P36 THE ROLE OF FATIGUE AND PSYCHOSOCIAL PHENOMENA IN THE DETERMINATION OF QUALITY OF LIFE FOR PATIENTS WITH MOTOR NEURONE DISEASE
GIBBONS C1,2, THORNTON E2, EALING J3, SHAW P4, TALBOT K5, TENNANT A6, YOUNG C1
1The Walton Centre for Neurology and Neurosurgery, Liverpool, UK, 2University of Liverpool, Liverpool, UK, 3Hope Hospital, Salford, UK, 4Sheffield Institute of Translational Neuroscience, Sheffield, UK, 5John Radcliffe Hospital, Oxford, UK, 6University of Leeds, Leeds, UK
Email address for correspondence: [email protected]
Keywords: quality of life, fatigue, depression
Background: Quality of life (QoL) for patients with MND has been shown to be unrelated to functional impairment. Whilst some research has been undertaken to evaluate factors that impact upon patient quality of life, to date no research has evaluated the direct and indirect causal links between psychological factors in a structured model for MND.
Methods: One hundred and seven patients with MND completed a suite of questionnaires containing measures for fatigue, depression, anxiety, functional status, social withdrawal and quality of life. The fatigue, depression, anxiety, coping and social withdrawal scales were modified in order to satisfy the demands of the Rasch model on a separate sample of 298 patients with MND. Hypothesised causal relationships between the study variables were tested using structural equation modeling (SEM).
Results: The final model was shown to have excellent fit characteristics (χ2(5) = 6.06, p = 0.30; CFI = 0.99; GFI = 0.98; RMSEA = 0.045). Quality of life (QoL) was primarily driven by strong direct effects from depression (β = −0.47, p < 0.001) and social withdrawal (β = −0.34, p < 0.001) in addition to an indirect effect of coping (β = 0.35, p < 0.001), mediated primarily though anxiety (β = −0.39, p < 0.001) and depression (β = −0.36, p < 0.001). Fatigue exerted a strong direct relationship with anxiety, depression and social withdrawal (p < 0.001).
Discussion: This study highlights the importance of depressive symptomology and social withdrawal in the determination of patient QoL in MND. Coping was found to be a strong modifier of both depression and anxiety, and had a strong indirect effect upon QoL. Fatigue was shown to impact strongly upon anxiety, depression and social withdrawal although it did not elicit a significant direct effect upon QoL in this model.
P37 THE DEVELOPMENT AND VALIDATION OF A BRIEF SCREENING QUESTIONNAIRE FOR COGNITIVE IMPAIRMENT IN ALS
ELAMIN M1,2, BEDE P1, BEDE S1, PHUKAN J1, JORDAN N1,2, GALLAGHER L1,2, LYNCH C1, PENDER N2, HARDIMAN O1,2
1Trinity College Institute of Neuroscience, Dublin, Ireland, 2Beuamont Hospital, Dublin, Ireland
Email address for correspondence: [email protected]
Keywords: cognition, dementia, screening
Background: It is estimated that 25-50% of ALS patients develop cognitive impairment during the course of their illness. The clinical significance of this cognitive impairment is becoming increasingly apparent. Cognitive dysfunction in ALS patients has been linked to poor decision making, reduced compliance with medical interventions, and increased carer burden as well as shorter survival. Comprehensive neuropsychological assessments do not constitute a practical option in busy multidisciplinary clinics. There is an urgent need for a validated screening tool that can be used by clinicians in busy clinics to identify ALS patients with cognitive impairment.
Objective: To describe a brief cognitive questionnaire (the Brief Screening Questionnaire for ALS Patients, BSQ-ALS), based on detailed neuropsychological assessment of a population-based cohort of incident ALS patients.
Methods: The questionnaire was developed following detailed assessment of 160 incident patients with ALS. It takes 7-10 minutes and includes a brief assessment of executive, memory and language functions. Within three months of completing the questionnaire, patients undergo a comprehensive home-based neuropsychological and clinical assessment. Similar neuropsychological evaluations are undertaken in age, sex and education matched healthy controls.
Results: We present the rationale for the current design of the questionnaire. Evaluation of executive function includes three tasks including similarities, response inhibition and a three minute verbal fluency task. Several aspects of memory function are assessed including registration, recall and recognition. Language function evaluation includes noun and verb naming as well as tests of comprehension. We also present the proposed methodology for validation of the questionnaire using the formal neuropsychological battery as the golden standard. We also compare the clinical utility of the information obtained using the questionnaire to other cognitive questionnaires, and to data obtained using only the verbal fluency task only.
