JOBNAME: No Job Name PAGE: 1 SESS: 10 OUTPUT: Fri Jan 8 14:22:36 2010 /v2451/blackwell/journals/head_v0_i0/head_1613 Headache ISSN 0017-8748 © 2010 the Authors doi: 10.1111/j.1526-4610.2010.01613.x Journal compilation © 2010 American Headache Society Published by Wiley Periodicals, Inc. 1 Expert Opinion 2 3 4 Trochleodynia and Migrainehead_1613 1..5 5 6 Randolph W. Evans, MD; Juan A. Pareja, MD 7 8 (Headache 2010;50:••-••) 9 10 11 The small region of the superior oblique muscle tearing or redness of the eye, right-sided ptosis, or 44 12 pulley (trochlea) may be the source of a distinctive nares congestion or drainage. 45 13 pain (trochleodynia) originated in the superior There was a many-year history of bilateral 46 14 oblique muscle-tendon-trochlea complex.1 Trochleo- migraine without aura occurring about once a month 47 15 dynia is mostly felt in the inner-upper angle of the and migraine aura without headache occurring 1-2 48 16 symptomatic orbit and may extend to the ipsilateral times per year. 49 17 forehead. Trochleodynia is commonly produced by His primary care physician placed him on an 50 18 trochleitis (primary or symptomatic)2,3 or primary tro- antibiotic for a presumed sinus infection without 51 19 chlear headache.4 Furthermore, trochleodynia may be improvement. An ENT physician obtained a CT scan 22 52 20 a trigger, common to different headaches. Concur- of the sinuses with normal findings.An Ophthalmolo- 53 21 rency with migraine may bring about a syndrome of gist found a normal exam except for 1 mm of left 54 22 trochlear migraine1,3,4 with important therapeutic eyelid ptosis with normal pupils. The first neurologist 55 23 implications. Trochleodynia has also been associated found a normal exam and obtained a normal MRI of 56 24 with tension-type headache4 and paroxysmal hemic- the brain with and without contrast, and a Westergren 57 25 rania5 and may, in the future, be an integral part erythrocyte sedimentation rate with normal findings. 58 26 of the pathogenic mechanisms of other concurrent He was tried on loratidine, mometasone furoate 59 27 headaches. monohydrate nasal spray, montelukast sodium, and 60 28 gabapentin without benefit. 61 29 CASE 1 There was a past medical history of well- 62 30 This 54-year-old man was seen in headache con- controlled hypertension on medication. Neurological 63 31 sultation with a 5-month history of a right superior examination was normal. There was tenderness of 64 32 medial orbital pressure type pain with an intensity of the right superior medial orbital area in the area of 65 33 5-6/10 which had been constant since onset. The pain the right trochlea. He was then seen by a neuro- 66 34 was not worse with eye movement, coughing, sneez- ophthalmologist with no additional findings. 67 35 ing, or bending over. He had no visual complaints, 68 36 CASE 2 69 37 Expert commentary by: Juan A. Pareja, MD, Departments of 38 Neurology, Hospital Quirón Madrid, Fundación Hospital A 36-year-old female patient had a family history 70 39 Alcorcón, Universidad Rey Juan Carlos, Madrid, Spain. of migraine in her father. 71 40 Case reports by: Randolph W. Evans, MD, 1200 Binz #1370, Since she was 10 years old she has been suffering 72 41 Houston, TX 77004, USA and Juan A. Pareja, MD, Tucanes 6, from attacks of left hemicranial headaches that were 73 42 Urb. Molino de la Hoz, 28230 Las Rozas, Madrid, Spain. 74 43 Accepted for publication ••. Conflict of Interest: None 75 1 JOBNAME: No Job Name PAGE: 2 SESS: 10 OUTPUT: Fri Jan 8 14:22:36 2010 /v2451/blackwell/journals/head_v0_i0/head_1613 2 •• 1 diagnosed as migraine without aura. The pain was can be conceived as a primary, non-inflammatory 46 2 severe in intensity and pulsatile in character. The trochleodynia. 47 3 attacks were regularly accompanied by nausea, vom- In trochleitis there is a typical induration and 48 4 iting, and photo-phonophobia. Each episode lasted swelling of the inflamed trochlea which can be easily 49 5 for about 12-24 hours. The usual frequency was of 1-2 assessed by palpation. Eye movement restriction is 50 6 episodes monthly but in the last 2 years progressively not a typical feature of idiopathic trochleitis. This 51 7 reached 2-4 attacks weekly. By the time she experi- remark is important to distinguish idiopathic trochlei- 52 8 enced such frequent attacks she also noticed almost tis from other syndromes of the superior oblique 53 9 continuous pain in the inner part of the left orbit, muscle-tendon-trochlea complex that typically 54 10 which persisted in between migrainous episodes. The present with restriction and diplopia. Brown’s 55 11 situation was disabling both socially and profession- syndrome consists in a restrictive inability to elevate 56 12 ally. The attacks had been treated with paracetamol, the eye into adduction specifically caused by