Clinical Features and Longterm Prognosis of Trochlear Headaches

European Journal of Neurology 2013 doi:10.1111/ene.12312

Clinical features and long-term prognosis of trochlear headaches

J. H. Smitha, J. A. Garrityb and C. J. Boesc aDepartment of Neurology, University of Kentucky, Lexington, KY; bDepartment of Ophthalmology, Mayo Clinic, Rochester, MN; and cDepartment of Neurology, Mayo Clinic, Rochester, MN, USA

Keywords: Background and purpose: Trochlear headaches are a recently recognized cause of chronic daily headache, headache, of which both primary and inflammatory subtypes are recognized. The migraine, neuro- clinical features, long-term prognosis and optimal treatment strategy have not been ophthalmology, ocular well defined. movements, secondary Methods: A cohort of 25 patients with trochlear headache seen at the Mayo Clinic headache disorders, between 10 July 2007 and 28 June 2012 were identified. trochlea, trochleitis Results: The diagnosis of trochlear headache was not recognized by the referring neurologist or ophthalmologist in any case. Patients most often presented with a new Received 13 September 2013 daily from onset headache (n = 22, 88%). The most characteristic headache syndrome Accepted 21 October 2013 was reported as continuous, achy, periorbital pain associated with photophobia and aggravation by eye movement, especially reading. Individuals with a prior history of migraine were likely to have associated nausea and experience trochlear migraine. Amongst individuals with trochleitis, 5/12 (41.6%) had an identified secondary mechanism. Treatment responses were generally, but not invariably, favorable to dexamethasone/lidocaine injections near the trochlea. At a median follow-up of 34 months (range 0–68), 10/25 (40%) of the cohort had experienced complete remission. Conclusions: Trochlear headaches are poorly recognized, have characteristic clinical features, and often require serial injections to optimize the treatment outcome. The identification of trochleitis should prompt neuroimaging to look for a secondary cause.

Introduction generally thought to be associated with a positive response. The trochlea is a saddle-like cartilaginous structure Unfortunately, only a very limited number of cases located in the superomedial orbit, which contains the have been reported in the literature [3–5]. Therefore, tendon of the superior oblique muscle. Histologically, information regarding the clinical features, treatment the trochlea is surrounded by a synovial membrane, and prognosis is limited. To help clarify these issues, analogous to the structure of a joint [1]. The trochlea our experience with trochlear headache in 25 previ- is innervated by an ophthalmic nerve branch and is ously unreported cases is reported. capable of generating pain in the setting of trochleitis, which is most often idiopathic, but may develop Methods secondary to autoimmune connective tissue disorders [2]. Trochleitis is characterized by swelling and tender- Prior to the initiation of the study, Mayo Clinic Insti- ness of the trochlea, and aggravation by vertical tutional Review Board approval was obtained. The ductions. Swelling may be observed clinically or docu- procedural records of one of the investigators (JAG) mented on orbital imaging studies [2]. A non-inﬂam- was searched from 10 July 2007 to 28 June 2012 to matory condition, termed primary trochlear headache identify all adult (age ≥ 18) patients who had received (PTRH), has also been described [3]. Treatment of all a trochlear injection. The electronic medical records trochlear headaches is presumed to require local were manually abstracted to identify individuals where steroid injection in the vicinity of the trochlea, and is a diagnosis of a trochlear headache could be deﬁni- tively assigned and adequate clinical documentation Correspondence: J. H. Smith, Department of Neurology, University was available. of Kentucky, 740 S. Limestone, L445, Lexington, KY 40503, USA (tel.: 859-323-5661; fax: 859-323-5943; e-mail: jonathan.smith@uky. In our paper, the general term trochlear headache is edu). used to describe any headache referable to the troch-

