The syndrome of progressive posterior cortical dysfunction: A multiple case study and review

Abstract

Dementia presenting with prominent higher order visual symptoms may be observed in a range of neurodegenerative conditions and is often challenging to diagnose.ObjectivesTo describe cases of progressive dementia presenting with prominent visual cortical symptoms.MethodsWe conducted a retrospective search of cases of progressive dementia with predominant visual symptoms, seen at our dementia unit from 1996 to 2006.ResultsTwelve patients (5 men, 7 women) were identified, with ages ranging from 49 to 67 years. At the first examination, the duration of the symptoms ranged from one to ten years and the Mini-Mental State Examination scores from 7 to 27. Eleven patients presented with predominant visuospatial symptoms (partial or complete Balint syndrome) and one with visuoperceptive impairment. Other reported manifestations were: constructional apraxia in 11 patients, partial or complete Gerstmann syndrome in ten, ideomotor apraxia in nine, hemineglect or extinction in four patients, alien hand phenomenon in three, and prosopagnosia in one patient. Memory loss was reported by ten patients, but was not the main complaint in any of these cases. Insight was relatively preserved in five patients even after a long period following the onset of symptoms. Six patients developed parkinsonism during evolution. Clinical diagnoses were possible or probable AD in seven patients, cortico-basal degeneration in four, and dementia with Lewy body in one.ConclusionsClinicians should consider this condition especially in presenile patients with slowly progressive higher-order visual symptoms. Although described in association with different conditions, it may also occur in Alzheimer disease.

Progressive posterior cortical dysfunction

“…In a multiple case study 4 of 12 patients with PPCD, only one patient presented visual agnosia, which is a relatively uncommon finding. 1 Agnosia is often defined as “a normal perception stripped of its meaning”.…”

“…Visuospatial processing disorders are more frequent, indicating a predilection for the dorsal occipito-parietal stream. 1 , 4 Our aim was to describe a patient with this syndrome and to present a video showing the main abnormalities.…”

Posterior cortical atrophy - a prototypical case of dementia beginning with visual symptoms: case report

“…These symptoms may suggest a primarily oph thalmologic disorder and add to the fact that PCA rarely presents with memory complains, this clinical syndrome may not evoke promptly the diagnosis of a dementia disorder, thus postponing its correct treatment. For that reason, it is essential to rule out primary ophthal mologic causes when diagnosing PPCD (6,7) . In fact, according to some authors (8) , PPCD can sometimes be misattributed to ocular causes, such as cataract or macular disease.…”

A patient with posterior cortical atrophy due to Alzheimer’s disease

“… 4 Symptoms such as space perception deficit, constructional dyspraxia, environmental agnosia, dressing apraxia, alexia, limb apraxia, prosopagnosia, and visual field defect are also described in PCA. 4 , 5 , 7 Other neurological features may be present including depression, hallucinations, myoclonus, limb rigidity, alien limb phenomena, tremor, and seizures. 4 , 5 , 7 …”

“… 4 , 5 , 7 Other neurological features may be present including depression, hallucinations, myoclonus, limb rigidity, alien limb phenomena, tremor, and seizures. 4 , 5 , 7 …”