NotSoSimple Carpal Tunnel Syndrome Kirsten Regalia MD Department

Not-So-Simple Carpal Tunnel Syndrome Kirsten Regalia, MD Department of Internal Medicine, University of Colorado Denver History of Present Illness • 63 year-old male presenting with generalized edema, worst in his lower extremities • Review of systems positive for: ü Exertional dyspnea with minimal activity ü Fatigue ü Recent weight gain, preceded by unintentional 30 pound weight loss 6 months prior ü Dysphagia requiring strict liquid diet ü Bilateral wrist pain with numbness and tingling Past Medical/Surgical History • Bilateral carpal tunnel syndrome ü Status-post left sided carpal tunnel release surgery without symptomatic benefit ü Severe pain requiring chronic narcotics • GERD Social/Family History • • • Images Problem List • • Macroglossia Lateral tongue scalloping Before Now Tongue Biopsies Laboratory and Radiographic Evaluation 137 100 11 93 3. 9 32 1. 1 8 5. 2 2. 9 18 26 306 33 UA: Mod protein, large LE, small blood MCV 103 Spot Pr/Cr: 4. 4 g/day 24 -hr urine protein: 3. 8 g TSH 10. 8 Free T 4 0. 52 181 Eosinophilic deposit Squamous epithelium Amyloid fibrils on Congo Red stain Further Workup and Diagnosis • Markedly elevated serum and urine kappa free light chains on SPEP/UPEP with immunofixation • Renal and bone marrow biopsies also consistent with amyloidosis • Diagnosed with systemic amyloidosis, poor prognosis given cardiac involvement • Underwent chemo and transitioned to hospice Born and raised in U. S. Previous 80+ pack-year tobacco history No Et. OH, illicits Never incarcerated, no recent travel Family h/o CAD Physical Exam • Vitals: 98. 2 104/69 94 18 93% on RA • Gen: Chronically-ill appearing • HEENT: Macroglossia, temporal muscle wasting • CV: Tachycardic but regular, JVD to ears, 3+ edema, + Kusssmaul’s • Pulm: Diminished bibasilar breath sounds • Abd: Distended, + shifting dullness, non-tender • Ext: Dupuytren’s contractures bilaterally • Neuro: Decreased sensation digits 1 st-5 th, + Tinel’s and Phalen’s Nephrotic-range proteinuria Macrocytic anemia Cholestatic LFTs and hepatic synthetic dysfunction Restrictive cardiomyopathy Macroglossia Peripheral polyneuropathy Echogenic organs Systemic Amyloidosis ECG Chest X-ray • Abdominal US: Echogenic hepatic parenchyma, moderate ascites • Renal US: Bilateral increased renal echogenicity, mildly elevated renal artery resistive indices • Trans-thoracic echo: Brightly echogenic myocardium, global LV hypokinesis, LVEF ~50%, PAP 28 mm. Hg + RAP • Right and left heart catheterization: Concordance of RV and LV systolic pressures with respiration and square-rooting of diastolic pressure tracings, suggestive of restrictive physiology • Extracellular deposition of pathologic insoluble fibrillar proteins in organs and tissues • AL (primary) amyloidosis involves deposition of monoclonal immunoglobulin light chains (λ or κ) • Plasma cell dyscrasia of bone marrow • Can involve kidneys, heart, peripheral nervous system, GI system, tongue • Tissue biopsy of involved organ for diagnosis • Treatment with chemo: melphalan and prednisone