Introduction: Paraneoplastic Encephalitis (PNE) is the neuropathological disorder of central nervous system, associated with a remote tumor, which presents with diverse of neuropsychological symptoms. There is still a deficiency of an aptly formulated management protocol.
Case report: We report the first case of anti- NMDA-R PNE secondary to ovarian teratoma from Pakistan, in a 17 year old girl that presented with acute psychosis. She underwent immediate tumor excision with plasmapheresis followed by a combination of corticosteroids and IV immunoglobulin therapy. The patient relapsed after a month despite the treatment presenting with mild psychosis, memory loss and cognitive impairment. Provided along with the case report is an extensive literature review (2010-2017) on previously reported cases of PNE with ovarian teratoma to improve understanding of current management of this form of encephalitis.
Conclusion: Paraneoplastic encephalitis is a critical yet reversible illness. Our comprehensive review and experience can be summarized in the following guidelines signifying the current clinical practices regarding PNE with ovarian teratoma. (I) PNE should be suspected in female patients presenting with an acute history of neuropsychiatric symptoms. (II) Tumor resection should be performed earliest to improve patient outcomes.(III) Administration of plasma exchange therapy in immediate postoperative period is recommended. (IV) Pulse therapy preceding intravenous immunoglobin therapy improves patient prognosis. (V) Patients should be discharged on maintenance dose of corticosteroids. (VI) Long term follow up is recommended to monitor for relapses.
Usama Khalid Choudry*, Areeba Nisar, Mahwish Amin, Waqar Saeed, Aisha Sanober, Qurat ul Ain Khan, Iram Aamir Siddiqa and Abdullah Khalid