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Apoptosis in cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy

Francoise Gray1, Marc Polivka, Anand Viswanathan

  • 1Department of Pathology, APHP Hôpital Lariboisière, Université Paris VII, 2 rue Ambroise Paré, 75475 Paris, France. francoise.gray@lrb.ap-hop-paris.fr

Journal of Neuropathology and Experimental Neurology
|July 11, 2007
PubMed

Insights

Apoptosis plays a key role in cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) pathogenesis. Increased vascular and neuronal apoptosis correlates with lesion severity and cognitive decline in CADASIL patients.

Area of Science:

  • Neurology
  • Pathology
  • Cell Biology

Background:

  • Cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a genetic small vessel disease.
  • The pathogenesis of cerebral lesions in CADASIL is not fully understood.
  • Apoptosis, or programmed cell death, is a potential mechanism in neurodegenerative diseases.

Purpose of the Study:

  • To investigate the role of apoptosis in the pathogenesis of cerebral lesions in CADASIL.
  • To compare apoptotic processes in CADASIL, Binswanger disease, and control brains.

Main Methods:

  • Human brain tissue samples from CADASIL, Binswanger disease, and control groups were analyzed.
  • In situ end labeling and activated caspase 3 immunostaining were used to detect apoptotic cells.
  • Immunolabeling for Notch3, beta-amyloid precursor protein, and phosphorylated neurofilament protein was performed.

Main Results:

  • Vascular cell apoptosis was significantly increased in CADASIL, particularly in the basal ganglia and subcortical white matter.
  • Neuronal apoptosis was observed in CADASIL, primarily in cortical layers 3 and 5.
  • The severity of neuronal apoptosis correlated with ischemic lesions, white matter axonal damage, and cognitive impairment in CADASIL patients.

Conclusions:

  • Apoptosis of vascular cells may contribute to the dilatation of Virchow-Robin spaces in CADASIL.
  • Neuronal apoptosis is implicated in cortical atrophy and cognitive decline in CADASIL.
  • Axonal damage in the white matter may contribute to neuronal apoptosis in CADASIL.

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