case records of the massachusetts general hospital The new england journal of medicine n engl j med 362;10 nejm.org march 11, 2010 929 Founded by Richard C. Cabot Nancy Lee Harris, m.d., Editor Eric S. Rosenberg, m.d., Associate Editor Jo-Anne O. Shepard, m.d., Associate Editor Alice M. Cort, m.d., Associate Editor Sally H. Ebeling, Assistant Editor Christine C. Peters, Assistant Editor From the Department of Neurology, Brigham and Women’s Hospital (A.H.R.); the Departments of Medicine (N.S.R.), Radiology (T.M.L.), and Pathology (S.C.-P., A.R.S.), Massachusetts General Hospi- tal; and the Departments of Neurology (A.H.R.), Medicine (N.S.R.), Radiology (T.M.L.), and Pathology (S.C.-P., A.R.S.), Harvard Medical School — all in Boston. This article (10.1056/NEJMcpc0907805) was updated on May 4, 2011, at NEJM .org. N Engl J Med 2010;362:929-40. Copyright © 2010 Massachusetts Medical Society. Presentation of Case Dr. Caron A. Jacobson (Medical Oncology): A 49-year-old man was admitted to this hospital because of ascites. The patient had been well until approximately 4 years earlier, when paresthesias of both feet, weakness of the lower legs, and bilateral foot drop developed gradu- ally. Three years before admission, he saw a neurologist at another hospital. Labo- ratory-test results, including testing for autoantibodies, were reportedly normal. Electromyography (EMG) reportedly showed prolonged F responses in both feet, normal motor and sensory responses in the distal segments of the arms, and no motor responses in the intrinsic foot muscles. A diagnosis of chronic inflamma- tory demyelinating polyneuropathy (CIDP) was made, and therapy with intravenous immune globulin was begun. Two and a half years before admission, the patient was referred to the neurol- ogy clinic of this hospital. On examination, the Romberg test was markedly ab- normal; there was bilateral foot drop, and the patient was unable to walk on his toes or heels. He was able to sign his name but not without visual control. Strength (on a scale of 0 to 5, where 5 is normal) in the ankle dorsiflexors mea- sured 1 to 2; in the muscles of ankle eversion and inversion, 4-; in the extensor hallucis longus, extensor digitorum brevis, and toe flexors, 0; and in the plantar flexors, 4- to 4. The left hand was mildly weak, particularly the abductor pollicis brevis, and strength was normal in the proximal arms. Deep-tendon reflexes were absent. Perception of vibration was absent in both first toes, decreased in both ankles, and normal at the knees and fingers. Position sense was diminished in both first toes. There was no muscle atrophy or fasciculation, and the remainder of the neurologic examination was normal. As compared with the previous study, EMG and nerve-conduction studies showed decreased sensory responses, absent sural-nerve responses, prolonged F-wave latencies, and more prominent denerva- tion in the tibialis anterior and medial gastrocnemius muscles, features consistent with a demyelinating polyneuropathy. During the next 6 months, weakness in the patient’s legs and arms increased, and intermittent stabbing pain in the lower legs developed. Intravenous immune Case 7-2010: A 49-Year-Old Man with Peripheral Neuropathy and Ascites Allan H. Ropper, M.D., Noopur S. Raje, M.D., Tara M. Lawrimore, M.D., Sandra Camelo-Piragua, M.D., and Aliyah R. Sohani, M.D. The New England Journal of Medicine Downloaded from nejm.org at UC SHARED JOURNAL COLLECTION on December 19, 2014. For personal use only. No other uses without permission. Copyright © 2010 Massachusetts Medical Society. All rights reserved.