BACKGROUNDThe spectrum of abnormal behaviors in amyotrophic lateral sclerosis/motor neuron disease (ALS/MND) has been described, but its practical meaning, namely its impact on caregiver burden, has not been clearly documented in Chinese population. This study aimed to assess the distribution of abnormal behaviors in Chinese population, and to analyze the relationship between behavior changes and caregiver burden.

METHODSSixty-five patients with ALS/MND have been consecutively enrolled into registry platform of Peking Union Medical College Hospital. An investigation was performed to these patients and their caregivers using the revised ALS function rating scale, Frontal Behavioral Inventory-ALS version, the Frontal Assessment Battery, and the Caregiver Burden Inventory.

RESULTSTwenty-eight (43.1%) patients displayed abnormal behaviors of varying degrees, with one fulfilling the diagnostic criteria of frontotemporal lobe degeneration. Irritability, logopenia, and inflexibility ranked top 3 of abnormal behavior list. Correlation analysis revealed that the degree of behavioral change and frontal cognitive status were significantly associated with caregiver burden, with more extensive impact from disinhibitive behaviors. Analysis of covariance analysis showed that after associated factors were corrected, caregivers of patients with moderate to severe behavior change reported significantly heavier developmental burden, physical burden, and total burden than those with no behavioral change.

CONCLUSIONSNeurobehavioral symptoms could present in around 40% of Chinese patients with ALS/MND, and the distribution of these behaviors was also unique. Besides, abnormal behaviors were highly related to caregivers' burden.

Adult ; Aged ; Amyotrophic Lateral Sclerosis ; physiopathology ; psychology ; Behavioral Symptoms ; physiopathology ; psychology ; Caregivers ; psychology ; Female ; Humans ; Male ; Middle Aged ; Quality of Life

PURPOSE: The purpose of this study was to identify and describe phenomenological structures of the lived experience of struggling against an illness for patients with Amyotrophic Lateral Sclerosis (ALS). METHODS: The participants were 7 patients with ALS recruited by snowball sampling who agreed to participate in this research and could verbally communicated with the researcher. Data were collected by long term-repeated interviews with participants in their own homes. Data were analyzed using Colaizzi's method of phenomenology. RESULTS: Four categories were extracted as follows: 'Being seized with fear of death', 'Living a marginal life', 'Accepting hard fate', and 'Clinging to faint life'. Seven theme clusters were identified as: 'Wandering to find a healing method with ominous signs in the body', 'Having a diagnosis of ALS is like a bolt from the blue and struggling against illness with faint hope', 'Being forced out to the edge of life with anguish', 'Filling one's heart with hatred and longing toward becoming estranged from the world', 'Living with stigma as a stumbling block with bitter grief in one's heart', 'Accepting every things as one's fate with self controlled fear of death', and 'Attaching to desire to live'. CONCLUSION: The results of this study can be used to develop the programs to support patients with ALS and their family.
Adaptation, Psychological ; Adult ; Amyotrophic Lateral Sclerosis/*psychology ; Female ; Grief ; Humans ; Interviews as Topic ; Male ; Middle Aged ; Quality of Life ; Stress, Psychological

OBJECTIVE: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease with high morbidity and mortality. There are about 5%-15% of ALS patients combining with frontotemporal lobe degeneration (FTLD) at the same time and 50% of patients combing with cognitive function changes. The analysis of cortical thickness based on MRI is an important imaging method to evaluate brain structure. The aim of the study was to explore the changes of brain structure in ALS patients by cortical thickness analysis, and to explore the correlation between the brain structure and cognitive function. METHODS: In the study, 18 ALS patients treated in Department of Neurology, Peking University Third Hospital and 18 normal controls (age, gender and education level matched) were included. 3D magnetization prepared rapid gradient echo imaging (MPRAGE) sequence MRI was performed and the cortical thickness was analyzed. At the same time, all the ALS patients took neuropsychology assessments, including: mini-mental state examination (MMSE), verbal fluency test (VFT), Stroop color word test (SCWT), prospective memory (PM), emotional picture perception and recognition, and faux pas story test. RESULTS: After cognitive assessment, two ALS patients had cognitive impairment. One was in accordance with ALS-frontotemporal dementia (FTD) diagnosis and the other one was in accordance with ALS cognitive impairment (ALSci) diagnosis. In all the 18 ALS patients and 18 normal controls, the cortical thickness of the left medial orbitofrontal lobe and the medial temporal lobe were significantly reduced (P < 0.05) in ALS group by the vertex-wise comparison. Cortical thickness of the left entorhinal cortex, the left inferior temporal gyrus, the left medial orbitofrontal lobe and the left insular lobe was significantly reduced (P < 0.05) by the region-wise comparison. However, when only concluded the 16 ALS non-cognitive impairment patients, there was no significant difference between the two groups (P>0.05). There were correlations between the scores of prospective memory, emotional picture perception and recognition, faux pas story test and the cortical thickness of their corresponding regions (P < 0.05). CONCLUSION The cortical thickness of ALS patients are correlated with neuropsychological scores which may reflect the changes of cortical structure corresponding to the cognitive assessment, and may provide help for the early diagnosis of cognitive changes in ALS patients.
Humans ; Amyotrophic Lateral Sclerosis/diagnostic imaging* ; Neurodegenerative Diseases ; Frontotemporal Dementia/psychology* ; Neuropsychological Tests ; Cognitive Dysfunction/etiology* ; Magnetic Resonance Imaging/methods*

