+ Resolve Article
+ Follow Us
Follow on FacebookFollow on Facebook
Follow on TwitterFollow on Twitter

+ Translate
+ Subscribe to Site Feed
GeoScience Most Shared ContentMost Shared Content

Neuroaxonal dystrophy in young adults: a clinicopathological study of two unrelated cases

, : Neuroaxonal dystrophy in young adults: a clinicopathological study of two unrelated cases. Annals of Neurology 11(4): 335-343

The clinical and pathological features of two unrelated sporadic cases of neuroaxonal dystrophy occurring in young adults are described. Initial clinical manifestations in both patients were those of primary psychiatric disorders. They subsequently developed extrapyramidal symptoms, dementia, cerebellar ataxia, and corticospinal dysfunction, but neither demonstrated myoclonic seizures. In one patient the diagnosis was made before death by brain biopsy. On pathological examination both patients showed a generalized distribution of spheroids within the central nervous system and, in one, in the peripheral nerves. In both patients, Lewy bodies were demonstrated in the pigmented brainstem nuclei. In addition, one patient showed ultrastructurally verified Lewy bodies in the cerebral cortex. Although these two cases could be considered examples of juvenile neuroaxonal dystrophy, we prefer to regard them as transitional forms in a disease spectrum with classic infantile neuroaxonal dystrophy and Hallervorden-Spatz disease at the two extremes.


PMID: 7103414

DOI: 10.1002/ana.410110403

Other references

Ellen, J.M.; Adler, N.E.; Gurvey, J.E.; Millstein, S.G.; Tschann, J.M., 2001: Adolescents perception of risk for STDS and condom use. International Journal of STD & AIDS 12(Supplement 2): 176

Stahlberg, R.; Van Volkenburgh, E., 1999: The effect of light on membrane potential, apoplastic pH and cell expansion in leaves of Pisum sativum L. var. Argenteum: Role of the plasma-membrane H+-ATPase and photosynthesis. The connection between three light responses of green leaf cells-membrane potential (Vm), H+ net efflux and growth, was analyzed. Illumination of mesophyll cells in leaves from Argenteum peas caused two rapid responses: (i) a de- and repolarizatio...

Stead,, R., 1989: Brewers lupine (Lupinus breweri), Sierra lupine (Lupinus confertus), Grays lupine (Lupinus grayi). Fact sheet College of Agriculture University of Nevada Reno Nevada Cooperative Extension: (89-49)

Rogers, N.M.; Matthews, T.J.; Kausman, J.Y.; Kitching, A.Richard.; Kitching, R.A.; Coates, P.Toby.H., 2009: Review article: Kidney dendritic cells: their role in homeostasis, inflammation and transplantation. There is definitive experimental proof that a lattice of dendritic cells (DC) exist within the renal parenchyma. Kidney-resident DC (KDC) are an important constituent of passenger leucocytes that initiate the direct component of allograft rejectio...

Lara-de-la-Fuente, R.; Alanís-Cruces, J.Marcelino., 2009: Postoperative fibrosis after lumbar surgery. Postoperative fibrosis is the excessive scarring resulting from any surgery; that is, the formation of more fibrous tissue than is normal. In some lumbar surgeries this fibrous tissue compresses or distends the dura mater and/or nerve roots, causi...

Piletta P.; Porchet H.C.; Dayer P., 1990: Lack of cns involvement in acidic nsaids analgesia. Clinical Pharmacology & Therapeutics 47(2): 134

Didier, R.; Gueux, E.; Rayssiguier, Y., 1984: Magnesium deficiency in the Japanese quail. Morphological effects of magnesium deficiency on liver cells and general aspects of its influence on the metabolism were investigated in young quails. Magnesium deficiency was characterized by a depressed growth, a high mortality rate, a decrease...

Del Balzo P.; Cappa M.; Salvatori R.; Gertner J.M., 1989: Pyridostigmine does not reverse dexamethasone induced inhibition of gh secretion. Hormone Research (Basel) 31(SUPPL 1): 52

Ito, S., 1993: Optical wave propagation in discrete random media with large particles: a treatment of the phase function. A treatment of the phase function in the radiative transfer equation is discussed for describing optical wave propagation in discrete random media with large particles. Unlike the conventional small-angle approximation, the phase function is norma...

Mamtani, M.; Mummidi, S.; Ramsuran, V.; Pham, M-Hieu.; Maldonado, R.; Begum, K.; Valera, M.Soledad.; Sanchez, R.; Castiblanco, J.; Kulkarni, H.; Ndung'u, T.; He, W.; Anaya, J.Manuel.; Ahuja, S.K., 2011: Influence of variations in CCL3L1 and CCR5 on tuberculosis in a northwestern Colombian population. We investigated the association of polymorphisms in CCR5, the major human immunodeficiency virus (HIV)-1 coreceptor, and copy number of its potent ligand CCL3L1 with tuberculosis in 298 individuals from Colombia. The CCR5-HHD haplotype, a known ge...