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Encephalitis ll: Pathophysiology01:26

Encephalitis ll: Pathophysiology

Encephalitis is inflammation of the brain parenchyma caused by direct viral invasion or immune-mediated mechanisms triggered by infections or tumors. Both processes lead to neuronal injury, disrupted neurotransmission, and diverse neurological symptoms, often with overlapping clinical and pathological features.Autoimmune EncephalitisIn autoimmune encephalitis, antibodies target neuronal antigens on cell surfaces, synapses, or within neurons. A key example is anti-NMDAR encephalitis, which can...

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Hereditary diffuse leukoencephalopathy with spheroids: ultrastructural and immunoelectron microscopic studies.

Wen-Lang Lin1, Zbigniew K Wszolek, Dennis W Dickson

  • 1Department of Neuroscience, Mayo Clinic, Jacksonville, Florida 32224, USA.

International Journal of Clinical and Experimental Pathology
|September 11, 2010
PubMed
Summary

Hereditary diffuse leukoencephalopathy with spheroids (HDLS) involves white matter degeneration. This study reveals new ultrastructural details of axonal spheroids and capillary basal lamina changes in HDLS brains.

Keywords:
Hereditary diffuse leukoencephalopathycapillary basal laminaimmunoelectron microscopyspheroidsultrastructure

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Area of Science:

  • Neuropathology
  • Neurodegenerative Diseases
  • White Matter Disorders

Background:

  • Hereditary diffuse leukoencephalopathy with spheroids (HDLS) is a rare autosomal dominant disorder causing progressive cognitive and motor decline.
  • While histopathology is known, ultrastructural details of HDLS pathology are lacking.

Purpose of the Study:

  • To provide ultrastructural and immunoelectron microscopic characterization of axonal spheroids and capillary basal lamina in HDLS white matter.
  • To elucidate the detailed morphology of pathological changes in HDLS.

Main Methods:

  • Ultrastructural analysis of HDLS brain white matter using electron microscopy.
  • Immunoelectron microscopy to identify components within spheroids and basal lamina.

Main Results:

  • Axonal spheroids showed aggregated neurofilaments (phosphorylated and non-phosphorylated) and other organelles, with variable myelin sheaths.
  • Capillary basal laminae exhibited dilation, deposition of collagen type I and III, and association with fibronectin.
  • Reactive astrocytes and lipid-laden macrophages were observed in the gliotic white matter.

Conclusions:

  • This study provides novel ultrastructural insights into HDLS pathology, detailing spheroid composition and capillary basal lamina abnormalities.
  • The findings highlight previously unemphasized pathological features that may be crucial for understanding HDLS pathogenesis.