171:
37:
410:, or help with adapting to visual changes may help. People with PCA and their caregivers are likely to have different needs than the more typical cases of Alzheimer's disease, and may benefit from specialized support groups, or other groups for young people with dementia. No study to date has been definitive to provide accepted conclusive analysis on treatment options.
310:
The cause of PCA is unknown, and there are no fully accepted diagnostic criteria for the disease. This is partially due to the gradual onset of PCA symptoms, their variety, the rare nature of the disease, and the younger age of onset typically 50–60 years. In 2012, the first international conference
278:
There is no standard definition of PCA and no established diagnostic criteria, so it is not possible to know how many people have the condition. Some studies have found that about 5 percent of people diagnosed with
Alzheimer's disease have PCA. However, because PCA often goes unrecognized, the true
928:
Crutch SJ, Schott JM, Rabinovici GD, Boeve BF, Cappa SF, Dickerson BC, Dubois B, Graff-Radford NR, Krolak-Salmon P, Lehmann M, Mendez MF, Pijnenburg Y, Ryan NS, Scheltens P, Shakespeare T, Tang-Wai DF, van der Flier WM, Bain L, Carrillo MC, Fox NC (Jul 2013). "Shining a light on posterior cortical
274:
Over time, however, atrophy in PCA may spread to regions that are commonly damaged in AD, leading to shared AD symptoms such as deficits in memory, language, learning, and cognition. Although PCA has an earlier onset, a diagnosis with
Alzheimer's is often made, suggesting that the degeneration has
202:
spreads, more severe symptoms emerge, including the inability to recognize familiar people and objects, trouble navigating familiar places, and sometimes visual hallucinations. In addition, difficulty may be experienced in making guiding movements towards objects, and a decline in literacy skills
377:
For some people with PCA, neuroimaging may not give a clear diagnosis; therefore, careful observation in relation to PCA symptoms can also assist in the diagnosis. The variation and lack of organized clinical testing has led to continued difficulties and delays in the diagnosis of PCA.
1553:
366:. This variation within the images will assist in early diagnosis of PCA; however, as the years go on the images will become increasingly similar, due to the majority of PCA also developing to AD later in life because of continued brain
235:(AD), with an emphasis on visual deficits. Although in primarily different, but sometimes overlapping, brain regions, both involve progressive neural degeneration, as shown by the loss of neurons and synapses, and the presence of
353:
are often compared to those of people with AD to assist diagnosis. Due to the early onset of PCA in comparison to AD, images taken at the early stages of the disease will vary from brain images in AD. At this early stage brain
386:
Specific and accepted treatment for PCA has yet to be discovered; this may be due to the rarity and variations of the disease. At times people with PCA are treated with AD treatments, such as cholinesterase inhibitors:
1387:
Möller C, van der Flier WM, Versteeg A, Benedictus MR, Wattjes MP, Koedam EL, Scheltens P, Barkhof F, Vrenken H (Feb 2014). "Quantitative
Regional Validation of the Visual Rating Scale for Posterior Cortical Atrophy".
322:. It has been suggested that depression or anxiety may result from the symptoms of decreased visual function, and the progressive nature of the disease. Early visual impairments have often led to a referral to an
1063:
Kennedy, Jonathan; Manja
Lehmann; Magdalena J. Sokolska; Hilary Archer; Elizabeth K. Warrington; Nick C. Fox; Sebastian J. Crutch (25 October 2011). "Visualizing the emergence of posterior cortical atrophy".
98:
PCA usually affects people at an earlier age than typical cases of
Alzheimer's disease, with initial symptoms often experienced in people in their mid-fifties or early sixties. This was the case with writer
2068:
131:. The atrophy is progressive; early symptoms include difficulty reading, blurred vision, light sensitivity, issues with depth perception, and trouble navigating through space. Additional symptoms include
2454:
2449:
2466:
2444:
1617:
1813:
1112:"Atypical Form of Alzheimer's Disease with Prominent Posterior Cortical Atrophy: a Review of Lesion Distribution and Circuit Disconnection in Cortical Visual Pathways"
690:
Mendez, Mario; Mehdi
Ghajarania; Kent Perryman (14 June 2002). "Posterior cortical atrophy: Clinical characteristics and differences compared to Alzheimer's disease".
