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Grand Rounds Vanderbilt Eye Institute 4/27/07 Ryan Tarantola M.D. PGY-2

Grand Rounds Vanderbilt Eye Institute 4/27/07 Ryan Tarantola M.D. PGY-2. Initial Evaluation 3/5/07. CC: Headache and acute bilateral vision loss HPI: 43 year-old female 24 hour hx of HA, nausea/vomiting Awoke with bilateral NLP vision.

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Grand Rounds Vanderbilt Eye Institute 4/27/07 Ryan Tarantola M.D. PGY-2

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  1. Grand Rounds Vanderbilt Eye Institute 4/27/07 Ryan Tarantola M.D. PGY-2

  2. Initial Evaluation 3/5/07 CC: Headache and acute bilateral vision loss HPI: • 43 year-old female • 24 hour hx of HA, nausea/vomiting • Awoke with bilateral NLP vision.

  3. PMH: Relapsing B cell lymphoma dx 10/05 S/P multiple courses of chemo and recent BMT POH: Wears glasses No eye disease No trauma or surgeries Allergies: Codeine, PCN FH: No eye disease

  4. SH: Married with 2 children. No tobacco, alcohol, or drug use. ROS: +HA, N/V Meds: Levaquin, Mycofungin, Valtrex, Nystatin, Cyclosporin, Prednisone, Zyrtec, Effexor, Prevacid, MVI

  5. General:Depressed level of consciousness  Developed right gaze preference and seizure-like episode. Intubated and treated with IV Ativan at that time. VA sc at near:OD: NLP OS: NLP Localization?

  6. Pupils: 42mm, no RAPD OU Visual Fields: unable Motility: Full OU Tp: OD:16 OS:13

  7. Pen Light Exam: WNL OU Dilated Fundus Exam: Vitreous: Clear OU Optic Nerves: C/D: 0.3 OU Macula: WNL OU Periphery: WNL OU

  8. Labs: Toxicology Screen: Negative CSF: clear, cell count wnl, protein: 43, gram stain -, culture - CBC c differential, CMP: WNL Imaging: CT Head s Contrast: No acute intracranial abnormality

  9. Acute onset of HA and N/V followed by bilateral post-geniculate visual loss, and SZ. Differential Diagnosis?

  10. Differential Diagnosis • 1. Vascular • a. Granulomatous angiitis • b. SLE • c. PAN • d. PRES: Posterior Reversible Encephalopathy Syndrome • 2. Ischemic/Thrombotic • a. Ischemic stroke of posterior circulation • b. Cerebral Venous Thrombosis • 3. Infectious • a. Progressive Multifocal Leukoencephalopathy • b. Infectious Encephalitis • 4. Inflammatory/Demylenating • a. Acute Disseminated Encephalomyelitis • 5. Mitochondrial Disease • a. MELAS: Mitochondrial Encephalomyopathy, Lactic Acidosis, and Stroke-Like • episodes • 6. Non-Organic

  11. Additional Studies?

  12. MRI of the Brain 3/5/07

  13. DWI ADC

  14. DWI ADC

  15. FINDINGS: • Patchy areas of increased signal on T2 and FLAIR involving the • cortical and subcortical white matter of the occipital and parietal • lobes bilaterally • No associated restricted diffusion or enhancement • Findings consistent with posterior reversible encephalopathy • syndrome (PRES). Consider Cyclosporine toxicity

  16. PRES: Posterior Reversible Encephalopathy Syndrome • Described in 1996 by Hinchey et al in NEJM • Transient, reversible, posterior cerebral edema • Headache, nausea, vomiting, altered mental status, seizures, • and visual disturbances • Blurred vision, visual field loss, and complete cortical blindness • MRI brain reveals characteristic findings

  17. PRES: Posterior Reversible Encephalopathy Syndrome • Involve the white matter > cortex • Completely reversible when promptly recognized and treated • Patients can progress to ischemia, massive infarction, and death • Multiple systemic associations

  18. PRES: Posterior Reversible Encephalopathy Syndrome Associations: • Toxemia of Pregnancy • Drugs: • Cyclosporin • Tacrolimus (FK506) • Interferon-alpha • Flutarabine • Cisplatin • Gemcitabine • Erythropoietin • Ifosfamide • 3. Secondary Hypertension: • SLE • Acute Glomerulonephritis • HTN with CRF • 4. Uncontrolled Essential Hypertension 5. Others: Hemolytic Uremic Syndrome Hepatorenal Syndrome Acute Intermittent Porphyria HIV Blood Transfusion Post-CEA TTP

  19. PRES: Posterior Reversible Encephalopathy Syndrome • Pathophysiology: • Posterior cerebral circulation has reduced perivascular sympathetic activity. • Autoregulation in response to hemodynamic challenge (HTN) is insufficient. • Local increases in hydrostatic pressure cause extravasation of fluid. • Regions of vasoconstriction and vasodilatation develop  hypoperfusion. • Focal areas of blood-brain barrier breakdown and further fluid extravasation

  20. PRES: Posterior Reversible Encephalopathy Syndrome • Radiologic Findings: • Vasogenic Edema: • DWI shows parieto-occipital signal changes caused by fluid • entrapment in the edematous region. • ADC map removes the underlying T2 signal (T2 shine through) • allowing distinction between vasogenic and cytotoxic edema.

