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Show LETTERS TO THE EDITOR Opsoclonus- Myoclonus Syndrome During Pregnancy To the Editor: Opsoclonus- myoclonus syndrome ( OMS) is a rare condition characterized by opsoclonus with oscillopsia, generalized myoclonus, ataxia ( mainly in the legs), and occasionally learning and behavioral disorders. Nearly 50% of children with OMS have neuroblastoma. In adults, the most common causes are idiopathic, with less common causes being encephalitis, drug intoxication, and paraneoplastic disorders ( 1). We have encountered two cases that developed during pregnancy. Case 1 A 3 3- year- old woman developed progressive dizziness, nausea, and gait difficulty in the ninth month of pregnancy. She had rapid, irregular, and oscillatory eye movements, and myoclonic jerks of the neck and limbs. She was unable to sit or stand because of truncal ataxia. Routine laboratory studies of blood and urine were normal. Serological tests for infectious pathogens were unremarkable; tumor markers and serum antibody to Ri antigen were not detectable. The cerebrospinal fluid contained 9/ mm3 lymphocytes, 60 mg/ dl protein, and 53 mg/ dl glucose. Brain magnetic resonance imaging showed no abnormalities. On the decision of the obstetrician, she delivered a healthy child by cesarean section. Thereafter, she was treated with oral prednisolone ( 60 mg per day), which was gradually tapered over two months. At the three- month follow- up, she had minimal residual opsoclonus and mild dizziness. Case 2 A 34- year- old woman developed nausea, vomiting, oscillopsia, dizziness, and unsteady gait in the fifth month of pregnancy. She showed opsoclonus and nonrhythmic myoclonic jerks involving the neck and upper extremities, mild dysmetria on knee- heel testing, and truncal ataxia. She was unable to stand without assistance. Routine laboratory studies, CSF studies, and brain MRI were normal. Serum antibody to Ri antigen, syphilitic serology, and autoantibody screen were negative. As her neurologic symptoms did not improve within the following three weeks, she was treated with intravenous prednisolone 50 mg per day. After the treatment, the myoclonus gradually disappeared, but the opsoclonus and severe dizziness remained almost unchanged for two weeks. In the sixth month of pregnancy, she suffered a spontaneous miscarriage. Thereafter, the clinical symptoms improved markedly. One month after the termination of pregnancy, she was walking steadily with some residual dizziness and mild ocular flutter. The relationship between OMS and pregnancy is unclear. Interestingly, chorea gravidarum, another movement disorder of uncertain etiology, occurs only in pregnancy. At Tokyo Metropolitan Neurological Hospital, eight patients have been diagnosed with clinically definite OMS in the past ten years. Considering the fact that this rare disorder occurred during pregnancy in 25% of our cases, we wonder if there is a susceptibility factor in pregnancy. Goto et al ( 2) previously reported a 35- year- old woman who developed vomiting, dizziness, and gait instability in the seventh month of pregnancy. Her examination resembled that of our patients. She was treated with clonazepam and oral prednisolone 60 mg per day and gradually improved within the following several months. She delivered a healthy child. Six months after the onset of OMS, she had recovered completely. In our two cases and that of Goto et al ( 2), the neurologic symptoms were entirely similar to those of OMS unrelated to pregnancy. The conditions of the fetuses were good, except in the case that miscarried. In all three cases, the OMS occurred in the middle to late stages of pregnancy. Whether the symptoms improved because the pregnancy ended or because of corticosteroid therapy remains unclear. However, the OMS gradually improved after spontaneous miscarriage in our Case 2. Taken together, these results raise the possibility that pregnancy influences the appearance of OMS. Reiji Koide, MD Masaki Sakamoto, MD Kozue Tanaka, MD Hideaki Hayashi, MD Department of Neurology Tokyo Metropolitan Neurological Hospital Tokyo, Japan reiji@ tmnh. fuchu. tokyo. jp REFERENCES 1. Moll JW, Vecht CH J. Paraneoplastic syndromes of the central nervous system. In: Vinken PJ, Brayn GW, eds. Neuro- Oncology, part III. ( Handbook of Clinical Neurology, Vol. 69). Amsterdam: Elsevier, 1997: 349- 71. 2. Goto T, Yonemitsu M, Araki Y, et al. Case of opsoclonus-myoclonus syndrome ( OMS) developing during pregnancy. Nippon Naika Gakkai Zasshi 1999; 88: 344- 46. Projector Light Visual Fields To the Editor: The article entitled " Laser Pointer Visual Field Screening" by Lee et al ( 1) fails to mention the historical J Neuro- Ophthalmol, Vol. 24, No. 3, 2004 273 JNeuro- Ophthalmol, Vol. 24, No. 3, 2004 Letters to the Editor legacy of the use of projector light visual fields in neuro- ophthalmology. During my fellowship year with J. Lawton Smith, MD ( Miami, FL) in 1979, Dr. Smith routinely used the projector light technique for doing central visual fields. Indeed, in his little book titled The Optic Nerve ( 2), he describes the technique and shows a photograph of how to do the test, as well as where to order a projector light. On Neuro- Ophthalmology Tape # 57, " Visual Fields- 2" ( 3), he goes into more detail about how to use this method of visual field testing. Dr. Smith learned the technique from David G. Cogan, MD, during his neuro- ophthalmology fellowship year in Boston in 1958, and used it in his clinical practice for the next 36 years until his retirement. Dr. Smith told me that he had tried a laser pointer, but liked the original incandescent projector light better because it was less expensive and it was easier to vary the light intensity by partially masking the light with a finger. Having used the projector light method of qualitatively testing visual fields on many occasions, I can personally vouch for its utility and versatility. Also, I vividly remember watching Dr. Smith test a supine bedridden patient. Dr. Smith lay on the floor next to the bed and used the hospital room ceiling as the " tangent screen!" Robert L. Tomsak, MD, PhD Case School of Medicine University Hospitals of Cleveland Cleveland, Ohio Robert. Tomsak@ uhhs. com REFERENCES 1. Lee MS, Baker LJ, Volpe NJ, et al. Laser pointer visual field screening. JNeuroophthalmol 2004; 23: 260- 263. 2. Smith JL. The Optic Nerve. Miami: Neuro- ophthalmology Tapes, 1977, pp. 55- 57. 3. Smith JL. Visual Fields - 2. Neuro- ophthalmology Tape # 57. Miami: Neuro- ophthalmology Tapes. 274 © 2004 Lippincott Williams & Wilkins |
