It's Not Just a FAD, (EHR Fatigue Syndrome)

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Identifier walsh_2015_s2_c5
Title It's Not Just a FAD, (EHR Fatigue Syndrome)
Creator Jacqueline A. Leavitt; John J. Chen; Diva R. Salomao
Affiliation Mayo Clinic Rochester, MN
Subject Vision loss; Field loss; Fatigue; Intracranial Tumor
Description MRI brain: enhancing chiasmal and hypothalamic lesion with surrounding vasogenic edema extending into the tracts, without leptomeningeal spread, most likely pilocytic astrocytoma. Endocrinology consult: hypopituitarism [AM cortisol 5.9, free thyroxine 0.7, and ACTH 83, LH and FSH low but on birth control pills, prolactin 120 (high)]. She was started on hydrocortisone and levothyroxine with increased energy. She had been a marathon runner but was no longer running because of right lower back pain. MRI pelvis: indeterminate lesions right hemi-sacrum. Sacral biopsy: marked histiocytic infiltrate with cells positive for Langerin, S100 and CD1a immunostains, consistent with Langerhans cell histiocytosis (LCH). Other negative tests: bone marrow biopsy, CT chest and PET scan (only uptake in known pelvic/ intracranial lesions).LCH is characterized by proliferation of CD1a+ dendritic cells. LCH encompasses a range of clinical presentations and includes diseases previously designated as eosinophilic granuloma, Letterer-Siwe disease and Hand-Schuller-Christian syndrome, which were initially separated on the basis of organ involvement and disease severity. (1-3) LCH can affect bone, lung, hypothalamus, pituitary gland, skin, lymph nodes, liver and other organs (1-3). This disease predominantly affects children; adult onset occurs in only ~ 10%.CNS involvement in LCH most commonly involves the hypothalamic-pituitary system causing DI, but any part of the CNS can be involved (4). LCH with CNS disease is more likely to be multi-systemic and have skull lesions (5). While visual compromise in LCH is common from orbital lesions, only a few cases have been reported of intracranial visual pathway involvement (6).Chemotherapy with cladribine was started with improvement in her symptoms (7-9). MRI 3 months after treatment: hypothalamic lesion significantly reduced; PET: markedly reduced activity in the pelvic lesions. Visual acuity 20/20- and color normal OU. Visual fields: normal foveal threshold, inferior bitemporal defects.
History A 29 year-old female nurse, nine months postpartum, presented with an inability to see her computerwell for the past two months. She denied eye pain, diplopia, numbness, tingling or weakness. Therewere no changes in vision in bright vs. dim lighting. She also had a headache at the back of her head fortwo months that was relieved with OTC medications. She denied any events immediately preceding theblurred vision. She also complained of shortness of breath, unexplained weight loss and extreme fatigue,sleeping 10 hours per night and taking naps over her lunch break at work. Workup by her primary caredoctor revealed a normal CXR, ECG with sinus bradycardia and anemia (Hgb 9.6, Hct 28.7). Towardsthe end of her recent pregnancy she was evaluated for polydipsia (drinking up to 9 L /d) and nocturia (6-7 x /night). Water deprivation testing during pregnancy was not possible but sodium of 133 made thediagnosis of diabetes insipidus (DI) unlikely. Symptoms improved after delivery and therefore thepolyuria and polydipsia was attributed to pregnancy. Postpartum she also developed fairly severeanxiety and depression that was managed with sertraline and clonazepam. On examination, bestcorrected visual acuity was 20/30 OU, Ishihara color plates were 11/13 OD, 13/13 OS. Pupils reactedbriskly without an RAPD. Visual fields were full to confrontation. Slit lamp and dilated fundusexamination was unremarkable, including absence of macular abnormalities or optic discpallor. Goldmann fields showed a suggestion of a homonymous field defect, but had variable responsesand the perimetrist noted that she was often falling asleep during the test. Optical coherencetomography showed a normal average retinal nerve fiber layer thickness OU. Lab workup showed anelevated ESR of 70 and an elevated ACE of 62.Tests were performed.
Disease/Diagnosis Langherhans cell histiocytosis involving the chiasm, hypothalamus and sacrum
Date 2015-02
References 1. Writing Group of the Histiocyte Society. Histiocytosis syndromes in children. Lancet. 1:208-209, 1987. 2. Harris NL, Jaffe ES, Diebold J, Flandrin G, Muller-Hermelink HK et al. World Health Organization classification of neoplastic diseases of the hematopoietic and lymphoid tissues: report of the Clinical Advisory Committee meeting-Airlie House, Virginia, November 1997. J Clin Oncol. 17:3835-3849,1999. 3. Howarth DM, Gilchrist GS, Mullan BP, Wiseman GA, Edmonson JH et al. Langerhans cell histiocytosis: diagnosis, natural history, management, and outcome. Cancer. 85:2278-2290, 1999. 4. Grois NG, Favara BE, Mostbeck GH, Prayer D. Central nervous system disease in Langerhans cell histiocytosis. Hematol Oncol Clin North Am. 12:287-305, 1998. 5. Grois N, Flucher-Wolfram B, Heitger A, Mostbeck GH, Hofmann J et al. Diabetes insipidus in Langerhans cell histiocytosis: results from the DAL-HX 83 study. Med Pediatr Oncol. 24:248-256,1995.
Language eng
Format application/pdf
Format Creation Microsoft PowerPoint
Type Text
Source 47th Annual Frank Walsh Society Meeting
Relation is Part of NANOS Annual Meeting 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 2013. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright
ARK ark:/87278/s6n0445t
Setname ehsl_novel_fbw
ID 179301
Reference URL https://collections.lib.utah.edu/ark:/87278/s6n0445t
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