Abstract
Presently, there is increasing interest in rare PSP (progressive supranuclear palsy) variants, including PSP-PGF (PSP-progressive gait freezing), PSP-PI (PSP-postural instability), PSP-OM (PSP-ocular motor dysfunction), PSP-C (PSP-predominant cerebellar ataxia), PSP-CBS (PSP-corticobasal syndrome), PSP-SL (PSP-speech/language disorders), and PSP-PLS (PSP-primary lateral sclerosis). Diagnosis of these subtypes is usually based on clinical symptoms, thus thorough examination with anamnesis remains a major challenge for clinicians. The individual phenotypes often show great similarity to various neurodegenerative diseases and other genetic, autoimmune, or infectious disorders, manifesting as PSP-mimicking syndromes. At the current stage of knowledge, it is not possible to isolate a specific marker to make a definite ante-mortem diagnosis. The purpose of this review is to discuss recent developments in rare PSP phenotypes and PSP-like syndromes.
Highlights
Progressive supranuclear palsy (PSP) has been known for more than half a century, but most of the reports are on PSP-Richardson syndrome (PSP-RS) and PSP-P (PSP-Predominant Parkinsonism) (Steele et al, 1964; Williams et al, 2005a)
This results in a higher sensitivity in the diagnosis of PSP compared with the National Institute of Neurological Disorders and Stroke and the Society for PSP (NINDS-SPSP) criteria issued in 1996 (87.9% for Movement Disorders Society (MDS) criteria versus 45.5% for NINDS-SPSP criteria) (Litvan et al, 1996; Ali et al, 2019)
Even more than 50% of patients with isolated nonfluent/agrammatic primary progressive aphasia or progressive apraxia of speech (AoS) develops other symptoms that could lead to a diagnosis of PSP-SL in subsequent years (Rohrer et al, 2010; Whitwell et al, 2019)
Summary
Manifestations of the Most Common Form of Atypical Parkinsonism. Patrycja Krzosek1*, Natalia Madetko, Anna Migda, Bartosz Migda, Dominika Jagus 4 and Piotr Alster. There is increasing interest in rare PSP (progressive supranuclear palsy) variants, including PSP-PGF (PSP-progressive gait freezing), PSP-PI (PSP-postural instability), PSP-OM (PSP-ocular motor dysfunction), PSP-C (PSP-predominant cerebellar ataxia), PSP-CBS (PSP-corticobasal syndrome), PSP-SL (PSPspeech/language disorders), and PSP-PLS (PSP-primary lateral sclerosis). Diagnosis of these subtypes is usually based on clinical symptoms, thorough examination with anamnesis remains a major challenge for clinicians. The individual phenotypes often show great similarity to various neurodegenerative diseases and other genetic, autoimmune, or infectious disorders, manifesting as PSP-mimicking syndromes. The purpose of this review is to discuss recent developments in rare PSP phenotypes and PSP-like syndromes
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