Conclusion: This study describes the clinical utility of a 10 minute cognitive screening questionnaire in a population based setting.
P38 Pragmatic language function in Motor Neurone Disease
FISHER F1, STAIOS M3, PAVLIS A2, MAULE R1, BRINKMANN S1, HOWE J1, REARDON K1, MATHERS S1
1Calvary Health Care Bethlehem, Melbourne, Victoria, Australia, 2Victoria University, Melbourne, Victoria, Australia, 3La Trobe University, Melbourne, Victoria, Australia
Email address for correspondence: [email protected]
Keywords: pragmatics, language, cognition
Background: Previous research had documented that persons with Motor Neurons Disease (PwMND) experience changes in recognizing the affective states of others. What is less clear is the extent that other aspects of social interaction are impaired by the disease.
Objectives: The current research study aimed to examine the profile of pragmatic language function in a sample of PwMND and healthy controls. It was anticipated that changes in aspects of pragmatic language would be apparent in PwMND.
Methods: Pragmatic language function was assessed in 31 persons with MND and 15 healthy controls, via speech pathologist-rated video-taped conversations. Nominated caregivers also completed questionnaires rating PwMND's overall pragmatic language functioning. Data were analyzed using non-parametric methods.
Results: Overall, PwMND were rated as more impaired in their pragmatic language function than healthy controls (p = 0.000). This difference was stable across both clinician and caregiver ratings. A profile of pragmatic difficulties was apparent in the MND group. PwMND were rated as having more frequent difficulties with providing ongoing contributions that maintained the flow of conversation. Conversational quality was abnormal, with content often sparse or verbose. Word finding difficulties also affected pragmatic communication. PwMND also showed reduced variation of communication style across conversational contexts. Consistent with clinical expectations, PwMND were rated as impaired on conversational prosody, fluency and voice quality. However, this is to be expected given the physical symptomology associated with MND.
Conclusion: In PwMND, pragmatic language function was significantly impaired, relative to controls. The profile of abnormal pragmatic language, in addition to well-recognized deficits in affect recognition, many have significant implications for interpersonal relationships and even everyday social interactions. These findings highlight the importance of pragmatic language assessment in tracking the development of non-motor symptoms in MND. It is recognised that the physical changes in PwMND do contribute to changes in pragmatic communication. However, physical changes alone did not explain the range of changes observed in this sample.
P39 A NATIONAL ALS MULTICENTER STUDY FOR THE CHARACTERIZATION OF EMERGING COGNITIVE AND BEHAVIORAL DECLINE
FLAHERTY-CRAIG C, BROTHERS A, SIMMONS Z
Penn State College of Medicine, Hershey, PA, USA
Email address for correspondence: [email protected]
Keywords: frontotemporal disease, language, behavior
Background: Current formulations of frontotemporal disease posit a synergism between the bilateral frontotemporal cortical regions in the emergence and progression of disease.
Objectives: Our objective was to investigate the pattern of emergence of cognitive and behavioral decline in ALS in a national sample, which would allow us to evaluate potential differences between rural, suburban, and urban population samples. Given that cognitive decline in ALS is generally associated with frontotemporal disease, characterized by disorders of language or behavior, we also sought a sample size large and diverse enough to examine male: female differences in language and behavior disorder incidence rates.
Methods: Designated personnel from 14 ALS Multidisciplinary clinics were trained by AB in standardized administration of the Penn State Brief Exam of Frontal and Temporal Dysfunction Syndromes (PSFTS). All patients evaluated met El-Escorial criteria from definite to possible ALS. Exclusion criteria included CNS co-morbidities potentially affecting cognition. Following exam administration, data were downloaded in a central depository for access by the Penn State research team.
