ABSTRACT
OBJECTIVES: To describe the characteristics of patients receiving a clinical referral for neuropsychological evaluation in two Huntington's Disease Society of America Centers of Excellence (HDSA COE). In this exploratory pilot study, we used an empirically supported clinical neuropsychological battery to assess differences in cognitive performance between premanifest and manifest HD patient groups (compared with each other and normative expectations). METHOD: Clinical data from 76 adult genetically confirmed patients referred for neuropsychological evaluations was retrospectively collected from two HDSA COEs. ANOVA and Chi-square tests were used to compare variables between pre-manifest (n = 14) and manifest (n = 62) groups for demographic, cognitive, neuropsychiatric, and disease severity variables. RESULTS: Our clinics serviced a disproportionate number of motor manifest patients. Six measures were excluded from analyses due to infrequent administration. The full WAIS-IV Digit Span was disproportionately administered to the manifest group. The premanifest group showed stronger cognitive performance with effect sizes in the large range on subtests of the WAIS-IV Digit Span, HVLT-R, SDMT, and verbal fluency. CONCLUSIONS: This is the first study to assess an empirically supported neuropsychological research battery in a clinical setting with a relatively large sample size given the rarity of HD. The battery adequately captured areas of impairment across the disease spectrum. Application of the current battery with larger premanifest samples is warranted.
Subject(s)
Huntington Disease , Adult , Humans , Huntington Disease/complications , Huntington Disease/psychology , Pilot Projects , Retrospective Studies , Neuropsychological TestsABSTRACT
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.
ABSTRACT
Hepatic encephalopathy (HE) is a consequence of liver disease and often diagnosed via psychometric testing. With inpatients, the Montreal Cognitive Assessment (MoCA) may be used as part of cognitive screening for transplant candidacy. However, the MoCA was developed to detect mild cognitive impairment in aging populations and its psychometric properties in inpatients with liver disease have not been determined. Retrospective chart review identified inpatient liver transplant candidates who were administered a MoCA as part of their neuropsychological screening and had either no cognitive dysfunction or a diagnosis of HE made by a neuropsychologist (n = 57, mean age = 48.8 ± 12.6 years). Psychometric analyses were conducted and regression analysis was performed to determine the predictive value of different variables on total MoCA scores. Internal consistency of MoCA domain scores was good (α = 0.80). Significant inverse relationships were found with Trail Making Test, Parts A and B (r's = -0.43 and -0.71, respectively). A cutoff score of 24 or below had the best sensitivity (0.72) and specificity (0.77) for identifying those with a diagnosis of HE. Increasing age and the presence of altered mental status were the strongest predictors of lower MoCA scores (both p's < 0.05, ηp2 = 0.10-0.14). The MoCA is appropriate to use with inpatient liver transplant candidates, with a cutoff of 24 or below to detect abnormal cognition. In addition to the clinical interview and other neuropsychological tests (including, but not limited to, the Trail Making Test, Parts A and B), low MoCA scores can help determine the presence of HE.
ABSTRACT
Following mild traumatic brain injury (mTBI), one or more symptoms commonly occur that are known as the postconcussion syndrome (PCS). While PCS typically resolves within a few weeks of injury for most concussed patients, some patients have a more prolonged or otherwise adverse recovery course. There is relatively little systematic research on the treatment of PCS. This report offers strategies for nonpharmacological treatment of PCS during the acute, subacute, and chronic stages of recovery following mTBI. The treatment strategies are supported by clinical consensus and the limited evidence-based research wherever possible. Core treatment strategies emphasize (1) education about mTBI, PCS, and the natural recovery course, (2) reassurance of a good outcome, (3) reduction in activity level and refrain from hazardous behaviors during the acute phase, (4) gradual return to lifestyle activities as symptoms permit, (5) careful monitoring and early intervention for adverse emotional responses, (6) symptom-specific treatment when needed, and (7) ready access to providers during acute and subacute recovery periods.
