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What Is the True Clinicopathologic Spectrum of Neuromyelitis Optica?

What Is the True Clinicopathologic Spectrum of Neuromyelitis Optica? We read with interest the article by Mealy at al1 on the clinicopathologic spectrum of neuromyelitis optica (NMO)/NMO spectrum disorder. We have the following additional comments on the NMO spectrum: Recurrent Conus Myeloradiculitis as a Presentation of NMO Spectrum Disorder A 42-year old woman presented with sudden-onset urinary retention that rapidly progressed to a flaccid paraplegia. Results of imaging studies were negative initially; repeat magnetic resonance imaging 5 days from symptom onset revealed conus myeloradiculitis. Results from extensive testing, including spinal angiogram as well as testing for NMO antibodies and visual evoked potentials, were negative. Results from repeat NMO antibody testing 1 year from the initial symptom onset were positive. Few reports have described conus involvement with NMO2; root involvement with NMO is even more rarely described. There are no cases in the current series where there is conus involvement or root involvement. Intractable Generalized Neuropathic Pruritus as a Heralding Symptom of NMO A 36-year-old woman presented with intractable generalized pruritus for 3 months, after which she developed quadriplegia with bulbar weakness. Cervical spine magnetic resonance imaging showed longitudinally extensive myelopathy from C2 to C5 with no enhancement. Results from tests for visual evoked potentials were normal, while test results for NMO antibodies and anti–Sjögren syndrome antigen A antibodies were positive. Few studies have described generalized pruritus with NMO.3 It would be interesting to know the prevalence of pruritus in NMO in the current study. Is Multiple Sclerosis Immunotherapy Beneficial in Some Patients With NMO? Although reports from previous case series have concluded that multiple sclerosis immunotherapy, including interferon and natalizumab, might not be beneficial in NMO, we reported a case where the patient initially diagnosed as having multiple sclerosis remained stable while taking interferons and natalizumab for many years and was eventually diagnosed as having NMO.4 It is unclear whether there is a subgroup of patients with NMO who might benefit from some of the multiple sclerosis immunomodulators. In conclusion, we agree with the authors that given the rarity of NMO, having a national registry or even a regional consortium would help answer some of these questions and shed more light on its protean manifestations. Back to top Article Information Correspondence: Dr Govindarajan, Cleveland Clinic Florida, 2950 Cleveland Clinic Blvd, Weston, FL 33331 (raghav316g@gmail.com). Conflict of Interest Disclosures: None reported. References 1. Mealy MA, Wingerchuk DM, Greenberg BM, Levy M. Epidemiology of neuromyelitis optica in the United States: a multicenter analysis. Arch Neurol. 2012;69(9):1176-118022733096PubMedGoogle ScholarCrossref 2. Elsone L, Townsend T, Mutch K, et al. Neuropathic pruritus (itch) in neuromyelitis optica [published online August 30, 2012]. Mult Scler22936333PubMedGoogle Scholar 3. Petelin Gadze Z, Hajnsek S, Basic S, Sporis D, Pavlisa G, Nankovic S. Patient with neuromyelitis optica and inflammatory demyelinating lesions comprising whole spinal cord from C2 level till conus: case report. BMC Neurol. 2009;9:5619852774PubMedGoogle ScholarCrossref 4. Govindarajan R, Salgado E. Is it too early to predict the failure of natalizumab in NMO? Arch Neurol. 2012;69(8):1085-1086, author reply 108622892674PubMedGoogle Scholar http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png JAMA Neurology American Medical Association

What Is the True Clinicopathologic Spectrum of Neuromyelitis Optica?

JAMA Neurology , Volume 70 (2) – Feb 1, 2013

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References (16)

Publisher
American Medical Association
Copyright
Copyright © 2013 American Medical Association. All Rights Reserved.
ISSN
2168-6149
eISSN
2168-6157
DOI
10.1001/jamaneurol.2013.963
Publisher site
See Article on Publisher Site

Abstract

We read with interest the article by Mealy at al1 on the clinicopathologic spectrum of neuromyelitis optica (NMO)/NMO spectrum disorder. We have the following additional comments on the NMO spectrum: Recurrent Conus Myeloradiculitis as a Presentation of NMO Spectrum Disorder A 42-year old woman presented with sudden-onset urinary retention that rapidly progressed to a flaccid paraplegia. Results of imaging studies were negative initially; repeat magnetic resonance imaging 5 days from symptom onset revealed conus myeloradiculitis. Results from extensive testing, including spinal angiogram as well as testing for NMO antibodies and visual evoked potentials, were negative. Results from repeat NMO antibody testing 1 year from the initial symptom onset were positive. Few reports have described conus involvement with NMO2; root involvement with NMO is even more rarely described. There are no cases in the current series where there is conus involvement or root involvement. Intractable Generalized Neuropathic Pruritus as a Heralding Symptom of NMO A 36-year-old woman presented with intractable generalized pruritus for 3 months, after which she developed quadriplegia with bulbar weakness. Cervical spine magnetic resonance imaging showed longitudinally extensive myelopathy from C2 to C5 with no enhancement. Results from tests for visual evoked potentials were normal, while test results for NMO antibodies and anti–Sjögren syndrome antigen A antibodies were positive. Few studies have described generalized pruritus with NMO.3 It would be interesting to know the prevalence of pruritus in NMO in the current study. Is Multiple Sclerosis Immunotherapy Beneficial in Some Patients With NMO? Although reports from previous case series have concluded that multiple sclerosis immunotherapy, including interferon and natalizumab, might not be beneficial in NMO, we reported a case where the patient initially diagnosed as having multiple sclerosis remained stable while taking interferons and natalizumab for many years and was eventually diagnosed as having NMO.4 It is unclear whether there is a subgroup of patients with NMO who might benefit from some of the multiple sclerosis immunomodulators. In conclusion, we agree with the authors that given the rarity of NMO, having a national registry or even a regional consortium would help answer some of these questions and shed more light on its protean manifestations. Back to top Article Information Correspondence: Dr Govindarajan, Cleveland Clinic Florida, 2950 Cleveland Clinic Blvd, Weston, FL 33331 (raghav316g@gmail.com). Conflict of Interest Disclosures: None reported. References 1. Mealy MA, Wingerchuk DM, Greenberg BM, Levy M. Epidemiology of neuromyelitis optica in the United States: a multicenter analysis. Arch Neurol. 2012;69(9):1176-118022733096PubMedGoogle ScholarCrossref 2. Elsone L, Townsend T, Mutch K, et al. Neuropathic pruritus (itch) in neuromyelitis optica [published online August 30, 2012]. Mult Scler22936333PubMedGoogle Scholar 3. Petelin Gadze Z, Hajnsek S, Basic S, Sporis D, Pavlisa G, Nankovic S. Patient with neuromyelitis optica and inflammatory demyelinating lesions comprising whole spinal cord from C2 level till conus: case report. BMC Neurol. 2009;9:5619852774PubMedGoogle ScholarCrossref 4. Govindarajan R, Salgado E. Is it too early to predict the failure of natalizumab in NMO? Arch Neurol. 2012;69(8):1085-1086, author reply 108622892674PubMedGoogle Scholar

Journal

JAMA NeurologyAmerican Medical Association

Published: Feb 1, 2013

Keywords: neuromyelitis optica

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