Significance of Atypical Presentation of Symptomatic SUNCT

J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.70.2.244 on 1 February 2001. Downloaded from 244 J Neurol Neurosurg Psychiatry 2001;70:244–246

SHORT REPORT

Signiﬁcance of atypical presentation of symptomatic SUNCT: a case report

J W M ter Berg, P J Goadsby

Abstract tinuum.6 There are three reported cases of A patient with a SUNCT-like syndrome symptomatic SUNCT which have in common caused by severe basilar impression in a posterior fossa lesion7–9 which may have association with osteogenesis imperfecta implications for understanding SUNCT. In is described. Initially symptoms of both this report we present a new symptomatic case the first and second branch of the trigemi- with clinical features resembling SUNCT, nal nerve were prominent, on which again with pathology in the posterior fossa. carbamazepine had only a temporary and mild eVect. Progressive symptoms with Case report prominent ipsilateral autonomic features, The patient, a wheelchair bound 30 kg man, unexplained triggering by photostimula- height 110 cm and normal sclera with tion, and increasing duration of pain non-familial osteogenesis imperfecta could attacks occurred with relentless progres- never walk because of severe bone fragility and sive basilar impression associated with progressive skeletal deformities. He had fre- pontomedullary compression. Patho- quent spontaneous bone fractures until the age physiologically a dysfunction in ephaptic of 22 and had increasing bilateral hearing loss transmission is hypothesised. from age 25. He had hypertension recognised (J Neurol Neurosurg Psychiatry 2001;70:244–246) from the age of 44. At the age of 45 he under- Keywords: SUNCT syndrome; symptomatic; basilar went a left sided stapes interposition because of impression stapes ankylosis. Family history disclosed that five of nine brothers and sisters died from myocardial infarction. SUNCT syndrome is a unusual form of At the age of 40, the patient presented primary headache, characterised by attacks of initially with attacks of intense lancinating pain short lasting, unilateral pain of a neuralgiform in the left supraorbital and infraorbital region character, accompanied by prominent con- present for 1 day with five discrete episodes http://jnnp.bmj.com/ junctival injection and tearing and rhinor- each of 100–300 attacks without autonomic rhoea.1 It is among the rarest of the headache symptoms, such as lacrimation. The patient syndromes.23To the best of our knowledge, 33 was not known to have had headaches before. cases have been described. It has been Brain CT disclosed a mild basilar impression, proposed that SUNCT syndrome is classified which was judged to be unrelated. The diagno- with other primary headaches with more or less sis of trigeminal neuralgia was made and treat- prominent autonomic features—such as cluster ment with carbamazepine (200 mg/day) was on September 26, 2021 by guest. Protected copyright. headache, chronic paroxysmal hemicrania initiated. Symptoms did not occur until 2 years Department of (CPH), episodic paroxysmal hemicrania later. Carbamazepine had to be increased to Neurology, Maasland (EPH), hemicrania continua, and the cluster- 600 mg/day because of recurrent neuralgiform Hospital, PO Box 5500, tic syndrome—as trigeminal-autonomic cepha- pain which was now associated with ipsilateral 6130 MB Sittard, The 3 Netherlands lalgias. This terminology recognises the physi- lacrimation. Three years later he presented to H W M ter Berg ology of the trigeminal-autonomic vasodilator one of us (HTB). reflex4 which has as its aVerent the ophthalmic On presentation the patient had left sided Institute of Neurology, (first) division of the trigeminal nerve and as its sharp, excruciating pain with a duration of 2–3 The National Hospital eVerent the cranial parasympathetic outflow minutes and a frequency of one to five times a for Neurology and through the VIIth (facial) nerve. day in association with nausea, ipsilateral lacri- Neurosurgery, Queen Square, London Clinical descriptions of SUNCT suggest a mation, facial flushing, conjunctival injection, WC1N 3BG, UK pain duration of between 5–250 seconds and and bilateral rhinorrhoea. After some months P J Goadsby frequencies of one attack/day to 30 attacks in 1 the duration increased to 30–60 minutes, and hour. The orbitotemporal pain localisation in the pain quality changed to a deep excruciat- Correspondence to: SUNCT is in the area of the first branch of the ing, boring pain with 10–15 episodes a day of Dr JWM ter Berg [email protected] trigeminal nerve. In some cases the diVerentia- superimposed lancinating pain. There were no tion between SUNCT and trigeminal neural- triggers. Neuralgiform attacks with a duration Received 12 June 2000 and gia may be diYcult5 and a case with transfor- of about 1 minute recurred in association with in revised form 3 October 2000 mation of trigeminal neuralgia into SUNCT the autonomic symptoms. The patient devel- Accepted 4 October 2000 syndrome is illustrative of a possible con- oped triggers of sudden exposure to light and

www.jnnp.com J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.70.2.244 on 1 February 2001. Downloaded from Signiﬁcance of atypical presentation of symptomatic SUNCT 245

