Riassunto
Le malattie cerebrali su base degenerativa che si caratterizzano per il prevalente coinvolgimento del sistema extrapiramidale, sebbene generalmente contraddistinte dalle alterazioni motorie, so no spesso associate a disturbi cognitivi e comportamentali, fino a delinearsi in alcuni casi come vere e proprie sindromi demenziali. Le malattie principali di questo gruppo sono la malattia di Parkinson (MP), la paralisi sopranucleare progressiva (PSP), la malattia di Huntington (HD) e le atrofie multisistemiche che rappresentano forme più rare di disturbi extrapiramidali associati a declino cognitivo. Queste malattie colpiscono principalmente, ma non necessariamente in maniera esclusiva, le strutture sottocorticali dell’encefalo come i gangli della base, il talamo, i nuclei rostrali del troncoencefalo nonché le proiezioni di queste strutture alle aree corticali del lobo frontale e al sistema limbico. In queste patologie tuttavia i disturbi motori sono generalmente preminenti sia in termini di comparsa che di gravità rispetto ai deficit cognitivi che appaiono invece secondari e di accompagnamento alla patologia motoria. Diversamente, nella demenza a corpi di Lewy (DLB), in cui le lesioni neuropatologiche si distribuiscono diffusamente alle strutture sottocorticali e corticali dell’encefalo, i disturbi cognitivi e motori (oltre a peculiari disturbi comportamentali) rappresentano entrambi i sintomi cardinali della patologia (McKeith et al., 2004). Anche nella degenerazione corticobasale (CBD) si assiste all’associazione di una sintomatologia di origine sia corticale che sottocorticale; in questo caso però, il complesso quadro neuropsicologico e le particolari caratteristiche motorie la rendono un’entità clinico-patologica distinta dalle altre malattie dementigene con disturbi motori.
Preview
Unable to display preview. Download preview PDF.
Bibliografia
Aarsland D, Litvan I, Salmon D et al (2003) Performance on the dementia rating scale in Parkinson’s disease with dementia and dementia with Lewy bodies: comparison with progressive supranuclear palsy and Alzheimer’s disease. J Neurol Neurosurg Psychiatry 74(9):1215–1220
Albert ML, Feldman RG, Willis AL (1974) The subcortical dementia of progressive supranuclear palsy. J Neurol Neurosurg Psychiatry 37:121–130
Andrews TC, Brooks DJ (1998) Advances in the understanding of early Huntington’s disease using the functional imaging techniques of PET and SPET. Mol Med Today 4(12):532–539
Bak TH, Caine D, Hearn VC, Hodges JR (2006) Visuospatial functions in a typical parkinsonian syndromes. J Neurol Neurosurg Psychiatry 77(4):454–456.
Bak TH, Craqwford LM, Hearn VC et al (2005) Subcortical dementia revisited: similarities and differences in cognitive function between progressive supranuclear palsy (PSP), corticobasal degeneration (CBD) and multiple system atrophy (MSA). Neurocase 11:268–273
Ballard C, Patel A, Oyebode F, Wilcock G (1996) Cognitive decline in patients with Alzheimer’s disease, vascular dementia and senile dementia of Lewy body type. Age Ageing 25(3):209–213
Bartzokis G, Cummings J, Perlman S et al (1999) Increased basal ganglia iron levels in Huntington disease. Arch Neurol 56(5):569–574
Beatty WW, Butters N (1986) Further analysis of encoding in patients with Huntington’s disease. Brain Cogn 5(4):387–398
Beatty WW, Salmon DP, Butters N et al (1988) Retrograde amnesia in patients with Alzheimer’s disease or Huntington’s disease. Neurobiol Aging 9(2):181–186
