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Show guidelines are lacking, reports in the literature of optic nerve sheath meningioma in children support surgical excision (2-5). In our patient, rapid progression documented with MRI and the immunohistochemical pattern of a Grade I meningioma with negative progesteron receptor combined with a strongly positive Bcl-2 immunoreaction and Ki-67 proliferative index .3% indicated aggressive behavior of the neoplasm (6). Distinguishing optic nerve sheath meningioma from optic nerve glioma with MRI early in the clinical course may be difficult with neuroimaging (2,3). Gliomas usually are characterized by fusiform enlargement with kinking and tortuosity, with no calcification and variable contrast en-hancement. Optic nerve sheath meningiomas often lead to tubular enlargement with homogeneous texture and smooth or slightly lobulated peripheral margins, presence of calcifications, and homogeneous contrast enhancement (7,8). Our case confirms that pediatric optic nerve sheath meningioma is uncommon, but may grow rapidly, exhibit-ing more aggressive clinical behavior than its adult counterpart. Serena Beccari, MD Luca Cima, MD Ilaria Posenato, MD Francesco Sala, MD Claudio Ghimenton, MD Matteo Brunelli, MD, PhD Albino Eccher, MD, PhD Departments of Pathology and Diagnostic (SB, LC, IP, MB) and Neurosciences (FS), Azienda Ospedaliera Universitaria Integrata di Verona, Verona, Italy; and Department of Pathology and Diagnostic (CG, AE), Azienda Ospedaliera Universitaria Integrata di Verona, Ospedale Civile Maggiore, Verona, Italy The authors report no conflicts of interest. REFERENCES 1. Nabavizadeh SA, Santi M, Belasco JB, Zimmerman RA. Primary atypical optic nerve sheath meniogioma in a child. J. Neuroophthalmol. 2014;34:173-176. 2. Monteiro ML, Gonçalves AC, Siqueira SA, Gebrim EM. Optic nerve sheath meningioma in the first decade of life: case report and review of the literature. Case Rep Ophthalmol. 2012;3:270-276. 3. Harold Lee HB, Garrity JA, Cameron JD, Strianese D, Bonavolontà G, Patrinely JR. Primary optic nerve sheath meningioma in children. Surv Ophthalmol. 2008;53:543-558. 4. Dutton JJ. Optic nerve sheath meningiomas. Surv Ophthalmol. 1992;37:167-183. 5. Eddleman CS, Liu JK. Optic nerve sheath meningioma: current diagnosis and treatment. Neurosurg Focus. 2007;23:E4. 6. Maiuri F, De Caro MB, Esposito F, Cappabianca P, Strazzullo V, Pettinato G, de Divitiis E. Recurrences of meningiomas: predictive value of pathological features and hormonal and growth factors. J Neurooncol. 2007;82:63-68. 7. Ortiz O, Flores RA. Clinical and radiologic evaluation of optic pathway lesions. Semin Ultrasound CT MR. 1998;19:225-239. 8. Ortiz O, Schochet SS, Kotzan JM, Kostick D. Radiologic-pathologic correlation: meningioma of the optic nerve sheath. AJNR Am J Neuroradiol. 1996;17:901-906. Optic Neuritis in the Setting of NMDA Receptor Encephalitis We read with great interest the report by Sawanura et al (1) on anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis.We evaluated a patient who experienced optic neu-ritis (ON)with optic disc swelling in the setting of anti-NMDAR encephalitis relapse. No evidence of tumor was detected. A 29-year-old otherwise healthy man experienced acute generalized tonic-clonic seizures preceded by severe daily headaches, acute left-sided weakness, and dysarthria. Systemic work-up was normal including autoantibodies (anti-nuclear, anti-DNA, anti-Ro, anti-La, lupus-like anticoagulant, anti-cardiolipin, anti-beta2-glycoprotein, and anti-neutrophyl cytoplasmic antibodies) as well as cryoglobulins, complement levels, angiotensin-converting enzyme, rheumatoid factor, ammonia and lactate levels, and Coomb test. Serologies were negative for Borrelia burgdorferi; HIV-1 and 2; hepatitis A, B, and C; venereal disease research laboratory test; chlamydia; and mycoplasma. A cerebrospinal fluid (CSF) study revealed lymphocytic pleocytosis with 512 cells per milliliter, elevated protein of 1.64 g/L, normal glucose, and positive oligoclonal bands. CSF PCR testing for herpes simplex virus, varicella-zoster virus, cytomegalovirus, human