Tuesday, December 08, 2009

The Horror of Dide-Botcazo Syndrome

At least it sounds pretty horrible...

Dide-Botcazo Syndrome, or "top of the basilar" syndrome, is a rare clinical condition caused by bilateral occlusion of the posterior cerebral arteries (labelled below in red).

The arteries of the base of the brain

A case report by Cappellari et al., 2009 describes a 72 year old man who had a major stroke affecting the territories of both posterior cerebral arteries, resulting in damage to L and R occipital cortex, R thalamus, and R medial temporal lobe (see below).

Fig. 2 (Cappellari et al., 2009). MRI-DWI [diffusion weighted imaging] demonstrates areas of altered signal in bilateral occipital regions, right thalamus and right mesial temporal lobe [MTL, critical for memory], suggesting an ischemic origin [caused by decreased blood supply]. NOTE: R side of brain on L side of scan.

Shortly thereafter, some of the ischemic areas started hemorrhaging (huge white areas in the CT scan below).

Fig. 3 (Cappellari et al., 2009). Brain CT demonstrates a hemorrhagic transformation of the left temporal and occipital ischemic lesions.

The resulting behavioral syndrome consisted of cortical blindness from the extensive damage to visual cortex, with anosognosia (denial or unawareness) for blindness, amnesia (from MTL damage), and topographical disorientation thrown in for good measure:
Stable neurological picture, several days after onset, was characterized by persistent cortical blindness with absence of awareness of blindness, confabulation and spatial disorientation, recent memory disturbance, apathy, inertia and left hemiparesis [weakness].
Along with all the other sensory and cognitive deficits, it seems like the co-occurrence of apathy and inertia was a good thing...


Cappellari, M., Tomelleri, G., Matteo, A., Carletti, M., Magalini, A., Bovi, P., & Moretto, G. (2009). Dide-Botcazo syndrome due to bilateral occlusion of posterior cerebral artery Neurological Sciences DOI: 10.1007/s10072-009-0179-7

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At December 09, 2009 3:12 AM, Blogger Neuroskeptic said...

It could be worse - at least you get anosognosia so don't know how bad it is.

At December 09, 2009 1:15 PM, Anonymous Geoffrey Aguirre said...

This is an odd eponymous syndrome -- it is essentially the collection of deficits one would expect from large, bilateral occipito-temporal lesions.

It is like coining a name for the syndrome of aphasia, hemiplegia, apraxia, and inferior quadrantanopsia that would follow a complete Left MCA distribution stroke!

At December 09, 2009 1:59 PM, Blogger The Neurocritic said...

I had never heard of it before...

The field is wide open for naming the aphasia, hemiplegia, apraxia, and inferior quadrantanopsia syndrome, so it's all yours!


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