Huntington Study Group's Neuropsychology Working Group: Implementing Non-Motor Diagnostic Criteria

Ciaran M Considine; M Agustina Rossetti; Victor A Del Bene; Kendra Anderson; Sharlet A Anderson; Andrea S Celka; Mary C Edmondson; Amelia L Nelson-Sheese; Adam Piccolino; Antonio L Teixeira; Julie C Stout

doi:10.1002/mdc3.13910

Huntington Study Group's Neuropsychology Working Group: Implementing Non-Motor Diagnostic Criteria

Mov Disord Clin Pract. 2023 Nov 8;10(12):1714-1724. doi: 10.1002/mdc3.13910. eCollection 2023 Dec.

Affiliations

¹ Department of Neurology Vanderbilt University School of Medicine Nashville Tennessee USA.
² Department of Neurology University of Virginia School of Medicine Charlottesville Virginia USA.
³ Department of Neurology University of Alabama at Birmingham Heersink School of Medicine Birmingham Alabama USA.
⁴ Department of Neurology, McGovern Medical School UT Health The University of Texas Health Science Center Houston Texas USA.
⁵ Department of Neurological Sciences Rush University Medical Center Chicago Illinois USA.
⁶ HD Reach Raleigh North Carolina USA.
⁷ Department of Neurological Sciences University of Nebraska Medical Center College of Medicine Omaha Nebraska USA.
⁸ Piccolino Psychological Services Burnsville Minnesota USA.
⁹ Turner Institute for Brain and Mental Health, and School of Psychological Science Monash University Melbourne Victoria Australia.

PMID: 38094638
PMCID: PMC10715363 (available on 2024-11-08)
DOI: 10.1002/mdc3.13910

Abstract

Background: The clinical diagnosis of manifest Huntington's disease (HD) relies on a high level of clinical confidence (99% confidence) of HD-consistent motor signs. Longitudinal data have reliably identified cognitive and behavioral dysfunction predating clinical motor diagnosis by up to 15 years. Reliance on motor signs to establish a diagnosis of HD increases risk of early misdiagnosis or delayed diagnosis. Clinical neuropsychologists are uniquely positioned to advise on the clinical application of the Movement Disorder Society Task Force's recently proposed non-motor diagnostic criteria for HD.

Objectives: To provide (1) a recommended clinical approach toward non-motor diagnostic criteria in persons with HD and facilitation of accurate diagnosis; (2) recommended practices for medical treatment providers to screen and longitudinally monitor non-motor signs of HD.

Methods: The Huntington Study Group re-established the Neuropsychology Working Group, then recruited a multi-disciplinary group of neuropsychologists, neurologists, and psychiatrists to conduct an unstructured literature review and discuss expert opinions on practice, to facilitate an informal consensus opinion to accomplish the objectives.

Results: The opinion and an example protocol for medical treatment providers to screen, monitor, and triage non-motor signs and symptoms of Huntington's disease is provided.

Conclusions: Clinical diagnosis of non-motor HD is empirically justified and clinically important. Screening and triage by non-neuropsychologist clinicians can aid in detecting and monitoring non-motor Huntington's disease manifestation. The Neuropsychology Working Group consensus advances good clinical practice, clinical research, and quality of life. A companion position paper presenting the details of our consensus opinion regarding evidence-based guidelines for neuropsychological practice is forthcoming.

Keywords: Huntington's disease; assessment; diagnosis; neuropsychology; non‐motor.

Publication types

Review