Results: To date, data of 100 subjects from rural (N = 28), suburban (N = 60) and urban (N = 12)regions have been collected, with rural and suburban data analyzed. Male (N = 40) and female (N = 48) subjects were equivalent in ALS FRS-R scores (32.8/29.6) and FVC percent of predicted (75.8/68.6). Significant differences were detected for age (M = 56.5/61.7) (p = 0.037) and education (M = 15.9/14.5) (p = 0.008) with the male sample somewhat younger and higher educated. Incidence rates of cognitive deficiency or behavioral change were found to be equivalent across rural and suburban regions: letter fluency (LF) (23.5%), category fluency (CF) (22.5%), reading comprehension (13.4%), 2-D constructions (CON) (30.2%), Frontal Behavioral Inventory (FBI) findings approaching (> 20) (15.7%) or exceeding (> 27) (4.5%) clinical significance. Despite lower education levels, females scored higher than males in CF (p = 0.014). Correlations were found for CF but not LF with respect to CON for the total (p = 0.009) group, for males (p = 0.05) and females (p = 0.044), and for the suburban (p = 0.010), but not the rural (p = 0.402) group.
Discussion and conclusions: The relationship between CF and CON lends support to the concept of a synergism between bilateral temporal cortical processing in lexical access for categorical information; with female gender potentially associated with delayed emergence of cognitive decline in the presence of temporal dysfunction. Findings were consistent with our recent validation study applying Guilford's Structure of Intellect Theory (1), as well as recent imaging evidence of a continuum of extra-motor cerebral and cognitive change in ALS related to cortical - subcortical neural network processes (2).
References
- Flaherty-Craig C, Brothers A, Svoboda R, et al. Cognitive and Behavioral Neurology 2011;24(1).
- Abrahams S, Goldstein L, Suckling J, et al. Journal of Neurology 2005;252:321–31.
P40 MND AND FTD: CONTINUUM OR OVERLAP?
LILLO P1,2, SAVAGE S1, HODGES J1,2
1Neuroscience Research Australia, Sydney, NSW, Australia, 2University of New South Wales, Sydney, NSW, Australia
Email address for correspondence: [email protected]
Keywords: cognition, behaviour, frontotemporal dementia
Background: Although previously considered as a pure motor disorder, it has become clear that a significant proportion of patients with motor neurone disease (MND) develop cognitive impairment and behavioural changes, which may be severe enough to meet criteria for frontotemporal dementia (FTD). Few studies have examined cognitive and behavioural changes in unselected cases and there have been no comparisons of MND with FTD.
Objectives: To compare the cognitive and behavioural profile of patients with MND versus those with behavioural variant FTD (bvFTD).
Methods: Twenty consecutive patients with MND, 20 with bvFTD and 20 healthy controls completed a neuropsychological assessment including cognitive screening (Addenbroke's Cognitive Examination Revised-ACE-R), working memory (Digit span), inhibitory control (Hayling test), decision making (Iowa Gambling Test-IGT) and evaluation of neuropsychiatric symptoms (Cambridge Behavioural Inventory CBI-R). Groups were matched by age and education.
Results: Nine of the 20 (45%) MND patients had marked cognitive impairment on testing and 5 of them met criteria for FTD. Controls performed better than both MND and bvFTD groups on the ACE-R as well as a wide range of other tasks. After excluding the 9 cases with frank cognitive impairment, the cases with pure MND had a worse performance than controls on the Hayling test of inhibitory control. Lack of motivation was the most prominent neuropsychiatric feature in MND, occurring in both those with and without cognitive impairment.
Discussion and conclusion: Significant heterogeneity exists in the cognitive and behavioural profiles of MND patients. MND patients with clear cognitive impairments presented a similar pattern to those with bvFTD. A subset of MND patients apparently cognitively intact, had a subtle impairment on control inhibition and a significant lack of motivation, reinforcing the idea of a clinical continuum between MND and FTD.
P41 NEUROPSYCHOLOGICAL PROFILE OF COGNITIVE CHANGE IN A SAMPLE OF SLOVENIAN ALS PATIENTS: THE RESULTS OF A PRELIMINARY STUDY
STUKOVNIK V1, REPOVS G2,1, PODNAR S1, ZIDAR J1
1Institute of Clinical Neurophysiology, University Medical Ccentre Ljubljana, Ljubljana, Slovenia, 2Faculty of Arts, University of Ljubljana, Ljubljana, Slovenia
Email address for correspondence: [email protected]
Keywords: cognitive functions, executive functions, everyday life performance
Background: Traditionally, ALS has been viewed as a disease of the motor neuron system, characterized by degeneration of both upper and lower motor neurons, with no compromise to cognitive functions. Recent studies have shown that structural and pathological changes are not confined to motor areas and that these changes correlate with cognitive dysfunction. However, the nature and the extent of cognitive change in patients with ALS are not clearly defined yet.
