de

sódio de liberação prolongada (DVP ER) e revisar a literatura

sobre as drogas utilizadas neste tratamento.

P P

aciente eaciente e

aciente e

MétodoMétodo

Método: Os autores descrevem um caso de um homem de

61 anos, com síndrome SUNCT que foi tratado com DVP ER.

Foi revisada a literatura sobre as diversas drogas que são

utilizadas no tratamento desta síndrome.

Conclusão:Conclusão:

Conclusão: A

CASE REPORT

síndrome SUNCT é clinicamente bem identificada, mas é

pobremente tratada. Este é o primeiro relato de síndrome

SUNCT responsiva ao DVP ER.

alavrasalavras

alavras

chave:chave:

chave: Síndrome SUNCT; cefaleia; divalproato

de sódio.

INTRODUCTION

Short-lasting unilateral neuralgiform headache attacks

with conjunctival injection and tearing (SUNCT) syndrome

is a primary rare headache, described initially by Sjaastad

and colleagues in 1989,

and characterized by trigeminal

pain accompanied by autonomic manifestations. The pain

is strictly unilateral, localized to the orbital region, stabbing

or burning in quality, abrupt, of short duration (5 to 240

seconds), frequent (3 to 200 per day), and accompanied

by lacrimation, conjunctival injection, and nasal congestion

or rhinorrhoea, all ipsilateral to the pain.

2,3

The pain is

neuralgiform, so it can be difficult to distinguish from

trigeminal neuralgia. The features that distinguish it from

other trigeminal autonomic cephalalgias are: very brief

duration of attacks that occur very frequently, and the

presence of prominent conjunctival injection and

lacrimation. SUNCT syndrome is clinically well identified,

but it is poorly treated. Several categories of drugs are

Raimundo Pereira da Silva Neto

, Kelson James Almeida

Neurologista e Membro da Sociedade Brasileira de Cefaleia, Centro de Neurologia e

Cefaleia do Piauí - Teresina PI, Brasil

Residente de Neurologia do HC-USP

Silva Neto RP, Almeida KJ. Successful treatment of SUNCT syndrome with divalproex sodium extended-

release: case report. Headache Medicine. 2010;1(1):34-35

Headache Medicine, v.1, n.1, p. 34-35, jan./fev./mar 2010 35

SUCCESSFUL TREATMENT OF SUNCT SYNDROME WITH DIVALPROEX SODIUM EXTENDED-RELEASE

used in the treatment to this syndrome, including

intravenous lidocaine,

anesthetic blockades, verapamil,

and anti-epileptic drugs such as gabapentin,

lamotrigine,

oxcarbamazepine,

and topiramate.

All are generally

of little benefit. Until recently, SUNCT was considered an

intractable headache or highly refractory to treatment.

We describe a patient with SUNCT syndrome who

was successfully treated with divalproex sodium extended-

release (DVPX ER). This drug has not previously been tried

in SUNCT syndrome, and to the best of our knowledge

this is the first report of SUNCT syndrome responsive to

DVPX ER.

CASE

A 61 year-old man with an 11-days history of

persistent throbbing headache localized to the orbital

region. The headache was always right-sided. Daily pain

was of severe intensity. The pain often lasted 5 to 10

seconds and recurred with an average of four to five times

per day. The pain was accompanied by autonomic

symptoms (conjunctival injection and lacrimation). The

headache had no known triggering factors. During this

period, the patient used different analgesics and other

antiepileptic drug without success. General physical and

neurologic examinations were normal. Magnetic

resonance imaging was normal. A diagnosis of SUNCT

syndrome was made. We started treatment with DVPX ER.

Daily dosage was initiated at 500 mg/day, and after 7

days, because of the reoccurrence of occasional attacks,

we increased the dosage to 500 mg twice daily. At a

dose of 1,000 mg per day (500 mg twice daily) he

became pain free. Two months later, DVPX ER was stopped

and his pain did not recur in the following 6 months.

DISCUSSION

Our patient's short-lasting headache meets the

diagnostic criteria for SUNCT.

This syndrome is known

to be refractory to pharmacotherapy. Various treatments

have been tried for SUNCT syndrome. Rare cases are

responsive to lidocaine,

anesthetic blockades, verapamil,

and anti-epileptic drugs such as gabapentin,

lamotrigine,

oxcarbamazepine,

and topiramate.

DVPX ER is an

antiepileptic drug used for treating other painful neurologic

syndromes such as migraine, cluster headache, and

chronic daily posttraumatic headache. DVPX ER stimulates

brain GABA synthesis and reduces glutamate levels, one

of the excitatory neurotransmitters, by increasing the activity

of glutamic acid decarboxylase.

In our case, the initial dose DVPX ER of 500 mg no

reduced the number of SUNCT attacks. When we

increased the dose to 1,000 mg/day (500 mg twice daily),

the attacks progressively reduced until they finally

disappeared. To induce a remission, the patient required

an average dose of DVPX ER to 1,000 mg/day.

From our experience with this case, DVPX ER should

be considered a possible prophylactic treatment for

SUNCT syndrome.

REFERENCES

1. Sjaastad O, Saunte C, Salvesen R, Fredriksen TA, Seim A, Roe

OD et al. Short-lasting unilateral neuralgiform headache attacks

with conjunctival injection, tearing, sweating, and rhinorrea.

Cephalalgia. 1989;9:147-56.

2. Pareja JA, Sjaastad O. SUNCT syndrome. A clinical review.

Headache. 1997;37:195-202.

3. Cohen AS. Short-lasting unilateral neuralgiform headache attacks

with conjunctival injection and tearing. Cephalalgia. 2007;

27(7):824-32.

4. Matharu MS, Cohen AS, Goadsby PJ. SUNCT syndrome

responsive to intravenous lidocaine. Cephalalgia. 2004; 24

(11):985-92.

5. Narbone MC, Gangemi S, Abbate M. A case of SUNCT syndrome

responsive to verapamil. Cephalalgia. 2005;25(6):476-8.

6. Hunt CH, Dodick DW, Bosch EP. SUNCT responsive to

gabapentin. Headache. 2002;42(6):525-6.

7. D'Andrea G, Granella F, Ghiotto N, Nappi G. Lamotrigine in

the treatment of SUNCT syndrome. Neurology. 2002;

57(9):1723-5.

8. Dora B. SUNCT syndrome with dramatic response to

oxcarbazepine. Cephalalgia. 2006;26(9):1171-3.

9. Rossi P, Cesarino F, Faroni J, Malpezzi MG, Sandrini G, Nappi

G. SUNCT syndrome successfully treated with topiramate: case

reports. Cephalalgia. 2003;23(10):998-1000.

10. Headache Classification Subcommittee of the International

Headache Society. The International Classification of Headache

Disorders. Cephalalgia. 2004;24(Suppl 1):1-160.

Endereço para correspondência

DrDr

. R. R

. R

aimundo Paimundo P

aimundo P

ereira da Silva Netoereira da Silva Neto

ereira da Silva Neto

Centro de Neurologia e Cefaleia do Piauí

Rua São Pedro, 2071/Centro

Ed. Raimundo Martins - Salas 303/304

64001-260 - Teresina-PI - Brasil

Tel./fax: + 55 86 3221.9000

E-mail: neurocefaleia@terra.com.br