Kortikobazine degeneracija: literaturos ap�valga ir triju klinikiniu atveju pristatymas

Taraseviciute, E.; �e�eikaite, M.; Sakalauskaite-Juodeikiene, E.; Kaubrys, G.; Budrys, V.
June 2012
Neurologijos Seminarai;2012, Vol. 16 Issue 2, p109
Academic Journal
Corticobasal degeneration is a rare disease of un known etiology. It was described by Rebeiz and colleagues as "corticodentatonigral degeneration with neuronal achromasia". Clinically corticobasal degeneration manifests as parkinsonian syndrome with unilateral or asymmetric signs of extrapyramidal rigidity, myoclonus and apraxia, limb dyspraxia occurs in most subjects, and many exhibit alien hand phenomena. Corticobasal degeneration can cause nonparkinsonian clinical syndromes: the behavioural, psychoneurologic disorders, progressive nonfluent aphasia, dementia. Diagnostic criteria have been proposed, but none have been validated in a prospective study with post mortemverification, so the definite corticobasal degeneration is a neuropathologic diagnosis characterized by gliosis and large achromatic neurons that are distributed asymmetrically in frontal or parietal corticalareas and in subcorticalregions. Gross pathologic similarities between corticobasal degeneration, progressive supranuclear palsy and frontotemporal dementia have been seen, and a specific taupolymorphism has been shown to link progressive supranuclear palsy and corticobasal degeneration. Pharmacologic treatment of parkinsonian mo tor signs has shown a modest response rate to levodopa, clonazepam may be useful for treating myoclonus and dystonia, limited clinical experience with acetylcholinesterase inhibitors has been unrewarding. Wepresent three clinical cases of corticobasal de generation which were diagnosed in Vilnius university hospital Santari�kiu klinikos Neurology department in 2011.


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