PMID- 16036798 OWN - NLM STAT- MEDLINE DCOM- 20050928 LR - 20181113 IS - 1355-0284 (Print) IS - 1355-0284 (Linking) VI - 11 IP - 2 DP - 2005 Apr TI - Clinicopathological and virological analyses of familial human T-lymphotropic virus type I--associated polyneuropathy. PG - 199-207 AB - Human T-lymphotropic virus type I (HTLV-I) is known to be the causative agent of the chronic myelopathy, HTLV-I--associated myelopathy (HAM), and on rare occasions infection is also associated with the development of polyneuropathy. Here the authors present an HTLV-I--positive family of whom four members developed a chronic demyelinating polyneuropathy without HAM. Four female patients in a family from Hokkaido in Japan developed distal dominant paresthesia and muscle weakness in the second and third decades of their life. Neurological findings at ages ranging from 50 to 65 years included mild painful sensorimotor disturbances with atrophy of the distal parts of the extremities but without pyramidal signs or hyperactive tendon reflexes. Magnetic resonance imaging (MRI) findings of brain and spinal cord were unremarkable. Serum HTLV-I antibody levels were elevated at 1:8192 to 1:32,768, whereas those in cerebrospinal fluid were low at 1:4 to 1:8. Electrophysiological studies revealed polyphasic compound muscle action potentials with denervation potentials on nerve conduction studies and neurogenic patterns by electromyography, which were consistent with signs of chronic motor dominant demyelinating polyneuropathy. Sural nerve biopsy showed decreased myelinated fibers, occurrence of globule formation, myelin ovoid and remyelinated fibers, and an infiltration of CD68-positive macrophages with occasional CD4-positive T cells in the nerve fascicles. The polyneuropathy was responsive to steroid therapy. Analyses of serological human leukocyte antigen (HLA) types indicated that none of the patients possessed a high-risk HLA type known to be associated with adult T-cell leukemia (ATL), whereas they did have high responsive alleles to HTLV-I env similar to that observed in HAM. Nucleotide sequence analysis of the HTLV-I tax region demonstrated the B subgroup in all patients. This study suggests that HTLV-I infection can result in the development of a familial form of polyneuropathy that is associated with distinct HLA class I alleles, which might possibly involve a distinct virus subtype. FAU - Sawa, Hirofumi AU - Sawa H AD - Laboratory of Molecular and cellular Pathology, Core Research of Evolutional Science and Technology (CREST), Japan. FAU - Nagashima, Toshiko AU - Nagashima T FAU - Nagashima, Kazuo AU - Nagashima K FAU - Shinohara, Toshiya AU - Shinohara T FAU - Chuma, Takayo AU - Chuma T FAU - Mano, Yukio AU - Mano Y FAU - Tachi, Nobutada AU - Tachi N FAU - Hall, William W AU - Hall WW LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - United States TA - J Neurovirol JT - Journal of neurovirology JID - 9508123 RN - 0 (Antibodies, Viral) RN - 0 (Gene Products, tax) RN - EC 3.1.3.48 (Leukocyte Common Antigens) SB - IM MH - Antibodies, Viral/blood/cerebrospinal fluid MH - Blood Cells/virology MH - Female MH - Gene Products, tax/genetics MH - HTLV-I Infections/blood/cerebrospinal fluid/*complications/diagnosis MH - *Human T-lymphotropic virus 1/genetics/immunology MH - Humans MH - Leukocyte Common Antigens/biosynthesis MH - Macrophages/immunology MH - Middle Aged MH - Muscle Weakness/pathology MH - Nerve Fibers, Myelinated/pathology MH - Paresthesia/pathology MH - Pedigree MH - Polyneuropathies/*etiology/genetics/*pathology MH - Sequence Analysis, DNA MH - Sural Nerve/immunology/pathology EDAT- 2005/07/23 09:00 MHDA- 2005/09/29 09:00 CRDT- 2005/07/23 09:00 PHST- 2005/07/23 09:00 [pubmed] PHST- 2005/09/29 09:00 [medline] PHST- 2005/07/23 09:00 [entrez] AID - H06300142111K324 [pii] AID - 10.1080/13550280590924197 [doi] PST - ppublish SO - J Neurovirol. 2005 Apr;11(2):199-207. doi: 10.1080/13550280590924197.