Reader Response: Timing and Impact of Psychiatric, Cognitive, and Motor Abnormalities in Huntington Disease
Ciaran M.Considine, Assistant Professor of Neurology, Vanderbilt University Medical Center
MaryEdmondson, Founding Medical Director of HD Reach, Vidant Behavioral Health
ErinStimming, Associate Professor of Neurology and Director of the Huntington Disease Center of Excellence, McGovern Medical School at University of Texas Health Science Center-Houston
ClaudiaTesta, Professor of Neurology and Founding Director of the Huntington Disease Center of Excellence, Virginia Commonwealth University
Karen E.Anderson, Professor of Neurology & Psychiatry, Director of the Huntington’s Disease Care, Education & Research Center at MedStar, Georgetown University Hospital
Daniel O.Claassen, Associate Professor of Neurology, Division Chief of Behavioral & Cognitive Neurology, Director of the Huntington Disease Cen, Vanderbilt University Medical Center
Submitted April 29, 2021
McAllister et al.1 concluded that psychiatric and cognitive symptoms should not be used for the clinical diagnosis of Huntington Disease (HD). We believe it is imperative for the HD community to embrace a diagnostic model that accounts for diverse non-motor symptoms, especially neuropsychological and psychiatric manifestations.
Using historical longitudinal registries that rely on motor-based diagnostic criteria to inform current best practices is self-limiting. These longitudinal databases select HD patients who convert to motor manifest disease, and poorly characterize the breadth of cognitive and psychiatric symptoms. Naturally, conclusions based on these registries will affirm the utility of motor manifestation and observe that cognitive or neuropsychiatric symptoms cannot lead to a viable alternative diagnostic pathway.
Recently, a position statement from the Movement Disorder Society2 contended that non-motor symptoms can be applied to the DSM-5 Minor and Major Neurocognitive Disorder paradigms. Psychiatric symptoms such as irritability, perseveration, delusions, and apathy, are helpful in this application. We also note that the Huntington Study Group’s Neuropsychology and Behavioral Working Groups will provide neuro-psychometric assessment guidelines to facilitate multidimensional diagnosis for clinical and research purposes. Expanding the application of diagnostic tools is necessary to improve clinical treatments for HD-related symptoms, and inform disease modifying research studies.3
Disclosure
The authors report no relevant disclosures. Contact journal@neurology.org for full disclosures.
References
McAllister B, Gusella JF, Landwehrmeyer GB, et al. Timing and Impact of Psychiatric, Cognitive, and Motor Abnormalities in Huntington Disease [published online ahead of print, 2021 Mar 25]. Neurology. 2021;10.1212/WNL.0000000000011893.
Ross CA, Reilmann R, Cardoso F, et al. Movement Disorder Society Task Force Viewpoint: Huntington's Disease Diagnostic Categories. Mov Disord Clin Pract. 2019;6(7):541-546. Published 2019 Aug 23. doi:10.1002/mdc3.12808
Biglan KM, Zhang Y, Long JD, et al. Refining the diagnosis of Huntington disease: the PREDICT-HD study. Front Aging Neurosci. 2013;5:12. Published 2013 Apr 2. doi:10.3389/fnagi.2013.00012
McAllister et al.1 concluded that psychiatric and cognitive symptoms should not be used for the clinical diagnosis of Huntington Disease (HD). We believe it is imperative for the HD community to embrace a diagnostic model that accounts for diverse non-motor symptoms, especially neuropsychological and psychiatric manifestations.
Using historical longitudinal registries that rely on motor-based diagnostic criteria to inform current best practices is self-limiting. These longitudinal databases select HD patients who convert to motor manifest disease, and poorly characterize the breadth of cognitive and psychiatric symptoms. Naturally, conclusions based on these registries will affirm the utility of motor manifestation and observe that cognitive or neuropsychiatric symptoms cannot lead to a viable alternative diagnostic pathway.
Recently, a position statement from the Movement Disorder Society2 contended that non-motor symptoms can be applied to the DSM-5 Minor and Major Neurocognitive Disorder paradigms. Psychiatric symptoms such as irritability, perseveration, delusions, and apathy, are helpful in this application. We also note that the Huntington Study Group’s Neuropsychology and Behavioral Working Groups will provide neuro-psychometric assessment guidelines to facilitate multidimensional diagnosis for clinical and research purposes. Expanding the application of diagnostic tools is necessary to improve clinical treatments for HD-related symptoms, and inform disease modifying research studies.3
Disclosure
The authors report no relevant disclosures. Contact journal@neurology.org for full disclosures.
References