X' Marks the Spot

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Identifier walsh_2021_s1_c3
Title X' Marks the Spot
Creator Luis Andre Leal Ferman; Leslie Bruch; John Buatti; Jeremy Greenlee
Affiliation (LALF) (LB) (JB) (JG) University of Iowa, Iowa City, Iowa
Subject Optic Chiasm; Intracranial Tumors; Magnetic Resonance Imaging (MRI); Vision Loss Binocular
Description In summary, this is a 24 year-old man without any significant past history who presented with a progressive chiasmal syndrome. He denied any other focal neurologic symptoms or symptoms of pituitary dysfunction. On exam, he had a bitemporal hemianopia and bilateral optic atrophy. MRI brain showed an enlarged chiasm and a left periventricular lesion abutting the genu of the corpus callosum. The differential diagnosis was thought to be either a primary intrinsic glioma or an inflammatory process. An extensive infectious, inflammatory, and rheumatologic work-up was unrevealing. CSF evaluation showed a lymphocytic pleocytosis and mildly increased protein concentration with positive oligoclonal bands. A course of high-dose steroids did not herald any clinical improvement. Neurosurgery was then consulted and a biopsy of the optic chiasm was performed; the pathology showed a germinoma. During that time period, he started complaining of polydipsia and polyuria. A diagnosis of central diabetes insipidus was made and he was started on DDAVP. Further endocrine testing also demonstrated a low testosterone level. Shortly after the diagnosis, he subsequently completed craniospinal irradiation (21 Gy) followed by ventricular boost (30 Gy) and primary site boost (45 Gy). His afferent visual function has remained stable since with a complete bitemporal hemianopsia and follow-up MRI brain showed no evidence of residual or recurrent disease.
History This 24 year-old man presented with several months of progressive visual loss in both eyes. His past medical, surgical and ocular history was unremarkable. There was no family history of phacomatosis. He complained of occasional binocular diplopia, but denied headaches or any other neurological symptoms. He denied galactorrhea, fatigue, and abdominal pain. On exam, his visual acuity was 20/125 OD and 20/50 OS. He had incomplete atypical bitemporal defects that respected the vertical meridian and pallor of both optic nerves, right more than left. Magnetic resonance imaging (MRI) of the brain showed an enlarged optic chiasm and a hyperintense left frontal periventricular lesion that was abutting the genu of the corpus callosum. Both lesions showed mild contrast enhancement. The differential diagnosis included a primary intrinsic chiasmal glioma versus an inflammatory etiology. An extensive infectious, inflammatory, and rheumatologic work-up was unrevealing. Cerebrospinal fluid (CSF) evaluation showed a mild lymphocytic pleocytosis (11/mL) and mildly increased protein concentration (54 mg/dL). The CSF showed 3 oligoclonal bands. CSF cytology and cultures were negative. Based on these findings, he received a 1-month course of high dose steroid treatment without clinical improvement and further visual loss in both eyes. Repeat MRI brain showed no radiological improvement. At this point, the patient started complaining of increased thirst. A diagnostic procedure was performed.
Disease/Diagnosis Primary intrinsic chiasmal germinoma.
Date 2021-02
References 1) Krolak-Salmon, Adrodias, Honnorat, Caudie Bret et al, Beware of optic neuritis!, The Lancet, vol1, 2002. 2) Chaudry, Ahmad, Whittington, Schatz, Morcos, Primary Intrinsic Chiasmal Germinoma, Journal of Neuro-Ophthalmology, 35, 171-174,2015. 3) Akiyama, Suzuki, Mikuni, Germinoma Mimicking Tumefactive Demyelinating Disease in Pediatric Patients, Pediatr Neurosurg, 2016. 4) Bowman, Bradley, Primary Chiasmal Germinoma, Journal of Clinical Neuro-Ophthalmology, 10, 9-17, 1990.
Language eng
Format video/mp4
Type Image/MovingImage
Source 53rd Annual Frank Walsh Society Meeting
Relation is Part of NANOS Annual Meeting 2021
Collection Neuro-Ophthalmology Virtual Education Library: Walsh Session Annual Meeting Archives: https://novel.utah.edu/Walsh/
Publisher North American Neuro-Ophthalmology Society
Holding Institution Spencer S. Eccles Health Sciences Library, University of Utah
Rights Management Copyright 2021. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright
ARK ark:/87278/s6674ckg
Setname ehsl_novel_fbw
ID 1697338
Reference URL https://collections.lib.utah.edu/ark:/87278/s6674ckg