1808:
1210:
Nagaratnam, Nages; Kujan
Nagaratnam; Daniel Jolley; Allan Ting (6 June 2001). "Dementia following posterior cortical atrophy—a descriptive clinical case report".
251:(cell body) loss in posterior regions, especially in the occipital, parietal, and temporal lobes, whereas in Alzheimer's there is typically more damage in the
1610:
1431:
Migliaccio R, Agosta F, Toba MN, Samri D, Corlier F, de Souza LC, Chupin M, Sharman M, Gorno-Tempini ML, Dubois B, Filippi M, Bartolomeo P (November 2012).
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including reading, writing, and spelling may develop. Furthermore, if neural death spreads into other anterior cortical regions, symptoms similar to
279:
percentage may be as high as 15 percent. Researchers and physicians are working to establish a standard definition and diagnostic criteria for PCA.
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1603:
567:
271:
intact, whereas in AD there is typically impaired episodic memory, suggesting some differences still lie in the primary areas of cortical damage.
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on PCA was held in
Vancouver, Canada. Continued research and testing will hopefully result in accepted and standardized criteria for diagnosis.
2406:
2079:
2431:
2063:
2269:
2257:
1719:
878:
Goenthals, Maartin; Patrick
Santens (20 February 2001). "Posterior cortical atrophy. Two case reports and a review of the literature".
2545:
2540:
2488:
2421:
1976:
1352:; Michael E. Silverman (May 2011). "Posterior Cortical Atrophy: An Exemplar for Renovating Diagnostic Formulation in Neurosychiaty".
2191:
1994:
2310:
1312:
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1801:
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507:"The topography of metabolic deficits in posterior cortical atrophy (the visual variant of Alzheimer's disease) with FDG-PET"
2017:
287:
108:
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2389:
2305:
2247:
1796:
1151:
Tsunoda, Ayami; Shuji
Iritani; Norio Ozaki (17 March 2011). "Presenile dementia diagnosed as posterior cortical atrophy".
972:
Tsunoda, Ayami; Shuji Iritani; Norio Ozaki (17 March 2011). "Presenile dementia diagnosed as posterior cortical atrophy".
660:
2473:
1847:
359:
346:
1983:
167:, leads to PCA symptoms related to impaired movements in response to visual stimuli, such as navigation and apraxia.
374:(collections of neuronal cell bodies) in the posterior and occipital temporal cortices within the right hemisphere.
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Crutch, Sebastian; Manja Lehmann; Jonathan Schott; Gil Rabinovici; Martin Rosser; Nick Fox (February 2012).
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Crutch, Sebastian; Manja Lehmann; Jonathan Schott; Gil Rabinovici; Martin Rosser; Nick Fox (February 2012).
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103:(1948–2015), who went public in 2007 about being diagnosed with PCA. In rare cases, PCA can be caused by
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1433:"Brain Networks in Posterior Cortical Atrophy: A Single Case Tractography Study and Literature Review"
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can also assist in the diagnosis of PCA. For PCA and AD neuroimaging is carried out using
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and occipital gyrus, while AD brain images show the majority of atrophy in the medial
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36:
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Caine, Diana (2004). "Posterior Cortical Atrophy: A Review of the Literature".
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298:. In addition, PCA may result in part from mutations in the presenilin 1 gene (
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330:
1966:
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1258:
Crutch, Sebastian J.; Manja Lehmann; Jonathan M. Schott; Gil D. Rabinovici;
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Hof, Patrick; Brent Vogt; Constantin Bouras; John Morrison (December 1997).
522:
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The main symptom resulting from posterior cortical atrophy is a decrease in
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may result – the inability to see stimuli on one half of the visual field.
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1137:
610:
587:
Benson DF, Davis RJ, Snyder BD (July 1988). "Posterior Cortical Atrophy".
