| Identifier |
walsh_2020_s1_c2 |
| Title |
From Brainstem to Stern (Video) |
| Creator |
Neena Cherayil; Angela Viane; Grant Liu; Ali Hamedani |
| Affiliation |
(NC) (AV) (GL) (AH) University of Pennsylvania, Philadelphia, Pennsylvania |
| Subject |
6th Nerve Palsy, Brain Stem Syndromes, Increased Intracranial Pressure, Intracranial Tumors |
| History |
A previously healthy 32-year-old man complained of diplopia and one month of worsening positional headaches. He then acutely developed confusion and chills, prompting ER referral. Further history was limited by altered mental status. On presentation, he was afebrile and encephalopathic. Serum WBC was 16.9/mL. Lactate was 6.2 mmol/L. Head CT was read as normal (Figure 1). Lumbar puncture revealed WBC 48/mL (80% monocytes, 19% lymphocytes, 1% neutrophils), RBC 234/ml, protein >600 mg/dL, and glucose 75 mg/dL. Vancomycin, ceftriaxone, acyclovir, ampicillin, and dexamethasone were started for infectious meningoencephalitis. The following day, he developed progressive obtundation with left mydriasis and ptosis. Head CT revealed evolving hydrocephalus (Figure 2). An external ventricular drain (EVD) was placed emergently. Head MRI showed diffuse leptomeningeal enhancement with expansile pontomedullary T2 hyperintensity (Figure 3). Mental status improved with EVD. Patient reported five months of binocular horizontal diplopia worse in right gaze. Neuro-ophthalmologic examination revealed normal afferent visual function and pupil reactivity. There was a right eye abduction deficit. Fundus examination was normal. Chronicity of diplopia suggested longstanding peripheral or fascicular 6th nerve lesion. Extensive testing for insidious infectious and inflammatory causes of rhombencephalitis was unrevealing. Repeat EVD sampling showed WBC count of 14/mL (89% neutrophils, 10% lymphocytes) and protein 60 mg/dl. Cytology and flow cytometry were normal. EVD was internalized. Listeria rhombencephalitis was presumptively diagnosed given acute decompensation, MRI findings, and inflammatory CSF, all of which stabilized on antibiotics. He was; discharged on 6-week course of ampicillin and gentamicin. A month later, persistent abduction deficit and new hyperreflexia were noted. Repeat MRI brain, cervical, and thoracic spine revealed diffuse subarachnoid space enhancement displacing the spinal cord (Figure 4a,b). Given thickened, infiltrative intra- and extra-axial lesions in a young patient, neurosarcoidosis was suspected. Patient deferred testing given clinical stability. Two months later, he developed painful bilateral leg weakness. |
| Disease/Diagnosis |
Diffuse midline glioma (DMG) is a midline CNS tumor which presents almost exclusively in childhood with cranial neuropathies and long tract signs. Obstructive hydrocephalus is typically a later finding of DMG (4). Radiographic appearance of DMG is variable, ranging from expansile, infiltrative lesion as in our patient to rim-enhancing mass with central necrosis mimicking astrocytomas found in adults (5)(6). Biopsy proves challenging given DMG's deep location and brainstem involvement. Traditional chemotherapy regimens have proven ineffective, and craniospinal radiation is the mainstay of treatment (7). Imipridone, a molecular therapy promoting tumor apoptosis, is currently being studied in adults with recurrent H3-K27M mutant gliomas (8) though disseminated gliomas are excluded. Median survival in pediatric DMG is 10 months but may be slightly longer in adults. (7)(9)(10 |
| Date |
2020-03 |
| References |
1. Louis,DN et al,"2016 WHO Classifications of CNS Tumors", Acta Neuropathol,131:803-20,2016. 2. Liang,JJ et al,'Rhombencephalitis Caused by Listeria with Hydrocephalus and ICH',World J Clin Cases,7:538-47,2019. 3.Nachmias,B et al,'Early Hydrocephalus in Listeria Meningitis', ID Cases,14:1-4,2018. 4. Guida,L et al,"Safety and Efficacy of ETV in DIPG Related Hydrocephalus" World Neurosurg,124:29-35,2019. 5. Aboian,MS et al,"Imaging Characteristics of Pediatric Diffuse Midline Gliomas with H3K27M Mutation" Am J Neuroradiol,38:795-800,2017. 6. Solomon,DA et al,"Diffuse Midline Gliomas with H3K27M Mutation", Brain Pathol,6:569-80,2016. 7.Vitanza,NA, Monje,M."DIPG: From Diagnosis to Next-Generation Clinical Trials", Curr Treat Opt N, 21:37,2016. 8.Arrillaga-Romany,I et al.'A Phase 2 Study of the First Imipridone ONC201' Oncotarget.8:79298-304,2017. 9. Daoud,EV et al."Adult Brainstem Gliomas With H3K27M mutation: Radiology, Pathology, and Prognosis" J Neuropath Exp Neur,77:302-11,2018. 10. Schreck,KC et al,"Incidence and Clinicopathologic Features of H3K27M Mutations in Adults with Midline Gliomas"J Neuro- Onc.143:87-93, 2019. |
| Language |
eng |
| Format |
video/mp4 |
| Type |
Image/MovingImage |
| Source |
2020 North American Neuro-Ophthalmology Society Annual Meeting |
| Relation is Part of |
NANOS Annual Meeting Frank B. Walsh Sessions; 2020 |
| 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 2020. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright |
| ARK |
ark:/87278/s60p67mn |
| Setname |
ehsl_novel_fbw |
| ID |
1538208 |
| Reference URL |
https://collections.lib.utah.edu/ark:/87278/s60p67mn |