Berent S, Giordani B, Gilman S et al (2002) Patterns of neuropsychological performance in multiple system atrophy compared to sporadic and hereditary olivopontocerebellar atrophy. Brain Cogn 50(2):194–206
Bigio EH, Brown DF, White CL 3rd (1999) Progressive supranuclear palsy with dementia: cortical pathology. J Neuropathol Exp Neurol 58(4):359–364
Blondel A, Eustache F, Schaeffer S et al (1997) Clinical and cognitive study of apraxia in corticobasal atrophy. A selective disorder of the production system. Rev Neurol 153(12):737–747
Bradshaw JM, Saling M, Anderson V et al (2006) Higher cortical deficits influence attentional processing in dementia with Lewy bodies, relative to patients with dementia of the Alzheimer’s type and controls. J Neurol Neurosurg Psychiatry 77(10):1129–1135
Caine ED, Bamford KA, Schiffer RB et al (1986) A controlled neuropsychological comparison of Huntington’s disease and multiple sclerosis. Arch Neurol 43(3):249–254
Calderon J, Perry RJ, Erzinclioglu SW et al (2001) Perception, attention, and working memory are disproportionately impaired in dementia with Lewy bodies compared with Alzheimer’s disease. J Neurol Neurosurg Psychiatry 70(2):157–164
Cambier J, Masson M, Viader F et al (1985) Frontal syndrome of progressive supranuclear palsy. Rev Neurol (Paris) 141(8–9):528–536
Carlesimo GA, Perri R, Caltagirone C (1998) Polimorfismo neuropsicologico nelle demenze degenerative. Nuova rivista di Neurologia 8:157–166
Colloby SJ, Fenwick JD, Williams ED et al (2002) A comparison of (99m)Tc-HMPAO SPET changes in dementia with Lewy bodies and Alzheimer’s disease using statistical parametric mapping. Eur J Nucl Med Mol Imaging 29(5):615–622
Connor DJ, Salmon DP, Sandy TJ et al (1998) Cognitive profiles of autopsy-confirmed Lewy body variant vs pure Alzheimer disease. Arch Neurol 55(7):994–1000
Cordato NJ, Pantelis C, Halliday GM et al (2002) Frontal atrophy correlates with behavioural changes in progressive supranuclear palsy. Brain 25 (Pt 4):789–800
Cormack F, Gray A, Ballard C, Tovée MJ (2004) A failure of ‘pop-out’ in visual search tasks in dementia with Lewy Bodies as compared to Alzheimer’s and Parkinson’s disease. Int J Geriatr Psychiatry 19(8):763–772
Cummings JL, Benson F (1984) Subcortical dementia. Review of an emerging concept. Arch Neurol 41:874–879
Dickson DW, Rademakers R, Hutton ML (2007) Progressive supranuclear palsy: pathology and genetics. Brain Pathol 17:74–82
Doubleday EK, Snowden JS, Varma AR, Neary D (2002) Qualitative performance characteristics differentiate dementia with Lewy bodies and Alzheimer’s disease. J Neurol Neurosurg Psychiatry 72(5):602–607
Dubois B, Deweer B, Pillon B (1996) The cognitive syndrome of progressive supranuclear palsy. Adv Neurol 69:399–403
Dubois B, Pillon B, Legault F et al (1988) Slowing of cognitive processing in progressive supranuclear palsy. A comparison with Parkinson’s disease. Arch Neurol 45(11):1194–1199
Dubois B, Slachevsky A, Litvan I, Pillon B (2000) The FAB: a frontal assessment battery at bedside. Neurology 55(11):1194–1199
Esmonde T, Giles E, Gibson M, Hodges JR (1996) Neuropsychological performance, disease severity, and depression in progressive supranuclear palsy. J Neurol 243(9):638–643
Faglioni P (1996) Lobo frontale. In: Denes Ge Pizzamiglio L (a cura di) Manuale di Neuropsicologia. Zanichelli, Bologna.
