Synonyms:
a.k.a. Cortical basal ganglionic degeneration CBGD formerly corticodentatonigral degeneration with neuronal achromasia a.k.a. corticonigral degeneration:
Diagnosis:
Clinical:
- Cortical signs: apraxia, visuospatial impairment, executive dysfunction, language dysfunction, spared memory function.
- +Basal ganglia signs: asymmetric rigidity, dystonia, myoclonus, alien hand,
- Relative Sparing of memory
Pathology:
Gross:
- Perisagittal atrophy of frontal & parietal lobes. Substantia nigra pallor
Microscopic:
- Cortical Microvacuolation, neuronal loss, gliosis. Neuronal loss in the substantia nigra & basal ganglia.
- Relatively spares the hippocampus & parahippocampal gyrus.
- Balloon neurons (chromatolytic-like)= large achromatic neurones (neuronal achromasia) in affected cortex (frontal & parietal) & basal ganglia.
Immunohistochemistry:
- Tau positive: Intraneuronal inclusions in cortical layer II.
- Astrocytic plaques
- Thread-like processes
- Oligodendroglial coiled bodies
- Neurofilament positive ballooned neurons
- Western blott: 4R type of Tau (4 microtubule repeats)