| Identifier |
walsh_2015_s1_c5-2 |
| Title |
Under Pressure |
| Creator |
Nathan H. Kung |
| 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 andpapilledema discovered 2 months earlier. She complained of recently blurred vision but no positionalheadache, pulsatile tinnitus, transient visual obscurations, or other neurologic issues. She used nomedications 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 acuitywith 20/20 VA OU, equal pupils without RAPD, and severe grade 4 papilledema OU with severalchoroidal folds through both maculae. No hemorrhages were noted. All other portions of theexamination were normal. Her BMI was 27. Humphrey SITA Standard visual fields were performedand showed slight enlargement of the blind spot OU with a nasal step in the L eye. An MRI of the brainand orbits with and without gadolinium was normal with normal venous flow voids. CSF analysisshowed an opening pressure of 26 cm H2O with 1 RBC, 1 WBC, Protein 75, Glucose 69, and noabnormalities on cytology. Although she had atypical features, a preliminary diagnosis of ProbablePseudotumor Cerebri was made and she was initiated on acetazolamide, with improved papilledemaover the next several months. Over the next three years, however, she developed worsening vision withmultiple 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 |
application/pdf |
| Type |
Text |
| 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/index3.html |
| Publisher |
Spencer S. Eccles Health Sciences Library, University of Utah |
| Holding Institution |
Spencer S. Eccles Health Sciences Library, University of Utah, 10 N 1900 E SLC, UT 84112-6213 |
| Rights Management |
Copyright 2013. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright |
| ARK |
ark:/87278/s68s7mj6 |
| Setname |
ehsl_novel_fbw |
| ID |
179296 |
| Reference URL |
https://collections.lib.utah.edu/ark:/87278/s68s7mj6 |