Figure 1 Midline sagittal T1 weighed 1 Tesla MRI of the cranium and craniocervical junction of the patient with osteogenesis imperfecta and symptomatic SUNCT.Note the severe platybasia with marked basilar impression and distortion of the posterior fossa anatomical relations. The odontoid process produces pronounced pontomedullary angulation Frontotemporal atrophy and ventricular enlargement are also visible.

Figure 2 Coronal T1 weighed MRI of the same patient intensive sounds, chewing, talking, and swal- showing a mass which is a part of the cerebellum located at lowing. He stayed for weeks in a dark environ- the left side of the pons (see white arrow). ment because of provocation by both light exposure and photophobia. Increasing car- by necropsy, which also confirmed the known bamazepine therapy to 1800 mg/day had only macroscopical intracranial deformations (fig temporary benefit. The trigger factors ex- 2). No abnormalities could be found by micro- panded to include upward positioning of the scopy in the area of the posterior structures and head and trunk which also caused vertical and the trigeminal system on the pontomesen- oblique diplopia, symptoms that could be cephalic level. improved by lying down. Between attacks there was a continuous mild left sided periorbital and Discussion auricular pain with low frequency ipsilateral The case is remarkable for both the symptoms blepherospasm. and the possible mechanisms of the presenta- Repeated neurological examination was nor- tion of a SUNCT-likesyndrome. We diagnosed mal, particularly coordination. Brain CT and this case as SUNCT-like syndrome because of MRI of the skull showed platybasia with the many short lasting attacks and the promi- marked basilar impression, bilateral frontotem- nent autonomic symptoms. The modest but poral atrophy, ventricular enlargement, and present eVect of carbamazepine and the nature marked pontomedullary angulation in associ- of the patient’s cranial anatomy with the ation with a left sided mass between the pons burden of abnormality falling on the posterior http://jnnp.bmj.com/ and cerebellum, defined as a part of a cerebel- fossa, are both interesting aspects of the case. lar lobe at the level of the trigeminal and facial The initial symptoms of this patient were nerve (fig 1). Blink reflex studies disclosed an similar to trigeminal neuralgia although it did absent left sided R1 response with left sided involve both the first and second trigeminal masseter reflex, and a normal right sided R1 divisions and the episodic pain duration and bilateral R2 response, indicating a V-VII increased up to 2–3 minutes, which would be lesion. Brain stem auditory evoked potential unusual in typical trigeminal neuralgia. This on September 26, 2021 by guest. Protected copyright. was inconclusive because of the inner ear overlap is somewhat similar to the cluster-tic lesions. Visual evoked potential was normal. syndrome.10 The triggering of pain attacks by A tentative diagnosis of symptomatic atypi- photostimulation is noteworthy and there is no cal SUNCT syndrome was made. During his obvious explanation. Prominent ipsilateral au- illness he was treated with carbamazepine (up tonomic features, which are an essential to 1800 mg/day), phenytoin (200 mg/day), component of SUNCT, do occur in the course verapamil (120 mg/day), sumatriptan (6 mg of this patient’s disease as the symptoms evolve. subcutaneously), amitriptyline (50 mg/day), The late occurrence of triggers and autonomic indomethacin (75 mg/day), lidocaine (4% in- features may be caused by the relentless tranasally), methysergide (3 mg/day), valproate progressive basilar impression and associated (900 mg/day), and also electrocoagulation of pontomedullary compression. the pterygopalatine ganglion. Trigeminal ther- Osteogenesis imperfecta is a heterogeneous mocoagulation was not possible for anatomical disorder of connective tissue. On clinical reasons. Operative treatment was considered grounds the patient could be classified as type but the patient thought the operative risks too III, because of the very severe progressive great. None of the treatments resulted in deformity of the long bones and spine.11 The acceptable pain reduction except car- adult onset conductive hearing loss is more bamazepine in the early phase of the illness. compatible with type I, which has less bone The patient died when nearly 50 years old deformity. However, some overlap between because of coronary heart disease, ascertained clinical types may occur.12 Neurological com-