herpesvirus 6, and en-terovirus were all negative. Brain magnetic resonance imaging (MRI) showed a right frontal cortical hyperintensity on T2 and fluid-attenuated inversion recovery (FLAIR) sequences. There was abnormal slow activity in the delta range, predom-inantly in the right hemisphere on the electroencephalogram (EEG). A diagnosis of presumed lymphocytic meningitis was established. The patient received intravenous methylprednis-olone (1 g/day) for 5 days and phenytoin with a clinical improvement. Three months later, brain MRI was unremark-able and the patient was symptom free. Three years later, he was readmitted to hospital because of headache and fluctuating motor and sensory deficits on the left side. Visual function was normal, but there was mild weakness in the left arm and touch impairment. Brain MRI revealed right frontotemporal hyperintensity without contrast 316 Letters to the Editor: J Neuro-Ophthalmol 2014; 34: 315-319 Letters to the Editor Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. enhancement (Fig. 1). EEG did not show epileptic discharges and CSF analysis was normal. Ten days after admission, the patient complained of painless blurred vision. Visual acuity was 20/60 in the right eye and 20/20 in the left eye, with a right relative afferent pupillary defect. On visual field test-ing, there was a central scotoma in the right eye and periph-eral constriction in the left eye. Ophthalmoscopy showed marked right optic disc swelling (Fig. 2). The patient was treated with intravenous methylprednis-olone (1 g/day) for 5 days. When steroids were tapered, he developed altered mental status with disorientation, psycho-motor agitation, visual hallucinations, and disorganized behavior. MRI revealed abnormal signal along the entire right optic nerve and similar change along the prechiasmatic portion of the left optic nerve (Fig. 3). Tumor serological markers and extensive panel of paraneoplastic antibodies in serum were negative (anti-Hu, anti-MA1/2, anti-CV2/ CRMP-5, anti-amphiphysin). NMDAR antibodies were detected in CSF (2). NMO-IgG/AQP4 antibodies were neg-ative, but antibodies to myelin oligodendrocyte glycoprotein (MOG-Ab) were found both in serum and CSF (3). Addi-tional work-up for malignancy, including thoracic, abdominal, and pelvic computed tomography, as well as a whole-body positron emission tomography and testicular ultrasound failed to reveal evidence of tumor. The patient was diagnosed with anti-NMDAR enceph-alitis and given 5 days of intravenous immunoglobulins (0.4 g/kg), which led to a dramatic improvement of symptoms and neuroimaging findings. Two months later, visual acuity was 20/20 in both eyes, with normal visual fields and mild optic disc pallor in the right eye. To the best of our knowledge, this is the first case of optic neuritis (ON) occurring in an adult patient with anti- NMDAR encephalitis. Two of the previously reported cases of ON were in Asian children (4,5), who experienced re-lapsing ON superimposed upon the typical symptoms of FIG. 1. Fluid-attenuated inversion recovery (FLAIR) imaging. A. At the onset of relapse, there is cortical hyperintensity (arrows) in the right frontal lobe. B. Improvement of the imaging abnormality (arrows) 3 months later. FIG. 2. There is right optic disc edema with peripapillary hemorrhage and tortuosity and dilation of retinal veins. The left fundus is normal. Letters to the Editor: J Neuro-Ophthalmol 2014; 34: 315-319 317 Letters to the Editor Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. NMDAR encephalitis. In both cases, no other antibodies were detected, including NMO-IgG/AQP4 antibodies. A third case was a 15-year-old girl who developed most of the symptoms of NMDAR encephalitis and had NMDAR antibodies (6). The patient later experienced recurrent lon-gitudinally extensive transverse myelitis and ON fulfilling the Wingerchuk criteria for NMO (7), despite NMO-IgG/ AQP4 seronegativity. In our patient, there was acute ON with right optic disc swelling and neuroimaging abnormalities of both optic nerves. In keeping with the favorable prognosis of NMDAR encephalitis (8), he was free of visual