an 57 13 metamizol, ibuprofen, and naproxen that provided abnormality of the superior oblique muscle-tendon- 58 14 partial relief. Sumatriptan and almotriptan were trochlea complex. Congenital Brown’s syndrome is a 59 15 effective in aborting migraine attacks but did not restrictive ophthalmopathy with shortening and 60 16 influence the course of the orbital pain. Preventive fibrosis of the superior oblique muscle tendon that 61 17 treatments with Nadolol, Flunarizine, and Amitrip- causes strabismus but no pain. Acquired Brown’s 62 18 tiline were of no avail. Upon examination the left syndrome6-9 may be idiopathic, but it is generally pro- 63 19 trochlear region was found extremely tender. Routine duced by inflammatory processes such as rheumatoid 64 20 blood analysis was normal. A contrast MRI of the arthritis, hyperthyroidism, lupus, psoriasis, or entero- 65 21 head and orbits was normal. Both ENT and Opthal- pathic artropathy (ulcerative colitis and Chron’s 66 22 mologic consultations were normal. disease). Exceptionally, it can be caused by sinusitis, 67 trauma, or metastasis.Acquired Brown’s syndrome of 68 23 inflammatory nature may produce local pain and ten- 69 24 QUESTIONS: WHAT IS THE DIAGNOSIS? derness in the superior nasal orbit.Therefore, in cases 70 25 WHAT TREATMENT WOULD YOU of trochleitis with restriction and diplopia appearing a 71 26 RECOMMEND? symptomatic form should be suspected, the disorder 72 27 Both patients are migraineurs and are also suffer- being better classified within the acquired Brown’s 73 28 ing from a chronic, continuous pain, in the upper- syndrome. 74 29 inner angle of the orbit. Upon examination, there was Trochleitis may be documented by orbital echog- 75 30 tenderness on the superior medial area of the symp- raphy or contrast enhanced CT or MRI of the brain, 76 31 tomatic orbit, ie, on the area of the trochlea. The centered in the orbits. Routine blood work, ESR, 33 77 32 clinical picture is consistent with pain stemming from standard biochemical determinations, thyroid func- 78 33 the trochlear region.Trochlear pain (trochleodynia) is tion with antitiroglobulin and antimicrosomal anti- 79 34 characterized by pain in the upper-inner angle of the bodies,ANAs, Rheumatoid factor, and urine analyses 44 80 35 orbit, occasionally spreading to the ipsilateral fore- are necessary to rule out a secondary form. Only 81 36 head, that typically increases upon palpation and exceptionally it is necessary to perform a biopsy. 82 37 supraduction1-4 and is accompanied by tenderness in Etiology of both primary trochleitis and primary 83 38 the trochlear region. The structures involved in the trochlear headache are largely unknown. However, 84 39 genesis of trochleodynia are the superior oblique several proposals have been intended. Idiopathic Tro- 85 40 muscle, its tendon, and the throclea itself. In a few chleitis could represent a highly restricted form of 86 41 patients the underlying process of trochleodynia is an orbital pseudotumor,2 or an inflammatory myopaty/ 87 42 inflammatory – usually primary – disorder of the enthesopaty of the superior oblique muscle/tendon.1 88 43 trochlear/peritrochlear area – ie, trochleitis.2,3 On the other hand, Primary Trochlear Headache 89 44 However, most patients have a recently recognized could be due to a mechanic enthesopathy, a 90 45 disorder named primary trochlear headache,4 which superior oblique muscle myofascial disorder,10 or an 91 JOBNAME: No Job Name PAGE: 3 SESS: 10 OUTPUT: Fri Jan 8 14:22:36 2010 /v2451/blackwell/journals/head_v0_i0/head_1613 Headache 3 1 intraorbital neuropathy of the nearby nerves liable to between both disorders. In such cases migraine was 47 2 persistent microtrauma by movements of the superior frequent or chronic, and strictly unilateral in the same 48 3 oblique muscle. side as trochleodynia. The term “trochlear migraine”1 49 4 The two presented patients neither had an abnor- has been proposed to name the coexistence of strictly 50 5 mal swelled and indured trochlea nor MRI showed unilateral migraine and ipsilateral trochleodynia, with 51 6 abnormal findings in the trochlear region. In addition, the improvement of migraine being dependent on the 52 7 analytical scrutiny was normal. Therefore, the plau- resolution of the trochlear complaint, thus indicating 53 8 sible diagnosis is Primary Trochlear Headache. The that the trochlear painful process could have contrib- 54 9 diagnosis of Primary Trochlear Headache requires uted to the perpetuation – or worsening – of 55 10 both the presence of trochleodynia and ruling out migraine.1,3,4,11 56 11 trochleitis and any structural abnormality in the tro- Painful inputs from both migraine and trochleo- 57 12 chlear region by both neuroimaging and analytical dynia processes follow the pathway of the first branch 58 13 exams.