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Show LETTERS TO THE EDITOR Recurrent Isolated Horner Syndrome We report a patient with recurrent isolated Horner syndrome without headache, rhinorrhea, ocular or nasal congestion, tearing, or any abnormalities on brain and neck imaging. A 28- year- old male resident physician reported a 2- week history of episodic right upper lid ptosis and miosis. During the first episode, the ptosis and miosis resolved within 30 minutes, and there was no associated headache, eye pain, tearing, nasal congestion, or rhinorrhea. The episode was witnessed by his fellow residents and an attending physician during morning report, and he was sent to the emergency room for evaluation. However, by the time he arrived, the episode had resolved. For the next 2 weeks, he had nearly daily recurrences of the same symptoms. The duration of the episodes ranged from 10 minutes to 11 hours, with an average of 30 to 45 minutes. During one of the episodes, he ran up several flights of stairs and noted that he had anhydrosis of the right side of his forehead. His past medical and surgical history were unremarkable. He denied head or neck trauma, neck pain, arm pain, migraine or cluster headache, or cigarette smoking. His mother had migraine headache, but there was no family history of cluster headache. On our initial neuro- ophthalmologic examination, best- corrected visual acuity was 20/ 20 in both eyes. Eye movements and alignment were normal, and confrontation visual fields were full. Pupils were 5 mm, round, and reactive to light in both eyes without an afferent defect. Cranial nerve examination was normal, as was the rest of the ophthalmologic examination. Results of MRI of the head and neck with contrast, MRA of the neck, and chest x- ray with apical and lordotic views were normal. Examination during a recurrence 1 week later, after he had run over from the hospital, showed 2 mm of right upper lid ptosis. The right pupil was 4 mm and the left pupil was 6.5 mm in darkness, both constricting normally to bright light. The ptosis and anisocoria resolved completely within 5 minutes of his arrival and 30 minutes from onset. Topical cocaine testing could therefore not be performed. He reported resolution of these episodes within 4 weeks of onset, and he has had neither a recurrence of the episodes nor the development of cluster headaches in 4 years of follow- up. Our patient's findings are highly suggestive of an intermittent Horner syndrome, and his report of anhydrosis limited to the forehead is suggestive, although not diagnostic, of a postganglionic etiology. Cervical cord lesions ( 1,2), carotid artery lesions ( 3,4), and cluster headache ( 5,6) have been associated with cases of intermittent Horner syndrome. The normal MRI and MRA results in our patient effectively ruled out cervical or carotid artery lesions as culprits. Cluster headache sine headache was first reported by Salvesen ( 5), who described a patient suffering from daily attacks of Horner syndrome and nasal congestion but no pain. Although our patient and the patient of Salvesen share a similar pattern of painless, intermittent Horner syndrome, normal neuroimaging studies, and normal examinations between episodes, the unique aspect of our case is the absence of associated parasympathetic symptoms such as rhinorrhea, nasal stuffiness, or lacrimation. Havalius ( 7) reported five patients ranging in age from 2 to 20 years with constant ptosis and miosis for years before the onset of an ipsilateral cluster headache. He documented the findings by photographs or patient reports, and there were no reports of an episodic Horner syndrome similar to that noted in our patient. Although the exact mechanism of the Horner syndrome in our patient is unknown, we believe it represents a variant of cluster headache sine headache. Marjorie A. Murphy, MD Lawrence C. Hou, MD Department of Ophthalmology Rhode Island Hospital and Brown Medical School Providence, Rhode Island MargieMurphy@ cox. net REFERENCES 1. Hopf HC. Intermittent Horner's syndrome on alternate sides: a hint for locating spinal lesions. J Neurol 1980; 224: 155- 7. 2. Zur PH. Intermittent Horner's syndrome: recurrent, alternate Horner's syndrome in cervical cord injury. Ann Ophthalmol 1975; 7: 955- 62. 3. Ameline- Audelan V Painful Claude Bernard Horner syndrome: apropos of a painless carotid artery dissection ( in French). J Fr Ophthalmol 1998; 21: 591- 5. 