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370:. A key aspect found through brain imaging of PCA patients is a loss of
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132:
80:
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207:, such as memory loss, may result. In PCA where there is significant
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Spinal muscular atrophy with lower extremity predominance (SMALED)
299:
169:
403:. Antidepressant drugs have also provided some positive effects.
1599:
1187:"Posterior Cortical Atrophy | Signs, Symptoms, & Diagnosis"
75:
which is considered a visual variant or an atypical variant of
338:
821:"Posterior Cortical Atrophy: Review of the Recent Literature"
657:"Terry Pratchett pledges $ 1 million to Alzheimer's research"
275:
simply migrated anteriorly to other cortical brain regions.
247:
in both diseases. In PCA there is more cortical damage and
505:
Nestor PJ, Caine D, Fryer TD, Clarke J, Hodges JR (2003).
873:
871:
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will be shown to be more centrally located in the right
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1311:
Croisile, MD. Bernard; Alexis Brice (September 2004).
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for PCA, neuropsychological examinations are advised.
95:. PCA was first described by D. Frank Benson in 1988.
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91:, resulting in the progressive disruption of complex
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326:, which can result in unnecessary cataract surgery.
243:
in affected brain regions; this eventually leads to
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26:
21:
1313:"Benson's syndrome or Posterior Cortical Atrophy"
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1251:
1249:
1247:
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1241:
482:"Visual variant of Alzheimer's disease - EyeWiki"
814:
812:
231:Studies have shown that PCA may be a variant of
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121:visuospatial and visuoperceptual capabilities
8:
819:Borruat, François-Xavier (18 October 2013).
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692:Dementia and Geriatric Cognitive Disorders
343:single-photon emission computed tomography
314:PCA is often initially misdiagnosed as an
35:
18:
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1287:
1127:
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772:
622:
620:
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452:
143:, an object recognition disorder. In the
123:, since the area of atrophy involves the
1066:Neurocase: The Neural Basis of Cognition
568:University of California, San Francisco
419:
2407:Distal hereditary motor neuronopathies
2080:Template:Demyelinating diseases of CNS
1212:Archives of Gerontology and Geriatrics
632:Martinos Center for Biomedical Imaging
554:
552:
550:
427:Schott JM, Crutch SJ (February 2019).
7:
2258:Idiopathic intracranial hypertension
603:10.1001/archneur.1988.00520310107024
476:
474:
472:
880:Clinical Neurology and Neurosurgery
139:, an impaired ability to read, and
135:, a disorder of movement planning,
2489:Infantile progressive bulbar palsy
1527:Posterior Cortical Atrophy support
14:
2551:Unsolved problems in neuroscience
2192:Template:Cerebrovascular diseases
1995:Frontotemporal lobar degeneration
227:Connection to Alzheimer's disease
2536:Central nervous system disorders
2212:For more detailed coverage, see
2190:For more detailed coverage, see
2161:For more detailed coverage, see
2127:For more detailed coverage, see
2078:For more detailed coverage, see
1165:10.1111/j.1479-8301.2011.00366.x
986:10.1111/j.1479-8301.2011.00366.x
282:PCA may also be correlated with
190:. Both streams originate in the
1366:10.1016/j.comppsych.2010.06.013
1262:; Nick C. Fox (February 2012).
211:in one hemisphere of the brain
1:
2505:Amyotrophic lateral sclerosis
2390:Hereditary spastic paraplegia
2248:Normal pressure hydrocephalus
1280:10.1016/s1474-4422(11)70289-7
1224:10.1016/s0167-4943(01)00179-0
1129:10.1016/s0042-6989(96)00240-4
1025:10.1016/s1474-4422(11)70289-7
892:10.1016/s0303-8467(01)00114-7
765:10.1016/s1474-4422(11)70289-7
511:J Neurol Neurosurg Psychiatry
161:dorsal, or "where/how" stream
2474:Progressive muscular atrophy
1449:10.1016/j.cortex.2011.10.002
1264:"Posterior Cortical Atrophy"
1078:10.1080/13554794.2011.588180
1009:"Posterior cortical atrophy"
746:"Posterior Cortical Atrophy"
663:. 2011-07-29. Archived from
628:"Posterior Cortical Atrophy"
564:UCSF Memory and Aging Center
560:"Posterior Cortical Atrophy"
445:10.1212/CON.0000000000000696
429:"Posterior Cortical Atrophy"
347:positron emission tomography
30:Biparietal Alzheimer disease
1984:Primary progressive aphasia
194:(blue) located posteriorly.
