Annals of Indian Academy of Neurology
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Year : 2007  |  Volume : 10  |  Issue : 1  |  Page : 52-54

Reversible paraneoplastic encephalomyelitis as the presenting feature of ovarian teratoma: A clinicopathological correlate

1 Department of Medical Oncology, Nizam's Institute of Medical Sciences, Hyderabad, India
2 Department of Pathology, Nizam's Institute of Medical Sciences, Hyderabad, India
3 Department of Neurology, Nizam's Institute of Medical Sciences, Hyderabad, India

Correspondence Address:
Senthil J Rajappa
Dept. of Medical Oncology, Nizam's Institute of Medical Sciences, Punjagutta, Hyderabad - 500 016
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0972-2327.31487

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Paraneoplastic encephalomyelitis (PEM) is a well-characterized neurological syndrome. Its association with ovarian teratoma is rare. A young lady presented with features suggestive of encephalomyelitis with predominant cerebellar syndrome. Magnetic resonance imaging brain was normal. Cerebrospinal fluid showed lymphocytic pleocytosis. Computerized tomography scan of the pelvis revealed a complex left ovarian cyst. With a clinical diagnosis of PEM she underwent a left salpingo-oopherectomy. This was followed by total recovery of the PEM in two weeks. The histopathology revealed immature teratoma. The interesting feature was the clinicopathological correlation between the finding of fetal cerebellar tissue in the tumor and the PEM with predominant cerebellar features.

Keywords: Onconeural antigen, ovarian teratoma, paraneoplastic encephalomyelitis

How to cite this article:
Rajappa SJ, Naidu NB, Uppin S, Prayaga AK, Borgohain R, Digumarti R. Reversible paraneoplastic encephalomyelitis as the presenting feature of ovarian teratoma: A clinicopathological correlate. Ann Indian Acad Neurol 2007;10:52-4

How to cite this URL:
Rajappa SJ, Naidu NB, Uppin S, Prayaga AK, Borgohain R, Digumarti R. Reversible paraneoplastic encephalomyelitis as the presenting feature of ovarian teratoma: A clinicopathological correlate. Ann Indian Acad Neurol [serial online] 2007 [cited 2022 Aug 15];10:52-4. Available from:

   Introduction Top

Paraneoplastic encephalomyelitis (PEM) is characterized by involvement of multiple areas of the nervous system. It usually precedes the diagnosis of cancer and is typically irreversible.[1] Previous instances of limbic encephalitis have been reported with ovarian teratoma.[2],[3],[4],[5] Immunologic mechanisms have been implicated in the pathogenesis of PEM as evidenced by isolation of antibodies directed against onconeural antigens in the tumor tissue and T-lymphocyte activation.[6] Imaging of the brain is usually unremarkable. Examination of the cerebrospinal fluid (CSF) reveals lymphocytic pleocytosis with oligoclonal bands supporting an inflammatory or immune etiology.[1] Timely diagnosis is critical for appropriate treatment of the underlying malignancy. Immunosuppressive therapy is rarely effective in reversing PEM.[1] We report a case of reversible PEM with ovarian teratoma with an interesting clinicopathological correlate.

   Case Report Top

A 24-year-old lady presented with headache, vomiting, involuntary movements of the eyes and unsteadiness of gait progressing over a week. There was no history of other cranial nerve involvement, weakness of limbs, sensory or autonomic dysfunction, fever or seizures. She was not a known hypertensive or diabetic. There was no history of a similar episode in the past.

Examination revealed a conscious, coherent, afebrile patient with normal pulse, blood pressure and general physical examination. She had scanning dysarthria. Ocular examination revealed normal visual acuity, pupils, extra ocular movements and fundus. There was downbeat nystagmus with a gaze evoked horizontal component. Bilateral cerebellar signs were present, right more than left side. Examination of the rest of the nervous system was unremarkable.

Her complete blood picture, liver and renal function tests and serum electrolytes were normal. The magnetic resonance imaging (MRI) of the brain [plain and contrast] was normal. The CSF analysis showed lymphocytic pleocytosis with normal sugar and protein. It was negative for antineuronal antibodies [anti Ri, Hu, Yo] and malignant cells.

During the course of the hospital stay, she developed altered sensorium and irrelevant talk. Meanwhile, a CT scan of the abdomen and pelvis revealed a complex cystic mass in the left ovary without any paraaortic nodes or acsites [Figure - 1]. The Cancer Antigen 125, beta Human Chorionic Gonadotropin, Alpha Feto Protein and Lactate Dehydrogenase were within normal limits. In view of the classical neurological syndrome with a left complex ovarian cyst, a clinical diagnosis PEM with predominant cerebellar syndrome was made.

She underwent a staging laprotomy with left salpingo-oopherectomy. The histopathological examination showed a combination of mature elements comprising of skin with adnexal structures and islands of hyaline cartilage [Figure - 2]. The immature elements comprised of monomorphous round cells with focal areas showing neuroectodermal rosettes [Figure - 3]. In addition, there were areas showing fetal type of cerebellar tissue with an additional external granular layer [Figure - 4]. A diagnosis of Grade III immature ovarian teratoma was made.

She was started on cisplatin-based chemotherapy. During the next two weeks she had a total recovery of neurological symptoms and signs.

