Rinsho Shinkeigaku (Clinical Neurology)

Case Report

A case of Sjögren syndrome with subacute combined degeneration-like posterior column lesion on cervical MRI

Yu Hongo, M.D.1), Hiroyuki Onoue, M.D., Ph.D.1), Shinichi Takeshima, M.D.1), Keiko Kamakura, M.D., Ph.D.2) and Ken-ichi Kaida, M.D., Ph.D.1)

1)Division of Neurology, Department of Internal Medicine 3, National Defense Medical College
2)Tokyo University of Technology

We report a case of a 67 year-old man with bilateral sensory ataxia of the upper extremities. He was diagnosed as having ANCA-related angitis and Sjögren syndrome at age 60. On admission to our hospital at age 67, he presented with severe sensory ataxia in his upper extremities, while his lower extremity neurological symptoms were limited to the absence of tendon reflexes. Cervical MRI showed an increased T2 signal intensity in an area limited to the bilateral cuneate fasciculus. Serum levels of vitamin B12 and folic acid were normal. Plasma homocysteine, serum and urine methylmalonic acid were also normal. Eight-week intramuscular administration of vitamin B12 did not improve either his disorder or the MRI findings. His sensory ataxia might be attributed to Sjögren syndrome-associated ganglionopathy at the cervical level, and the MRI findings might reflect centripetal Wallerian degeneration in the cuneate fasciculus. Gracilis fasciculus are well-known as vulnerable regions in Sjögrenassociated myelopathy, whereas cervical myelopathy, limited to cuneate fascicules, can emerge as Sjögrenassociated disorders.
Full Text of this Article in Japanese PDF (334K)

(CLINICA NEUROL, 52: 491|494, 2012)
key words: Subacute combined degeneration, Sjögren syndrome, Cuneate fasciclus

(Received: 9-Dec-11)