WBR0879

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Author [[PageAuthor::Serge Korjian M.D. (Reviewed by Serge Korjian)]]
Exam Type ExamType::USMLE Step 1
Main Category MainCategory::Pathology
Sub Category SubCategory::Neurology
Prompt [[Prompt::A 14-year-old girl presents to the pediatrics clinic for 1 week of dull headache, with recent onset nausea and vomiting. The girl's mother also reports that her daughter is assuming weird head postures in order to look at objects or persons higher that her eye level. On physical exam, the physician notices total loss of vertical gaze, with nystagmus on attempted convergence. He also notes large pupils that only faintly react to light. Brain MRI detects a significant intracranial mass in close proximity to the brainstem. Which tumor is most likely associated with this patient's presentation?]]
Answer A AnswerA::Meningioma
Answer A Explanation AnswerAExp::Meningiomas are rarely diagnosed in patients with Parinaud syndrome. Menigiomas can have many different presentations depending on their location.
Answer B AnswerB::Acoustic schwannoma
Answer B Explanation AnswerBExp::Classically, schwannomas are associated with facial nerve palsies not with Parinaud syndrome.
Answer C AnswerC::Oligodendroglioma
Answer C Explanation AnswerCExp::Oligodendroglioma is not linked to the presentation of Parinaud syndrome.
Answer D AnswerD::Germinoma
Answer D Explanation AnswerDExp::Germinoma, the most common pineal gland tumor, is classically associated with Parinaud syndrome.
Answer E AnswerE::Ependymoma
Answer E Explanation AnswerEExp::Ependymoma is not associated with Parinaud syndrome. Classically ependymomas present with hydrocephalus.
Right Answer RightAnswer::D
Explanation [[Explanation::Parinaud syndrome, characterized by paralyis of conjugate upward gaze, is the most common presentation of pineal gland tumors particularly germinomas seen in approximately half of presenting patients. It is also known as dorsal midbrain syndrome, occuring secondary to compression of the tectum. Other signs of Parinaud syndrome include pseudo-argyll robertson pupils, and excessive eyelid retraction. Germinomas are the most common pineal gland tumors classically presenting with Parinaud syndrome, hydrocephalus, or precocious puberty in young children. Germinomas usually have a good prognosis, and most patients improve with radiation therapy.

Educational Objective: Germinomas are the most common form of pineal gland tumors usually presenting with Parinaud syndrome in up to 50% of patients.
References: Packer RJ, Cohen BH, Cooney K, Coney K. Intracranial germ cell tumors. Oncologist. 2000;5(4):312-20.]]

Approved Approved::Yes
Keyword WBRKeyword::Parinaud syndrome, WBRKeyword::Pineal gland, WBRKeyword::Germinoma, WBRKeyword::Pinealoma
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