Lamb, R;
Rohrer, JD;
Lees, AJ;
Morris, HR;
(2016)
Progressive Supranuclear Palsy and Corticobasal Degeneration: Pathophysiology and Treatment Options.
Current Treatment Options in Neurology
, 18
(9)
, Article 42. 10.1007/s11940-016-0422-5.
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Abstract
There are currently no disease-modifying treatments for progressive supranuclear palsy (PSP) or corticobasal degeneration (CBD), and no approved pharmacological or therapeutic treatments that are effective in controlling their symptoms. The use of most pharmacological treatment options are based on experience in other disorders or from non-randomized historical controls, case series, or expert opinion. Levodopa may provide some improvement in symptoms of Parkinsonism (specifically bradykinesia and rigidity) in PSP and CBD; however, evidence is conflicting and where present, benefits are often negligible and short lived. In fact, “poor” response to levodopa forms part of the NINDS-SPSP criteria for the diagnosis of PSP and consensus criteria for the diagnosis of CBD (Lang Mov Disord. 20 Suppl 1:S83–91, 2005; Litvan et al. Neurology. 48:119–25, 1997; Armstrong et al. Neurology. 80(5):496–503, 2013). There is some evidence that intrasalivery gland botulinum toxin is useful in managing problematic sialorrhea and that intramuscular botulinum toxin and baclofen are helpful in reducing dystonia, including blepharospasm. Benzodiazepines may also be useful in managing dystonia. Myoclonus may be managed using levetiracetam and benzodiazepines. Pharmacological agents licensed for Alzheimer’s disease (such as acetylcholinesterase inhibitors and N-Methyl-D-aspartate receptor antagonists) have been used off-label in PSP, CBD, and other tauopathies with the aim of improving cognition; however, there is limited evidence that they are effective and risk of adverse effects may outweigh benefits. The use of atypical antipsychotics for behavioural symptoms is not recommended in the elderly or those with demetia associated conditions and most antipsychotics will worsen Parkinsonism. Antidepressants may be useful for behavioral symptoms and depression but are often poorly tolerated due to adverse effects. In the absence of an effective drug treatment to target the underlying cause of CBD and PSP, management should focus on optimizing quality of life, relieving symptoms and assisting patients with their activities of daily living (ADL). Patients should be managed by a multidisciplinary team consisting of neurologists, physiotherapists (PT), occupational therapists (OT), speech and language therapists (SALT), dieticians, ophthalmologists, psychologists, and palliative care specialists.
| Type: | Article |
|---|---|
| Title: | Progressive Supranuclear Palsy and Corticobasal Degeneration: Pathophysiology and Treatment Options |
| Open access status: | An open access version is available from UCL Discovery |
| DOI: | 10.1007/s11940-016-0422-5 |
| Publisher version: | http://dx.doi.org/10.1007/s11940-016-0422-5 |
| Language: | English |
| Additional information: | The Author(s) 2016 Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. |
| Keywords: | Science & Technology, Life Sciences & Biomedicine, Clinical Neurology, Neurosciences & Neurology, Atypical Parkinsonism, Progressive supranuclear palsy, Steele-Richardson-Olszewski syndrome, Richardson's syndrome, Corticobasal degeneration, Corticobasal syndrome, Movement disorders, Neurodegenerative diseases, Parkinson-plus syndromes, Treatment, Tauopathies, Tau protein, FRONTOTEMPORAL DEMENTIA, PARKINSONS-DISEASE, CONTROLLED-TRIAL, BOTULINUM TOXIN, DOUBLE-BLIND, PHARMACOLOGICAL THERAPY, ALZHEIMERS-DISEASE, LEXICAL RETRIEVAL, SYMPTOMS, APHASIA |
| UCL classification: | UCL UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology > Clinical and Movement Neurosciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology > Neurodegenerative Diseases |
| URI: | https://discovery.ucl.ac.uk/id/eprint/1514063 |
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