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June 2015; 2 (3) Clinical/Scientific NotesOpen Access

Neuromyelitis optica phenotype associated with therapy-responsive acute peripheral neuropathy

Anteneh M. Feyissa, Elena Shanina, Rahul Shah, Robert G. Smith
First published March 12, 2015, DOI: https://doi.org/10.1212/NXI.0000000000000083
Anteneh M. Feyissa
From the Department of Neurology (A.M.F.), Mayo Clinic, Rochester, MN; and Department of Neurology (E.S., R.S., R.G.S.), University of Texas Medical Branch at Galveston, Galveston, TX.
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Elena Shanina
From the Department of Neurology (A.M.F.), Mayo Clinic, Rochester, MN; and Department of Neurology (E.S., R.S., R.G.S.), University of Texas Medical Branch at Galveston, Galveston, TX.
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Rahul Shah
From the Department of Neurology (A.M.F.), Mayo Clinic, Rochester, MN; and Department of Neurology (E.S., R.S., R.G.S.), University of Texas Medical Branch at Galveston, Galveston, TX.
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Robert G. Smith
From the Department of Neurology (A.M.F.), Mayo Clinic, Rochester, MN; and Department of Neurology (E.S., R.S., R.G.S.), University of Texas Medical Branch at Galveston, Galveston, TX.
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Citation
Neuromyelitis optica phenotype associated with therapy-responsive acute peripheral neuropathy
Anteneh M. Feyissa, Elena Shanina, Rahul Shah, Robert G. Smith
Neurol Neuroimmunol Neuroinflamm Jun 2015, 2 (3) e83; DOI: 10.1212/NXI.0000000000000083

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Case report.

A 51-year-old Hispanic woman with a history of Churg-Strauss syndrome (in remission for 20 years)–associated mesangial glomerulonephritis and end-stage renal disease with a recent renal transplant and previously cured cytomegalovirus (CMV) infection who was on a tapering dose of tacrolimus presented with sudden onset of lower extremity numbness. Symptoms progressed to complete paraplegia and sensory loss over 24 hours. The following day, ascending paresis extended to bilateral upper extremities, with high thoracic sensory level and complete blindness. Initial MRI revealed contrast enhancement of bilateral prechiasmatic optic nerve as well as T2 hyperintensities involving the central gray matter of the entire spinal cord with associated edema. CSF analysis documented neutrophilic pleocytosis (182 neutrophils per mm3) and increased total protein level (142 mg/dL) without oligoclonal banding. Bacterial, fungal, and mycobacterial cultures from the CSF were negative, as was PCR for CMV, Epstein-Barr virus, herpes simplex virus, human herpesvirus 6, and varicella-zoster virus. Serologic autoimmune assessment, including onconeuronal, anti-neutrophil cytoplasmic antibody, and other rheumatologic, Lyme, and aquaporin-4 (AQP4) antibody testing, was negative. Moreover, testing for sarcoidosis and HIV antibody was unrevealing. Nerve conduction studies (NCS) performed 2 weeks after symptom onset documented severe reduction in compound motor action potential amplitudes (worse in the lower extremities), <10% slowing of distal latencies but not conduction velocities, and mildly slowed upper extremity f-wave latencies. Sensory nerve action potentials were pathologically reduced in the lower extremities (table). Needle examination (EMG) showed active, severe denervation changes in lower extremity muscles and less severe changes in upper extremity muscles. Subsequent GM1, GM2, GD1a, GD1b, and GQ1b ganglioside antibody testing was negative. The patient was diagnosed with concurrent clinically definite neuromyelitis optica (NMO) phenotype1 and acute axonal polyradiculoneuropathy with primarily axonal features.

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Table

Compound motor and sensory nerve action potential amplitudes before and after immunomodulating therapies

The patient was treated initially with methylprednisolone followed by 5 cycles of plasma exchange, with minimal improvement in upper extremity muscle strength and sensation despite repeat MRI showing partial resolution of the previously seen lesions. Subsequently she received 4 weekly cycles of IV rituximab therapy. Follow-up at 7 and 14 months demonstrated normal strength in her upper extremities with decreased reflexes, and she was able to walk with use of a walker. Moreover, the patient regained complete right eye vision with improved left eye vision (20/400), clinically consistent with a monophasic process. Repeat NCS/EMG at 7 months revealed improvement, and at 14 months NCS were normal and EMG was significantly improved (table). Repeat AQP4 antibody testing was negative at 7 months posttreatment.

Discussion.

Until recently, NMO was believed to be confined to the optic nerves and spinal cord, with no involvement of the peripheral nervous system (PNS). Routine EMG/NCS are not commonly indicated for patients with NMO, so the prevalence of PNS damage coexisting with this disorder is unknown. There have been 5 case reports of seropositive NMO with possible involvement of the PNS,2–4 speculated to result from AQP4 antibody–mediated astrocyte dysfunction or complement activation associated with peripheral AQP4 antibody targets.5 However, to the best of our knowledge, this association has not been described in the setting of seronegative NMO phenotype disease.

