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Progressive Supranuclear Palsy

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Movement Disorders Curricula

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Abstract

Progressive supranuclear palsy (PSP) is an adult-onset neurodegenerative disease, clinically characterized by prominent postural instability and falls, vertical supranuclear gaze palsy, and frontal-subcortical dementia. The etiology is still unknown but recent scientific advances have led to a consensus that the main pathogenetic event is the abnormal 4R tau deposition in the brainstem, basal ganglia, and neocortical areas. The clinical spectrum has been expanded and includes several clinical syndromes that can be underpinned by the pathological hallmark features of PSP. Attempts to identify a biomarker in the cerebrospinal fluid or a specific and sensitive neuroimaging technique have so far failed to contribute significantly to the diagnostic process. Symptomatic pharmacologic treatment of PSP is still unsatisfactory, and the effect of dopaminergic medication on the parkinsonian symptoms is limited. Although the first disease-modifying trials have failed to show any clinical benefit, they have signaled the beginning of a new era towards finding an effective treatment for this devastating disorder.

The original version of this chapter was revised. An erratum to this chapter can be found at DOI 10.1007/978-3-7091-1628-9_45

An erratum to this chapter can be found at http://dx.doi.org/10.1007/978-3-7091-1628-9_45

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Abbreviations

CBD:

Corticobasal degeneration

DATScan:

Dopamine transporter imaging

EIF2AK3:

Eukaryotic translation initiation factor 2-α kinase 3

ER:

Endoplasmic reticulum

FDG-PET:

Fluorodeoxyglucose positron emission tomography

GSK-3:

Glycogen synthase kinase 3

GWAS:

Genome-wide association study

IBZM:

123I-iodobenzamide

MAPT:

Microtubule-associated protein tau

MIBG:

123 I-meta-iodobenzylguanidine

MOBP:

Myelin-associated oligodendrocyte basic protein

MRS:

Magnetic resonance spectroscopy

MSA:

Multiple system atrophy

NFTs:

Neurofibrillary tangles

NINDS-SPSP:

National Institute of Neurological Disorders and Stroke and Society for Progressive Supranuclear Palsy

PAGF:

Pure akinesia with gait freezing

PD:

Parkinson’s disease

PEG:

Percutaneous endoscopic gastrostomy

PSP:

Progressive supranuclear palsy

SPECT:

Single-photon emission computed tomography

SSRIs:

Serotonin reuptake inhibitors

STX6:

Syntaxin 6

UPR:

Unfolded protein response

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Authors and Affiliations

  1. Department of Neurology, University Clinic, Philipps University Marburg, Baldingerstrasse, 35039, Germany

    Maria Stamelou MD, PhD & Wolfgang H. Oertel MD

  2. Movement Disorders Department, Hygeia Hospital, Erythrou Stavrou 4 & Kifisiaw, Marousi, Athens, 15123, Greece

    Maria Stamelou MD, PhD

  3. Second Department of Neurology, Kapodistrian University of Athens, Rimini 1, Athens, 12462, Greece

    Maria Stamelou MD, PhD

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  1. Maria Stamelou MD, PhD
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  2. Wolfgang H. Oertel MD
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Correspondence to Maria Stamelou MD, PhD .

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Editors and Affiliations

  1. Faculty of Medicine, Transilvania University, Brasov, Romania

    Cristian Falup-Pecurariu

  2. Laboratory of Clinical Pharmacology and Therapeutics, Faculty of Medicine, Lisbon, Portugal

    Joaquim Ferreira

  3. National Center of Epidemiology and CIBERNED, Carlos III Institute of Health, Madrid, Spain

    Pablo Martinez-Martin

  4. Centre of Excellence, King’s College London, National Parkinson Foundation Centre of Excellence King’s College, London, United Kingdom

    Kallol Ray Chaudhuri

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Stamelou, M., Oertel, W.H. (2017). Progressive Supranuclear Palsy. In: Falup-Pecurariu, C., Ferreira, J., Martinez-Martin, P., Chaudhuri, K. (eds) Movement Disorders Curricula. Springer, Vienna. https://doi.org/10.1007/978-3-7091-1628-9_18

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