Finke K, Schneider WX, Redel P et al (2007) The capacity of attention and simultaneous perception of objects: a group study of Huntington’s disease patients. Neuropsychologia 45(14):3272–3284
Frasson E, Moretto G, Beltramello A et al (1998) Neuropsychological and neuroimaging correlates in corticobasal degeneration. Ital J Neurol Sci 19(5):321–328
Frattali CM, Grafman J, Patronas N et al (2000) Language disturbances in corticobasal degeneration. Neurology 54(4):990–992
Gibb WR, Luthert PJ, Marsden CD (1989) Corticobasal degeneration. Brain 112(Pt 5):1171–1192
Grafman J, Litvan I, Gomez C, Chase TN (1990) Frontal lobe function in progressive supranuclear palsy. Arch Neurol 47(5):553–558
Graham NL, Bak T, Patterson K, Hodges JR (2003) Language function and dysfunction in corticobasal degeneration. Neurology 61(4):493–499
Graham NL, Bak TH, Hodges JR (2003) Corticobasal degeneration as a cognitive disorder. Mov Disord 18(11):1224–1232
Graham NL, Zeman A, Young AW et al (1999) Dyspraxia in a patient with corticobasal degeneration: the role of visual and tactile inputs to action. J Neurol Neurosurg Psychiatry 67(3):334–344
Hamilton JM, Salmon DP, Galasko D et al (2004) A comparison of episodic memory deficits in neuropathologically-confirmed Dementia with Lewy bodies and Alzheimer’s disease. J Int Neurosurg ropsychol Soc 10(5):689–697
Hauw JJ, Danile SE, Dickson D et al (1994) Preliminary NINDS neuropathologic criteria for Steele-Richardson-Olszewski syndrome (progressive supranuclear palsy). Neurology 44:2015–2019
Heindel WC, Salmon DP, Shults CW et al (1989) Neuropsychological evidence for multiple implicit memory systems: a comparison of Alzheimer’s, Huntington’s, and Parkinson’s disease patients. J Neurosci 9(2):582–587
Heyman A, Fillenbaum GG, Gearing M et al (1999) Comparison of Lewy body variant of Alzheimer’s disease with pure Alzheimer’s disease: Consortium to Establish a Registry for Alzheimer’s Disease, Part XIX. Neurology 52(9):1839–1844
Higuchi M, Tashiro M, Arai H et al (2000) Glucose hypometabolism and neuropathological correlates in brains of dementia with Lewy bodies. Exp Neurol 162(2):247–256
Ho AK, Nestor PJ, Williams GB et al (2004) Pseudo-neglect in Huntington’s disease correlates with decreased angular gyrus density. Neuroreport 15(6):1061–1064
Ho AK, Sahakian BJ, Brown RG et al (2003) Profile of cognitive progression in early Huntington’s disease. Neurology 61(12):1702–1706
Hodges JR, Spatt J, Patterson K (1999) “What” and “how”: evidence for the dissociation of object knowledge and mechanical problem-solving skills in the human brain. Proc Natl Acad Sci US A96(16):9444–9448
Huber SJ, Shuttleworth EC, Paulson GW et al (1986) Cortical vs subcortical dementia. Neuropsychological differences. Arch Neurol 43:392–394
Jacobs DH, Adair JC, Macauley B et al (1999) Apraxia in corticobasal degeneration. Brain Cogn 40(2):336–354
Johnson R Jr (1992) Event-related brain potentials. In: Litvan I, Agid Y (Eds) Progressive supranuclear palsy: clinical and research approaches. Oxford United Press, New York
Johnson R Jr, Litvan I, Grafman J (1991) Progressive supranuclear palsy: altered sensory processing leads to degraded cognition. Neurology 41(8):1257–1262
Kaat LD, Boon AJ, Kamphorst W et al (2007) Frontal presentation in progressive supranuclear palsy. Neurology 69(8):723–729
Kawai Y, Suenaga M, Takeda A et al (2008) Cognitive impairments in multiple system atrophy: MSAC vs MSA-P. Neurology 70(16 Pt 2):1390–1396
Kertesz A, Martinez-Lage P, Davidson W, Munoz DG (2000) The corticobasal degeneration syndrome overlaps progressive aphasia and frontotemporal dementia. Neurology 55(9):1368–1375
