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Show Neuro-Behçet Disease Presenting With Oculopalatal Tremor Michael L. Morgan, MD, PhD, Angelina Espino Barros Palau, MD, Andrew G. Lee, MD, Rod Foroozan, MD Abstract: A 39-year-old woman with a history of Behçet disease presented for evaluation of oscillopsia that began postpartum. Examination showed oculopalatal tremor (OPT), documented videographically. Brain magnetic reso-nance imaging revealed bilateral pseudohypertrophy of the inferior olivary nuclei. Treatment with gabapentin was initiated for OPT presumed secondary to neuro-Behçet disease. Journal of Neuro-Ophthalmology 2015;35:51-53 doi: 10.1097/WNO.0000000000000159 © 2014 by North American Neuro-Ophthalmology Society Behçet disease (BD) is an autoimmune disorder geo-graphically associated with the Silk Road and featuring recurrent oral and genital ulcers and uveitis as well as poten-tial involvement of multiple organ systems, including the central nervous system (1). As with many autoimmune dis-eases, pregnancy provides some protection with potentially increased relapse risk in the postpartum period (2). Involve-ment of the central nervous system is termed neuro-Behçet disease (NBD), with the brainstem most frequently affected. Palatal tremor (PT), also termed palatal myoclonus, refers to rhythmic jerking movements of the throat and soft palate associated with damage to the dento-rubro-olivary pathways, referred to as the triangle of Guillain and Mollaret (3). Several cases of PT have been associated with NBD (4-7). When pendular ocular oscillations synchronous with PT are also present, the syndrome is referred to as oculopalatal tremor (OPT) or oculopalatal myoclonus (8). Our patient with BD developed OPT in the postpartum period. CASE REPORT A 39-year-old woman with a history of BD, Hashimoto thyroiditis, and hypertension was referred for evaluation of nystagmus. She was diagnosed with BD 16 years prior after biopsy of recurrent genital ulcers. She also had developed oral ulcers and uveitis in the past and had been treated with etanercept and methotrexate in the early years after diagnosis. She also had been treated intermittently with dapsone, which was discontinued several times after development of methe-moglobinemia and corticosteroids 3 months before our evaluation and azathioprine more recently. Her family history was remarkable for rheumatoid arthritis and hypothyroidism. She drank alcohol moderately and was a former tobacco smoker. Six weeks after delivering a healthy baby, the patient noted poor balance on attempted resumption of physical exercise and soon thereafter rotary oscillopsia involving the right eye. Two months later, she developed a throbbing sensation in her throat and observed her uvula jerking rhythmically. These symptoms continued uninterrupted. Magnetic resonance imaging (MRI) of the brain revealed pseudohypertrophy of the inferior olivary nuclei bilaterally. Her neurologist detected nystagmus corresponding to her oscillopsia. A vestibular evaluation was consistent with right vestibular dysfunction and benign paroxysmal posi-tional vertigo. Multiple Epley maneuvers provided no improvement. Department of Ophthalmology (MLM, AEBP, AGL), Houston Meth-odist Hospital, Houston, Texas; Department of Ophthalmology, (AGL, RF), Baylor College of Medicine, Houston, Texas; Departments of Ophthalmology, Neurology, and Neurosurgery (AGL), Weill Cornell Medical College, Houston, Texas; Department of Ophthalmology (AGL), UTMB, Galveston, Texas; Department of Head and Neck Surgery (AGL), UTMD Anderson Cancer Center, Houston, Texas; and Department of Ophthalmology (AGL), University of Iowa Hospitals and Clinics, Iowa City, Iowa. The authors report no conflicts of interest. Supplemental digital content is available for this article. Direct URL citations appear in the printed text and are provided in the full text and PDF versions of this article on the journal's Web site (www. jneuro-ophthalmology.com). Address correspondence to Rod Foroozan, MD, Department of Ophthalmology, Baylor College of Medicine, 1977 Butler Boulevard, Houston, TX 77030; E-mail: foroozan@bcm.edu Morgan et al: J Neuro-Ophthalmol 2015; 35: 51-53 51 Clinical Observation Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Nine months before our evaluation, neurological exam-ination was remarkable for flattening of the left nasolabial fold, PT, proximal weakness (4/5 strength) in the upper extremities and in the right lower extremity, bilateral patellar hyperreflexia, and unsteady gait. Cerebrospinal fluid analysis was unremarkable. Twenty-two months after onset of symptoms, brain MRI showed T2 hyperintensity bilaterally in the ventral medulla with faint contrast enhancement (Fig. 1). Neuro-ophthalmic examination demonstrated rotary pendular