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Posterior Cortical Atrophy

PCA


Presentation

  • Atypical focal cortical presentations have been described, including the logopenic variant of primary progressive aphasia (lvPPA) which presents with language impairment, and posterior cortical atrophy (PCA) which presents with prominent visuospatial[ncbi.nlm.nih.gov]
Progressive Dementia
  • Three individuals with progressive dementia that began with higher visual dysfunction (posterior cortical atrophy) were followed up to definitive neuropathologic diagnosis.[ncbi.nlm.nih.gov]
  • • Five patients had progressive dementia heralded by disorders of higher visual function. All eventually developed alexia, agraphia, visual agnosia, and components of Balint's, Gerstmann's, and transcortical sensory aphasia syndromes.[archneur.ama-assn.org]
Auditory Hallucination
  • Auditory hallucinations were only present in patients meeting diagnosis criteria for DLB. CONCLUSION: Prevalence of the 12 NPS examined was similar between patients with PCA and AD.[ncbi.nlm.nih.gov]
Dysmetria
  • Balint's syndrome is characterized by faulty visual scanning, dysmetria secondary to a visual perceptual deficit, and an inability to recognize more than one object at a time.[ncbi.nlm.nih.gov]
Slow Speech
  • RESULTS: Relative to healthy controls, PCA patients exhibited language impairments across all of the domains examined, but with anomia, reduced phonemic fluency and slowed speech rate the most prominent deficits.[ncbi.nlm.nih.gov]

Workup

  • Subsequently the development of uncommon symptoms, such as extrapyramidal signs and primitive reflexes, has complicated the clinical profile, requiring more diagnostic workup with neuropathologic, genetic and neuroradiologic detailed analysis, to confirm[jneuro.com]
Gliosis
  • Three separate neuropathologic entities were discovered: subcortical gliosis, Alzheimer's disease, and Creutzfeldt-Jakob disease. Posterior cortical atrophy is a clinically homogeneous but pathologically heterogeneous syndrome.[ncbi.nlm.nih.gov]
  • Patients with non-specific subcortical gliosis and, more occasionally, Creutzfeldt Jacob disease, neither of which have an obvious affinity with PCA, have also been described.[doi.org]

Treatment

  • CASE REPORT A 58-year-old PCA patient underwent a cognitive rehabilitation treatment followed by 2 cycles of tDCS stimulation.[ncbi.nlm.nih.gov]
  • Management and treatment There is no cure for PCA. Medications like acetylcholinesterase inhibitors, used in the treatment of Alzheimer's disease, are available and might bring relief for some symptoms.[orpha.net]
  • Treatment and outcomes There are no treatments for posterior cortical atrophy known to slow or halt its progression.[alz.org]
  • Antidepressant drugs have also provided some positive effects.Patients may find success with non-prescription treatments such as psychological treatments.[en.wikipedia.org]

Prognosis

  • Prognosis Prognosis is poor as PCA is progressive disorder. Life expectancy after PCA diagnosis is thought to be similar (8-12 years) or superior at times compared to individuals affected with Alzheimer's disease.[orpha.net]
  • There is also paucity of research on prevalence, course, prognosis and management of PCA. In this article we describe a middle aged gentlemen presenting with clinical features suggestive of PCA. We also discussed relevant literature.[ncbi.nlm.nih.gov]
  • Treatment and prognosis As is the case with most neurodegenerative diseases, no cure is available. Management is medical and centers on behavioral techniques targeted at overcoming visual disabilities as well as the use of antidepressants.[radiopaedia.org]

Etiology

  • Etiology Etiology is unknown.[orpha.net]
  • This array of neuropsychological manifestations may help to distinguish among different clinical and etiological types of pCA, and to elucidate the pathophysiology of a syndrome that has been associated with conditions as diverse as Alzheimer's disease[ncbi.nlm.nih.gov]

Epidemiology

  • UCL) Institute of Neurology, Queen Square, London, UK. 3 Dementia Research Centre, Department of Neurodegenerative Disease, University College London (UCL) Institute of Neurology, Queen Square, London, UK; Department of Medical Statistics, Faculty of Epidemiology[ncbi.nlm.nih.gov]
  • Summary Epidemiology Prevalence is unknown, largely due to the lack of awareness of the syndrome and the inaccurate terminology referring to it. Clinical description All patients with PCA show impairment in at least one basic visual process.[orpha.net]
Sex distribution
Age distribution

Pathophysiology

  • The pathophysiology of this disorder may be related to an impairment of the ventral "what" stream's ability to interact with the dorsal "where" stream that mediates the allocation of spatial attention, or a deficit in the systems that allocate contralateral[ncbi.nlm.nih.gov]

Prevention

  • The new dementia blog from Alzheimer’s Research UK will share the ideas and experiences of people living with dementia, scientists working to improve diagnosis, prevention and treatment, and the fundraisers who support the UK’s leading dementia research[alzheimersresearchuk.org]
  • Ruling out AD may also prevent inappropriate treatment, renouncing to use someone of the many medications available for AD, which are unnecessary for the conditions mimicking the symptoms of AD.[omicsonline.org]
  • Varying the position of the finger is important to prevent an anticipatory response.[pn.bmj.com]

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