www.jnnp.com J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.70.2.244 on 1 February 2001. Downloaded from 246 ter Berg, Goadsby

plications of osteogenesis imperfecta include In summary, a case of secondary, sympto- basilar impression with brainstem compres- matic, SUNCT syndrome in a patient with sion, macrocephaly, cortical atrophy, increased osteogenesis imperfecta is reported. The poste- intracranial pressure caused by hydrocepha- rior fossa abnormalities emphasise the absolute lus,12 and trigeminal neuralgia.13 Pathophysi- need for MRI in any suspected case of SUNCT ological mechanisms associated with basilar as all symptomatic patients have had findings in impression may include impaired CSF outflow, the posterior fossa. SUNCT in all its forms, but deformation of the skull base, forces generated particularly when there are overlaps with by neck muscles to keep the head upright, and trigeminal neuralgia at some clinical level, venous outflow obstruction through the skull underscores the importance of the trigeminal base.12 autonomic reflex and its role in primary head- The association of short lived unilateral pain ache syndromes. and ipsilateral autonomic features in the primary headache disorders, such as cluster 1 Sjaastad O, Saunte C, Salvesen R, et al. Shortlasting unilat- headache, paroxysmal hemicrania, SUNCT, eral neuralgiform headache attacks with conjunctival injec- and cluster-tic syndrome was recently classified tion, tearing, sweating, and rhinorrhea. Cephalalgia as trigeminal autonomic cephalgias (TACs).3 1989;9:147–56. 2 Pareja JA, Sjaastad O. SUNCT syndrome. A clinical review. In our patient without any neurological feature Headache 1997;37:195–202. with exception of SUNCT, both compression 3 Goadsby PJ, Lipton RB. A review of paroxysmal hemicra- nias, SUNCT syndrome and other short-lasting headaches and traction forces at the level of the brainstem with autonomic features, including new cases. Brain 1997; may have been relevant to the clinical presenta- 120:193–209. 4 May A, Goadsby PJ. The trigeminovascular system in tion. It is remarkable that the three known humans: pathophysiological implications for primary head- symptomatic patients presented with long ache syndromes of the neural influences on the cerebral circulation. J Cereb Blood Flow Metab 1999;19:115–27. standing cerebellopontine angle pathology, an 5 Benoliel R, Sharav Y. Trigeminal neuralgia with lacrimation arteriovenous malformation,7 cavernous he- or SUNCT syndrome? Cephalalgia 1998;18:85–90. 6 Bouhassira D, Attal N, Esteve M, et al. SUNCT syndrome. mangioma in the anterolateral part of the pons A case of transformation from trigeminal neuralgia. Cepha- in close proximity to the trigeminal nerve root lalgia 1994;14:168–70. 9 7 Bussone G, Leone M, Volta GD, et al. Short-lasting unilat- entry zone, and a venous angioma described eral neuralgiform headache attacks with tearing and as being in the region of the brainstem and conjunctival injection: the first symptomatic case. Cephala- 8 lgia 1991;11:123–7. right cerebellar hemisphere. In our patient 8 Morales F, Mostacero E, Marta J, et al. Vascular malforma- there was a longstanding gradually progressive tion of the cerebellopontine angle associated with SUNCT syndrome. Cephalalgia 1994;14:301–2. parenchymal distortion by the basilar impres- 9 De Benedittis G. SUNCT syndrome associated with sion which subsequently may have caused an cavernous angioma of the brain stem. Cephalalgia 1996;16: 503–6. ephaptic transmission disturbance. It could be 10 Mulleners WM, Verhagen WIM. Cluster-tic syndrome. suggested that this alone, or more likely, in Neurology 1996;47:302. 11 Sillence DO. Craniocervical abnormalities in osteogenesis combination with an underlying genetic predis- imperfecta: genetic and molecular correlation. Pediatr position, is the likely basis for the headache Radiol 1994;24:427–30. 12 Charnas LR, Marini JC. Communicating hydrocephalus, syndrome The initial transient eVect of car- basilar invagination, and other neurological features in bamazepine supports the relation with trigemi- osteogenesis imperfecta. Neurology 1993;43:2603–8. 13 Reilly MM, Valentine AR, Ginsberg L. Trigeminal neuralgia nal neuralgia and has been also reported in two associated with osteogenesis imperfecta. 67 J Neurol Neurosurg other symptomatic SUNCT cases. Psychiatry 1995;58:665. http://jnnp.bmj.com/ on September 26, 2021 by guest. Protected copyright.