Nerve Inclusion bodies

Salajegheh M, Pinkus JL, Taylor JP et al. (2009) Sarcoplasmic redistribution of nuclear TDP-43 in inclusion body myositis. Muscle Nerve 40, 19-31. 

Koffman BM, Rugiero M, Dalakas MC. (1998) Autoimmune diseases and autoantibodies associated with sporadic inclusion body myositis. Muscle Nerve 21,115-117. 

Greenberg SA Pinkus GS, Amato AA et al. (2007) Myeloid dendritic cells in inclusion-body myositis and polymyositis. Muscle Nerve 35, 17-23. 

Layzer R, Lee HS, Iverson D, Margeta M. (2009) Der-matomyositis with inclusion body myositis pathology. Muscle Nerve 40(3), 469-471. 

Jackson CE, Barohn RJ, Gronseth G et al. (2008) Inclusion body myositis functional rating scale a reliable and valid measure of disease severity. Muscle Nerve 37(4), 113 16. 

Felice KJ, Relva GM, Conway Sr., (1998) Further observations on forearm flexor weakness in inclusion body myositis. Muscle Nerve 21(5), 659-661. 

Lindberg C, Oldfors A, Hedstrom A. (1990) Inclusion body myositis peripheral nerve involvement. Combined morphological and electrophysiolog-ical studies on peripheral nerves. J Neurol Sci 99(2-3) 327-338. 

Khurana H, Luciano CA. (1996) Nerve conduction studies in inclusion body myositis Observations in 63 patients. Muscle Nerve 19(9), 1192. 

Needham M, MastagUa FL, Garlepp MJ. (2007) Genetics of inclusion-body myositis. Muscle Nerve 35 (5), 549-561. 

OhSJ, ClaussenGC. (1995) Single-fiberBMGfindings in inclusion body myopathy. Muscle Nerve 18, 1050. 

Barkhaus PE, Periquet MI, Nandedkar SD. (1999) Quantitative electrophysiologic studies in sporadic inclusion body myositis. Muscle Nerve 22(4), 480-487. 

Phillips BA, Cala LA, Thickbroom G.W et al. (2001) Patterns of muscle involvement in inclusion body myositis clinical and magnetic resonance imaging study. Muscle Nerve 24(11), 1526-1534. 

DelBoR, BaronP, Prelie Aetal. (2003) Novelmissense mutation and large deletion of GNE gene in autosomal-recessive inclusion-body myopathy. Muscle Nerve 28, 113-117. 

Djamshidian A, Schaefer J, Haubenberger D et al. (2009) A novel mutation in the VCP gene (G157R) in a German family with inclusion-body myopathy with Paget disease of bone and frontotemporal dementia. Muscle Nerve 39(3), 389-391. 

Gidaro T, Modoni A, Sabatelli M et al. (2008) An Italian family with inclusion-body myopathy and frontotemporal dementia due to mutation in the VCP gene. Muscle Nerve 37( ), 111-114. 

Broccohni A, Gidaro T, Morosetti R, Mirabella M. (2009) Hereditary inclusion-body myopathy clues on pathogenesis and possible therapy. Muscle Nerve 40, 340-349. 

The new work has established that a neurodegenerative pathway leading from soluble to insoluble, filamentous a-synuclein is central to Lewy body diseases and multiple system atrophy. The development of experimental models of a-synucleinopathies has opened the way to the identification of the detailed mechanisms by which the formation of inclusions causes disease. These model systems have also made it possible to identify disease modifiers that may well lead to the development of the first mechanism-based therapies for these diseases. At a conceptual level, it will be important to understand whether a-synuclein has a role to play in disorders, such as autosomal-recessive juvenile forms of parkinsonism caused by mutations in the Parkin, DJ-1 and PINK-1 genes, or whether there are entirely separate mechanisms by which the dopaminergic nerve cells of the substantia nigra degenerate in Parkinson s disease and in inherited disorders with parkinsonism. 

PD pathology is characterized by threadlike proteinaceous neurite inclusions and intracellular Lewy bodies (Fig. 14.1) occurring exclusively in unmyelinated fibers. In pathological stage I and II of the disease, first Lewy neurites, then Lewy bodies appear almost simultaneously in the olfactory bulb and the dorsal visceromotor nucleus of the vagal nerve. Degeneration in the olfactory bulb seems to be very slow. 

Patients with GMi-gangliosidosis have rarefaction of the bones and deformed vertebrae. The neuronal cells in the brain are distended with membranous cytoplasmic bodies which resemble in some degree the inclusions found in nerve cells of Tay-Sachs patients. The spleen and liver are enlarged, and the bone marrow contains foam cells distended with stored material. The glomeruli of the kidneys are also swollen with accumulating substance. 

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