PURPOSE: The purpose of this cross-sectional study was to examine the relationship between characteristics of severe ALS patient-caregiver couples and health related quality of life (HRQoL) in family caregivers. METHODS: The participants in this study were 89 pairs of ALS patients using ventilators and a family caregiver. The characteristics of the ALS patients and caregivers, Korean-Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised, Zarit Burden Interview and SF-36 were measured in this study. The data were collected from August 2008 to April 2009. Descriptive statistics, Pearson correlation coefficients, and canonical correlation were used for data analysis. RESULTS: The physical component summary and mental component summary of the HRQoL score for family caregivers were 147.49+/-31.63 and 129.09+/-35.83, respectively. HRQoL for caregivers was related to characteristics of the ALS patient-caregiver couples, such as patient's gender, caregiver's age, gender, marital status, daily time spent in caregiving and burden with one significant canonical variable. The significant variate showed that the lower the age, the time spent in caregiving and the burden of caregivers, the higher the HRQoL of caregivers. CONCLUSION: The support systems for caregivers considering caregiver characteristics such as demographics and burden should be implemented to improve the HRQoL of caregivers.
Adult ; Aged ; Amyotrophic Lateral Sclerosis/*psychology ; Caregivers/*psychology ; Cross-Sectional Studies ; Dependency (Psychology) ; Female ; Humans ; Interviews as Topic ; Male ; Middle Aged ; *Quality of Life ; Questionnaires ; Ventilators, Mechanical

PURPOSE: The purpose of this study was to describe depression, caregiving burden and the correlation of the two variables in the families of patients with amyotrophic lateral sclerosis (ALS) and to clarify factors predicting caregiving burden. METHODS: A descriptive and cross-sectional study was conducted with 139 family members who provided care to patients with ALS. The characteristics of patients and families, Korean-Beck Depression Inventory (K-BDI), Korean version of Zarit Burden Interview (K-ZBI) and Korean-Amyotrophic Lateral Sclerosis Functional Rating Scale - Revised (K-ALSFRS-R) were used as study measures. RESULTS: The mean score for K-BDI was 19.39 out of 63 suggesting sub-clinical depression and 38.2% of the family members exhibited depression. The mean score for K-ZBI was 66.03 out of 88. The predictors for K-ZBI were K-BDI, age of family member, length of time spent per day in caring, relationship to patient and K-ALSFRS-R. CONCLUSION: The results of this study suggest that levels of depression and caregiving burden are high among family members caring for patients with ALS. As depression is associated with caregiving burden, screening and emotional supports should be provided to reduce the burden of care for these family. Support programs to alleviate the care burden are also needed, considering family demographics, time per day in caring giving and K-ALSFRS-R.
Adult ; Aged ; Amyotrophic Lateral Sclerosis/pathology ; Caregivers/*psychology ; Cross-Sectional Studies ; Depression/etiology/*psychology ; Family ; Female ; Humans ; Male ; Middle Aged ; Psychiatric Status Rating Scales ; Quality of Life ; Surveys and Questionnaires

The authors reviewed the medical records of 78 patients diagnosed as amyotrophic lateral sclerosis (ALS) at the Department of Neurology of Seoul National University Hospital from January 1980 to June 1986. The clinical features, serum CPK value, electromyographic findings and muscle biopsy findings were analysed. Results obtained were as follows: 1. The mean age of onset was 44.4 (17-68) which was about 10 years earlier than that in the reports from other countires. The sex ratio (male to female) was two to one (52:26). 2. The most common from was conventional type (48.7%), followed by pseudopolyneuritic type (30.8%) and bulbar type (20.5%). 3. Muscle weakness (100%), muscle atrophy (97%), hyperreflexia (94%), fasciculations (82%), bulbar signs (67%), frontal release signs (31%), Babinski signs (27%) and sensory deficits (5%) were observed. Four cases with sensory deficits proved that they were accompanied with carpal tunnel syndromes. 4. The mean interval from onset to generalization of symptoms was shorter in bulbar type (10.6 months) than in non-bulbar type (16.6 months), suggesting a more progressive course in the former. 5. Serum CPK value was checked in 62 cases. An increase up to four times the normal value was observed in 17 cases (27%). 6. EMG (N=73) revealed such abnormal findings in 70 cases (95.9%) as increased insertional activities, fibrillations, positive sharp waves, reduced interfernece patterns, fasciculations and giant motor unit potentials. No abnormal findings were found in three cases, however. 7. Muscle biopsy (N=61) showed neurogenic atrophy with various changes in 56 cases (92%), massive fatty infiltration in 4 cases and muscle fascia only in one case.
Age of Onset ; Amyotrophic Lateral Sclerosis* ; Atrophy ; Biopsy ; Carpal Tunnel Syndrome ; Fascia ; Fasciculation ; Generalization (Psychology) ; Humans ; Medical Records ; Muscle Weakness ; Muscular Atrophy ; Neurology ; Reference Values ; Reflex, Abnormal ; Reflex, Babinski ; Seoul ; Sex Ratio