2572:
2306:Hashimoto's encephalopathy
2012:Posterior cortical atrophy
1833:Striatonigral degeneration
1684:Cavernous sinus thrombosis
943:10.1016/j.jalz.2012.11.004
931:Alzheimer's & Dementia
661:Alzheimer's Research Trust
223:are also common symptoms.
186:(purple) runs through the
61:Posterior cortical atrophy
22:Posterior cortical atrophy
2546:Ailments of unknown cause
2541:Aging-associated diseases
2412:Spinal muscular atrophies
2380:Primary lateral sclerosis
2238:Intracranial hypertension
2018:Creutzfeldt–Jakob disease
1494:10.1080/13554790490892239
1402:10.1007/s00330-013-3025-5
840:10.1007/s11910-013-0406-8
406:Other treatments such as
288:Creutzfeldt–Jakob disease
178:(green) runs through the
149:ventral, or "what" stream
109:Creutzfeldt–Jakob disease
105:dementia with Lewy bodies
79:(AD). The disease causes
43:
34:
2479:Progressive bulbar palsy
2270:Intracranial hypotension
2253:Choroid plexus papilloma
1667:Herpesviral encephalitis
1531:Dementia Research Centre
1354:Comprehensive Psychiatry
433:Continuum (Minneap Minn)
44:Lobes of the human brain
1991:Frontotemporal dementia
1677:Encephalitis lethargica
523:10.1136/jnnp.74.11.1521
237:neurofibrillary tangles
226:
2296:Hepatic encephalopathy
1348:Goldstein, Martin A.;
259:. PCA tends to impair
195:
145:two-streams hypothesis
2354:Ataxia–telangiectasia
2311:Static encephalopathy
2034:Mitochondrial disease
1872:Spasmodic torticollis
1782:Basal ganglia disease
1320:Orphanet Encyclopedia
590:Archives of Neurology
173:
2301:Toxic encephalopathy
2007:Lewy bodies dementia
1268:The Lancet Neurology
1159:(171–176): 171–176.
1013:The Lancet Neurology
753:The Lancet Neurology
408:occupational therapy
71:, is a rare form of
2349:Friedreich's ataxia
1732:Meningoencephalitis
1672:Limbic encephalitis
1329:on 12 November 2013
828:Neuro-Ophthalmology
329:Due to the lack of
233:Alzheimer's disease
213:hemispatial neglect
205:Alzheimer's disease
77:Alzheimer's disease
2385:Pseudobulbar palsy
2123:Status epilepticus
2074:Multiple sclerosis
1911:Myoclonic epilepsy
1774:movement disorders
1720:Acute disseminated
1662:Viral encephalitis
1570:External resources
1390:European Radiology
636:Harvard University
296:Gerstmann syndrome
265:anterograde memory
196:
2523:
2522:
2519:
2518:
2515:
2514:
2319:
2318:
2224:
2223:
2163:Template:Headache
2129:Template:Epilepsy
2050:
2049:
2024:Vascular dementia
1867:Status dystonicus
1740:
1739:
1715:Encephalomyelitis
1593:
1592:
1443:(10): 1298–1309.
1122:(24): 3609–3625.
704:10.1159/000058331
517:(11): 1521–1529.