4. Venketasubramanian N, Singh J, Hui F, et al. Carotid artery dissection presenting as a painless Horner's syndrome in a pilot: fit to fly? Aviat Space Environ Med 1998; 69: 307- 10. 5. Salvesen R. Cluster headache sine headache: case report. Neurology 2000; 55: 451. 6. Leone M, Rigamonti A, Bussone G. Cluster headache sine headache: two new cases in one family. Cephalalgia 2002; 22:\ 2- A. 7. Havalius H. A Horner- like syndrome and cluster headache. What comes first? Acta Ophthalmol Scand 2001; 79: 374- 5. Isolated Sixth Cranial Nerve Palsy in Preeclampsia Preeclampsia is a multisystem disorder of unclear etiology often seen in pregnancy ( 1). Its common maternal 296 J Neuro- Ophthalmol, Vol. 26, No. 4, 2006 Letters to the Editor J Neuro- Ophthalmol, Vol. 26, No. 4, 2006 manifestations include hypertension and proteinuria. The neurologic abnormalities seen in association with preeclampsia include seizures and stroke. Isolated cranial nerve palsies are, however, an uncommon manifestation. We report a woman who developed an isolated sixth cranial nerve palsy. An asymptomatic 3 5- year- old woman, gravida 3 para 2, was transferred to our institution for management of severe preeclampsia when she was at 30 weeks of gestation. She reported an unremarkable medical history apart from untreated " white coat" hypertension during and before her current pregnancy. There was no history of diabetes mellitus or neuro- ophthalmologic disorders. Her blood pressure at the time of presentation was 230/ 110 mm Hg. Neurologic examination revealed symmetrically brisk reflexes but was otherwise unremarkable. Laboratory studies revealed a urea concentration of 7.1 mmol/ L, creatinine concentration of 112 | jimol/ L, platelet count of 109 X 109/ L, and normal liver function and coagulation tests. Urinalysis revealed 4+ proteinuria. She was subsequently treated with betamethasone 11.4 mg intramuscularly, clonidine 150 | Jig orally QID, hydralazine 25 mg orally QID, and magnesium sulfate 1 g/ h intravenously over 24 hours. As her blood pressure was not adequately controlled with aggressive medical therapy, the baby was delivered by urgent cesarean section with epidural anesthesia. The procedure was uncomplicated. However, her blood pressure remained elevated after delivery. Ongoing antihypertensive therapy resulted in markedly improved blood pressure control over the following days. On day 11 after delivery, she reported the abrupt onset of a mild frontal headache with horizontal diplopia on looking to the left. At this time, she was continuing to receive clonidine 150 | Jig orally twice daily, enalapril 10 mg orally twice daily, hydralazine 25 mg orally twice daily, labetalol 200 mg orally twice daily, controlled- release nifedipine 60 mg orally daily, and prazosin 1.5 mg orally QID. Physical examination revealed a blood pressure of 120/ 70 mm Hg and reduced abduction of the left eye consistent with a left sixth cranial nerve palsy. No other abnormality was detected on neuro- ophthalmologic and cranial nerve examination. Examination of her limbs revealed symmetrically brisk reflexes with flexor plantar responses. The remainder of the examination was unremarkable. Brain MRI revealed no abnormalities. There was no improvement in the abduction deficit after an intravenous edrophonium test. Results of other laboratory studies were unremarkable. No renal, endocrine, or cardiovascular abnormality could be found to account for her hypertension. A provisional diagnosis of an ischemic sixth cranial nerve palsy was made. The palsy was noted to slowly resolve in the months after discharge. Isolated sixth cranial nerve palsy is rarely associated with preeclampsia. To the best of our knowledge, there are only two case reports of sixth cranial nerve palsy occurring in association with preeclampsia, with symptoms commencing before delivery in both patients ( 2,3). It has also been reported in a woman at gestation who had only a single elevated blood pressure reading at the time of onset ( 4). The nerve palsy has been variably attributed to downward displacement of the nerve due to cerebral edema and nerve infarction due to vasospasm or hypertension ( 2- 4). In a recent retrospective study of nontraumatic sixth cranial nerve palsies in adults younger than 50 years of age, an ischemic cause was identified in only 4% of patients ( 5). The majority of patients in this series were found to have mass lesions or multiple sclerosis, although many of these patients also had other neurologic signs. There are, however, a number of reports of isolated sixth cranial nerve palsies