A case of a patient with sunct syndrome treated with jannetta procedure

A case of a patient with SUNCT syndrome treated with L Gardella, A Viruega, H Rojas & J NagelSanatorio Parque, Cordoba, Rosario, Santa Fe, Argentina Gardella L, Sanatorio Parque, Cordoba 2324, Rosario (CP 2000), Santa Fe, Argentina.
Received 12 September 2000, accepted 18 June 2001 wind, lateral head movements to the right and rapid eye movements also triggered the pain.After the painful SUNCT syndrome is a rare disorder.Sjaastad (1) ®rst episodes she suffered from hyperesthesia in the affected described this syndrome in 1978.The patient suffers area and the skin of the ipsilateral frontal region looked short-lasting, unilateral crisis of a neuralgic-type pain rough and irregular like `sandpaper'.Neurological of a severe intensity, centred in the orbital/periorbital examination, except for the above description, was area.This pain is accompanied by conjuctival injection, always normal.The previous clinical history and the CT scan were unremarkable.Indomethacin, amitripty- This syndrome has some similarities with cluster line, ergotamine and verapamil treatments were not headache (17) and ®rst-division trigeminal neuralgia.
helpful.The later use of prednisolone (60 mg daily for The treatment is carbamacepine, amitrptyline, pred- 6 days and then 20 mg daily for 10 days) and carbama- nisolone, indomethacin, verapamil, ergotamine, with cepine (800 mg per day for 11 weeks) brought bene®t unlikely and uncertain results (18).Very few cases by reducing the painful crises to one or two a day, but Because of these negative therapeutic results, we considered surgical methods (17, 20, 21).
A 48-year-old woman consulted a physician in April 1997 due to a painful condition in the right orbitofrontal area.She did not remember the exact date when symp- The patient was studied with cerebral magnetic reso- toms began, but reported that they started approxi- nance imaging (MRI) using paramagnetic contrast.
mately 4 or 5 years before our consultation.During those Different pulse sequences were applied with a General years she consulted lots of other physicians without Electric Signa superconductive magnet of 1.5 Tesla satisfactory results.She experienced between six and equipment.Axial, coronal and saggital images were seven episodes a day.The temporal pattern was about made in T1 y T2.Paramagnetic contrast was also given three or four times per week; of course this pattern was to the patient. She was examined using F.L.A.I.R. effect, related to exposition to pain triggers that she had saggital T1 effect and Fast Spin ECO T2.We also identi®ed.Each one consisted of an abrupt and intense performed spectroscopic and perfusion studies.
peak of 30±45 s, followed by a painful burning feeling The Fast Spin ECO T2 (Fig.1) study showed absence that lasted for 35 min to 2 h.The after-attack burning of demyelinating lesions in the axial as well as in the coronal series.The region of the cerebellopontine The neuralgic pain began in the medial canthus of angle was free of expansive processes.We observed an the right eye, involving later a triangular orbitofrontal important change in a superior cerebellar artery root.
area and, with less intensity, the upper half of her This vessel made contact with the right trigeminal nerve right cheek.During the peak of the pain there was ocular that appeared toned down (Figs 1, 2 and 3).
congestion, tearing, oedema (observed only in the eye- lids) and ipsilateral palpebral ptosis.Crises were not only spontaneous, but also were triggered by touching the periorbital area, brushing her teeth, yawning, chew- Under the effects of general anaesthesia with endo- ing and washing her hair.In addition, exposure to strong tracheal intubation and ECG, arterial pressure and # Blackwell Science Ltd Cephalalgia, 2001, 21, 996±999 Figure 3 MRI axial T1WI: vascular structure (arrow) touching Figure 1 In the FAST SPIN ECHO effect a vascular structure the trigeminal nerve (with hyper-intense ¯ow signal in can be observed, like empty signal (arrow), in close relationship Figure 4 In this ®gure the trigeminal nerve can be seen (full arrow) entering into the pons.At this level it is being fenestrated by a branch (small empty arrow) of the superior cerebellar artery (big empty arrow).
the cerebellum.Once the Dandy's petrous vein wasfound, it was coagulated and cut, which allowed us to Figure 2 MRI Coronal plain: appreciable vascular structure (arrow) in contact with the right trigeminal nerve.
identify the ®fth cranial nerve in a vertical position,between the tentorium and the petrous bone, penetratingits border towards Gasser's ganglion.
gasometry monitoring, on left lateral decubitus (park Focusing on an area rather proximate to the entry zone bench position), a right suboccipital retro-mastoid cran- of this cranial nerve into the pons (Fig.4, fenesmacro) iectomy was performed.The duramatter was opened we noticed that a superior cerebellar artery branch in a semicircular shape following the latero-sigmoid penetrated into the trigeminal nerve and fenestrated it.
sinus limits, exposing the superior and later faces of This artery branch was dissected and separated from the # Blackwell Science Ltd Cephalalgia, 2001, 21, 996±999 reported in the literature.This syndrome has some similarities with cluster headache (17) and ®rst-division trigeminal neuralgia.A great many medical treatments (18) with uncertain results are proposed in order to diminish the intensity or the duration of the symptoms.
By means of the neuroimaging techniques we have employed, an important change in a superior cerebellar artery root was oberved.This vessel contacted with the right trigeminal nerve.Because of the lack of bene®ts brought by the large number of medical treatments tried with this patient, we proposed a surgical treatment with the `Jannetta technique' (microvascular decompression), which is frequently used to treat trigeminal neuralgia.
