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Neuro Diagnostic Dilemmas: Dissection of a Case (1 hour interactive lecture format) Kelly A. Malloy, OD and Erin M. Draper, OD Kelly A. Malloy and Erin M. Draper have no financial interests to disclose. ● The content of this COPE Accredited CE activity was prepared independently by Kelly A. Malloy and Erin M. Draper without input from members of the optometric community. ● Kelly A. Malloy and Erin M. Draper have no direct financial or proprietary interest in any companies, products or services mentioned in this presentation. ● The content and format of this course is presented without commercial bias and does not claim superiority of any commercial product or service. ABSTRACT: This interactive lecture revolves around one patient, who presents with asymmetric optic neuropathy. Join us as we determine the necessary work-up to rule out causes of non-glaucomatous optic neuropathy. With this interactive format, help us analyze the results of this testing and determine the appropriate management plan for our patient. Couse Objectives: 1. 2. 3. 4. 5. 6. 7. 8. To recognize the importance of providing comprehensive patient care To recognize when additional testing is warranted based on the clinical exam findings. To understand how the differential diagnosis for optic neuropathy includes systemic etiologies To understand how to interpret the results of lab testing and imaging studies. To appropriately refer patients to other medical specialties outside of eye care. To better understand the causes for an abnormal SPEP. To understand the possible causes of lytic lesions of the calvarium. To understand multiple myeloma. OUTLINE 1. Pertinent Exam Findings a. Optic Neuropathy i. Causes 1. Glaucomatous 2. Non-Glaucomatous a. Compression b. Inflammation c. Infection d. Nutritional/Toxic b. Optociliary Shunt Vessels i. Causes 1. Congenital 2. End-stage glaucoma 1 3. 4. 5. Retinal Vein Occlusion Papilledema Meningioma a. Optic Nerve Sheath b. Sphenoid Wing 2. Necessary Work-Up a. Lab Testing i. Inflammation 1. CBC with differential 2. ESR 3. C-reactive protein ii. Auto-immune 1. ANA iii. Inflammatory 1. ACE iv. Infectious 1. Lyme titer 2. RPR 3. FTA-ABS v. Nutritional 1. Vitamin B 12 2. Folate 3. Homocysteine 4. Methylmalonic Acid vi. Other 1. SPEP (serum protein electrophoresis) vii. Kidney Function 1. BUN 2. Creatinine 3. GFR b. Imaging i. MRI brain ii. MRI orbits iii. Carotid ultrasound 3. Interpreting the Results a. Abnormal Lab Results i. Elevated homocysteine ii. Elevated creatinine iii. Low GFR iv. Abnormal SPEP 1. M-spike in gamma globulin region, IgG kappa monoclonal band, Elevated Immunoglobulin G, Reduced Immunoglobulin M 2. Discussion a. Serum contains a variety of different proteins that will be separated by electrophoresis into five or six fractions b. Monoclonal proteins (M proteins) are identical and have the same electrical charge. On electrophoresis a monoclonal protein will migrate as a narrow spike which most often appears in the gamma zone. c. Production of a single, monoclonal protein (M-protein) is a characteristic feature of multiple myeloma. d. Monoclonal gammopathies i. multiple myeloma and spectrum of disease 2 ii. plasma cell leukemia iii. Waldenstrom’s macroglobulemia iv. AL amyloidosis b. 4. 5. MRI Results i. Small vessel ischemic disease ii. Azygous ectatic anterior cerebral artery with dominant A1 segment which contacts and possibly compresses the right optic nerve iii. Enhancing 1.7 cm lytic lesion in the right frontal calvarium 1. Discussion of lytic lesions a. Destruction of bone b. Causes: i. Primary bone cancers 1. Osteosarcoma 2. Ewing’s sarcoma ii. Multiple Myeloma iii. Metastasis to bone Additional Work-Up a. Skeletal Survey vs Bone Scan i. Skeletal Survey 1. Series of X-rays performed systemically to cover the entire skeleton or the anatomic regions for clinical indications 2. Identifies focal and diffuse abnormalities of the skeleton 3. Indications: a. Abuse b. Skeletal dysplasias c. Infections d. Metastatic bone disease e. Multiple myeloma f. Metabolic bone disease ii. Bone Scan 1. Procedure that uses small amounts of radioactive material 2. To view the metabolic activity of bones 3. Indications a. Malignancy b. Osteomyelitis c. Occult Fractures iii. Results 1. Skeletal survey completed a. No lytic lesions or fractures identified Referral a. Hematology / Oncology i. Concern for multiple myeloma 1. About Multiple Myeloma a. Part of a spectrum of diseases ranging from monoclonal gammopathy of unknown significance (MGUS) b. Multiple myeloma is a cancer of plasma cells that produce monoclonal immunoglobulin and invade and destroy adjacent bone tissue. c. Systemic effects of multiple myeloma i. Hyperviscosity ii. Inhibition of normal immune response; recurrent infections iii. Interfere with RBC production: leukopenia, anemia and thrombocytopenia iv. Increased bone destruction resulting in hypercalcemia, bone pain and fractures (lytic bone lesions) 3 v. Cast nephropathy caused by excess light chains (Bence Jones protein) vi. Infiltration of cranial nerves vii. Infiltration of the leptomeninges of the cerebrum, brain stem, optic chiasm, pituitary gland, cranial bone marrow, and subarachnoid blood vessels ii. Order bone marrow biopsy 1. About bone marrow biopsy a. Determine percentage of plasma cells in bone marrow b. Look for plasma cell tumor iii. Order PET scan 1. About PET scan a. Nuclear medicine procedure that uses a small amount of radioactive substance to evaluate the metabolism of a particular organ or tissue b. Indications i. ii. iii. iv. Cancer Neurodegenerative disease Epilepsy Trauma iv. Treatment Options and Prognosis 4