Hostname: page-component-586b7cd67f-l7hp2 Total loading time: 0 Render date: 2024-11-26T02:40:54.570Z Has data issue: false hasContentIssue false
  • English
  • Français

Reversible Paraneoplastic Encephalomyelitis Associated with a Benign Ovarian Teratoma

Published online by Cambridge University Press:  02 December 2014

R. Blaine Taylor ,
Warren Mason ,
Kester Kong  and
Richard Wennberg
R. Blaine Taylor
Affiliation:
Division of Neurology, The Toronto Hospital, University of Toronto, ON
Warren Mason
Affiliation:
Division of Neurology, The Toronto Hospital, University of Toronto, ON
Kester Kong
Affiliation:
Division of Neurology, The Toronto Hospital, University of Toronto, ON
Richard Wennberg
Affiliation:
Division of Neurology, The Toronto Hospital, University of Toronto, ON
Rights & Permissions [Opens in a new window]

Abstract

Core share and HTML view are not available for this content. However, as you have access to this content, a full PDF is available via the ‘Save PDF’ action button.
Background:

Paraneoplastic encephalomyelitis (PEM) is a well characterized, and typically irreversible, paraneoplastic syndrome, usually associated with small cell lung cancer or other malignancy. We describe a case of a young woman with a benign ovarian teratoma who presented with a reversible PEM.

Case report:

A 24-year-old woman presented with a three week history of memory impairment, unusual behavior, personality changes, auditory hallucinations, hypersomnolence and binocular diplopia. On admission she was disoriented and inattentive with impaired short term memory. Small doses of lorazepam (1 mg), given for episodic agitation, repeatedly induced multidirectional bilateral nystagmus and a skew deviation, but her neurological examination was otherwise normal. A left-sided pelvic mass was palpable. Brain MRI pre- and post-gadolinium was normal. There was a mild CSF pleocytosis and an EEG showed minimal bilateral background activity irregularities. There were no other laboratory abnormalities. Two weeks after admission, she clinically deteriorated developing central respiratory failure and a flaccid paraplegia. Repeat MRI showed an area of increased T2 weighted signal in the medulla and three similar areas in the spinal cord. Following removal of her tumor, treatment with high dose corticosteroids and intravenous immunoglobulin, she ultimately made a full recovery. Pathology revealed the tumor to be a benign ovarian cystic teratoma.

Conclusion:

This is the first report of a reversible PEM seen in association with a benign tumor, in this case a mature ovarian teratoma. Presumably, an immune response directed against neural elements of the teratoma cross-reacted with normal brain, brainstem and spinal cord antigens to cause neurologic symptoms. Tumor removal was followed by neurologic recovery.

Résumé

RÉSUMÉIntroduction:

L’ encéphalomyélte paranéoplasique (EMP) est un syndrome paranéoplasique réversible bien caractérisé, habituellement associé à un cancer du poumon à petites cellules ou à un autre type de cancer. Nous décrivons le cas d’une jeune femme porteuse d’un tératome bénin de l’ovaire et qui a présenté une EMPréversible.

Préntation clinique:

Une jeune femme de 24 ans a consulté pour un problème de mémoire évoluant depuis 3 semaines, un comportement inhabituel, des changements de la personnalité, des hallucinations auditives, de la somnolence et de la diplopie binoculaire. À l’admission, elle était désorientée et inattentive et présentait un déficit de la mémoire à court terme. De petites doses de lorazépam (1mg), administré pour contrôler une agitation épisodique, ont induit à plusieurs reprises un nystagmus bilatéral multidirectionnel et une disjonction des mouvements du regard. Son examen neurologique demeurait par ailleurs normal. La palpation de l’abdomen a révélé une masse pelvienne gauche. L’IRM cérébrale pré et post-gadolinium était normale. Il existait une pléiocytose légère du LCR et l’ÉEG montrait des irrégularités minimes bilatérales de l’activité de fond. Il n’y avait pas d’autre anomalie de laboratoire. Deux semaines après l’admission, elle a présenté une défaillance respiratoire centrale et une paraplégie flasque. Des études IRM répétées ont montré une région de signal pondéré en T2 augmenté dans le bulbe rachidien et trois zones semblables dans la moelle épinière. Suite à l’ablation de sa tumeur, elle a reçu des doses élevées de corticostéroïdes et des immunoglobulines intraveineuses. Elle a finalement récupéré complètement. L’examen anatomopathologique de la tumeur a montré qu’il s’agissait d’un tératome kystique bénin de l’ovaire.

Conclusions:

C’est le premier rapport d’un cas d’EPM réversible observé en association avec une tumeur bénigne, soit un tératome ovarien mature. Il semble qu’une réponse immunitaire dirigée contre des éléments nerveux du tératome a induit une réaction croisée avec des antigènes du tissu cérébral normal, du tronc cérébral et de la moelle épinière provoquant des symptômes neurologiques. Elle a récupéré complètement suite à l’ablation de la tumeur.

