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Reversible Paraneoplastic Encephalomyelitis Associated with a Benign Ovarian Teratoma

  • R. Blaine Taylor (a1), Warren Mason (a1), Kester Kong (a1) and Richard Wennberg (a1)

Abstract

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 Introduction:

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.

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References

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