title: Ultrastructural pathology of a Chilean case of tropical spastic paraparesis/human T-cell lymphotropic type I-associated myelopathy (TSP/HAM).
abstract: Human T-cell lymphotropic virus type I (HTLV-I), is the cause of endemic tropical spastic paraparesis (TSP) or HTLV-I-associated myelopathy (HAM). Because TSP/HAM is not a fatal disease, the neuropathology of this disease, albeit relatively well understood, is based on the examination of just a few incidental cases. Previously, we demonstrated peculiar lamellated structures, called "multilamellar bodies" (MLB). In this report, we present the ultrastructural neuropathology of a TSP/HAM case from Chile, with further detailed descriptions of MLB. It is tempting to suggest that MLB may represent specific ultrastructural markers of TSP/HAM. The pathology of the anterior and posterior horns was similar and was comprised of axonal degeneration, accompanied by extensive astrocytic gliosis. Lymphocytic infiltration, particularly observed as "cuffs" around blood vessels, was scattered among other cellular elements. Ultrastructurally, myelin sheaths were relatively well preserved, and some demyelinated but not remyelinated fibers were observed. Moreover, axons with abnormal accumulations of neurofilaments, suggestive of axonal degeneration, were detected. Several axons contained Hirano bodies. In many samples, glial processes replaced most of the remaining neuropil. In a few specimens of the anterior and posterior horns of the spinal cord, MLB were observed. These structures consisted of stacks of 30 to 40 electron-dense lamellae, which were interrupted by narrow electron-lucent spaces. All of the lamellae were immersed within an amorphous substance of intermediate density. Neurons of the dorsal root ganglia were basically normal except for increased lipofuscin accumulation. As in the spinal cord, myelinated axons were well preserved, but a few were demyelinated and surrounded by concentric arrays of Schwann cell membranes. Also, axons of the dorsal roots accumulated increased number of neurofilaments. Mast cells and Schwann cells were increased in number, the latter containing abundant pi granules and myelin fragments.
authors.affiliation: Laboratory of Central Nervous System Studies, National Institutes of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD, USA.
authors.forename: P P
authors.initials: PP
authors.lastname: Liberski
authors.forename: J
authors.initials: J
authors.lastname: Buczyński
authors.forename: R
authors.initials: R
authors.lastname: Yanagihara
authors.forename: C
authors.initials: C
authors.lastname: Mora
authors.forename: C J
authors.initials: CJ
authors.lastname: Gibbs
authors.forename: C
authors.initials: C
authors.lastname: Gajdusek
authors.forename: L
authors.initials: L
authors.lastname: Cartier
authors.forename: A
authors.initials: A
authors.lastname: Verdugo
authors.forename: F
authors.initials: F
authors.lastname: Araya
authors.forename: L
authors.initials: L
authors.lastname: Castillo
mesh_terms: D001253:Astrocytes / Q000648:ultrastructure; D001369:Axons / Q000648:ultrastructure; D002677:Chile; D005625:Frontal Lobe / Q000648:ultrastructure*; D005727:Ganglia, Spinal / Q000648:ultrastructure*; D006003:Glycogen / Q000378:metabolism; D006801:Humans; D008062:Lipofuscin / Q000378:metabolism; D008214:Lymphocytes / Q000473:pathology; D018482:Muscle, Skeletal / Q000648:ultrastructure*; D009186:Myelin Sheath / Q000648:ultrastructure; D009210:Myofibrils / Q000648:ultrastructure; D009454:Neurofibrils / Q000648:ultrastructure; D015493:Paraparesis, Tropical Spastic / Q000473:pathology*; D013116:Spinal Cord / Q000648:ultrastructure*; D014617:Vacuoles / Q000648:ultrastructure
chemical_list: D008062:Lipofuscin; D006003:Glycogen