The purpose of this technique is to separate an artery branch, or more rarely a vein, in close relationship with Figure 5 In this photo, the fenestrating artery branch (small the entry zone of the ®fth cranial nerve into the pons.
empty arrow) is disjoining from the superior cerebellar artery In the case we report (Figs 1, 2 and 3) we found (big empty arrow) perpendicularly.It has just been separated from the fenestration of the trigeminal nerve (full arrow).
a vascular compression of the trigeminal nerve in the neuroradiological images, similar to that found in the essential neuralgias successfully treated with this proce- dure.This should be, according to our knowledge, the third case in the literature of a SUNCT syndrome treated with surgical procedures.The ®rst (20) was treated with a similar technique and the other (21) with a per- cutaneous compression of the Gasser's ganglion.The present case stands as a new option in the therapeutic management of SUNCT syndrome.This case, success- fully treated with a surgical procedure, should encour- age neurologists to require neuroradiological studies with the purpose of evidencing a neurovascular com- pression.Consequently, when medical treatment is not successful, this surgery, performed by professionals with proven experience in this ®eld, becomes a possible choice with minimal morbidity or mortality.
Figure 6 Final position: a foam rubber sponge is placed between the fenestrating vessel (empty arrow) and the nerve (full arrow), precluding the direct contact of both structures.
nerve, retracting it from the nerve fenestration (Fig.5, 1 Sjaastad O, Saunte C, Salvesen R et al.Shortlasting, unilateral sin fenesmicro).A foam rubber sponge was placed neuralgiform headache attacks with conjunctival injection, between both structures (Fig.6, esponga 3).The dura- tearing, sweating, and rhinorrhea.Cephalalgia 1989; 9:147±56.
matter and the other plains were closed in accordance 2 Sjaastad O, Zhao J-M, Kruszewski P, Stovner L-J.Shortlasting, unilateral, neuralgiform headache attacks with conjunctivalinjection, tearing, etc.(SUNCT): III.Another Norwegian case.
The patient stayed in the intensive care unit for 12 h, and then spent 2 days in the general care room.
3 Kruszewski P, Fasano ML, Brubakk AO, Shen JM, Sand T, She left hospital on the third day with total pain relief.
Sjaastad O.Shortlasting, unilateral, neuralgiform headache The patient has remained asymptomatic and there was attacks with conjunctival injection, tearing, and subclinical no recurrence up to now, 17 months after the surgery.
forehead sweating (`Sunct' syndrome).II.Changes in heartrate and arterial blood pressure during pain paroxysms.
Headache 1991; 31:399±405.
4 Pareja JA, Sjaastad O.SUNCT syndrome in the female.
5 Sjaastad O, Kruszewski P.Trigeminal neuralgia and SUNCT Ottar Sjaastad decribed the ®rst case of SUNCT syn- syndrome: similarities and differences in the clinical pictures.
drome in 1978 (1).Since then, many cases have been An overview.Funct Neurol 1992; 7:103±7.
# Blackwell Science Ltd Cephalalgia, 2001, 21, 996±999 6 Bouhassira D, Attal N, Esteve M, Chauvin M.SUNCT syndrome.A case of transformation from trigeminal headache attacks with conjunctival injection and tearing neuralgia? Cephalalgia 1994; 14:168±70.
(SUNCT syndrome): V.Orbital phlebography.Cephalalgia 7 Kruszewski P, Zhao JM, Shen JM, Sjaastad O.SUNCT syndrome: forehead sweating pattern.Cephalalgia 1993; 15 Sjaastad O, Kruszewski P, Fostad K, Elsis T, Qvigstad G.
SUNCT syndrome.VII.Ocular and related variables.
8 Becser N, Berky M.SUNCT syndrome: a Hungarian case.
16 Zhao JM, Sjaastad O.SUNCT syndrome.VIII.Pupillary 9 Pareja JA, Pareja J, Palomo T, Caballero V, Pamo M.SUNCT reaction and corneal sensitivity.Funct Neurol 1993; 8:409±14.
syndrome: repetitive and overlapping attacks.Headache 17 Headache Classi®cation Committee of the International Headache Society.Classi®cation and diagnostic criteria for 10 Bussone G, Leone M, Dalla Volta G, Strada L, Gasparotti R, headache disorders cranial neuralgias and facial pain.
Di Monda V.Short-lasting unilateral neuralgiform headache Cephalalgia 1988; 8 (Suppl.7):1±96.
attacks with tearing and conjunctival injection: the ®rst 18 Pareja JA, Kruszewski P, Sjaastad O.SUNCT syndrome: `symptomatic' case? Cephalalgia 1991; 11:123±7.
trials of drugs and anesthetic blockades.Headache 1995; 11 Morales F, Mostacero E, Marta J, Sanchez S.Vascular malformation of the cerebellopontine angle associated 19 Raimondi E, Gardella LA.SUNCT syndrome.Two cases in with `SUNCT' syndrome.Cephalalgia 1994; 14:301±2.
Argentina.Headache 1998; 38:369±71.
12 Hannerz J, Greitz D, Hansson P, Ericson K.SUNCT may be 20 Lenaerts M, Diederich N, Phuce K.A patient with SUNCT another manifestation of orbital venous vasculitis.Headache cured by the Jannetta procedure.Poster presentations, Session IV, 460, 8th Congress of the IHS.Cephalalgia 1997; 17.
13 Wober C, Wober-Bingol C, Wessley P.Das SUNCT syndrom.
21 Morales-AsõÂn F, Espada F, LoÂpez-Obarrio LA, Navas I, Fallbericht und Literaturubersicht.Fortschr Neuro Psychiatr Escalza I, InÄõÂguez C.A SUNCT case with response to surgical treatment.Cephalalgia 2000; 20:67±8.
# Blackwell Science Ltd Cephalalgia, 2001, 21, 996±999

Source: http://www.hectorrojas.com.ar/publicaciones/SUNCT.pdf