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Neuropsychiatric Symptoms as a Reliable Phenomenology of Cerebellar Ataxia.

Authors
  • Kronemer, Sharif I1, 2
  • Slapik, Mitchell B3
  • Pietrowski, Jessica R3
  • Margron, Michael J4
  • Morgan, Owen P3
  • Bakker, Catherine C3
  • Rosenthal, Liana S3
  • Onyike, Chiadi U4
  • Marvel, Cherie L5, 6
  • 1 Interdepartmental Neuroscience Program, Yale University, New Haven, CT, USA.
  • 2 Neurology, Yale University, New Haven, CT, USA.
  • 3 Neurology, Johns Hopkins University, Baltimore, MD, USA.
  • 4 Psychiatry and Behavioral Sciences, Johns Hopkins University, Baltimore, MD, USA.
  • 5 Neurology, Johns Hopkins University, Baltimore, MD, USA. [email protected]
  • 6 Psychiatry and Behavioral Sciences, Johns Hopkins University, Baltimore, MD, USA. [email protected]
Type
Published Article
Journal
Cerebellum (London, England)
Publication Date
Apr 01, 2021
Volume
20
Issue
2
Pages
141–150
Identifiers
DOI: 10.1007/s12311-020-01195-7
PMID: 33000380
Source
Medline
Keywords
Language
English
License
Unknown

Abstract

While cerebellar ataxia (CA) is a neurodegenerative disease known for motor impairment, changes in mood have also been reported. A full account of neuropsychiatric symptomology in CA may guide improvements in treatment regimes, measure the presence and severity of sub-clinical neuropsychiatric disturbance symptomology in CA, and compare patient versus informant symptom recognition. Neuropsychiatric phenomena were gathered from CA patients with genetic and unknown etiologies and their informants (e.g., spouse or parent). Information was obtained from in-person interviews and the Center for Epidemiologic Studies Depression Scale. Responses were converted to the Neuropsychiatric Inventory-Questionnaire (NPI-Q) scores by consensus ratings. Patient NPI-Q scores were evaluated for symptom prevalence and severity relative to those obtained from healthy controls. Patient-informant NPI-Q score disagreements were evaluated. In this cohort, 95% of patients presented with at least one neuropsychiatric symptom and 51% of patients with three or more symptoms. The most common symptoms were anxiety, depression, nighttime behaviors (e.g., interrupted sleep), irritability, disinhibition, abnormal appetite, and agitation. The prevalence of these neuropsychiatric symptoms was uniform across patients with genetic versus unknown etiologies. Patient and informant symptom report disagreements reflected that patients noted sleep impairment and depression, while informants noted irritability and agitation. Neuropsychiatric disturbance is highly prevalent in patients with CA and contributes to the phenomenology of CA, regardless of etiology. Clinicians should monitor psychiatric health in their CA patients, considering that supplemental information from informants can help gauge the impact on family members and caregivers.

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