Title "Parkinson plus" sindrom Title (english) "Parkinson plus" syndrome Author Doroteja Lehpamer Mentor Srđana Telarović (mentor)Committee member Zdravka Poljaković (predsjednik povjerenstva)Committee member Mario Habek (član povjerenstva)Committee member Srđana Telarović (član povjerenstva)Granter University of Zagreb Defense date and country 2018-07-13, Croatia Scientific / art field, BIOMEDICINE AND HEALTHCARE Abstract „Parkinson plus“ sindrom označava neurodegenerativne bolesti koje se očituju kliničkim znakovima parkinsonizma (bradikinezija, tremor, rigiditet, posturalna nestabilnost), ali se po određenim kliničkim značajkama razlikuju od Parkinsonove bolesti. U novijoj literaturi se te bolesti još nazivaju i atipičnim parkinsonizmom. Po patohistološkom supstratu možemo ih podijeliti na α-sinukleionopatije i tauopatije. U α-sinukleionopatije ubrajamo multiplu sistemsku atrofiju i demenciju Lewyjevih tjelešaca. Obilježene su nakupljanjem α-sinukleina u oligodendrocitima, odnosno neuronima. Multipla sistemska atrofija obilježena je autonomnom disfunkcijom, parkinsonizmom ili cerebelarnim sindromom. Demencija Lewyjevih tjelešaca druga je demencija po učestalosti nakon Alzheimerove bolesti, a izazov je razlikovati je od demencije koja se javlja u Parkinsonovoj bolesti i Alzheimerovoj bolesti. U tauopatije su uključene progresivna supranuklearna paraliza, frontotemporalna lobarna degeneracija i kortikobazalna degeneracija, a zajedničko im je nakupljanje tau-proteina u obliku netopljivih neurofibrilarnih ili glijofibrilarnih čvorova. Treba napomenuti da frontotemporalna lobarna degeneracija može biti obilježena patološkim nakupljanjem drugih vrsta proteina različitih od tau-proteina. Progresivna supranuklearna paraliza očituje se aksijalnim parkinsonizmom, supranuklearnom paralizom pogleda i posturalnom nestabilnošću s padovima. Frontotemporalna lobarna degeneracija se prezentira kao bihevioralna varijanta frontotemporalne demencije i kao primarna progresivna afazija. Kortikobazalna degeneracija prezentira se progresivnim, asimetričnim parkinsonizmom koji ne pokazuje odgovor na levodopu, distonijom, mioklonusom i znakovima zahvaćanja moždane kore. Točan patofiziološki uzrok ovih promjena još uvijek nije poznat te se polažu nade da će se razumijevanjem tih procesa otvoriti mogućnosti za potencijalnu etiološku terapiju.
Abstract (english) ˝Parkinson plus˝ syndrome designates a group of neurodegenerative diseases which present with parkinsonian features (bradykinesia, tremor, rigidity, postural instability), but can be differentiated from Parkinson's disease by certain clinical features. In the recent literature these diseases are also called atypical parkinsonism. Considering their histopathological substrate they can be divided into α-synucleinopathies and tauopathies. α-synucleinopathies include multiple system atrophy and dementia with Lewy bodies. They are characterized by accumulation of α-synuclein in oligodendrocytes and neurons. Multiple system atrophy presents with autonomic dysfunction, parkinsonism, or cerebellar syndrome. Dementia with Lewy bodies is the second most common dementia after Alzheimer's disease and it is challenging to differentiate it from Parkinson's disease dementia and Alzheimer's disease. Tauopathies include progressive supranuclear palsy, frontotemporal lobar degeneration and corticobasal degeneration, all having in common the accumulation of tau protein, which then forms the insoluble neurofibrillary or gliofibrillary tangles. It is important to notice that frontotemporal lobar degeneration can be associated with accumulation of other proteins different from tau. Regarding the brain regions affected by pathological processes, a variety of complex clinical presentations are seen. Progressive supranuclear palsy presents with axial parkinsonism, supranuclear gaze palsy and postural instability associated with falls. Frontotemporal lobar degeneration presents as a behavioral variant of frontotemporal dementia and primary progressive aphasia. Corticobasal degeneration presents with progressive, asymmetric parkinsonism with poor response to levodopa, dystonia, myoclonus, and signs of cortical affection. The precise pathophysiological cause of these changes is still unknown and it is expected that with the understanding of these processes a new potential etiologic therapy will emerge.
Keywords
atipični parkinsonizam
multipla sistemna atrofija
demencija Lewyjevih tjelešaca
progresivna supranuklearna paraliza
frontotemporalna lobarna degeneracija
kortikobazalna degeneracija
Keywords (english)
atypical parkinsonism
multiple system atrophy
dementia with Lewy bodies
progressive supranuclear palsy
frontotemporal lobar degeneration
corticobasal degeneration
Language croatian URN:NBN urn:nbn:hr:105:347201 Study programme Title: Medicine Type of resource Text File origin Born digital Access conditions Open access Terms of use Created on 2019-01-08 08:33:44
