| Identifier |
walsh_2015_s1_c5-1 |
| Title |
Under Pressure |
| Creator |
Nathan H. Kung; Collin M. McClelland; Gregory P. Van Stavern |
| Affiliation |
(NHK) Department of Neurology, Washington University School of Medicine St Louis, MO; (CMM) (GPV) Department of Ophthalmology, Washington University School of Medicine St Louis, MO |
| Subject |
Paraneoplastic Disorders, Polyneuropathy, Papilledema, Optic Nerve Sheath Fenestration |
| History |
A 29-year-old woman was referred to neuro-ophthalmology clinic for 1 year of headaches and papilledema discovered 2 months earlier. She complained of recently blurred vision but no positional headache, pulsatile tinnitus, transient visual obscurations, or other neurologic issues. She used no medications and denied any recent illnesses except for thrombocythemia (591k) on recent blood work, and had no significant past medical history. Her initial examination in 4/2009 revealed normal acuity with 20/20 VA OU, equal pupils without RAPD, and severe grade 4 papilledema OU with several choroidal folds through both maculae. No hemorrhages were noted. All other portions of the examination were normal. Her BMI was 27. Humphrey SITA Standard visual fields were performed and showed slight enlargement of the blind spot OU with a nasal step in the L eye. An MRI of the brain and orbits with and without gadolinium was normal with normal venous flow voids. CSF analysis showed an opening pressure of 26 cm H2O with 1 RBC, 1 WBC, Protein 75, Glucose 69, and no abnormalities on cytology. Although she had atypical features, a preliminary diagnosis of Probable Pseudotumor Cerebri was made and she was initiated on acetazolamide, with improved papilledema over the next several months. Over the next three years, however, she developed worsening vision with multiple additional symptoms. |
| Disease/Diagnosis |
POEMS syndrome. She fulfilled all major criteria: 1) lambda-restricted plasma cell population, 2) polyneuropathy, 3) sclerotic bone lesion, 4) Castleman disease; and all minor criteria: 1) organomegaly (splenomegaly), extravascular volume overload (body wall anasarca), 3) endocrinopathy (elevated TSH/ACTH/Prolactin), 4) skin changes (hyperpigmented rash), 5) papilledema, and 6) thrombocytosis. |
| Date |
2015-02 |
| References |
1. Dispenzieri A. POEMS Syndrome: 2014 Update on Diagnosis, Risk-Stratification, and Management. Am J Hematol. 89:214-223, 2014. 2. Dispenzieri A. How I Treat POEMS Syndrome. Blood. 119(24):5650-5658, 2012. 3. Dispenzieri A, Kyle RA, Lacy MQ, Rajkumar V, Therneau TM. POEMS Syndrome: Definitions and Long-Term Outcome. Blood. 101:2496-2506, 2003. 4. Kaushik M, Pulido JS, Abreu R, Amselem L, Dispenzieri A. Ocular Findings in Patients with Polyneuropathy, Organomegaly, and Endocrinopathy, Monoclonal Gammopathy, and Skin Changes Syndrome. Ophthalmology. 118:778-782, 2011. 5. Latov N. Diagnosis and Treatment of Chronic Acquired Demyelinating Polyneuropathies. Nat Rev Neurol. 10:435-446, 2014. |
| Language |
eng |
| Format |
video/mp4 |
| Type |
Image/MovingImage |
| Source |
47th Annual Frank Walsh Society Meeting |
| Relation is Part of |
NANOS Annual Meeting Frank B. Walsh Sessions; 2015 |
| 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 2015. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright |
| ARK |
ark:/87278/s6448j2p |
| Setname |
ehsl_novel_fbw |
| ID |
179274 |
| Reference URL |
https://collections.lib.utah.edu/ark:/87278/s6448j2p |