© 2013 The Author(s) European Journal of Neurology © 2013 EFNS 1 2 J. H. Smith, J. A. Garrity and C. J. Boes lear apparatus. Given the lack of validated criteria, sheath meningioma. One individual developed the specific diagnoses of PTRH and trochlear migraine headache 6 months following removal of an orbital were assigned based on previously suggested dermoid tumor (case 5), 1 month following orbital definitions [3,5]. Trochlear migraine refers to a troch- decompression for Graves ophthalmopathy (case 6), lear headache which then triggers a secondary 3 months post-partum (case 11) and 1 month follow- migraine attack [2,5]. The diagnostic criteria for head- ing a Roux-en-Y gastric bypass (case 13). No enlarge- ache secondary to trochleitis were used as suggested ment of the superior oblique muscle had been by the newly published International Classification of radiographically noted in case 6. There were no com- Headache Disorders, 3rd edition (online beta version) plications reported in review of any of the above men- [6]. A diagnosis of secondary trochleitis was assigned tioned procedures. if there was clinical, radiographic and/or histopatho- Trochlear headaches had an acute, daily from logical evidence for orbital localization of an inflam- onset, presentation in the majority of cases (n = 22, matory or neoplastic process. The diagnosis of either 88%), being subacute, daily within 1 month, in the trochleitis or PTRH could be supported by aggrava- remainder. A final diagnosis of PTRH (n = 13, 52%) tion and/or reproduction of pain by action of the was made slightly more often than trochleitis (n = 12, superior oblique muscle. 48%). Bilateral involvement was seen in 8/13 (61.5%) Patients were excluded if an alternative diagnosis cases of PTRH and in 4/12 (33.3%) cases of trochle- for the headache was established, such as a periorbital itis. In all bilateral cases this always occurred sequen- cranial neuralgia, a trigeminal autonomic cephalalgia tially, and within 1 year of onset. Amongst patients or carotid-cavernous fistula. with trochleitis, five (41.6%) had an identified second- Acuity of onset was summarized as acute if the ary mechanism. These included incomplete Behcet’s headache was daily from onset, subacute if daily (n = 1), idiopathic TolosaÀHunt (n = 1), granuloma- within 1 month of onset, and insidious if daily within tosis polyangiitis (GPA) (n = 2) and orbital lymphoma 1 year of onset. Due to the retrospective nature of the (n = 1). All five cases of secondary trochleitis under- study, treatment efficacy was summarized as complete, went biopsy of an orbital mass located near the troch- partial or ineffective based on the information avail- lea. In case 11, the trochlear headache had been the able in the medical record. If the patient reported a presenting symptom of GPA. Secondary trochleitis quantitative indicator of pain relief, this information was most often bilateral, but was observed unilaterally was specifically recorded. in one individual (case 11). The location of the trochlear headache was most Results often focused at the medial eyebrow, orbit or forehead (n = 9, 36%), with radiation into the forehead, tem- Over a 5-year period of chart review, 25 individuals ple, periorbit or retro-orbitally. The pain was reported were identified with sufficient clinical characterization as continuous (n = 25, 100%), and achy, dull or pres- for study inclusion (Table 1). Patients were generally sure-like (n = 19, 76%). The average intensity as rated female (n = 20, 80%), with a median age at diagnosis on a numerical rating scale was severe (7–10) in seven of 46 (range 18–77). Patients had a median time from (35%), moderate (4–6) in 10 (50%) and mild (0–3) in symptom onset to diagnosis of 6.7 months (range three (15%) cases. The most common associated 2 weeks to 10 years). The diagnosis of a trochlear symptoms were photophobia (n = 15, 68.1%) and bin- headache had not been made in any patient prior to ocular diplopia (n = 10, 45.4%). Four out of the five referral, despite all patients having been seen previ- patients who reported associated nausea also had co- ously by either a neurologist or ophthalmologist prior morbid migraine. The headache was characteristically to presentation. The most common mis-diagnoses aggravated by eye movements (especially reading) by prior to presentation were chronic migraine (n = 13), 18 (75%) patients. new daily persistent headache (n = 5), no diagnosis Amongst individuals with a prior diagnosis of (n = 4), hemicrania continua (n = 2) and atypical migraine (n = 7), six were considered to have attacks facial pain (n = 1). of trochlear migraine following the onset of the new All patients with a prior headache diagnosis self- headache syndrome. These headaches were reported reported a new superimposed pain syndrome, which to be ipsilateral to the trochlear pain. was very apparent to all patients as distinct. Anteced- Diagnostic evaluation consisted of either a magnetic ent events were only occasionally noted by patients. resonance imaging (MRI) of the head or a computed The most concrete association was in case 3, where a tomography (CT) study of the orbits in all patients new daily trochlear headache began immediately fol- except one (case 13). Three patients were found to lowing orbital surgery for removal of an optic nerve have an imaging abnormality directly involving the

