Leber Hereditary Optic Neuropathy

New findings from E. Jaros and co-researchers in the area of neurodegenerative disease described

Leber Hereditary Optic Neuropathy Library Library Home This article was published in Pain & Central Nervous System Week, which you can subscribe to online.

"The brain and cerebellum show atrophic changes. Diffuse symmetric abnormality is present in the posterior medial aspects of the whole spinal cord as well as part of the gray matter. ( H through P) Neuropathology and immunohistochemistry (IHC). Neuropathology examination was limited to the spinal cord. Formalin-fixed paraffin-embedded sections of the cervical, upper mid and thoracic, lumbar and sacral regions were stained with hematoxylin and eosin, Nissl, and Loyez stains, followed by IHC using relevant antibodies. (H) Myelin loss in the gracile fasciculus of the lower thoracic spinal cord outlined by dashed/dotted line. (I) Macrophage activity in posterior column of the lower thoracic spinal cord. (J) Normal myelination in the sacral spinal cord; posterior funiculus is outlined by dashed/dotted line. (K) Axon loss in the gracile fasciculus of the lower thoracic spinal cord outlined by dashed/dotted line. (L) Axon loss in the posterior thoracic spinal roots. (M) Intact axonal population in the anterior thoracic spinal roots. (N) Macrophage activity in the intermediate zone of gray matter bilaterally (filled arrows) and in the ventral zone of the gracile fasciculus (empty arrow) of the upper lumbar spinal cord; the inset represents an enlarged portion of the right intermediate zone of gray. (O) Neuropil vacuolation, disintegration, neuron loss, and capillary proliferation in the intermediate zone of gray matter of the upper lumbar spinal cord. (P) Neuronal necrosis and apoptosis (arrows) in the intermediate zone of gray matter of the upper lumbar spinal cord. ( H and J) Loyez myelin stain; (I and N) antimacrophage IHC, CD68 monoclonal antibody (McAb), 1:50, M0876 from DAKO; (K) anti-phosphorylated neurofilament 200-kd subunit IHC, SMI31 McAb, 1:50,000, NA1219 from Affiniti; (L and M) antiphosphorylated and nonphosphorylated neurofilament 200-kd subunit IHC, N52 McAb, 1:1,600, N0142 form Sigma; (O and P) hematoxylin and eosin stain. The incubation with all the primary antibodies was followed by secondary biotinylated antibody and ABC reagent (Vectastain Elite ABC peroxidase kit; Vector Laboratories), diaminobenzidine and hematoxylin counterstain," wrote E. Jaros and colleagues.

The researchers concluded: "Scale bars for H, J, K, and N ( main) = 920 mu m, ( I and O) = 400 mu m, (P) = 100 mu m, (L, M) = 50 mu m, N (inset) = 40 mu m, (P) = 30 mu m."

Jaros and colleagues published their study in Neurology (Primary spinal cord neurodegeneration in leber hereditary optic neuropathy. Neurology, 2007;69(2):214-216).

For additional information, contact P.F. Chinnery, Medical School Newcastle Upon Tyne, M41014, Framlington Pl, Newcastle Upon Tyne NE2 4HH, Tyne & Wear, UK.

Publisher contact information for the journal Neurology is: Lippincott Williams & Wilkins, 530 Walnut St., Philadelphia, PA 19106-3621, USA.

Keywords: United Kingdom, Neurodegenerative Disease, Biotechnology, Diagnosis, Diagnostics, Medical Device, Mental Health, Monoclonal Antibody, Neurodegenerative, Neurology, Neuropathology, Neuropathy, Optic Nerve Disease.

This article was prepared by Pain & Central Nervous System Week editors from staff and other reports. Copyright 2007, Pain & Central Nervous System Week via NewsRx.com.