Lambon Ralph MA, Powell J, Howard D et al (2001) Semantic memory is impaired in both dementia with Lewy bodies and dementia of Alzheimer’s type: a comparative neuropsychological study and literature review. J Neurol Neurosurg Psychiatry 70(2):149–156
Lang C, Balan M, Reischies F (2000) Recall and recognition in Huntington’s disease. Arch Clinical Neuorpsychol 15:361–371
Leiguarda R, Lees AJ, Merello M et al (1994) The nature of apraxia in corticobasal degeneration. J Neurol Neurosurg Psychiatry 57(4):455–459
Leiguarda R, Merello M, Balej J (2000) Apraxia in corticobasal degeneration. Adv Neurol 82:103–121
Leiguarda RC, Marsden CD (2000) Limbapraxias: higher-order disorders of sensorimotor integration. Brain 123 (Pt 5):860–879
Leiguarda RC, Merello M, Nouzeilles MI et al (2003) Limb-kinetic apraxia in corticobasal degeneration: clinical and kinematic features. Mov Disord 18(1):49–59
Leiguarda RC, Pramstaller PP, Merello M et al (1997) Apraxia in Parkinson’s disease, progressive supranuclear palsy, multiple system atrophy and neuroleptic-induced parkinsonism. Brain 120 (Pt 1):75–90
Lemiere J, Decruyenaere M, Evers-Kiebooms G et al (2002) Longitudinal study evaluating neuropsychological changes in so-called asymptomatic carriers of the Huntington’s disease mutation after 1 year. Acta Neurol Scand 106(3):131–141
Lemiere J, Decruyenaere M, Evers-Kiebooms G et al (2004) Cognitive changes in patients with Huntington’s disease (HD) and asymptomatic carriers of the HD mutation—a longitudinal followup study. J Neurol 251(8):935–942
Lemstra AW, Eikelenboom P, van Gool WA (2003) The cholinergic deficiency syndrome and its therapeutic implications. Gerontology 49(1):55–60
Lippa CF, Duda JE, Grossman M et al (2007) DLB and PDD boundary issues: diagnosis, treatment, molecular pathology, and biomarkers. Neurology 68(11):812–819
Lippa CF, McKeith I (2003) Dementia with Lewy bodies: improving diagnostic criteria. Neurology 60(10):1571–1572
Litvan I (1994) Cognitive disturbances in progressive supranuclear palsy. J Neural Transm Suppl 42:69–78
Litvan I, Agid Y, Calne D et al (1996) Clinical research criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome): report of the NINDS-SPSP international workshop. Neurology 47:1–9
Litvan I, Agid Y, Goetz C et al (1997) Accuracy of the clinical diagnosis of corticobasal degeneration: a clinicopathologic study. Neurology 48(1):119–125
Litvan I, Grafman J, Gomez C, Chase TN (1989) Memory impairments in patients with progressive supranuclear palsy. Arch Neurol 46:765–767
Magherini A, Litvan I (2005) Cognitive and behavioural aspects of PSP since Steele, Richardson and Olszewski’s description of PSP 40 years ago and Albert’s delineation of the subcortical dementia 30 years a go. Neurocase 11:250–62
McHugh PR, Folstein MF (1975) Psychiatric syndromes of Huntington’s chorea: a clinical and phenomenologic study. In Benson DF, Blumer D (eds) Psychiatric aspects of neurologic disease. Grune & Stratton Inc., New York
McKeith IG (2002) Dementia with Lewy bodies. Br J Psychiatry 180:144–147
McKeith IG, Dickson DW, Lowe J et al (2005) Diagnosis and management of dementia with Lewy bodies: third report of the DLB Consortium. Neurology 65(12):1863–1872
McKeith IG, Mintzer J, Aarsland D et al (2004) Dementia with Lewy bodies. Lancet Neurol 3:19–28
Meco G, Gasparini M, Doricchi F (1996) Attentional functions in multiple system atrophy and Parkinson’s disease. J Neurol Neurosurg Psychiatry 60(4):393–398
Mendez MF (2000) Corticobasal ganglionic degeneration with Balint’s syndrome. J Neuropsychiatry Clin Neurosci 12(2):273–275
Metzler-Baddeley C (2007) A review of cognitive impairments in dementia with Lewy bodies relative to Alzheimer’s disease and Parkinson’s disease with dementia. Cortex 43(5):583–600