292:Bálint's syndrome
284:Lewy body disease
253:prefrontal cortex
200:neurodegeneration
163:, located in the
155:, located in the
129:visual processing
93:visual processing
69:Benson's syndrome
58:
57:
16:Medical condition
2563:
2337:
2328:
2286:Brain herniation
2097:
1937:Intention tremor
1932:Essential tremor
1797:Postencephalitic
1765:
1756:
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1626:Diseases of the
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1322:. Archived from
1317:
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1302:
1301:
1291:
1260:Martin N. Rossor
1255:
1236:
1235:
1207:
1201:
1200:
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1153:Psychogeriatrics
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974:Psychogeriatrics
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316:anxiety disorder
267:, while leaving
147:, damage to the
127:responsible for
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2556:1988 in science
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2437:Congenital DSMA
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2208:Sleep disorders
2196:
2173:Cerebrovascular
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1899:Choreoathetosis
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486:eyewiki.aao.org
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384:
364:temporal cortex
324:ophthalmologist
308:
269:episodic memory
229:
117:
101:Terry Pratchett
89:cerebral cortex
67:), also called
17:
12:
11:
5:
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2265:Cerebral edema
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2015:
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2009:
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1769:Extrapyramidal
1762:
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1751:encephalopathy
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1540:Classification
1534:
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1522:
1521:External links
1519:
1516:
1515:
1488:(5): 382–385.
1472:
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1396:(2): 397–404.
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1360:(3): 326–333.
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964:
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886:(2): 115–119.
861:
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759:(2): 170–178.
725:
677:
648:
616:
597:(7): 789–793.
579:
546:
497:
468:
418:
417:
415:
412:
383:
380:
360:posterior lobe
307:
304:
261:working memory
241:senile plaques
228:
225:
192:occipital lobe
184:ventral stream
141:visual agnosia
125:occipital lobe
116:
113:
56:
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46:
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2291:Reye syndrome
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2256:
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2243:Hydrocephalus
2241:
2240:
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2056:Demyelinating
2053:
2043:
2040:
2039:
2037:
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2031:
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2022:
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2020:
2019:
2013:
2010:
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1998:
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1989:
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1982:
1978:
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1950:
1947:
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1944:Restless legs
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1882:Blepharospasm
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1838:Hemiballismus
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1689:Brain abscess
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667:on 2013-01-14
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349:(PET scans).
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234:
224:
222:
218:
214:
210:
206:
201:
193:
189:
188:temporal lobe
185:
181:
180:parietal lobe
177:
176:dorsal stream
172:
168:
166:
165:parietal lobe
162:
158:
157:temporal lobe
154:
153:visual system
150:
146:
142:
138:
134:
130:
126:
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53:
51:
47:
42:
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33:
29:
25:
20:
2499:
2398:
2371:
2332:Degenerative
2069:Inflammatory
2016:
2011:
1949:Stiff-person
1787:Parkinsonism
1760:Degenerative
1657:Encephalitis
1640:Inflammation
1630:, primarily
1577:
1552:
1485:
1481:
1475:
1440:
1436:
1426:
1393:
1389:
1382:
1357:
1353:
1343:
1331:. Retrieved
1324:the original
1319:
1306:
1271:
1267:
1215:
1211:
1205:
1194:. Retrieved
1190:
1181:
1156:
1152:
1146:
1119:
1115:
1069:
1065:
1016:
1012:
1002:
977:
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967:
937:(4): 463–5.
934:
930:
883:
879:
831:
827:
756:
752:
698:(1): 33–40.
695:
691:
669:. Retrieved
665:the original
651:
640:. Retrieved
638:. 2009-01-19
631:
594:
588:
582:
571:. Retrieved
563:
514:
510:
500:
489:. Retrieved
485:
439:(1): 52–75.
436:
432:
422:
405:
393:rivastigmine
385:
376:
335:Neuroimaging
328:
313:
309:
281:
277:
273:
230:
197:
118:
97:
87:part of the
68:
64:
60:
59:
2484:Fazio–Londe
2324:Both/either
2118:Generalised
1977:Early-onset
1972:Alzheimer's
1707:spinal cord
1333:11 November
834:(12): 406.
