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Neuropathol Appl Neurobiol. 2019 Apr;45(3):230-243. doi: 10.1111/nan.12495. Epub 2018 Jun 10.

Pathoarchitectonics of the cerebral cortex in chorea-acanthocytosis and Huntington's disease.

Author information

1
Department of Neurology, Xuanwu Hospital, Capital Medical University, Beijing, China.
2
Neurologische Klinik und Poliklinik, Ludwig-Maximilians-Universität München, München, Germany.
3
Department of Psychiatry, Psychosomatics and Psychotherapy, Center of Mental Health, University Hospital Würzburg, Würzburg, Germany.
4
Ageing Brain Study Group, Department of Pathology, University of São Paulo Medical School, São Paulo, Brazil.
5
Department of Neurology, University of California, San Francisco, San Francisco, CA, USA.
6
Praça Amadeu Amaral, São Paulo, Brazil.
7
Department of Radiology, University of São Paulo Medical School, São Paulo, Brazil.
8
Experimental Neurobiology (Anatomical Institute II), Goethe-University, Frankfurt/Main, Germany.
9
Anatomy & Cell Biology, Medical Faculty, Anatomical Institute, University of Bonn, Bonn, Germany.
10
Department of Pathology and Medical Biology, University Medical Center Groningen University of Groningen, Groningen, The Netherlands.
11
Center for Neuropathology and Prion Research, Ludwig-Maximilians-University Munich, Munich, Germany.
12
Department of Psychiatry and Psychotherapy, Ludwig-Maximilians-University Munich, Munich, Germany.
13
Department of Neuroanatomy, Ludwig-Maximilians-Universität München, Munich, Germany.
14
Clienia Privatklinik für Psychiatrie und Psychotherapie, Oetwil am See, Switzerland.

Abstract

AIMS:

Quantitative estimation of cortical neurone loss in cases with chorea-acanthocytosis (ChAc) and its impact on laminar composition.

METHODS:

We used unbiased stereological tools to estimate the degree of cortical pathology in serial gallocyanin-stained brain sections through the complete hemispheres of three subjects with genetically verified ChAc and a range of disease durations. We compared these results with our previous data of five Huntington's disease (HD) and five control cases. Pathoarchitectonic changes were exemplarily documented in TE1 of a 61-year-old female HD-, a 60-year-old female control case, and ChAc3.

RESULTS:

Macroscopically, the cortical volume of our ChAc cases (ChAc1-3) remained close to normal. However, the average number of neurones was reduced by 46% in ChAc and by 33% in HD (P = 0.03 for ChAc & HD vs. controls; P = 0.64 for ChAc vs. HD). Terminal HD cases featured selective laminar neurone loss with pallor of layers III, V and VIa, a high density of small, pale, closely packed radial fibres in deep cortical layers VI and V, shrinkage, and chromophilia of subcortical white matter. In ChAc, pronounced diffuse astrogliosis blurred the laminar borders, thus masking the complete and partial loss of pyramidal cells in layer IIIc and of neurones in layers III, V and VI.

CONCLUSION:

ChAc is a neurodegenerative disease with distinct cortical neurodegeneration. The hypertrophy of the peripheral neuropil space of minicolumns with coarse vertical striation was characteristic of ChAc. The role of astroglia in the pathogenesis of this disorder remains to be elucidated.

KEYWORDS:

minicolumn; neurodegenerative disease; neurone number; selective vulnerability; stereology

PMID:
29722054
PMCID:
PMC6994229
[Available on 2020-04-01]
DOI:
10.1111/nan.12495

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