Case Acuity Continuous Quality/ Associated Aggravating Relieving Relevant no. Age/gender Diagnosis/laterality of onset or episodic Location Radiation intensity symptoms factors factors comorbidity

1 55/F PTRH, TM/R Acute Continuous Orbit None Pressure/6 NA, PHT ‘Reading’ None CMA 2 29/M Trochleitis, TM/Bil Acute Continuous Orbit, medial None Throb/6 NA, PHT, DIP EM None EMO 3 77/F PTRH/R Acute Continuous Medial None Ache NR None None Left optic nerve eyebrow meningioma 4 34/F PTRH, TM/Bil Acute Continuous Periorbital Holocephalic Throb NA, PHT, DIP None None EMO, IED

03EFNS 2013 dermoid 6 57/F PTRH/Bil Acute Continuous ‘Eye’ NR NR/7 DIP EM NR Graves ophthalmopathy s/p decompression 1 month prior to onset 7 38/F Trochleitis, Acute Continuous Orbit None Ache/7 NA, DIP EM None CMO incomplete Behcet’s/Bil 8 36/F Trochleitis/ Acute Continuous Periorbital Forehead Pressure/7 NA, DIP EM Eye patch TolosaÀHunt/Bil 9 63/M Trochleitis/R Acute Continuous Periorbital None Ache/8 NR None None History of encephalitis in childhood, unknown cause 10 18/F Trochleitis, TM/L Acute Continuous Medial None NR NR NR NR EMO eyebrow 11 56/F Trochleitis, GPA/L Acute Continuous Eyebrow None Ache/2 PHT, DIP EM None GAD 12 42/F PTRH/R Acute Continuous Medial Forehead Pressure/5 NR EM None eyebrow 13 71/F PTRH/Bil Acute Continuous Periorbital None Dull/3 PHT EM Warmth History of gastric bypass 14 65/F Trochleitis/Bil Acute Continuous Retroorbital Eyebrow Pressure, sharp/4 PHT, DIP EM None 15 57/F PTRH/R Acute Continuous Retroorbital None Pressure/4 PHT EM Warmth headaches trochlear of Prognosis 16 40/F Trochleitis/R Acute Continuous Medial Retro-orbital Pressure/4 PHT, DIP EM None forehead 17 74/F PTRH/Bil Acute Continuous Medial Forehead, Pressure/6 PHT EM Warmth EMO, depression eyebrow temple 18 58/F PTRH/Bil Acute Continuous Medial None Ache PHT, PHN Touch None forehead 19 46/F PTRH, TM/Bil Acute Continuous Medial Forehead, Pressure/4 PHT EM None Depression eyebrow bridge of nose 20 23/F Trochleitis, GPA/L Acute Continuous Eyebrow Orbit Throb/7 PHT, DIP EM None GPA 21 48/M Trochleitis/L Acute Continuous Orbit None Pressure/2 None EM None ETTH 3 4 J. H. Smith, J. A. Garrity and C. J. Boes

trochlear apparatus (Fig. 1). In the patient with Tol- osaÀHunt (case 8), no radiographic abnormalities were visualized in the orbit. Two other patients were noted to have radiographic enlargement of the lacri- Radiation-

+ mal glands (cases 11 and 16). Clinical examination was generally unremarkable except for trochleodynia anticardiolipin antibody induced retinopathy and optic neuropathies (1 year before onset) Relevant comorbidity GBM, s/p WBRT (all cases), clinically apparent trochlear edema (all cases of trochleitis) and ﬁndings attributable to neuro- ophthalmic comorbidities (Table 1).

