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Progressive Supranuclear Palsy: Diagnosis and Management Review Progressive supranuclear palsy: Pract Neurol: first published as 10.1136/practneurol-2020-002794 on 2 July 2021. Downloaded from diagnosis and management James B Rowe,1,2 Negin Holland,1,3 Timothy Rittman1,3 1Department of Clinical ABSTRACT symptom.1 That is halfway through Neurosciences, University of Treating patients with progressive the illness. The lateness of diagnosis Cambridge, Cambridge, UK 2MRC Cognition and Brain supranuclear palsy (PSP) is both effective is multifactorial with delays seeking Sciences Unit, Cambridge, UK and rewarding. This review aims to share general practitioner advice, failure to 3 Cambridge University our experience in the proactive management recognise the significance of early symp- Hospitals NHS Foundation Trust, of PSP, considering the patient, the family Cambridge, UK toms and misdiagnosis as depression and the medical context in which the illness and/or Parkinson’s disease. Part of the Correspondence to unfolds. There are many opportunities problem is the terminology of ‘atypical Dr James B Rowe, University to assist your patients, ameliorate their of Cambridge Department Parkinsonism’. There is nothing ‘atyp- of Clinical Neurosciences, symptoms, reduce their risks and harm, ical’ about PSP: it is typical of PSP, and Cambridge, Cambridgeshire, UK; and guide them through the complex readily distinguished from Parkinson’s james. rowe@ mrc- cbu. cam. ac. uk medical, social and legal minefield that disease. For example, the limb signs in characterises life with chronic neurological Accepted 13 April 2021 PSP are symmetrical, without tremor illness. We summarise the challenges of early and rigidity is marked in the trunk and diagnosis, consider PSP mimics and the role neck, and minimal in the periphery— of investigations in excluding these, and the opposite of Parkinson’s disease on discuss the available pharmacological and all counts. non- pharmacological treatment strategies The diagnosis of PSP is usually not to tackle the common and challenging difficult with a few easy tips, set out symptoms of PSP. The best treatment in table 1. Many of these apply even will be patient centred and as part of a soon after symptom onset. Is there multidisciplinary team. a tendency to fall over easily, in the first year of symptoms? Yes for PSP, no for Parkinson’s disease. Are the eyes http://pn.bmj.com/ INTRODUCTION ‘staring’, with an odd, fixed smile or Progressive supranuclear palsy (PSP) is grimace? Yes for PSP, no for Parkinson’s not yet curable, but many aspects are disease. Do they walk with head up and certainly treatable. Indeed, the support forward (poetically called sniffing the and management of people with PSP morning breeze)? These features take can be both effective and rewarding. seconds to take in as the patient walks on September 24, 2021 by guest. Protected copyright. This article shares our experience in to the consulting room. its proactive management, considering Once in the consulting room, further the patient, the family and the medical differences are obvious. Typical PSP context in which the illness unfolds. speech is not the quiet, hypophonia Effective management starts with of Parkinson’s disease, but a more achieving an early, accurate diagnosis. We review new concepts in the diag- chaotic dysarthrophonia: distorted, nosis of PSP that have emerged over slow and effortful, sometimes inap- the last 10 years. We then consider propriately loud then indistinct, often the differential diagnosis and the role nasal in quality or low pitched with a of investigations. We go on to review ‘gravelly drawl’. Speech may be mixed © Author(s) (or their pharmacological treatment options in with laughter, perhaps inappropri- employer(s)) 2021. Re- use and non-pharmacological aspects to ately. There is much less spontaneous permitted under CC BY. speech—known as adynamic aphasia. Published by BMJ. effective holistic care, support and management. There may be changes in personality. To cite: Rowe JB, Holland N, In the history, families may have noticed Rittman T. Pract Neurol Epub ahead of print: [please include Diagnosis of PSP the person with PSP becoming apathetic Day Month Year]. doi:10.1136/ By the time of diagnosis, patients with (losing ‘get up and go’, zest, motivation, practneurol-2020-002794 PSP are typically 3 years from their first or interest); or becoming self- centred and Rowe JB, et al. Pract Neurol 2021;0:1–9. doi:10.1136/practneurol-2020-002794 1 Review stubborn. These reflect the common cognitive impair- Pract Neurol: first published as 10.1136/practneurol-2020-002794 on 2 July 2021. Downloaded from Table 1 Symptoms and signs of PSP ments of PSP, and its relationship to frontotemporal dementias.2 Despite a history of apathy, patients may Symptoms Signs also be impulsive, leaping up from their chair as you Cognitive change (apathy, Akinetic rigidity—neck and axial rigidity approach, or in anticipation of your request to stand, impulsivity) >limbs ‘sniffing morning breeze sign’ even if