occurring with lesions involving the sixth cranial nerve fascicles as they pass through the basis pontis, indicating that some cases presumed to be due to peripheral lesions are in fact due to central lesions ( 6- 8). Our patient was unusual in that her sixth cranial nerve palsy occurred some days after the delivery of the baby, when her blood pressure was relatively well controlled with antihypertensive agents. The possibility that the palsy occurred as a consequence of intracranial hypotension was therefore considered, because both unilateral and bilateral sixth cranial nerve palsies have been reported after unintentional dural puncture with epidural anesthesia ( 9,10). Our patient did not, however, complain of orthostatic headache or any other symptom to suggest intracranial hypotension. Furthermore, the palsy came on suddenly over a week after the epidural catheter was removed. Consequently, we believe that an ischemic etiology was most likely. Our patient demonstrates that an ischemic sixth cranial nerve palsy can occur rarely in the setting of preeclampsia, even when blood pressure is relatively well controlled. In such patients, full investigation for other possible causes is necessary to exclude more serious underlying pathologic conditions. When no serious cause is found, with ongoing control of blood pressure, the prognosis for recovery is excellent and the patient can be reassured. Matthew J. Thurtell, MBBS Kaitlyn L. Sharp, MBBS Judith M. Spies, MBBS, PhD G. Michael Halmagyi, MD Department of Neurology Royal Prince Alfred Hospital Sydney, Australia matthewt@ icn. usyd. edu. au 297 J Neuro- Ophthalmol, Vol. 26, No. 4, 2006 Letters to the Editor REFERENCES 1. Sibai B, Dekker G, Kupferminc M. Preeclampsia. Lancet 2005; 365: 785- 99. 2. Blade J, Peborde J, Darleguy P. Paralysis of the 6th cranial nerve in eclampsia ( in French). Bull Soc Ophthalmol Fr 1968; 68: 284- 7. 3. Barry- Kinsella C, Milner M, McCarthy N, et al. Sixth nerve palsy: an unusual manifestation of preeclampsia. Obstet Gynecol 1994; 83: 849- 51. 4. Fung TY, Chung TKH. Abducens nerve palsy complicating pregnancy: a case report. Eur J Obstet Gynecol Reprod Biol 1999; 83: 223^. 5. Peters GB 3rd, Bakri SJ, Krohel GB. Cause and prognosis of nontraumatic sixth nerve palsies in young adults. Ophthalmology 2002; 109: 1925- 8. 6. Donaldson D, Rosenberg NL. Infarction of abducens nerve fascicle as cause of isolated sixth nerve palsy related to hypertension. Neurology 1988; 38: 1654. 7. Barr D, Kupersmith MJ, Turbin R, et al. Isolated sixth nerve palsy: an uncommon presenting sign of multiple sclerosis. J Neurol 2000; 247: 701^. 8. Thomke F. Brainstem diseases causing isolated ocular motor nerve palsies. Neuroophthalmology 2002; 28: 53- 67. 9. Szokol JW, Falleroni MJ. Lack of efficacy of an epidural blood patch in treating abducens nerve palsy after an unintentional dura puncture. Reg Anesth Pain Med 1999; 24: 470- 2. 10. Arcand G, Girard F, McCormack M, et al. Bilateral sixth cranial nerve palsy after unintentional dural puncture. Can J Anesth 2004; 51: 821- 3. Topical Apraclonidine Testing of Pupillary Sympathetic Denervation in Diabetic Patients I read with interest the report by Koc et al ( 1) about topical apraclonidine testing in diabetic patients to show pupillary sympathetic denervation. Using a Rosenbaum pocket vision screener, they concluded that " apraclonidine testing identified pupillary sympathetic denervation in nearly half of diabetes melhtus patients ( DM), the degree of mydriasis being correlated to the duration of DM and presence of diabetic retinopathy." However, I believe this conclusion cannot be supported because of significant limitations of the study design. The pupil diameter was measured using the gauge on the Rosenbaum pocket vision screener in normal room lighting. The precise illumination conditions are critical in studies evaluating pupil dilatation. However, the authors did not specify the use of a light meter or any other specific method to standardize the amount of extraneous light falling on the subject's retina. In addition, the handheld Rosenbaum card had been described as being among the least reliable methods of measurement, because of high inter- observer and intra- observer variation, a tendency to overestimate pupil size, and greater inter- examiner variation than with infrared systems ( 2- 4). Boxer, Wachler and Krueger ( 5) concluded that the comparison method consistently overestimates the pupil diameter and recommended that doctors apply a 0.5 mm adjustment