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 ±

Podocyte Depletion Causes Glomerulosclerosis

“…Other examples of diseases that stem from cell depletion include Alzheimer's disease (46), Parkinson's disease (47), Huntington's disease (48), blindness caused by loss of retinal photoreceptor cells (49), deafness caused by cochlear hair cell loss (50), and diabetes resulting from ␤ cell depletion (51). In each case, the depletion of highly specialized "terminally differentiated" cells caused by a range of mechanisms and often superimposed on genetic susceptibilities seems to be a common pathway driving progressive organ failure.…”

The Neuropathology of Huntington’s Disease

“…A more detailed quantitative study using stereological cell counting has been addressed by Heinsen et al (1994), and the authors found a pronounced pyramidal cell loss in the supragranular layers in the primary sensory areas including primary somatosensory cortex (areas 3, 1, 2), primary visual cortex (area 17), primary auditory cortex (area 41), and association areas of the frontal, parietal, and temporal lobes. Similarly, Macdonald et al (1997) reported a significant reduction of pyramidal cells across layers III and V, and also found atrophy of cell bodies of the remaining cells in the angular gyrus of the parietal lobe.…”

The Ventral Striatopallidum and Extended Amygdala in Huntington Disease

“…The most characteristic brain pathology is the atrophy of the caudate nucleus and putamen, which is accompanied by a secondary enlargement of the lateral ventricles (Roos et al, 1985;Vonsattel et al, 1985). The striatal atrophy is due to the progressive loss of medium-sized GABAergic striatal neurons (Heinsen et al, 1994), which comprise approximately 90% of all neurons in the striatum. Cortical, subcortical and brainstem areas with grey and white matter changes are also affected (de la Monte et al, 1988;Dumas et al, 2012;Heinsen et al, 1996;Heinsen et al, 1999;Rosas et al, 2003;Rüb et al, 2009;Schmitz et al 1999;Tabrizi et al, 2011).…”