closure The treatment outcomes are summarized in Table 2. Relieving factors

ut aura; ETTH, episodic tension-type –

. Overall, at a median follow-up of 34 months (range 0 68), 10 patients had reported complete remission, 10 disorder; L, left; MALT, mucosa-associated continued to have either persistent or recurrent trochlear headache but with signiﬁcantly improved pain lev- Aggravating factors None None History of els and four had not experienced any substantial improvements at all. Treatments with standard migraine preventive medications were generally ineﬀec- tive (Table 2). Complete remission was achieved with = blurry vision immunotherapy (n 2) or external beam radiation Associated symptoms (n = 1) in cases of secondary trochleitis. Trochlear injections were performed in a non-standardized way with a single injection, generally containing 2–3 mg of dexamethasone, with or without triamcinolone, and lidocaine. When reported, responses occurred either

Quality/ intensity immediately (n = 4) or within 7 days (n = 5). Following the injection, patients reported complete resolution of trochlear pain (n = 10), partial improvement (n = 12) or no effect (n = 8). When effective, the reported dura- tion of effect was of the order of weeks to months (range 12 h to 12 months) (Table 2). The median number of Forehead Pressure/6 PHT None Eye injections per patient was three (range 1–12). Amongst the 17 individuals receiving repeat injections several patterns of response were seen. Most commonly, an ini-

eyebrow tially effective injection would continue to have either a similar (n = 6) or increased (n = 4) benefit on subsequent injections. Of patients with an initially ineffective first injection, subsequent injections were either ineffective (n = 5) or subsequently effective (n = 2). Overall, Continuous or episodic Location Radiation the only adverse event noted was injection site bruising in one case. Acuity of onset Subacute Continuous Periorbital Hemicranial/neck Throbbing/7 PHT, Discussion

In our specialty headache practice, trochlear headaches were very poorly recognized by referring neurology and ophthalmology physicians. The 25 patients in our cohort presented with a fairly characteristic clini-

MALT lymphoma/L cal syndrome of moderately severe, achy, periorbital

) (especially medial) pain, variably associated with photophobia, and aggravation by eye movements (especially reading). Individuals with a prior history Continued ( of migraine were likely to have associated nausea and experience trochlear migraine as part of their clinical lymphoid tissue; NA, nausea; NR, not reported; PHN, phonophobia; PHT, photophobia; R, right TM, trochlear migraine; WBRT, whole brain radiotherapy 25 39/FBil, bilateral; CMA, chronicheadache; migraine GAD, with generalized aura; anxiety CMO, disorder; chronic GBM, migraine PTRH/BilIntensity glioblastoma without refers aura; multiforme; to what DIP, GPA, the diplopia; granulomatosis patient EM, reported with eye as polyangiitis; movements; average IED, on EMO, a intermittent episodic numerical explosive migraine rating witho scale from 0 to 10. Subacute Continuous Retroorbital None Dull/7 None EM None None 24 41/F Trochleitis, orbital Table 1 Case no. Age/gender Diagnosis/laterality 2223 35/F 48/M Trochleitis, TM/L PTRH/L Subacute Continuous Periorbital Acute Eyebrow Continuous Medial Pressurecourse. PHT, DIP EM It is notable None EMO that the daily from onset

(a) (b)

