Sunday, 12 February 2012

Corticobasal Ganglionic Degeneration

A 75 yo male ataxia. On neurological examination marked apraxia and cortical sensory loss like astereognosis). Increased tone in lower limbs.
MRI Axial T1 image show atrophy with marked widening of cortical sulci in parietal region. No signal abnormality in brain parenchyma on T2w images suggestive of cerebral cortical atrophy confined to parietal lobes.
Imaging finding of bilateral parietal lobe atrophy with history is consistent with a clinical diagnosis of Corticobasal Ganglionic Degeneration.


Corticobasal ganglionic degeneration (CBGD)
A rare Neurodegenerative disorder.
Imaging wise cerebral cortical atrophy prominently involving parietal lobes along with basal ganglia.
Clinically characterised by prominent cortical sensory loss and Apraxia. Alien hand syndrome, whereby pt complaints that he feels that his hands are disconnected from the rest of their body.
Dysfunction of the basal ganglia results in features of Parkinsonism, including increased tone, bradykinesia and gait disturbance. However, in contrast to Parkinson's Disease, Levodopa and other similar drugs are ineffective in this condition.
CBGD has significant overlap with other rare neurodegenerative disorders, including frontotemporal dementia, primary progressive aphasia (PPA), Pick's disease and progressive supranuclear palsy (PSP).

2 comments:

Brett Metelerkamp said...

Hello

Interesting case! Was there any evidence of medial temporal lobe atrophy or ventricular enlargement?

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