Type
Case Report
Information
Canadian Journal of Neurological Sciences , Volume 26 , Issue 4 , November 1999 , pp. 317 - 320
Copyright
Copyright © The Canadian Journal of Neurological 1999

References

1. Voltz, RD, Posner, JB, Dalmau, J, Graus, F. Paraneoplasticencephalomyelitis: an update of the effects of the anti-Hu immune response on the nervous system and tumour. J Neurol Neurosurg Psychiatry 1997; 63: 133–136.Google Scholar
2. Benyahia, B, Liblau, R, Merle-Béral, H, et al. Cell-mediatedautoimmunity in paraneoplastic neurological syndromes with anti-Hu antibodies. Ann Neurol 1999; 45: 162–167.Google Scholar
3. Dalmau, J. Paraneoplastic syndromes of the nervous system:diagnostic approaches. In: Vinken, PJ, Bruyn, GW, eds. Handbook of Clinical Neurology, Vol. 69. Amsterdam: Elsevier Science, 1997: 319–328.Google Scholar
4. Dalmau, J, Graus, F, Rosenblum, MK, Posner, JB. Anti-Hu-associatedparaneoplastic encephalomyelitis/sensory neuronopathy. Medicine 1992; 71: 59–72.Google Scholar
5. Deodhare, S, O’Connor, P, Ghazarian, D, Bilbao, JM. Paraneoplasticlimbic encephalitis in Hodgkin’s disease. Can J Neurol Sci 1996; 23: 138–140.Google Scholar
6. Antoine, JC, Honnorat, J, Anterion, CT, et al. Limbic encephalitis andimmunological perturbations in two patients with thymoma. J Neurol Neurosurg Psychiatry 1995; 58: 706–710.CrossRefGoogle Scholar
7. Henson, RA, Hoffman, HL, Urich, H. Encephalomyelitis withcarcinoma. Brain 1965; 88: 449–464.Google Scholar
8. Baloh, RW, DeRossett, SE, Cloughesy, TF, et al. Novel brainstemsyndrome associated with prostate carcinoma. Neurology 1993; 43: 2591–2596.Google Scholar
9. Scully, RE, Mark, EJ, McNeely, BU. Case records of the MassachusettsGeneral Hospital. N Engl J Med 1985; 313: 249–257.Google Scholar
10. Burton, GV, Bullard, DE, Walther, PJ, Burger, PC. Paraneoplasticlimbic encephalopathy with testicular carcinoma. A reversible neurologic syndrome. Cancer 1988; 62: 2248–2251.3.0.CO;2-X>CrossRefGoogle ScholarPubMed
11. Tsukamoto, T, Mochizuki, R, Mochizuki, H, et al. Paraneoplastic cerebellar degeneration and limbic encephalitis in a patient with adenocarcinoma of the colon. J Neurol Neurosurg Psychiatry 1993; 56: 713–716.Google Scholar
12. Langston, JW, Dorfman, LJ, Forno, LS. “Encephalomyeloneuritis” inthe absence of cancer. Neurology 1975; 25: 633–637.Google Scholar
13. Kohler, J, Hufschmidt, A, Hermle, L, Volk, B, Lucking, CH. Limbicencephalitis: two cases. J Neuroimmunol 1988; 20: 177–178.Google Scholar
14. Daniel, SE, Love, S, Scaravilli, F, Harding, AE. Encephalomyeloneuropathy in the absence of a detectable neoplasm. Clinical and postmortem findings in three cases. Acta Neuropathol 1985; 66: 311–317.Google Scholar
15. Horoupian, DS, Kim, Y. Encephalomyeloneuropathy withganglionitis of the myenteric plexuses in the absence of cancer. Ann Neurol 1981; 11: 628–632.CrossRefGoogle Scholar
16. Torvik, A, Sletteb, M. Encephalomyelitis with polyneuropathy. Acta Neurol Scand 1980; 61: 287–297.Google Scholar
17. Aydiner, A, Gurvit, H, Baral, I. Paraneoplastic limbic encephalitiswith immature ovarian teratoma. J Neurooncol 1998; 37: 63–66.Google Scholar
18. Nokura, K, Yamamoto, H, Okawara, Y, et al. Reversible limbicencephalitis caused by ovarian teratoma. Acta Neurol Scand 1997; 95: 367–373.Google Scholar
19. Kawaguchi, K, Kishida, S, Okeda, R, Funata, N, Koike, M. Encephalomyeloneuritis with mediastinal germ cell tumor. Acta Pathol Jpn 1988; 38: 351–359.Google Scholar
20. Kaplan, AM, Itabashi, HH. Encephalitis associated with carcinoma. Central hypoventilation syndrome and cytoplasmic inclusion bodies. J Neurol Neurosurg Psychiatry 1974; 37: 1166–1176.Google Scholar
21. Corsellis, JAN, Goldberg, GJ, Norton, AR. Limbic encephalitis and itsassociation with carcinoma. Brain 1968; 91: 481–496.Google Scholar
22. Caldemeyer, KS, Smith, RR, Harris, TM, Edwards, MK. MRI in acutedisseminated encephalomyelitis. Neuroradiology 1994; 36: 216–220.Google Scholar
23. Byrne, T, Mason, WP, Posner, JB, Dalmau, J. Spontaneousneurological improvement in anti-Hu associated encephalo-myelitis. J Neurol Neurosurg Psychiatry 1997; 62: 276–278.CrossRefGoogle Scholar
24. Alamowitch, S, Graus, F, Uchuya, M, et al. Limbic encephalitis andsmall cell lung cancer. Clinical and immunological features. Brain 1997; 120: 923–928.Google Scholar