Overview
Progressive supranuclear palsy (PSP) is a neurodegenerative disorder characterized by early postural instability, supranuclear gaze palsy, and cognitive decline, often mimicking other parkinsonian syndromes 14.Diagnosis
Key Diagnostic Criteria: Supranuclear vertical gaze palsy, postural instability, and cognitive impairment 14.
Recommended Tests: Pathological confirmation may be required; clinical diagnosis often relies on exclusion of other parkinsonian syndromes 4.
Downward Gaze Palsy: Highly specific but not always present; its absence does not rule out PSP 4.Management
First-Line Treatments: No specific disease-modifying therapy; management focuses on symptomatic relief 15.
Adjunctive Therapies:
- Thiamine Supplementation: Consider in patients with overlapping symptoms suggestive of Wernicke's encephalopathy, especially if thiamine deficiency is identified 1.
- Speech Therapy (LSVT®): Effective for improving vocal intensity and articulation in PSP patients 2.
- Electroconvulsive Therapy (ECT): May be considered for treatment-resistant depression without worsening neurological symptoms 5.
- Delayed Auditory Feedback (DAF): Can improve speech intelligibility and rate in patients with dysarthria 7.Special Populations
Elderly: Commonly affected; management focuses on supportive care and symptom management 156.
Comorbidities: Superimposed conditions like thiamine deficiency should be actively screened and treated 1.Key Recommendations
Screen for and treat thiamine deficiency in PSP patients presenting with overlapping symptoms of Wernicke's encephalopathy to potentially alleviate symptoms 1 (Evidence: Moderate).
Consider Lee Silverman Voice Treatment (LSVT®) for PSP patients experiencing speech difficulties to improve vocal volume and articulation 2 (Evidence: Moderate).
Evaluate and manage neuropsychiatric symptoms, including depression, with ECT if pharmacological treatments fail, noting no exacerbation of neurological symptoms 5 (Evidence: Weak).References
1 Prakash S, Joshi H, Patel J. Progressive supranuclear palsy responding to intravenous thiamine: superimposed Wernicke's encephalopathy?. BMJ case reports 2018. link
2 Sale P, Castiglioni D, De Pandis MF, Torti M, Dall'armi V, Radicati FG et al.. The Lee Silverman Voice Treatment (LSVT®) speech therapy in progressive supranuclear palsy. European journal of physical and rehabilitation medicine 2015. link
3 Suzuki K, Miyamoto T, Miyamoto M, Hirata K. The core body temperature rhythm is altered in progressive supranuclear palsy. Clinical autonomic research : official journal of the Clinical Autonomic Research Society 2009. link
4 Brusa A, Cammarata S. Frequency, distribution and characteristics of progressive supranuclear palsy in Italy: preliminary observations. Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology 2002. link
5 Netzel PJ, Sutor B. Electroconvulsive therapy-responsive depression in a patient with progressive supranuclear palsy. The journal of ECT 2001. link
6 Lee S. The neuropsychiatric evolution of a case of progressive supranuclear palsy. The British journal of psychiatry : the journal of mental science 1991. link
7 Hanson WR, Metter EJ. DAF as instrumental treatment for dysarthria in progressive supranuclear palsy: a case report. The Journal of speech and hearing disorders 1980. link