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Show Journal of Neuro- Ophthalmology 14( 4): 210- 213, 1994. 11994 Raven Press, Ltd., New York Medical and Surgical Management of Acute Disseminated Encephalomyelitis Brian D. Ellis, M. D., Gregory S. Kosmorsky, D. O., and Bruce H. Cohen, M. D. Two children with a recent history of viral illness developed visual loss secondary to optic neuritis. Clinical findings and neuroimaging were consistent with acute disseminated encephalomyelitis ( ADEM). Markedly elevated opening pressures were noted on lumbar puncture. The patients demonstrated an initial favorable response to high- dose corticosteroid administration. Both had recurrence of symptoms after being tapered off oral corticosteroids. High- dose corticosteroids were reinsti-tuted and a bilateral optic nerve sheath decompression was performed on one patient who developed profound visual loss. A second patient underwent a lumboperito-neal shunt. Both children had resolution of their symptoms and had a recovery of normal visual acuity. Key Words: Encephalitis- Neuroimaging- Corticosteroid therapy. From the Departments of Ophthalmology ( B. D. E., G. S. K.) and Pediatric Neurology ( B. H. C.), Cleveland Clinic Foundation, Cleveland, Ohio, U. S. A. This paper was presented at the North American Neuro- Ophthalmology Society Meeting, Big Sky, Montana, February 9, 1993, and at the Association for Research in Vision and Ophthalmology Meeting, Sarasota, Florida, May 4, 1993. Address correspondence and reprint requests to Dr. Gregory S. Kosmorsky, Department of Ophthalmology, A31, 9500 Euclid Avenue, Cleveland, OH 44195- 5024, U. S. A. Two children presented with bilateral optic neuritis and encephalomyelitis. Both patients had histories consistent with a parainfectious etiology. Management included both medical and surgical modalities. CASE1 A 4- year- old girl presented with a 3- week history of fever, headache, and photophobia. Three weeks prior to presentation, the patient had an episode of gastroenteritis. One week prior to presentation, she was treated with amoxicillin for presumed sinusitis. On admission the patient was alert. Her white blood cell count was 28,000/ mm3. A computed tomography ( CT) scan showed small ventricles with loss of normal sulci. Lumbar puncture results included an opening pressure of 530 mm H20; white blood cells, 22/ mm3; red blood cells, 0/ mm3; protein, 23 mg/ dl; glucose, 58 mg/ dl; and negative latex agglutination for Streptococcus pneumoniae, Neisseria meningitidis, and Hemophilus influenzae. No oligoclonal bands were found. Viral studies for adenovirus, cytomegalovirus, enterovirus, herpes simplex, measles, and mumps were negative. Magnetic resonance imaging ( MRI) demonstrated a right centrum semiovale lesion most prominent on T2 images. ( Fig. 1). Her visual acuity was 20/ 30 Allen OD, 20/ 30 Allen OS. No relative afferent pupillary defect was detected. There was mild disc edema OU. She was treated with both intravenous dexamethasone 4 mg every 6 hours and intravenous ceftriaxone. After 2 days of intravenous steroid therapy and with resolution of symptoms, she was discharged on an oral dexamethasone taper. Approximately 1 month after discharge and after completion of an oral corticosteroid taper, the patient had recurrence of headache with decreased 220 ACUTE DISSEMINATED ENCEPHALOMYELITIS 211 FIG. 1. Magnetic resonance imaging of Case 1, demonstrating right centrum semiovale lesion at presentation. ( TE = 90, TR = 2500.) vision. Examination showed no light perception OD, 20/ 70 Allen OS. Fundus examination demonstrated severe bilateral disc edema. MRI scan showed decreased size of the previously noted right centrum semiovale lesion. Opening pressure on a repeat lumbar puncture done under general anesthesia was 200 mm H20. She was again started on intravenous dexamethasone 2 mg every 6 hours and an optic nerve sheath decompression was performed OU. Nine days later, still receiving her intravenous steroids, the visual acuity was 15/ 30 Allen OD, and 20/ 30 Allen OS. She was discharged on dexamethasone 4 mg by mouth twice daily. On her most recent examination, 2 months after the bilateral optic nerve sheath decompression, a visual acuity of 20/ 30 Allen OU was noted, as well as bilateral optic atrophy. No relative afferent pupillary defect was noted. She was being tapered off her steroids, and was currently on a dose of dexamethasone 0.25 mg by mouth every 3 days. CASE 2 A 5- year- old girl presented with generalized headache and malaise, and an inability to read her school work for 1 week. The patient was in her usual state of good health until 3 weeks prior to presentation, when she had an episode of gastroenteritis. A remote history of febrile seizures was elicited. Her neurologic examination was normal except for her visual changes. Best corrected visual acuity at presentation was 20/ 400 OD and 20/ 400 OS. A trace relative afferent pupillary defect was noted OS. Fundus examination revealed bilateral disc edema with fine peripapillary exudates. Gold-mann visual field testing showed an enlarged blind spot with a paracentral scotoma OD and an enlarged blind spot OS. CT scanning of the head was normal. An MRI study showed a left frontal lesion and a left thalamic abnormality, which had increased signal on T2- weighted images ( Fig. 2). Lumbar puncture showed an opening pressure of 360 mmH20; white blood cells, 6/ mm3; red blood cells, 0/ mm3; protein, 26 mg/ dl; and glucose, 55 mg/ dl. No oligoclonal bands were found. Lyme and mycoplasma titers, as well as both bacterial and fungal cultures were negative. She was placed on oral dexamethasone 4 mg every 8 hours and gradually tapered to a regimen of 1 mg every 8 hours. A follow- up lumbar puncture done 5 days after admission