Figure 1 (a) Coronal computed tomography (CT) of the orbits showing calcifi- cation of the right trochlear tendon (c) (d) (solid arrow) and thickening of the left trochlea tendon (broken arrow) (case 21). (b) Magnetic resonance imaging (MRI) of the orbits showing gadolinium uptake at the left trochlear tendon (arrow) (case 21). (c) Axial T2-weighted fluid attenuated inversion recovery (FLAIR) MRI of the head showing dif- fuse left orbital involvement by GPA (arrow) (case 20). (d) Axial CT of the head with contrast showing lymphoma- tous involvement of the left trochlear tendon (arrow) (case 24). presentation could be easily confused with new-daily which may represent a topographically restricted form persistent headache, which is generally thought of as of persistent idiopathic facial pain [8]. extremely difficult to treat [7]. However, the preva- The largest case series of trochleitis was provided lence of undiagnosed trochlear headache in patients by Tychsen et al. in 1984, who reported on 13 patients with presumed new-daily persistent headache has not with a syndrome of subacute onset inflammatory tro- been studied. chleodynia [4]. Ages ranged from 27 to 69, and 9/13 In our cohort, patients were almost as likely to were women. Three patients developed recurrences receive a diagnosis of PTRH as trochleitis. However, over a mean 8 months of follow-up. Two previously the distinction had important implications, as 5/12 anophthalmic patients underwent excisional biopsy, (41.6%) patients with trochleitis (clinically apparent demonstrating perivascular lymphocytic infiltration trochlear edema) had an identified secondary mecha- involving not only the tendon but also extending into nism. It is therefore concluded that all individuals the superior oblique muscle itself. It was hypothesized with trochleitis should undergo diagnostic imaging, that the condition represented a localized form of but CT versus MRI cannot be recommended based orbital pseudotumor. In our cohort, neuroimaging on our limited experience. Dedicated orbital imaging abnormalities were confined to the trochlear tendon in is likely to be important, on the basis that the trochlea idiopathic cases and never involved the superior obli- is often not visualized on routine head imaging. que muscle. Based on our retrospective data, dexamethasone/lido- Limitations of our study included possible selection caine injections of the trochlea appeared to be a gener- bias, as consecutive cases were not included, and lack ally efficacious strategy for many patients. As many of a standardized approach to evaluation and treat- patients reported incremental success (and at times ment. Strengths of our study include the relatively remission) with serial injections, at least a second round large number of patients, long-term follow-up data of of injection is recommended for patients who are ini- treatment outcomes, and fairly complete clinical data tially non-responders. This is especially important as for the cohort. many of these patients do not seem to respond robustly In conclusion, the diagnosis of a trochlear headache to typical migraine preventive treatments. In cases of should be considered especially in patients presenting secondary trochleitis, treatments directed at the disease- with a new daily eye pain, aggravated by eye move- specific process were generally efficacious. The reasons ments, especially reading. Our data provides a new for treatment success in some patients but not others are perspective on long-term treatment outcomes in this not known. An alternative diagnostic consideration poorly recognized, but important, headache syn- amongst refractory cases is idiopathic ophthalmodynia, drome.

© 2013 The Author(s) European Journal of Neurology © 2013 EFNS 6 Table 2 Treatment outcomes of patients with trochlear headache .H mt,J .GriyadC .Boes J. C. and Garrity A. J. Smith, H. J. Time from Prior treatments Eﬃcacy of treatments Total number Injected material Onset of diagnosis to Case other than other than trochlear of trochlear (total volume 1 ml action of Outcome of last follow-up Outcome at no. trochlear injection injections injections for all cases) injections injections (months) last follow-up

1 a. Indomethacin 50 mg TID a. Partial 1 2 mg dexamethasone, <1 week 90% reduction 62 Remission b. Botulinum toxin b. Partial 20 mg triamcinolone with in pain intensity A injections, c. Ineffective 0.25 ml 2% lidocaine 150 units 9 1 c. Melatonin 3 mg 2 a. Nortriptyline 25 mg a. Ineffective 7 3 mg dexamethasone with <1 week 50% reduction 8 Improved, recurrent 0.25 ml 2% lidocaine in pain intensity trochleitis with epinephrine lasting 4 weeks 3 a. Ibuprofen 600 mg, a. Partial 1 3 mg dexamethasone with NR Complete relief 39 Remission as needed 0.25 ml 2% lidocaine with epinephrine 4 a. Nortriptyline 75 mg a. Ineffective 1 3 mg dexamethasone with Immediate Partial relief 7 Chronic trochlear 0.25 ml 2% lidocaine with for only 12 h headache epinephrine 5 None NA 3 2 mg dexamethasone, NR Complete relief, 47 Remission 20 mg triamcinolone with durability of 0.25 ml 2% lidocaine 7 months, then 12 months, and then ongoing relief 6 None NA 1 4 mg dexamethasone NR Complete relief 10 Remission 7 a. OxyContin 30 mg a. Partial 12 2 mg dexamethasone, 40 mg 2–4 days Injections 1–8, 10: 27 Improved, persistent twice daily triamcinolone with 0.25 ml partial relief, average trochleitis 2% lidocaine durability of 22 days Injections 9, 11–12: ineffective uoenJunlo Neurology of Journal European 8 a. Amitriptyline 50 mg a. Ineffective 8 3 mg dexamethasone, Immediate Partial relief, 42 Remission b. Gabapentin 1800 mg b. Ineffective 40 mg triamcinolone durability c. Intravenous c. Ineffective with 0.25 ml 2% lidocaine range 2–5 days methylprednisolone d. Ineffective 500 mg e. Ineffective d. Dilaudid, as needed f. Ineffective e. Methotrexate g. Complete f. Azathioprine ©

