Journal Article (Review Article) DZNE-2026-00991

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4-Repeat Tauopathien – Progressive Supranukleäre Blickparese und kortikobasale Degeneration | 4-Repeat Tauopathies: Progressive Supranuclear Palsy andCorticobasal Degeneration

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2026
Thieme Stuttgart [u.a.]

Fortschritte der Neurologie, Psychiatrie 94(9), 389 - 399 () [10.1055/a-2895-4480]

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Abstract: Progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) are primary tauopathies that are neuropathologically characterized by deposits of 4-repeat tau proteins. Both diseases lead to a progressive impairment of motor skills, balance, speech, cognition, and everyday functioning. While PSP is often associated with axially emphasized symptoms and characteristic midbrain atrophy, CBD usually presents with asymmetric frontoparietal atrophy with cortical and subcortical deficits. Due to overlapping clinical phenotypes, it is often difficult to distinguish the two diseases from other neurodegenerative diseases in everyday clinical practice. A distinction must be made between neuropathologically defined CBD and the clinical phenotype of corticobasal syndrome (CBS), which can also be caused by other pathologies, such as Alzheimer's disease. The diagnosis of PSP or CBD is made according to current clinical criteria, based on the evaluation of multiple functional domains and the severity of presenting symptoms. Disease-modifying therapies are not yet available. Treatment currently follows symptom-oriented approaches with the aim of maintaining the quality of life and independence of patients for as long as possible. This article provides an up-to-date overview of the diagnosis, clinical presentation, management strategies, and treatment options for individuals affected by PSP and CBD.

Keyword(s): Humans (MeSH) ; Supranuclear Palsy, Progressive: therapy (MeSH) ; Supranuclear Palsy, Progressive: diagnosis (MeSH) ; Supranuclear Palsy, Progressive: pathology (MeSH) ; Supranuclear Palsy, Progressive: genetics (MeSH) ; Corticobasal Degeneration: therapy (MeSH) ; Corticobasal Degeneration: diagnosis (MeSH) ; Corticobasal Degeneration: genetics (MeSH) ; Corticobasal Degeneration: pathology (MeSH) ; tau Proteins: genetics (MeSH) ; Tauopathies: therapy (MeSH) ; Tauopathies: diagnosis (MeSH) ; Tauopathies: pathology (MeSH) ; Tauopathies: genetics (MeSH) ; Diagnosis, Differential (MeSH) ; tau Proteins

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Contributing Institute(s):
  1. Clinical Research (Munich) (Clinical Research (Munich))
Research Program(s):
  1. 353 - Clinical and Health Care Research (POF4-353) (POF4-353)

Database coverage:
Medline ; BIOSIS Previews ; Biological Abstracts ; Clarivate Analytics Master Journal List ; Current Contents - Clinical Medicine ; Essential Science Indicators ; IF < 5 ; JCR ; NationallizenzNationallizenz ; SCOPUS ; Science Citation Index Expanded ; Web of Science Core Collection
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Institute Collections > M DZNE > M DZNE-Clinical Research (Munich)
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 Record created 2026-09-21, last modified 2026-09-21


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