  21. MRI • T1: Short TR, Short TE • Fat>White Matter>Gray Matter>Vitreous/CSF>Air • Demonstrates normal anatomy • T2: Long TR, Long TE • Vitreous/CSF>Gray Matter>White Matter>Fat. • Demonstrates inflammation, ischemia, and neoplasia • FLAIR: Very Long TR, Long TE • Fat>Gray Matter>White Matter>Vitreous/CSF>Air • Similar to T2 without CSF signal. Demonstrates demylelination • DWI: Cellular swelling  slow diffusion of molecules into cells • Demonstrates regions of edema • Reflects both diffusion weighting and T2 effects • ADC: Constructed from diffusion data • Removes underlying T2 signal

  22. PRES • CT, T2: Bilateral occipital lesions with a white matter predominance. • DWI: Slight hyperintensity, left more than right. ? cytotoxic edema due to infarction vs. T2 effects • ADC: Shows T2 shine through supporting a vasogenic pattern of edema

  23. Acute Occipital Infarction DWI T2 ADC • T2: hyperintensity along the right calcarine cortex • DWI: Marked hyperintensity (lightbulb sign) characteristic of acute infarction • ADC:Hypointensity confirms that this is restricted diffusion related to cytotoxic edema

  24. How Reversible is PRES? • Pande et al, 2006 • Multicenter retrospective study • 53 patients July 1999-June 2003 • PRES lesions in the subcortical white matter showed significantly higher • reversibility (76-91%) than those in the brain stem (44%), deep white matter • (47%) and thalamus (60%). • Complete resolution of radiographic findings occurred in 12/12 with eclampsia, • 7/11 with hypertension, 12/21 drug-induced, 2/5 renal failure. • Conclusions: Location of lesions and etiology are significant factor in • reversibility of PRES.

  25. Cyclosporin and PRES • Cyclosporin inhibits gene transcription of IL-2, IL-3, IFN-g and directly inhibits • activation / differentiation of T cells • Most common medication associated with PRES • Likely secondary to its ability to increase bp and cause fluid retention • Toxic effect on vascular endothelium • Low magnesium, aluminum overload, and high-dose steroids also potentiate • toxicity of cyclosporin • Frequently causes hypomagnesaemia and lower seizure threshold

  26. Treatment of PRES • Reduction of bp and removal of causative agent • IV Nicardipine and Labetalol are 1st line agents • Avoid: Clonidine  CNS depression • NTG  aggravation of edema due to cerebral vasodilatation • Identify and treat any systemic disease that may be underlying etiology • In eclampsia delivery and IV magnesium typically rapid improvement • Seizure management • ICU admission for monitoring and supportive care in severe cases

  27. Our Patient • Cyclosporin discontinued, IV antihypertensives, and seizures • treated with IV Ativan • 3/6/07: A+Ox3, HA and N/V resolved. C/O constant blurry vision, palinopsia (immediate type), and polyopia. Able to read newspaper print with some difficulty • 3/7/07: Vision returned to baseline per patient • 3/8/07: Started on FK-506 to replace Cyclosporin • 3/13/07: Va 20/20 OD, 20/20-1 OS at near, no field loss

  28. Take Home Points PRES should be considered in all patients with acute cerebral visual loss Diffuse white matter hyperintensity on T2/FLAIR, DWI, and ADC are characteristic, and allow differentiation from ischemic cause Treatment is aimed at controlling bp and determining underlying cause

  29. References Miller et al. Walsh and Hoyt’s Clinical Neuro-Ophthalmology Kline et al. Neuro-Ophthalmology Review Manual Kahana et al. Cortical Blindness: Clinical and Radiologic Findings in Reversible Posterior Leukoencephalopathy Syndrome. Ophthalmology. 2005;112:e7-e11. Eckard et al. Cerebral Hemodynamics and Autoregulation in Reversible Posterior Leukoencephalopathy Syndrome. Cerebrovasc Dis. 2006;22:204-208. Covarrubias et al. Posterior Reversible Encephalopathy Syndrome: Prognostic Utility of Quantitative Diffusion-Weighted MR Images. AJNR. 2002;23:1038-1048. Pande et al. Clinicoradiological factors influencing the reversibility of posterior reversible Encephalopathy Syndrome: a multicenter study. Radiat Med. 2006;24:659-668. Hinchey et al. A Reversible Posterior Leukoencephalopathy Syndrome. N Engl J Med. 1996;334:494-500 Ayoub, Mirza. Posterior reversible encephalopathy syndrome: A variant of hypertensive Encephalopathy. J of Clin Neurosci. 2006; 590-596.

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