Flaherty-Craig C, Eslinger P, Stephens B, et al. A rapid screening battery to identify frontal dysfunction in patients with ALS

Department of Neurology, Penn State College of Medicine, Penn State Hershey Medical Center, Hershey, PA 17033, USA.
Neurology (Impact Factor: 8.29). 01/2007; 67(11):2070-2. DOI: 10.1212/01.wnl.0000247667.89251.43
Source: PubMed


We studied the relationship between verbal associative fluency, verbal abstract reasoning, and judgment in ALS using a 20-minute screening evaluation. Deficiencies in these measures were found in 20.0%, 18.6%, and 35.7% of patients with limb-onset ALS and in 37.5%, 25.0%, and 60.0% of patients with bulbar-onset ALS. This simple screen identifies deficits that affect discussions of treatment interventions and end-of-life issues.

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Available from: Helen Stephens, Sep 08, 2015
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    • "There are three principal circuits in frontal lobe for cognitive process, the dorsolateral, ventromedial and orbitofrontal [3]. Among them the dorsolateral-caudate nucleus circuit related to the executive functions has been the most investigated in ALS patients [4,5]. However, no widely accepted and sensitive screening tool is available for early detection because traditional neuropsychological examinations can have normal results in early-stage ALS patients. "
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    ABSTRACT: Background: Cognitive change is prevalent in patients with amyotrophic lateral sclerosis (ALS), but still lack a widely accepted and sensitive screening method. In this study, we try to find a sensitive screening battery for detecting subtle cognitive deficits in patients with ALS. Methods: Eighty consecutive ALS patients and 57 matched normal controls underwent the Mini-Mental Status Examination (MMSE), the verbal fluency test (VFT), the Stroop Color Word Interference Test (CWT), and the prospective memory (PM) tests, including event-based (EBPM) and time-based (TBPM). Results: The patients did not differ from the controls in the MMSE, the VFT and the CWT. By contrast, statistically significant differences were found in the PM tests (EBPM: P=0.043; TBPM: P<0.001). More interestingly, TBPM was more sensitive than EBPM in the early-phase patients. Conclusions: Prefrontal lobar dysfunction does exist among ALS patients and may spread from the medial to the lateral region. The PM tests seem more sensitive in ALS patients with frontotemporal dysfunction than are the classical cognitive measures.
    BMC Neurology 11/2012; 12(1):142. DOI:10.1186/1471-2377-12-142 · 2.04 Impact Factor
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    • "The ALS Cognitive Behavioral Screen, developed by Wooley and colleagues, includes a 15-item ALS specific behavioral questionnaire filled out by the caregiver, and an 8-item cognitive assessment of the patient that is estimated to take only 5 minutes; it has been validated [76]. The Penn State Screen Battery of Frontal and Temporal Dysfunction Syndromes takes approximately 20 minutes; and is currently being used in a nationwide study to establish its validity [77, 78]. The UCSF screen battery is the longest of the screens, taking approximately 45 minutes to complete. "
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    ABSTRACT: Though once believed to be a disease that was limited to the motor system, it is now apparent that amyotrophic lateral sclerosis (ALS) may be associated with cognitive changes in some patients. Changes are consistent with frontotemporal dysfunction, and may range from mild abnormalities only recognized with formal neuropsychological testing, to profound frontotemporal dementia (FTD). Executive function, behavior, and language are the most likely areas to be involved. Screening helpful in detecting abnormalities includes verbal or categorical fluency, behavioral inventories filled out by the caregiver, and evaluation for the presence of depression and pseudobulbar affect. Patients with cognitive dysfunction have shortened survival and may be less compliant with recommendations regarding use of feeding tubes and noninvasive ventilation. Evolving knowledge of genetic and pathological links between ALS and FTD has allowed us to better understand the overlapping spectrum of ALS and FTD.
    Neurology Research International 08/2012; 2012:806306. DOI:10.1155/2012/806306
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    ABSTRACT: We wished to determine whether a screening test battery for cognitive impairment can be given practicably in a busy multidisciplinary ALS clinic, and to assess initial test performance in a sequentially drawn ALS population. We administered a word generation task (letter fluency), the Frontal Behavioral Inventory (FBI), and the Beck Depression Inventory (BDI) to 49 ALS patients and their caregivers during a visit to our ALS clinic. We also computed Clinical Dementia Rating (CDR) scale and ALS Functional Rating Scale (ALSFRS-R) scores for patients. Pearson correlation coefficients and regression analyses assessed associations between outcome measures. The test battery took 30 min to administer. Word generation was associated with the FBI score (r = -0.36, p = 0.01), and time to ALS diagnosis (p = 0.01). Caregiver depressive symptoms (BDI) correlated with the FBI (r = 0.40, p = 0.005) and motor severity (r = -0.47, p<0.01) in patients. CDR scores were associated with behavioral abnormalities and lower ALSFRS-R scores. We concluded that a screen of cognition could be administered during multidisciplinary ALS clinics. Frontostriatal cognitive impairment may be associated with behavioral syndromes and more rapid forms of ALS. Behavioral and motor impairment is associated with depressive symptoms in caregivers. Studies with formal neuropsychological tests are needed to determine the sensitivity and specificity of the screen in ALS.
    Amyotrophic Lateral Sclerosis 07/2007; 8(6):362-5. DOI:10.1080/17482960701500817 · 2.37 Impact Factor
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