03TeAuthor(s) The 2013 g. Inﬂiximab 9 None NA 1 3 mg dexamethasone, 0.125 ml Immediate Partial None Unknown

© 2% lidocaine with epinephrine 03EFNS 2013 10 a. Nortriptyline 50 a. Improved migraines, 1 3 mg dexamethasone, 0.25 ml Immediate Complete 25 Remission but not trochleitis pain 2% lidocaine with epinephrine uoenJunlo Neurology of Journal European © 03TeAuthor(s) The 2013 Table 2 (Continued )

Time from Prior treatments Eﬃcacy of treatments Total number Injected material Onset of diagnosis to Case other than other than trochlear of trochlear (total volume 1 ml action of Outcome of last follow-up Outcome at no. trochlear injection injections injections for all cases) injections injections (months) last follow-up

11 a. Propranolol 120 mg a. Ineﬀective 5 3 mg dexamethasone, Unknown 1–2, 4–5: partial, 30 Remission b. Gabapentin 1800 mg b. Ineﬀective 0.25 ml 2% lidocaine durability range

03EFNS 2013 d. Azathioprine d. Partial 3: Ineffective e. Rituximab e. Partial f. External beam f. Complete radiation (2000 cGy in 10 fractions) 12 a. Indomethacin a. Partial 18 3 mg dexamethasone, Unknown 1–3, 5–7, 9–1: partial, 32 Improved, recurrent 50 mg three b. Partial 0.25 ml 2% lidocaine durability range trochlear headache times daily with epinephrine 2–6 weeks, except b. Acetazolamide 250 mg last two injections twice daily lasted 5, then 7 months 4, 8: ineffective 13 a. Prednisone a. Complete 3 2 mg dexamethasone, Unknown Complete, durability 68 Improved, recurrent b. Azathioprine b. Partial 0.1 ml 2% lidocaine 9–12 months trochlear headache with epinephrine 14 None NA 5 3 mg dexamethasone, Unknown Complete, durability 20 Improved, recurrent 0.25 ml 2% lidocaine of 3–4 months trochleitis with epinephrine 15 a. Amitriptyline a, b. Unable to 3 3 mg dexamethasone, and NA Ineffective 34 Chronic trochlear b. Carbamazepine tolerate either 0.25 ml 2% lidocaine with headache epinephrine 16 None NA 4 2 mg dexamethasone, Unknown 1–3: partial, durability 44 Persistent trochleitis 20 mg triamcinolone range of 8–12 months 0.25 ml 2% lidocaine 4: ineffective 17 None NA 2 2 mg dexamethasone, 20 mg Unknown 1, 2: partial, durability 6 Improved, recurrent headaches trochlear of Prognosis triamcinolone 0.25 ml 2% of 2 months each trochlear headache lidocaine 18 None NA 1 3 mg dexamethasone, Unknown Complete 37 Remission 0.125 ml 2% lidocaine with epinephrine 19 a. Topiramate 100 mg a. Ineffective 2 3 mg dexamethasone, 0.25 ml 10 days 1, 2: complete 1 Improved, low-grade b. Amitriptyline 40 mg b. Ineffective 2% lidocaine with epinephrine (right eye), partial (2/10) trochlear c. Prednisone 40 mg c. Partial (left eye) headache on left side, remission on right side 7 8

Table 2 (Continued ) Boes J. C. and Garrity A. J. Smith, H. J.