   Discussion Top

PEM includes a spectrum of clinical syndromes, which include limbic and brain stem encephalitis, sub acute cerebellar degeneration and myelitis.[1] PEM usually presents with features that have considerable overlap between these classical syndromes. A high index of suspicion is necessary to make a diagnosis. Our patient had predominant sub acute cerebellar degeneration suggested by limb incoordination and nystagmus with symptoms of limbic encephalitis appearing later, in the form of altered sensorium and irrelevant talk.

PEM is most commonly associated with small cell lung cancer followed by breast, ovarian, testicular cancers and Hodgkin's lymphoma.[1] Limbic encephalitis with ovarian teratoma is extremely rare.[2],[3],[4],[5] PEM with predominant cerebellar syndrome has been reported with ovarian carcinoma but has never been reported with ovarian teratoma.

Immunologic mechanisms are believed to be responsible for the pathogenesis of PEM as evidenced by T - lymphocyte responses and presence of antibodies like anti Yo, anti Hu, anti Purkinje cell antibody , which may be detected in the CSF or serum.[6] Immune responses appear to be elicited against onconeural antigens in the tumor, which then act on the nervous system to produce the syndrome. In contrast to the well-defined role of antibodies to surface antigens like acetylcholine receptor [as in myasthenia gravis], the role of antibodies to cytoplasmic and nuclear antigens [as in PEM] in the pathogenesis of these syndromes is unclear. Paraneoplastic antibodies may represent a surrogate for T-lymphocyte activation.[7] The relative contributions of cellular and humoral immunity in the pathogenesis of these syndromes have not been resolved.[8]

Although our patient did not have any antineuronal antibodies in the CSF, the interesting feature was the presence of fetal cerebellar tissue in the tumor. Finding cerebellar tissue in an ovarian teratoma is exceedingly rare.[9] The expression of cerebellar onconeural antigens in the tumor probably triggered an immune response leading to manifestations of PEM with predominant cerebellar signs. However, in the absence of demonstrable antibodies, a T-lymphocyte mediated immune response cannot be ruled out. To our knowledge, this is the first instance of a clinico-histopathological correlation between ovarian tumor tissue and PEM.

Previous instances of reversal of PEM associated with ovarian teratoma have been reported.[2],[4] Outcome of PEM with predominant sub acute cerebellar syndrome is poor with rare instances of recovery.[1] However, our patient's total recovery after removal of the ovarian tumor without use of immunosuppressive therapy was remarkable.

This clinico-histopathological correlation adds further evidence to the theory of a possible immune response to onconeural antigens in the tumor tissue as the basis for PEM. This is the first instance of a PEM with predominant cerebellar syndrome associated with ovarian teratoma. Reversibility of PEM associated with ovarian teratoma as in our patient, stresses the importance of recognizing this syndrome so that appropriate treatment can be directed against the primary.

   References Top Beukelaar JW, Sillevis Smitt PA. Managing paraneoplastic neurological disorders. Oncologist 2006;11:292-305.  Back to cited text no. 1  [PUBMED]  [FULLTEXT]
2.Taylor RB, Mason W, Kong K, Wennberg R. Reversible paraneoplastic encephalomyelitis associated with a benign ovarian teratoma. Can J Neurol Sci 1999;26:317-20.  Back to cited text no. 2  [PUBMED]  
3.Vitaliani R, Mason W, Ances B, Zwerdling T, Jiang Z, Dalmau J. Paraneoplastic encephalitis, psychiatric symptoms and hypoventilation in ovarian teratoma. Ann Neurol 2005;58:594-604.  Back to cited text no. 3  [PUBMED]  [FULLTEXT]
4.Muni RH, Wennberg R, Mikulis DJ, Wong AM. Bilateral Horizontal gaze palsy in presumed paraneoplastic brain stem encephalitis associated with Benign Ovarian Teratoma. J Neuro Ophthalmol 2004;24:114-8.  Back to cited text no. 4  [PUBMED]  [FULLTEXT]
5.Sutton I, Winer JB. The immunopathogenesis of paraneoplastic neurological syndromes. Clin Sci 2002;102:475-86.  Back to cited text no. 5  [PUBMED]  [FULLTEXT]
6.Aydiner A, Gurvit H, Baral I. Paraneoplastic limbic encephalitis with immature ovarian teratoma. J Neurooncol 1998;37:63-6.  Back to cited text no. 6  [PUBMED]  [FULLTEXT]
7.Tanaka M, Tanaka K, Tokiguchi S, Shinozawa K, Tsuji S. Cytotoxic T cells against a peptide of Yo protein in patients with paraneoplastic cerebellar degeneration and anti-Yo antibody. J Neurol Sci 1999;168:28-31.  Back to cited text no. 7  [PUBMED]  [FULLTEXT]
8.Yu Z, Kryzer TJ, Griesmann GE, Kim K, Benarroch EE, Lennon VA. CRMP-5 neuronal auto antibody: Marker of lung cancer and thymoma-related autoimmunity. Ann Neurol 2001;49:146-54.  Back to cited text no. 8  [PUBMED]  
9.Remadi S, Burkhardt K, Straccia AT, Pizzolato G, Mac Gee W. Well differentiated cerebellar tissue within a mature cystic teratoma. Pathol Res Pract 1998;194:371-4.  Back to cited text no. 9  [PUBMED]  


[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]


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