when using the Rosenbaum card for pupillometry. Problems also arise when a printed pupil card is used because of difficulty examiners have in viewing and accurately measuring pupil sizes in low- light conditions ( 3). How did the authors conduct the standardization of their examination or compensate for inherent disadvantages of their chosen piece of equipment? A recent article that controlled for accommodation and patient alertness concluded that 5 minutes of dark adaptation is necessary for consistent pupillary measurements ( 6). However, in the methods section of the article by Koc et al, there is no information about how the authors compensated for the measurement flaws originating from inadequate dark adaptation. Furthermore, the authors did not state whether the study was masked or whether the same examiner ( with the potential for bias toward a positive outcome) conducted the analysis before and after apraclonidine instillation. If more than one observer used the Rosenbaum card, there is the risk for significant inter- observer error, especially in small sample groups. Although I applaud the authors' attempt to address an interesting hypothesis, there is no clear reassurance that the difference in pupil dilatation after instillation of apraclonidine occurred as a result of pupillary sympathetic denervation in diabetic patients. Hasan Basri Cakmak, MD Ankara Ataturk Research Hospital Ophthalmology Clinic Ankara, Turkey hasanbasricakmak@ yahoo. com. tr REFERENCES 1. Koc F, Kansu T, Kavuncu S, et al. Topical apraclonidine testing discloses pupillary sympathetic denervation in diabetic patients. J Neuroophthalmol 2006; 26: 25- 9. 2. Lord- Feroli K, Maguire- McGinty M. Toward a more objective approach to pupil assessment. J Neurosurg Nurs 1985; 17: 309- 12. 3. Colvard M. Preoperative measurement of scotopic pupil dilation using an offfice pupillometer. J Cataract Refract Surg 1998; 24: 1594- 7. 4. Rosen ES, Gore CL, Taylor D, et al. Use of a digital infrared pupillometer to assess patient suitability for refractive surgery. J Cataract Refract Surg 2002; 28: 1433- 8. 5. Boxer Wachler BS, Krueger RR. Agreement and repeatability of infrared pupillometry and the comparision method. Ophthalmology 1999; 106: 319- 23. 6. Boxer Wachler BS, Durrie DS, Assil KK, et al. Improvement of visual function with glare testing after photorefractive keratectomy and radial keratotomy Am J Ophthalmol 1999; 128: 582- 7. 298 © 2006 Lippincott Williams & Wilkins Letters to the Editor J Neuro- Ophthalmol, Vol. 26, No. 4, 2006 Authors' Reply: The authors thank Dr. Cakmak for his interest in our work. This study was not planned in a masked manner because we did not think it was necessary. The dilatation response to apraclonidine was observed in a diabetic patient by chance while testing its effect on the pupil. The agent we were using was not standardized for pupillary testing yet, and all responses were important for us. We were curious about its effect in the diabetic population and impressed with the positive responses as we observed more patients. Non- diabetic patients did not show the same response. We completed testing on the number of patients and control subjects for statistically meaningful values. The precise illumination was not measured with a light meter, but to standardize the test as much as possible, all measurements were done at the same spot of the examination room with 1 hour temporal difference only. To prevent inter- observer variations, all patients and control subjects were referred to the same examiner ( FK). The same distant fixation target was used during all measurements to standardize the accommodative miosis effect. We agree that the Rosenbaum pocket vision screener is not as precise as an infrared pupillometer, but it is easily available and is the most widely used method for routine pupillary measurements that is also appropriate for screening purposes. We wanted to share and document our observations that apraclonidine may be helpful in establishing the diagnosis of ocular sympathetic involvement in diabetes mellirus, especially if other tests are not easily accessible. Further studies with more ideal setting, including a light meter and pupillometer, will be needed to support our findings. Feray Koc, MD Sevim Kavuncu, MD Esin Firat, MD Neuro- Ophthalmology Unit SB Ulucanlar Eye Hospital Ankara, Turkey dr_ feray@ yahoo. com Tulay Kansu, MD Department of Neurology Neuro- Ophthalmology Unit Hacettepe University Faculty of Medicine Ankara, Turkey 299 |