J Clin Neurol.  2014 Jul;10(3):262-266. 10.3988/jcn.2014.10.3.262.

Anti-Ma2 Paraneoplastic Encephalitis in Association with Recurrent Cervical Cancer

• Ney DE
• Messersmith W
• Behbakht K

• KMID: 2179455
• DOI: http://doi.org/10.3988/jcn.2014.10.3.262

Abstract

BACKGROUND
Paraneoplastic neurological syndromes are rare, and although they are frequently associated with gynecological malignancies, cervical cancer is a rare cause. The symptoms of anti-Ma2 encephalitis are diverse and often present prior to the diagnosis of malignancy.
CASE REPORT
We report a case of a 37-year-old woman with a history of cervical cancer presenting with unexplained weight gain and vertical supranuclear gaze palsy. Magnetic resonance imaging of the brain revealed lesions within the bilateral hypothalami and midbrain. Anti-Ma2 antibodies were eventually found in the serum, prompting a search for malignancy. Recurrent metastatic cervical cancer was found in the retroperitoneal lymph nodes.
CONCLUSIONS
This is the first report of cervical cancer in association with anti-Ma2 encephalitis, and highlights the need for a high degree of suspicion in patients with a cancer history presenting with neurological symptoms. The symptoms associated with anti-Ma2 encephalitis are diverse and typically precede the diagnosis of cancer in patients, and should trigger a search for an underlying malignancy.

Keyword

paraneoplastic syndromes; cervical cancer; anti-Ma2 encephalitis

MeSH Terms

Adult
Antibodies
Brain
Diagnosis
Encephalitis*
Female
Humans
Lymph Nodes
Magnetic Resonance Imaging
Mesencephalon
Paralysis
Paraneoplastic Syndromes
Uterine Cervical Neoplasms*
Weight Gain
Antibodies

Figure

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  Fig. 1 Postgadolinium T1 (A) and fluid-attenuated inversion recovery (FLAIR) (B) sequences obtained upon presentation. Postgadolinium T1 (C) and FLAIR (D) sequences after treatment with high-dose steroids, chemotherapy, and directed radiation to the involved pelvic lymph nodes.

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  Fig. 2 Fused PET/CT showing hypermetabolic activity in enlarged left retroperitoneal and common iliac lymph nodes.

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  Fig. 3 Hematoxylin-and-eosin-stained slide of retroperitoneal lymph node biopsy tissue showing metastatic adenocarcinoma of the cervix (magnification ×400).

Reference

1. Voltz R, Gultekin SH, Rosenfeld MR, Gerstner E, Eichen J, Posner JB, et al. A serologic marker of paraneoplastic limbic and brain-stem encephalitis in patients with testicular cancer. N Engl J Med. 1999; 340:1788–1795.

2. Graus F, Delattre JY, Antoine JC, Dalmau J, Giometto B, Grisold W, et al. Recommended diagnostic criteria for paraneoplastic neurological syndromes. J Neurol Neurosurg Psychiatry. 2004; 75:1135–1140.

3. Ahern GL, O'Connor M, Dalmau J, Coleman A, Posner JB, Schomer DL, et al. Paraneoplastic temporal lobe epilepsy with testicular neoplasm and atypical amnesia. Neurology. 1994; 44:1270–1274.

4. Dalmau J, Graus F, Villarejo A, Posner JB, Blumenthal D, Thiessen B, et al. Clinical analysis of anti-Ma2-associated encephalitis. Brain. 2004; 127(Pt 8):1831–1844.

5. Hoffmann LA, Jarius S, Pellkofer HL, Schueller M, Krumbholz M, Koenig F, 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; 79:767–773.

6. Adams C, McKeon A, Silber MH, Kumar R. Narcolepsy, REM sleep behavior disorder, and supranuclear gaze palsy associated with Ma1 and Ma2 antibodies and tonsillar carcinoma. Arch Neurol. 2011; 68:521–524.

7. Darnell RB, Posner JB. Paraneoplastic Syndromes. New York: Oxford University Press;2011.

8. Johannis W, Renno JH, Wielckens K, Voltz R. Ma2 antibodies: an evaluation of commercially available detection methods. Clin Lab. 2011; 57:321–326.

9. Okagbue UE, Wang LY, Cymet T, Rabin B, Erlich RB. A rare case of anti-Hu paraneoplastic neurologic syndrome in association with cervical cancer. Compr Ther. 2008; 34:48–50.

10. Power DG, McVey GP, Delaney DW, Rea D, D'arcy T, Daly PA, et al. Papillary serous carcinomas of the uterine cervix and paraneoplastic cerebellar degeneration: a report of two cases. Acta Oncol. 2008; 47:1590–1593.

11. Sutton GP, Siemers E, Stehman FB, Ehrlich CE. Eaton-Lambert syndrome as a harbinger of recurrent small-cell carcinoma of the cervix with improvement after combination chemotherapy. Obstet Gynecol. 1988; 72(3 Pt 2):516–518.

12. Darnell RB, Posner JB. Paraneoplastic syndromes involving the nervous system. N Engl J Med. 2003; 349:1543–1554.

13. Linke R, Schroeder M, Helmberger T, Voltz R. Antibody-positive paraneoplastic neurologic syndromes: value of CT and PET for tumor diagnosis. Neurology. 2004; 63:282–286.