A rapid screening battery to identify frontal dysfunction in patients with ALS
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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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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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.Cognitive and Behavioral Neurology 07/2007; 20(2):79-82. DOI:10.1097/WNN.0b013e31804c700b · 1.14 Impact Factor
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ABSTRACT: Amyotrophic lateral sclerosis (ALS) is a motor neuron disease that has sporadic and inherited forms. ALS is the most common neurodegenerative disorder of young and middle-aged adults, and few treatments are available. Although the degeneration predominantly affects the motor system, cognitive and behavioural symptoms have been described for over a century, and there is evidence that ALS and frontotemporal dementia overlap clinically, radiologically, pathologically, and genetically. Cognitive decline in ALS is characterised by personality change, irritability, obsessions, poor insight, and pervasive deficits in frontal executive tests. This presentation is consistent with the changes to character, social conduct, and executive function in frontotemporal dementia. We highlight genetic, imaging, and neuropathological evidence that non-motor systems are affected in ALS and explain the importance of recent discoveries. We review studies of cognitive impairment in ALS and common neuropsychological test results. We also provide advice about clinical assessment of frontotemporal dysfunction in patients with ALS, and suggest future research. Understanding of cognitive impairment in ALS will improve care for patients and their families and provide valuable insights into the pathogenesis of neurodegeneration.The Lancet Neurology 12/2007; 6(11):994-1003. DOI:10.1016/S1474-4422(07)70265-X · 21.82 Impact Factor