20 a. Oxycodone 15 mg a. Partial 2 1 mg dexamethasone, Unknown 1: ineffective 49 Chronic trochlear b. Methotrexate 0.5 ml b. Ineffective 0.1 ml 2% lidocaine 2: partial headache per week c. Partial with epinephrine c. Cyclophosphamide d. Partial d. Rituximab e. Partial e. Prednisone 60 mg 21 None NA 3 3 mg dexamethasone, Unknown 1, 2: ineffective 16 Remission 0.1 ml 2% lidocaine 3: complete with epinephrine 22 a. Indomethacin 75 mg a–f, i. Ineffective 6 3 mg dexamethasone, Unknown Partial, durability 64 Improved, low-grade three times daily g. Partial 20 mg triamcinolone, 2–3 weeks (1-2/10) persistent b. Amitriptyline 50 mg h. Near-complete 0.25 ml 2% lidocaine trochlear headache c. Gabapentin 1800 mg d. Propranolol 80 mg e. Topiramate 50 mg f. Botulinum Toxin A injections, 150 units 9 2 g. Prednisone 80 mg h. Methotrexate i. External beam radiation (25 CGy over 14 fractions) 23 None NA 4 1–3: 2 mg dexamethasone, 20 mg <7 days Complete, durability 57 Remission triamcinolone and 0.25 ml 2% of 1–2: 3 months, 3: lidocaine with 6 months, 4: remission uoenJunlo Neurology of Journal European epinephrine 4: 4 mg dexamethasone, 20 mg triamcinolone, 0.25 ml 2% lidocaine with epinephrine 24 a. Nortriptyline 50 mg a. Partial 1 3 mg dexamethasone, NA Ineffective 40 Chronic trochlear b. Prednisone 60 mg b. Partial 0.25 ml 2% lidocaine headache

03TeAuthor(s) The 2013 25 None NA 7 3 mg dexamethasone, <7 days Partial, average 54 Recurrent trochlear 0.2 ml 2% lidocaine durability of 6 weeks headache © with epinephrine 03EFNS 2013

NA, not applicable. Prognosis of trochlear headaches 9

Disclosure of conflicts of interest 4. Tychsen L, Tse DT, Ossoinig K, Anderson RL. Trochle- itis with superior oblique myositis. Ophthalmology 1984; The authors declare no financial or other conflicts of 91: 1075–1079. interest. 5. Yanguela J, Pareja JA, Lopez N, Sanchez Del Rio M. Trochleitis and migraine headache. Neurology 2002; 58: 802–805. References 6. The International Classification of Headache Disorders, 3rd edition (beta version). Cephalalgia 2013; 33: 629–808. 1. Helveston EM, Merriam WW, Ellis FD, Shellhamer RH, 7. Robbins MS, Grosberg BM, Napchan U, Crystal SC, Gosling CG. The trochlea. A study of the anatomy and Lipton RB. Clinical and prognostic subforms of new – physiology. Ophthalmology 1982; 89: 124 133. daily-persistent headache. Neurology 2010; 74: 1358– 2. Pareja JA, Sanchez del Rio M. Primary trochlear head- 1364. ache and other trochlear painful disorders. Curr Pain 8. Pareja JA, Cuadrado ML, Porta-Etessam J, et al. Idio- – Headache Rep 2006; 10: 316 320. pathic ophthalmodynia and idiopathic rhinalgia: two 3. Yanguela J, Sanchez-del-Rio M, Bueno A, et al. Primary topographic facial pain syndromes. Headache 2010; 50: trochlear headache: a new cephalgia generated and modu- 1286–1295. lated on the trochlear region. Neurology 2004; 62: 1134– 1140.