(Posterior Cortical Atrophy)

Rare neurodegenerative disorder  of unknown origin, sometimes considered as an atypical form of Alzheimer's disease, the symptoms of which  appear in the 5th decade. At MRI:  bilateral cortical occipito-parietal and occipito-temporal atrophy with a predominance on the right side. Histology shows lesions similar to those found in Alzheimer's disease in these regions: neurofibrillar tangles,  β-amyloid proteins.

Clinical picture: 

gradual decline of vision but speech and memory are retained:

-         associative visual agnosia, visual extinction, deficiency of the visual field in quadrant or hemi-fields

-         sometimes loss of writing skills: alexia, spatial agraphia, dyscalculia

-         dressing and ideomotor apraxia

Treatment: antidepressants and visual aids.

Anesthetic implications: 

not known; it is probably useful to monitor brain oxygenation and maintain normal cerebral perfusion according to age.

References : 

Updated: November 2019