399:, and also
397:galantamine
372:grey matter
351:Neuroimages
257:hippocampus
249:gray matter
27:Other names
2530:Categories
2092:paroxysmal
2064:Autoimmune
1857:Dyskinesia
1727:Meningitis
1705:Brain and
1196:2016-07-25
1116:Vision Res
929:atrophy".
671:2011-07-29
642:2011-10-22
573:2011-10-22
491:2017-11-07
414:References
331:biomarkers
320:depression
221:depression
182:, and the
2089:Episodic/
1967:Tauopathy
1918:Akathisia
1906:Myoclonus
1889:Athetosis
1821:Tauopathy
1529:from the
1482:Neurocase
401:memantine
389:donepezil
382:Treatment
306:Diagnosis
151:, of the
85:posterior
54:Neurology
50:Specialty
2455:SMALED2B
2450:SMALED2A
2147:Migraine
2139:Headache
2105:epilepsy
2101:Seizures
1959:Dementia
1862:Dystonia
1579:Orphanet
1510:45491962
1502:15788276
1467:22099855
1418:14187936
1410:24092044
1374:21497228
1298:22265212
1232:15374033
1191:Dementia
1173:21951958
1094:20617012
1086:22026812
1043:22265212
994:21951958
959:16404876
951:23274153
908:46582748
900:11516556
856:45579994
848:24136454
783:22265212
720:12291848
712:12053130
541:14617709
463:30707187
245:dementia
115:Symptoms
73:dementia
2467:SMA-PME
2462:SMA-PCH
2445:SMALED1
2157:Tension
2152:Cluster
1877:Meige's
1694:Amoebic
1561:: G31.1
1458:4813795
1289:3740271
1138:9425534
1034:3740271
774:3740271
611:3390033
532:1738241
454:6548537
368:atrophy
356:atrophy
341:scans,
217:Anxiety
209:atrophy
133:apraxia
83:of the
81:atrophy
2186:Stroke
2000:Pick's
1927:Tremor
1894:Chorea
1508:
1500:
1465:
1455:
1437:Cortex
1416:
1408:
1372:
1296:
1286:
1230:
1171:
1136:
1092:
1084:
1041:
1031:
992:
957:
949:
906:
898:
854:
846:
781:
771:
718:
710:
609:
539:
529:
461:
451:
345:, and
294:, and
137:alexia
2500:both:
2432:DSMA1
2427:SMAX2
2422:SMAX1
2402:only:
2375:only:
2279:Other
2201:Other
2113:Focal
1746:Brain
1649:Brain
1584:54247
1506:S2CID
1414:S2CID
1327:(PDF)
1316:(PDF)
1090:S2CID
955:S2CID
904:S2CID
852:S2CID
824:(PDF)
749:(PDF)
716:S2CID
300:PSEN1
2103:and
1814:PKAN
1809:NBIA
1498:PMID
1463:PMID
1406:PMID
1370:PMID
1335:2013
1294:PMID
1228:PMID
1169:PMID
1134:PMID
1082:PMID
1039:PMID
990:PMID
947:PMID
896:PMID
844:PMID
779:PMID
708:PMID
607:PMID
537:PMID
459:PMID
263:and
255:and
239:and
219:and
174:The
107:and
2417:SMA
2400:LMN
2373:UMN
2364:MND
2230:CSF
2181:TIA
1826:PSP
1802:NMS
1771:and
1632:CNS
1554:ICD
1490:doi
1453:PMC
1445:doi
1398:doi
1362:doi
1284:PMC
1276:doi
1220:doi
1161:doi
1124:doi
1074:doi
1029:PMC
1021:doi
982:doi
939:doi
888:doi
884:103
836:doi
769:PMC
761:doi
700:doi
599:doi
527:PMC
519:doi
449:PMC
441:doi
339:MRI
318:or
302:).
198:As
65:PCA
2532::
2341:SA
1848:OA
1843:HD
1792:PD
1582::
1558:10
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1461:.
1451:.
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63:(
Text is available under the Creative Commons Attribution-ShareAlike License. Additional terms may apply.