revealed an opening pressure of 360 mmH20 with WBC, 0/ mm3. Her dexamethasone dose was again increased to 2 mg orally every 8 hours. The patient became verbally and physically violent, which was believed to be due to the dexamethasone. Outpatient examinations documented an improvement of visual acuity to 20/ 30 OD and 20/ 50 OS. Optic atrophy with trace disc edema was noted OU. After completing her steroid taper, she developed headache, vomiting, and lethargy. Her affect became withdrawn. Repeat serial lumbar punctures, despite the addition of oral acetazol- FIG. 2. Magnetic resonance imaging of Case 2 demonstrating left thalamic lesion noted at presentation. ( TE = 80, TR = 2500.) / Neuro- Ophthalmol, Vol. 14, No. 4, 1994 222 B. D. ELLIS ET AL. amide, had opening pressures ranging from 230 to 310 mmH20. A lumboperitoneal shunt was placed. Postoperatively, the patient was symptom-free on no medications with a visual acuity of 20/ 20 OU and a normal affect. DISCUSSION Infections such as measles, mononucleosis, mumps, varicella, pertussis, and vaccinations often precede childhood optic neuritis ( 1,2). The accompanying symptoms of headache, nausea, vomiting, and findings of spinal fluid lymphocytosis are more frequently seen in children than in adults ( 3,4). While these symptoms suggest a component of encephalomyelitis ( 4), the visual prognosis in children with optic neuritis is usually good ( 4- 6). Encephalomyelitis and optic neuritis of parainfectious etiology, or acute disseminated encephalomyelitis ( ADEM), may produce a variety of central nervous system signs, which can include drowsiness and coma. Of particular importance, ADEM may cause intracranial hypertension ( 7). Usually following viral infections or immunizations, the underlying pathologic process is likely to be an autoimmune reaction as viral particles have not been isolated or cultured from cerebrospinal fluid specimens ( 4,7). Stereotactic biopsy of these lesions has demonstrated perivascular inflammation, with no evidence of viral particles ( 4). While corticosteroids are viewed as the treatment of choice ( 8- 10), intracranial pressure monitoring has shown that some patients have wide fluctuations in intracranial pressure and may be unresponsive to this therapy ( 7). MRI has been found to be the most sensitive imaging modality in detecting the extent of the multifocal brain lesions typical of ADEM ( 11). CT scanning was normal in three cases in which Farris and Pickard ( 1) detected high- intensity white matter signals by MRI. Both of our patients had initial CT scans that did not reveal focal parenchymal lesions. Both of our patients described a recent gastroenteritis prior to presentation. Additionally, both patients had lesions on MRI, spinal fluid pleocytosis, and a marked increase in intracranial pressure. While initial treatment with high- dose corticosteroids achieved favorable results, Case 1 had a relapse with vision decreasing to no light perception in one eye after completing an outpatient steroid taper. As the underlying pathologic process included components of both inflammation and increased intracranial pressure, intravenous corticosteroids were reinstituted and a bilateral optic nerve sheath decompression was performed. The second patient developed chronic symptoms of headache and fluctuations in personality. Her papilledema persisted, and she eventually underwent a lumboperitoneal shunt with relief of symptoms. Whether these relapses are due to an inadequacy in steroid treatment duration or strength is uncertain. Hamed and colleagues ( 4) described two patients with parainfectious optic neuritis and encephalomyelitis and normal intracranial pressure that had subsequent relapses with completion of an outpatient steroid taper. High- dose steroids were reinstituted in these patients with return of normal vision. In a patient with multiple subsequent relapses, Imuran was eventually instituted ( 4). Ziegler ( 8) reported a patient with acute disseminated encephalitis who had recurrence of blindness with reduction in corticosteroid dose. An elevated intracranial pressure was recorded during a relapse. We used both surgical and medical modalities to treat acute visual loss, which occurred approximately 1 month after hospital discharge in Case 1. While optic nerve sheath decompression may relieve ischemia in the setting of papilledema ( 12), the efficacy of the individual surgical and the individual medical components of treatment in our patients with acute disseminated encephalomyelitis cannot be determined by our case reports. We suspect the hydrodynamic forces on the optic nerve created by the increase in cerebrospinal fluid pressure would create more damage than the presumed demyelination alone. 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Kriss A, Francis D, Cuendet F, et al. Recovery after optic neuritis in childhood. / Neurol Neurosurg Psychol 1988; 51: 1254- 8. / Neuro- Ophthalmol, Vol. 14, No. 4, 1994 ACUTE DISSEMINATED ENCEPHALOMYELITIS 213 7. Garcia- Merino A, Garcia- Sola R, Vela L, Martin- Gonzalez E. Intracranial pressure monitoring in acute disseminated encephalomyelitis in childhood. Crit Care Med 1990; 18: 1481- 3. 8. Ziegler DK. Acute disseminated encephalitis. Arch Neurol 1966; 14: 476- 88. 9. Pasternak JF, De Vivo DC, Prensky AL. Steroid- responsive encephalomyelitis in childhood. Neurology 1980; 30: 481- 6. 10. Adams RD, Victor M. Multiple sclerosis and allied demy-elinative diseases. In: Principles of neurology, 2nd ed. New York: McGraw- Hill; 1981: 647- 63. 11. Atlas SW, Grossman RI, Goldberg HI, et al. MR diagnosis of acute disseminated encephalomyelitis. / Comput Assist Tomogr 1986; 10: 798- 801. 12. Mittra RA, Sergott RC, Flaharty PM, et al. 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