Article

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

ABSTRACT

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.
    Full-text · Article · Nov 2012 · BMC Neurology
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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.
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    ABSTRACT: Cognitive deficits associated with frontal lobe dysfunction can occur in amyotrophic lateral sclerosis (ALS), particularly in individuals with bulbar ALS who can also suffer pathologic emotional lability. Because frontal pathophysiology can alter emotional perception, we examined whether emotional perception deficits occur in ALS, and whether they are related to depressive or dementia symptoms. Bulbar ALS participants (n=13) and age-matched healthy normal controls (n=12) completed standardized tests of facial emotional and prosodic recognition, the Geriatric Depression Scale, and the Mini-Mental State Examination. Participants identified the basic emotion (happy, sad, angry, afraid, surprised, disgusted) that matched 39 facial expressions and 28 taped, semantically neutral, intoned sentences. ALS patients performed significantly worse than controls on facial recognition but not on prosodic recognition. Eight of 13 patients (62%) scored below the 95% confidence interval of controls in recognizing facial emotions, and 3 of these patients (23% overall) also scored lower in prosody recognition. Among the 8 patients with emotional perceptual impairment, one-half did not have depressive, or memory or cognitive symptoms on screening, whereas the remainder showed dementia symptoms alone or together with depressive symptoms. Emotional recognition deficits occur in bulbar ALS, particularly with emotional facial expressions, and can arise independent of depressive and dementia symptoms or comorbid with depression and dementia. These findings expand the scope of cognitive dysfunction detected in ALS, and bolsters the view of ALS as a multisystem disorder involving cognitive and also motor deficits.
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