Cortical and striatal neurone number in Huntington's disease

Abstract

The total cortical and striatal neurone and glial numbers were estimated in five cases of Huntington's disease (three males, two females) and five ageand sex-matched control cases. Serial 500-l-lm-thick gallocyanin-stained frontal sections through the left hemisphere were analysed using Cavalieri's principle for volume and the optical disector for cell density estimations. The average cortical neurone number of five controls (mean age 53±13 years, range 36-72 years) was 5.97x 10 9 ±320x 10 6 , the average number of small striatal neurones was 82 X 10 6 ± 15.8 X 10 6 • The left striatum (caudatum, putamen, and accumbens) contained a mean of 273 X 10 6 ±53 X 10 6 glial cells (oligodendrocytes, astrocytes and unc1assifiable glial profiles). The mean cortical neurone number in Huntington's disease patients (mean age 49±14 years, range 36-75 years) was diminished by about 33 % to 3.99x10 9 ±218x10 6 nerve cells (P : : ; : : : : 0.012, MannWhitney V-test). The mean number of small striatal neurones decreased tremendously to 9.72 X 10 6 ±

Cerebral cortex structure in prodromal Huntington disease

“…The observations of relative gray matter sparing in early prodromal HD (Far group) are interesting, given that widespread cortical involvement is evident in later stages (Heinsen et al, 1994; Selemon et al, 2004; Wagster et al, 1994). Yet lack of cortical findings in other neuroimaging studies of subjects who are prodromal HD or newly diagnosed suggest that cortical degeneration is a relatively late phenomenon in the course of the illness, occurring in a slow and patchy process.…”

The Neuropathology of Huntington’s Disease: Classical Findings, Recent Developments and Correlation to Functional Neuroanatomy

“…The neuropathological phenotype of adult HD can be almost cryptic or outstanding within the same nucleus, but at different sites. 1.4 ) (Birnbaum 1941 ;Braak and Braak 1992a , b ;Bruyn et al 1979 ;De la Monte et al 1988 ;Dom et al 1976 ;Dunlap 1927 ;Estrada-Sanchez and Rebec 2013 ;Fennema-Notestine et al 2004 ;Ferrante et al 1987 ;Forno and Jose 1973 ;Hedreen et al 1991 ;Heinsen et al 1992Heinsen et al , 1994Heinsen et al , 1996Heinsen et al , 1999Heinsen and Rüb 1997 ;Hodges et al 2006 ;Kiesselbach 1914 ;Landwehrmeyer et al 1995 ;Lange 1981 ;Lange and Aulich 1986 ;Lange et al 1976 ;Lewy 1923 ;McCaughey 1961 ;Myers et al 1988 ;Neustaedter 1933 ;Roos et al 1985 ;Rüb et al 2013aRüb et al , b , 2014aSchroeder 1931 ;Selemon et al 2004 ;Sotrel et al 1991 ;Terplan 1924 ;Vonsattel 2008 ;Vonsattel and DiFiglia 1998 ;Vonsattel et al 1985 ). 1.4 ) (Braak and Braak 1992a , b ;Bruyn et al 1979 ;De la Monte et al 1988 ;Dom et al 1976 ;…”

Genetics and Neuropathology of Huntington's Disease

“…The neuron loss is reflected in regional brain atrophy. For example, late in disease, volumetric losses of the following magnitudes are observed: 20% in cortex, 30% in cerebral white matter, 60% in striatum, 55% in globus pallidus, and 30% in thalamus (de la Monte et al , 1988; Lange et al , 1976; Heinsen et al , 1994). Caudate shrinkage is significant already 10 years from estimated disease onset, while putamen and globus pallidus shrinkage is not significant until 3 years before estimated disease onset (Aylward et al , 1996).…”