and neurological symp-toms 2 months after beginning the treatment. Our patient did not have an underlying teratoma, which is not surprising given that only 6% of young male patients with anti- NMDAR encephalitis have an associated tumor (8). We detected MOG-Ab in the serum and CSF of our patient. The presence of this antibody may indicate an overlap of autoimmune mechanisms in patients with blend phenotypes. Unlike IgG/AQP4, which appears to be the pathogenic in NMO, the role of MOG-Ab remains to be established and deserves further investigation. Álvaro Cobo-Calvo, MD Multiple Sclerosis Unit, Neurology Department, Hospital Universitari de Bellvitge, IDIBELL, L'Hospitalet de Llobregat, Barcelona, Spain Cristina Izquierdo Gracia, MD Neurology Department, Hospital Universitari de Bellvitge, IDIBELL. L'Hospitalet de Llobregat, Barcelona, Spain Silvia M. Quiñones, MD Neuro-ophtalmology Unit, Ophtalmology Department, Hospital Universitari de Bellvitge, IDIBELL. L'Hospitalet de Llobregat, Barcelona, Spain Carles M. Torro, MD, PhD Institut de Diagnòstic per la Imatge, Hospital Duran i Reynals, L'Hospitalet de Llobregat, Barcelona, Spain Albert Saiz, MD, PhD Josep Dalmau, MD, PhD Department of Neurology, Hospital Clinic, and Institut d´ Investigacio Biomedica August Pi i Sunyer (IDIBAPS), and University of Barcelona. Barcelona, Spain Sergio Martínez-Yélamos, MD, PhD Multiple Sclerosis Unit, Neurology Department, Hospital Universitari de Bellvitge, IDIBELL, L'Hospitalet de Llobregat, Barcelona, Spain The authors report no conflicts of interest. FIG. 3. With short tau inversion recovery sequences, coro-nal magnetic resonance imaging shows increased signal in the right orbital optic nerve (arrow) (A), and the pre-chiasmatic portion of both optic nerves (arrows) (B). C. Postcontrast T1 coronal scan demonstrates enhance-ment of both optic nerves (arrows). 318 Letters to the Editor: J Neuro-Ophthalmol 2014; 34: 315-319 Letters to the Editor Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. REFERENCES 1. Sawamura H, Yamamoto T, Ohtomo R, Bannai T, Wakakura M, Tsuhi S. Anti-NMDA receptor encephalitis associated with transient cerebral dyschromatopsia, prosopagnosia, and lack of stereopsis. J Neuroophthalmol. 2014;34:144-148. 2. Graus F, Saiz A, Lai M, Bruna J, López F, Sabater L, Blanco Y, Rey MJ, Ribalta T, Dalmau J. Neuronal surface antigen antibodies in limbic encephalitis: clinical-immunologic associations. Neurology. 2008;71:930-936. 3. Titulaer MJ, McCracken L, Gabilondo I, Armangué T, Glaser C, Iizuka T, Honig LS, Benseler SM, Kawachi I, Martinez- Hernandez E, Aguilar E, Gresa-Arribas N, Ryan-Florance N, Torrents A, Saiz A, Rosenfeld MR, Balice-Gordon R, Graus F, Dalmau J. Treatment and prognostic factors for long-term outcome in patients with anti-NMDA receptor encephalitis: an observational cohort study. Lancet Neurol. 2013;12:157-165. 4. Ishikawa N, Tajima G, Hyodo S, Takahashi Y, Kobayashi M. Detection of autoantibodies against NMDA-type glutamate receptor in a patient with recurrent optic neuritis and transient cerebral lesions. Neuropediatrics. 2007;38:257-260. 5. Motoyama R, Shiraishi K, Tanaka K, Kinoshita M, Tanaka M. Anti- NMDA receptor antibody encephalitis with recurrent optic neuritis and epilepsy [in Japanese]. Rinsho Shinkeigaku. 2010;50:585-588. 6. Kruer MC, Koch TK, Bourdette DN, Chabas D, Waubant E, Mueller S, Moscarello MA, Dalmau J, Woltjer RL, Adamus G. NMDA receptor encephalitis mimicking seronegative neuromyelitis optica. Neurology. 2010;74:1473-1475. 7. Wingerchuk DM, Lennon VA, Pittock SJ, Lucchinetti CF, Weinshenker BG. Revised diagnostic criteria for neuromyelitis optica. Neurology. 2006;66:1485-1489. 8. Titulaer MJ, Höftberger R, Lizuka T, Leypoldt F, McCracken L, Cellucci T, Benson L, Shu H, Irioka T, Hirano M, Shing G, Cobo Calvo A, Kaida K, Morales PS, Wirtz PW, Yamamoto T, Reindl M, Rosenfeld MR, Graus F, Saiz A, Dalmau J. Overlapping demyelinating syndromes and anti-NMDA receptor encephalitis. Ann Neurol 2014;75:411-428. Letters to the Editor: J Neuro-Ophthalmol 2014; 34: 315-319 319 Letters to the Editor Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. |