balance is poor. Impaired balance Slow saccades and ‘round the houses’ Look at their eyes. You may notice a blank, staring vertical saccades appearance, with few spontaneous blinks, a furrowed Early falls (with increased Vertical supranuclear gaze palsy fracture risk) brow and raised eyebrows. Look closely for small Blurry or double vision Frontalis overactivity, reduced blink, staring square wave jerks in neutral gaze. These are small expression (2°–4°), horizontal, brief (less than a second before Sleep difficulties Tendency to lose balance spontaneously or returning to target), and frequent (every few seconds). on the ‘pull test’ Early in the course of PSP, there can be a full range of Dysphagia (especially liquids) Uncontrolled decent into a chair eye movements, but a key feature for the diagnosis is Drooling, sialorrhoea Dystonia, cervical, axial >limbs the presence of either (1) restricted vertical saccadic Urinary urgency or Apraxia (CBS overlap) movements in a supranuclear pattern or (2) slow incontinence saccades (tip: compare vertical to horizontal saccades). Constipation Emotional lability (pseudobulbar affect) A valuable sign that is not in the formal diagnostic Depression or anxiety Reduced verbal fluency criteria, but which can be seen before restriction and Hyperphagia and change in Dysarthrophonia slowing, is a curvilinear path on downward saccades food preferences (‘round- the- houses’ sign, see figure 1). Slowing and Weight loss (with possible ‘round-the- houses’ are best seen with saccadic move- malnutrition) ments on command to a target. The supranuclear gaze CBS, corticobasal syndrome; PSP, progressive supranuclear palsy. palsy from which PSP is named causes restriction of voluntary eye movements in the vertical plane (up, down or both). The patient overcomes the restriction with Parkinson’s disease that I have seen?’ The answer, (at least in part) by reflexive eye movements when you, with PSP, will be ‘No’. the clinician, tilt the patient’s head with them fixating on a target (Tip: if the neck becomes too rigid to tilt New variants of PSP the head, try holding gently with both hands either When Steele et al,3 first described PSP, seven of their nine side, and wobbling it a few degrees left and right first, cases had dementia or severe cognitive and behavioural before a downward tilt). Other dementia syndromes change. Despite this, the illness became known as a can cause a ‘pseudo-gaze palsy’ due to ocular apraxia movement disorder, allied to Parkinson’s disease—a (eg, posterior cortical atrophy, Alzheimer’s disease problem exacerbated by terms like ‘Parkinson- plus’ http://pn.bmj.com/ and corticobasal degeneration, Huntington’s disease), and ‘atypical parkinsonism’. However, the importance in which a patient initially appears unable to look up of cognitive and behavioural change has increased or down to a target, while reflexive movements are with recent recognition that cognitive change is almost preserved with head tilting. However, with repeated attempts, it is possible in these conditions to elicit a fast and full saccade to the vertical target. on September 24, 2021 by guest. Protected copyright. Are they dopa- responsive or not dopa- responsive? One of the criteria for the diagnosis of PSP is that it is poorly responsive to levodopa. This is poor with respect to the level of response in Parkinson’s disease. But ~30% of patients report some benefit to levodopa or dopamine agonists. The effect is mild, and they may describe vague benefits in mobility, speech, ‘energy’ and other symptoms. Only conclude non- responsiveness if a high dose has been given (750–1000 mg daily). Note that PSP does not cause dyskinesia, or dopa-induced Figure 1 Left: ‘Round- the- houses’ sign, illustrated for a dyskinesia (unlike Parkinson’s disease). The dose can downward saccade. Note the lateral curvature of downward be rapidly increased over 6–8 weeks and decreased path of eye movement (yellow arrows). The velocity may also rapidly if not effective. be slow. Right: the face gives many clues to the diagnosis: So, PSP is not Parkinson’s disease! It is usually not as shown by this gentleman, there may be retrocollis, raised eyebrows and frontalis overactivity. People with PSP may have difficult to diagnose the classical presentation, known a rather fixed smile, with lips drawn back rather than up. The commonly as Richardson’s syndrome. Table 2 gives eyebrows sometimes appear knitted together (not shown). a list of distinguishing features, but just ask yourself, For video clips of other signs of PSP, refer to the ‘quick links to ‘does this person actually look like the other people further information section’. PSP, progressive supranuclear palsy. 2 Rowe JB, et al. Pract Neurol 2021;0:1–9. doi:10.1136/practneurol-2020-002794 Review case, be honest about your suspicion of PSP, initiate Pract Neurol: first published as 10.1136/practneurol-2020-002794 on 2 July 2021. Downloaded from Table 2 Key differences in symptoms and signs in PSP and Parkinson’s disease symptomatic treatment and follow- up.
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