Mondon K, Gochard A, Marqué A et al (2007) Visual recognition memory differentiates dementia with Lewy bodies and Parkinson’s disease dementia. J Neurol Neurosurg Psychiatry 78(7):738–741
Montoya A, Pelletier M, Menear M et al (2006a) Episodic memory impairment in Huntington’s disease: a meta-analysis. Neuropsychologia 44(10):1984–1994
Montoya A, Price BH, Menear M, Lepage M (2006b) Brain imaging and cognitive dysfunctions in Huntington’s disease. J Psychiatry Neurosci 31(1):21–29
Mosimann UP, Mather G, Wesnes KA et al (2004) Visual perception in Parkinson disease dementia and dementia with Lewy bodies. Neurology 63(11):2091–2096
Noe E, Marder K, Bell KL et al (2004) Comparison of dementia with Lewy bodies to Alzheimer’s disease and Parkinson’s disease with dementia. Mov Disord 19(1):60–67
Okuda B, Tachibana H, Kawabata K et al (2000) Cerebral blood flow in corticobasal degeneration and progressive supranuclear palsy. Alzheimer Dis Assoc Disord 14(1):46–52
Okuda B, Tanaka H, Kawabata K et al (2001) Truncal and limb apraxia in corticobasal degeneration. Mov Disord 16(4):760–762
Paviour DC, Price SL, Stevens JM et al (2005) Quantitative MRI measurement of superior cerebellar peduncle in progressive supranuclear palsy. Neurology 64(4):675–679
Peigneux P, Salmon E, Garraux G et al (2001) Neural and cognitive bases of upper limb apraxia in corticobasal degeneration. Neurology 57(7):1259–1268
Perriol MP, Dujardin K, Derambure P (2005) Disturbance of sensory filtering in dementia with Lewy bodies: comparison with Parkinson’s disease dementia and Alzheimer’s disease. J Neurol Neurosurg Psychiatry 76(1):106–108
Perry EK, Haroutunian V, Davis KL et al (1994) Neocortical cholinergic activities differentiate Lewy body dementia from classical Alzheimer’s disease. Neuroreport 5(7):747–749
Pharr V, Uttl B, Stark M et al (2001) Comparison of apraxia in corticobasal degeneration and progressive supranuclear palsy. Neurology 56(7):957–963
Pillon B, Blin J, Vidailhet M et al (1995) The neuropsychological pattern of corticobasal degeneration: comparison with progressive supranuclear palsy and Alzheimer’s disease. Neurology 45(8):1477–1483
Pillon B, Deweer B, Michon A et al (1994) Are explicit memory disorders of progressive supranuclear palsy related to damage to striato-frontal circuitsw? Comparison with Alzheimer’s, Parkinson’s and Hungtinton’s diseases. Neurology 44:1264–1270
Pillon B, Dubois B (2000) Memory and executive processes in corticobasal degeneration. Adv Neurol 82:91–101
Pillon B, Dubois B, Lhermitte F, Agid Y (1986) Heterogeneity of cognitive impairment in progressive supranuclear palsy, Parkinson’s disease, and Alzheimer’s disease. Neurology 36(9):1179–1185
Pillon B, Dubois B, Ploska A, Agid Y (1991) Severity and specificity of cognitive impairment in Alzheimer’s, Parkinson’s and Hungtinton’s diseases and progressive supranuclear palsy. Neurology 41(5):634–643
Pillon B, Gouider-Khouja N, Deweer B et al (1995) Neuropsychological pattern of striatonigral degeneration: comparison with Parkinson’s disease and progressive supranuclear palsy. J Neurol Neurosurg Psychiatry 58(2):174–179
Posner MI, Petersen SE (1990) The attention system of the human brain. Annu Rev Neurosci 13:25–42
Rafal RD (1992) Visually guided behavior. In Litvan I, Agid Y (eds) Progressive supranuclear palsy: clinical and research approaches. Oxford United Press, New York
Rafal RD, Posner MI, Friedman JH et al (1998) Orienting of visual attention in progressive supranuclear palsy. Brain 111 (Pt 2):267–280