Aim: The purpose of this study was to examine the profile and the extent of cognitive deficits in a sample of Slovenian ALS patients.
Participants and methods: A total of 22 non-demented ALS patients (median age = 59.5 years, interquartile range 51.7-64.0 years) and 21 age, sex and education matched healthy controls were compared on a comprehensive battery of neuropsychological tests of cognitive functions (executive, language, visuo-spatial functions and memory). A special emphasis was placed on executive functions. Standard neuropsychological tests were appropriately controlled for motor impairment in patients and all participants were evaluated also on ecologically valid motor-free test of cognitive functions. All patients were being treated at the ALS Centre at the Institute of Clinical Neurophysiology, University Clinical Centre Ljubljana, at the time of the inclusion into the study.
Results: The results show that participants with ALS are significantly impaired on tests of executive functions, language and memory but not on tests of visuo-spatial functions. The differences between ALS and control group are smaller after appropriately controlling for motor dysfunction, however, the robust differences still remain. According to Schulz criteria of cognitive dysfunction 15 patients (68%) can be classified as severely cognitively impaired (scores lower than 5th percentile on at least two measures in two different cognitive domains or on more than two measures within a single cognitive domain).
Conclusion: We conclude that the studied sample of ALS patients shows significant cognitive dysfunction. The results further show that motor dysfunction can present a significant confounding factor when using standard neuropsychological measures, but even when appropriately controlling for motor dysfunction, ALS patients show cognitive deficits, including those with possible effects on their everyday life performance. Further research work is needed to precisely map and confirm its exact nature and extent.
P42 NEUROPSYCHOLOGICAL STUDY OF PATIENTS WITH AMYOTROPHIC LATERAL SCLEROSIS/PARKINSONISM-DEMENTIA COMPLEX OF THE KII PENINSULA OF JAPAN
SHINDO A1, KUZUHARA S2, KOKUBO Y1
1Department of Neurology, Mie Graduate School of Medicine, Tsu, Mie, Japan, 2Department of Medical Welfare, Faculty of Health Science, Suzuka University of Medical Science, Suzuka, Mie, Japan
Email address for correspondence: [email protected]
Keywords: parkinsonism-dementia complex, neuropsychological study, Kii
Background: The Kii peninsula of Japan, together with Guam, has been one of the highest incidence foci of amyotrophic lateral sclerosis and parkinsonism-dementia complex (ALS/PDC) in the world. The purpose of this study is to clarify neuropsychological features of patients with ALS/PDC of the Kii peninsula (Kii ALS/PDC) of Japan.
Methods: Fourteen patients with Kii ALS/PDC (ALS 4, PDC 8 and parkinsonism-dementia ALS: P-D-ALS 2), 12 patients with Alzheimer disease (AD), 10 patients with progressive supranuclear palsy (PSP), 10 patients with frontotemporal lobar degeneration (FTLD) and 10 patients with dementia with Lewy body (DLB) were subjected to history taking about clinical symptoms, brain MRI, SPECT, and neuropsychological tests. The neuropsychological tests consisted of Mini Mental State Examination (MMSE), Raven's Colored Progressive Matrices (RCPM), verbal fluency, paired associate word-learning test (PAWLT) and the Frontal Assessment Battery (FAB).
Results: Two patients with Kii ALS had no cognitive dysfunction, and the rest of the patients with Kii ALS/PDC had dementia. Brain MRI showed atrophy of the frontal and temporal lobes, and SPECT revealed a decrease in CBF of the frontal and temporal lobes in all patients with cognitive dysfunction. Disorientation, difficulty of serial 7's and 3 word recall of MMSE, delayed reaction time of RCPM, difficulty of word recall of PAWLT and low score of FAB were recognized in Kii ALS/PDC patients with cognitive dysfunction. Personality change and aphasia were not present, but hallucination (5/12) and abulia (9/12) were recognized in Kii ALS/PDC with cognitive dysfunction.
Conclusions: The neuropsychological features of patients with Kii ALS/PDC were characterized by marked abulia and bradyphrenia, hallucination, disorientation and deterioration of recent memory. Kii ALS/PDC showed features characteristic of frontal-subcortical dementia.