J Mov Disord.  2019 Sep;12(3):177-183. 10.14802/jmd.19015.

Clinical Milestones Preceding the Diagnosis of Multiple System Atrophy and Progressive Supranuclear Palsy: A Retrospective Cohort Study

• Wiblin L
• Durcan R
• Galna B
• Lee M
• Burn D

• KMID: 2464636
• DOI: http://doi.org/10.14802/jmd.19015

Abstract

OBJECTIVE
Multiple System Atrophy (MSA) and progressive supranuclear palsy (PSP) are rapidly progressive forms of degenerative Parkinsonism. The difficulties of diagnosing MSA and PSP in their early stages may lead to delayed referral to appropriate specialists and distress to patients, as well as delaying symptomatic treatment and participation in clinical trials. This work aimed to describe the symptoms that patients with MSA and PSP developed and plot their emergence relative to final diagnosis using a median onset in months.
METHODS
Forty-seven patients from the United Kingdom with MSA or PSP diagnosed by a movement disorder specialist were interviewed with carers or relatives to establish milestone onset. This was corroborated using clinical notes and letters.
RESULTS
In the MSA cohort (n = 23), autonomic symptoms (median 5.5 months before diagnosis) and falls (median 1 month before diagnosis) were the two clinical milestones which occurred before diagnosis. In the PSP cohort (n = 24), falling was the only milestone which occurred before diagnosis (median of 18.5 months).
CONCLUSION
This study shows that PSP patients experience falling more than a year and a half an average before receiving a diagnosis and although MSA patients also tended to fall, this was much closer to the time of diagnosis. Further work with larger cohorts may illustrate whether these preliminary findings can be generalised to guide diagnosis and management.

Keyword

Advance care planning; Delayed diagnosis; Multiple System Atrophy; Progressive supranuclear palsy

MeSH Terms

Accidental Falls
Advance Care Planning
Caregivers
Cohort Studies*
Delayed Diagnosis
Diagnosis*
Great Britain
Humans
Movement Disorders
Multiple System Atrophy*
Parkinsonian Disorders
Referral and Consultation
Retrospective Studies*
Specialization
Supranuclear Palsy, Progressive*