Rebeiz JJ, Kolodny EH, Richardson EP Jr (1968) Corticodentatonigral degeneration with neuronal achromasia. Arch Neurol 18(1):20–33
Robbins TW, James M, Owen AM et al (1994) cognitive deficits in progressive supranuclear palsy, Parkinson’s disease, and multiple system atrophy in tests sensitive to frontal lobe dysfunction. J Neurol Neurosurg Psychiatry 57(1):79–88
Robinson G, Shallice T, Cipolotti L (2006) Dynamic aphasia in progressive supranuclear palsy: a deficit in generating a fluent sequence of novel thought. Neuropsychologia 44(8):1344–1360
Rosser A, Hodges JR (1994) Initial letter and semantic category fluency in Alzheimer’s disease, Huntington’s disease, and progressive supranuclear palsy. J Neurol Neurosurg Psychiatry 57(11):1389–1394
Salmon DP, Filoteo VJ (2007) Neuropsychology of cortical versus subcortical dementia syndromes. Semin Neurol 27:7–21
Salmon DP, Galasko D, Hansen LA et al (1996) Neuropsychological deficits associated with diffuse Lewy body disease. Brain Cogn 31(2):148–65
Silveri MC, Almonti S, Di Trapani G (1995) Unusual pattern of mental deterioration in a patient with signs of corticobasal degeneration. Ital J Neurol Sci. 16(8):572
Snowden JS, Craufurd D, Thompson J, Neary D (2002) Psychomotor, executive, and memory function in preclinical Huntington’s disease. J Clin Exp Neuropsychol 24(2):133–145
Soliveri P, Monza D, Paridi D et al (2000) Neuropsychological follow up in patients with Parkinson’s disease, striatonigral degeneration-type multisystem atrophy, and progressive supranuclear palsy. J Neurol Neurosurg Psychiatry 69(3):313–318
Steele JC, Richardson JC, Olszewski J (1964) Progressive supranuclear palsy. A heterogeneous degeneration involving the brain stem, basal ganglia and cerebellumwith vertical gaze and pseudobulbar palsy, nuchal dystonia and dementia. Arch Neurol 10:333–359
Tang-Wai DF, Josephs KA, Boeve BF et al (2003) Pathologically confirmed corticobasal degeneration presenting with visuospatial dysfunction. Neurology 61(8):1134–1135
Tekin S, Cummings J (2002) Frontal-subcortical neuronal circuits and clinical neuropsychiatry: an update. J Psychosom Res 53(2):647–654
Thieben MJ, Duggins AJ, Good CD et al (2002) The distribution of structural neuropathology in pre-clinical Huntington’s disease. Brain 125 (Pt 8):1815–1828
Verny M, Duyckaerts C, Agid Y, Hauw JJ (1996) The significance of cortical pathology in progressive supranuclear palsy: clinico-pathological data in 10 cases. Brain 119:1123–1136
Wakabayashi K, Hayashi M, Kakita A et al (1998) Accumulation pf alpha-sinuclein/NACP is a cytopathological feature common to Lewy body disease and multiple system atrophy. Acta Neuropathol 96:445–452
Wakabayashi K, Masumoto K, Takaiama K et al (1997) NACP a presynaptic protein, immunoreactivity in Lewy bodies in Parkinson’s disease. Neurosci Lett 239:45–48
Wenning GK, Colosimo C, Geser F, Poewe W (2004) Multiple system atrophy. Lancet Neurol 3(2):93–103
Zadikoff C, Lang AE (2005) Apraxia in movement disorders. Brain 128(Pt 7):1480–97
Zakzanis KK (1998) The subcortical dementia of Huntington’s disease. J Clin Exp Neuropsychol 20(4):565–78
Author information
Authors and Affiliations
Rights and permissions
Copyright information
© 2009 Springer-Verlag Italia
About this chapter
Cite this chapter
Perri, R., Carlesimo, G.A. (2009). I deficit cognitivi nelle sindromi extrapiramidali con demenza. In: Malattia di Parkinson e parkinsonismi. Springer, Milano. https://doi.org/10.1007/978-88-470-1490-9_4
Download citation
DOI: https://doi.org/10.1007/978-88-470-1490-9_4
Publisher Name: Springer, Milano
Print ISBN: 978-88-470-1489-3
Online ISBN: 978-88-470-1490-9
eBook Packages: Biomedical and Life SciencesBiomedical and Life Sciences (R0)