Recent studies have shown that some AQP4 antibody–seronegative patients have antibodies against myelin oligodendrocyte glycoprotein (MOG) and other proteins.6 Conceivably, although MOG is localized to the CNS, other targeted complement-activating antibodies might damage the PNS similarly to the mechanism hypothesized for AQP4 antibodies.1,6 Moreover, fulminant NMO spectrum disorders (NMOSDs) are characterized by the presence of severe inflammation, evidenced by neutrophilic pleocytosis and elevated protein in the CSF.7 Reduced local blood flow, hypoxia, and the blood–brain barrier destruction from severe inflammation may partly explain the involvement of the peripheral nerves. Coexistence of multiple autoimmune diseases with NMOSDs has been recently recognized.7 Similar to other immune-mediated diseases, NMO may be a manifestation of a genetic predisposition to multiple autoimmune conditions.7 Immune reconstitution in the face of acute viral syndromes, medications, or other causes for immune activation has also been well-described in patients with HIV, as well as in transplant patients following tapering of immunomodulation. It is unknown whether simultaneous onset of the peripheral and CNS involvement in our patient results from an identical antigen targeting 2 different parts of the neuraxis. Lastly, peripheral B cell–mediated antibody activation of the complement system, inflammatory neuronal damage, and necrosis in NMOSDs may benefit from treatment agents like plasmapheresis and rituximab. It is possible that a common mechanism, likely autoimmune in nature, caused the concurrent damage to the central and peripheral neural elements seen in our patient.

This report highlights the association of NMOSDs with peripheral neuropathies and polyradiculopathies. It is essential that clinicians are aware of this unusual association and obtain NCS/EMG whenever there is a clinical suspicion for concomitant involvement of peripheral nerves with NMOSD.

Footnotes

  • ↵* These authors contributed equally to this manuscript.

  • Author contributions: Study concept and design: Drs. Feyissa, Shanina, Shah, and Smith. Acquisition of data: Drs. Feyissa, Shanina, Shah, and Smith. Analysis and interpretation of data: Drs. Feyissa, Shanina, and Smith. Drafting of the manuscript: Drs. Feyissa, Shah, and Shanina. Critical revision of the manuscript for important intellectual content: Drs. Feyissa, Shanina, and Smith.

  • Study funding: No targeted funding reported.

  • Disclosure: A.M. Feyissa, E. Shanina, and R. Shah report no disclosures. R.G. Smith received research support from the University of Texas system. Go to Neurology.org/nn for full disclosure forms. The Article Processing Charge was paid by the authors.

  • Received November 19, 2014.
  • Accepted in final form January 20, 2015.
  • © 2015 American Academy of Neurology

This is an open access article distributed under the terms of the Creative Commons Attribution-Noncommercial No Derivative 3.0 License, which permits downloading and sharing the work provided it is properly cited. The work cannot be changed in any way or used commercially.

References

  1. 1.↵
    1. Wingerchuk DM,
    2. Lennon VA,
    3. Lucchinetti CF,
    4. et al
    . The spectrum of neuromyelitis optica. Lancet Neurol 2007;6:805–815.
    OpenUrlCrossRefPubMed
  2. 2.↵
    1. Laemmer AB,
    2. Maihöfner C,
    3. Gölitz P,
    4. et al
    . Possible second motor neuron damage in neuromyelitis optica. Clin Neurophysiol 2014;125:859–861.
    OpenUrl
  3. 3.↵
    1. Kitada M,
    2. Suzuki H,
    3. Ichihashi J,
    4. et al
    . Acute combined central and peripheral demyelination showing anti-aquaporin 4 antibody positivity. Intern Med 2012;51:2443–2447.
    OpenUrlCrossRefPubMed
  4. 4.↵
    1. Warabi Y,
    2. Yamazaki M,
    3. Shimizu T,
    4. Nagao M
    . Abnormal nerve conduction study findings indicating the existence of peripheral neuropathy in multiple sclerosis and neuromyelitis optica. Biomed Res Int 2013;2013:847670.
    OpenUrl
  5. 5.↵
    1. Kato J,
    2. Takai Y,
    3. Hayashi MK,
    4. et al
    . Expression and localization of aquaporin-4 in sensory ganglia. Biochem Biophys Res Commun 2014;451:562–567.
    OpenUrl
  6. 6.↵
    1. Kitley J,
    2. Woodhall M,
    3. Waters P,
    4. et al
    . Myelin-oligodendrocyte glycoprotein antibodies in adults with a neuromyelitis optica phenotype. Neurology 2012;79:1273–1277.
    OpenUrlCrossRef
  7. 7.↵
    1. Pittock SJ,
    2. Lennon VA,
    3. de Seze J,
    4. et al
    . Neuromyelitis optica and non organ-specific autoimmunity. Arch Neurol 2008;65:78–83.
    OpenUrlCrossRefPubMed

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