oscillations of both eyes, greater on the right (see Supplemental Digital Content, Video, http://links. lww.com/WNO/A108). Additionally, she had rhythmic movements of the angles of her mouth, uvula, and palate, and her speech was dysarthric. The rotary pendular ocular oscillations and palatal movements were synchronous with a frequency of approximately 1-2 Hz. The remainder of her ophthalmic examination was normal. Given her clinical and MRI findings, she was diagnosed with OPT and prescribed gabapentin 300 mg 3 times daily. Her symptoms and examination remained unchanged 2 months later, and consideration was being given for treatment with memantine or increased immunosuppression. DISCUSSION OPT arises from disruption of the dento-rubro-olivary pathways (8). Brainstem ischemia and hemorrhage account for 60%-70% of cases with additional causes including trauma, infection, inflammation, demyelination, and possibly toxicity from metronidazole, lithium, carbamazepine, and ciprofloxacin. Pseudohypertrophy of one or both inferior olivary nuclei (ION) is the neuroimaging hallmark of OPT. Our patient had bilateral OPT and bilateral ION pseudohypertrophy. In a review of the literature, Pearce (3) reported that in 287 cases of PT, no cause could be found in 25% and these patients typically lacked radio-graphic abnormalities. The finding of bilateral ION pseu-dohypertrophy without other radiographic abnormalities in our patient is unusual. However, her first MRI was performed over a year after symptoms began, and a brain-stem or cerebellar abnormality might have been present transiently. Our patient experienced gait imbalance 6 weeks after delivery. We presume that she developed NBD at that time with bilateral damage to the dento-rubro-olivary pathways. OPT symptoms began approximately 2 months postpar-tum. As with many autoimmune diseases, BD often improves during pregnancy with relapses sometimes occur-ring in the postpartum period. In 27 BD exacerbations across 84 pregnancies in 50 patients, 19 occurred during 9 months of pregnancy (0.025/mo) compared with 18 in 3 months postpartum (0.071/mo) (2). The occurrence of PT due to NBD has been docu-mented in case reports (4-7). However, OPT occurring in patients with NBD is rare. In a series of 4 cases with PT, 1 patient had NBD (9). In that patient, the tremor was more extensive, affecting the palate, throat, and diaphragm with subsequent involvement of the face, eyes, neck, and shoul-der girdles. FIG. 1. Pseudohypertrophy of the bilateral inferior olivary nuclei (ION). A, Fluid-attenuated inversion recovery magnetic resonance imaging shows hyperintensity and enlargement of the bilateral ION (arrows). B, T1 imaging also demonstrates enlargement. C, The ION faintly enhanced with gadolinium contrast (arrowheads). 52 Morgan et al: J Neuro-Ophthalmol 2015; 35: 51-53 Clinical Observation Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. REFERENCES 1. Saip S, Akman-Demir G, Siva A. Neuro-behçet syndrome. Handb Clin Neurol. 2014;121:1703-1723. 2. Noel N, Wechsler B, Nizard J, Costedoant-Chalumeau H, Boutis du LT, Dommergues M, Vauthler-Brouzes D, Cacoub P, Saadour D. Behçet's disease and pregnancy. Arthritis Rheum. 2013;65:2450-2456. 3. Pearce JMS. Palatal myoclonus (syn. palatal tremor). Eur Neurol. 2008;60:312-315. 4. Shuttleworth EC, Voto S, Sahar D. Palatal myoclonus in Behçet's disease. Arch Intern Med. 1985;145:949-950. 5. Yokota T, Hirashima F, Furukawa T, Tsukagoshi H, Yoshikawa H. MRI findings of inferior olives in palatal myoclonus. J Neurol. 1989;236:115-116. 6. Sakurai N, Koike Y, Kaneoke Y, Yasuda T, Takahashi A. Sleep apnea and palatal myoclonus in a patient with neuro-Behçet syndrome. Intern Med. 1993;32:336-339. 7. Iwasaki Y, Kinoshita M, Ikeda K, Shiojima T. Palatal myoclonus following Behçet's disease ameliorated by ceruletide, a potent analogue of CCK octapeptide. J Neurol Sci. 1991;105:12-13. 8. Borruat FX. Oculopalatal tremor: current concepts and new observations. Curr Opin Neurol. 2013;26:67-73. 9. Nagaoka M, Narabayashi H. Palatal myoclonus: its remote influence. J Neurol Neurosurg Psychiatry. 1984;47:921-926. STATEMENT OF AUTHORSHIP Category 1: a. Conception and design: M. L. Morgan and R. For-oozan; b. Acquisition of data: M. L. Morgan and R. Foroozan; c. Analysis and interpretation of data: M. L. Morgan and R. Foroozan. Category 2: a. Drafting the manuscript: M. L. Morgan, A. Espino Barros Palau, and R. Foroozan; b. Revising it for intellectual content: A. Espino Barros Palau, A. G. Lee, M. L. Morgan, and R. Foroozan. Category 3: a. Final approval of the completed manuscript: A. Espino Barros Palau, A. G. Lee, M. L. Morgan, and R. Foroozan. Morgan et al: J Neuro-Ophthalmol 2015; 35: 51-53 53 Clinical Observation Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. |
