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Subacute Progressive Ptosis, Ophthalmoplegia, Gait Instability, and Cognitive Changes

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A 70-year-old woman with long-term tobacco use presented with 2 weeks of bilateral ptosis and binocular horizontal diplopia. She denied weakness, difficulty swallowing, or breathing. On initial examination, she had a normal mental status, no pupillary abnormalities, normal visual acuity, and full visual fields. She had moderate exotropia, severe bilateral ptosis, impaired bilateral adduction and vertical ductions, and impaired convergence. Her abduction and vertical oculocephalic reflexes were intact. She had full strength and normal sensation and reflexes but had a slightly wide-based and unsteady gait. Over the subsequent weeks, she experienced worsening gait, falls, inattention, and short-term memory loss.

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D. Anti-Ma2 encephalitis

The patient also developed seizures, hypersomnolence, and the syndrome of inappropriate antidiuretic hormone secretion. Considering a history of long-term smoking, the new onset of progressive neurologic deficits with associated abnormalities on brain MRI raised concern for a paraneoplastic etiology. Computed tomography imaging scans of the chest, abdomen, and pelvis were obtained, revealing multiple lung nodules. Nodule biopsy results confirmed pulmonary adenocarcinoma. Given the constellation of ocular findings that were localized to the midbrain, paroxysmal hypersomnolence, and encephalopathy, anti-Ma encephalitis was suspected, and testing for the presence of anti-Ma antibodies in the CSF revealed elevated levels of anti-Ma2 antibodies.

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Article Information

Corresponding Author: Jessica Lin, MD, Department of Neurology, New York University Langone Medical Center, 222 E 41st St, 14th Floor, New York, NY 10017 (jessica.lin@nyumc.org).

Published Online: June 25, 2018. doi:10.1001/jamaneurol.2018.1588

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient for granting permission to publish this information.

References
1.
Dalmau  J, Graus  F, Villarejo  A,  et al.  Clinical analysis of anti-Ma2-associated encephalitis.  Brain. 2004;127(pt 8):1831-1844. doi:10.1093/brain/awh203PubMedGoogle ScholarCrossref
2.
Darnell  RB, Posner  JB.  Paraneoplastic syndromes involving the nervous system.  N Engl J Med. 2003;349(16):1543-1554. doi:10.1056/NEJMra023009PubMedGoogle ScholarCrossref
3.
Hoffmann  LA, Jarius  S, Pellkofer  HL,  et al.  Anti-Ma and anti-Ta associated paraneoplastic neurological syndromes: 22 newly diagnosed patients and review of previous cases.  J Neurol Neurosurg Psychiatry. 2008;79(7):767-773. doi:10.1136/jnnp.2007.118588PubMedGoogle ScholarCrossref
4.
Ko  MW, Dalmau  J, Galetta  SL.  Neuro-ophthalmologic manifestations of paraneoplastic syndromes.  J Neuroophthalmol. 2008;28(1):58-68. doi:10.1097/WNO.0b013e3181677fccPubMedGoogle ScholarCrossref
5.
Bennett  JL, Galetta  SL, Frohman  LP,  et al.  Neuro-ophthalmologic manifestations of a paraneoplastic syndrome and testicular carcinoma.  Neurology. 1999;52(4):864-867. doi:10.1212/WNL.52.4.864PubMedGoogle ScholarCrossref
6.
Bien  CG, Vincent  A, Barnett  MH,  et al.  Immunopathology of autoantibody-associated encephalitides: clues for pathogenesis.  Brain. 2012;135(pt 5):1622-1638. doi:10.1093/brain/aws082PubMedGoogle ScholarCrossref
7.
Dalmau  J, Graus  F.  Antibody-mediated encephalitis.  N Engl J Med. 2018;378(9):840-851. doi:10.1056/NEJMra1708712PubMedGoogle ScholarCrossref
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