Subject(s)
Brain/pathology , Charcot-Marie-Tooth Disease/complications , Genetic Diseases, X-Linked/genetics , Genetic Predisposition to Disease/genetics , Hereditary Central Nervous System Demyelinating Diseases/diagnosis , Hereditary Central Nervous System Demyelinating Diseases/genetics , Adult , Brain/physiopathology , Brain Stem/pathology , Brain Stem/physiopathology , Cerebellum/pathology , Cerebellum/physiopathology , Charcot-Marie-Tooth Disease/genetics , Connexins/genetics , DNA Mutational Analysis , Diagnosis, Differential , Disease Progression , Genetic Diseases, X-Linked/diagnosis , Genetic Diseases, X-Linked/physiopathology , Genetic Testing , Hereditary Central Nervous System Demyelinating Diseases/physiopathology , Humans , Magnetic Resonance Imaging , Male , Methylprednisolone/therapeutic use , Multiple Sclerosis/diagnosis , Multiple Sclerosis/physiopathology , Mutation/genetics , Oligoclonal Bands/cerebrospinal fluid , Recurrence , Treatment Failure , Gap Junction beta-1 ProteinABSTRACT
BACKGROUND: Understanding the determinants of quality of life (QoL) in amyotrophic lateral sclerosis (ALS) has become increasingly important with the recent emphasis on the comprehensive management of patients. OBJECTIVE: To evaluate the determinants of QoL in ALS using two scales with different theoretical constructs: the Schedule for the Evaluation of QoL-Direct Weighting (SEIQoL-DW), which evaluates subjective aspects of QoL, and the McGill QoL Questionnaire (MQOL), which evaluates both health related and non-health related factors of QoL. METHODS: Eighty consecutive patients with ALS underwent a battery of tests evaluating QoL and a series of physical, emotional, psychological, and socioeconomic predictor variables. A stepwise linear regression model was used to compare QoL scores and explicatory variables. RESULTS: SEIQoL-DW score was related to social support, depression, religiosity, and socioeconomic status. Total MQOL score was related to social support, socioeconomic status, and clinical status. MQOL single item score (MQOL-SIS) was related to social support, depression, social withdrawal, and socioeconomic status. SEIQoL-DW score was not related to total MQOL score. Conversely, a significant correlation was found between SEIQoL-DW and MQOL-SIS. CONCLUSIONS: With both QoL scales, the most important explicatory variable of QoL was the self perceived quality of social support. Physical status was not relevant in determining QoL. This study indicates that health related QoL measures are not adequate to assess QoL in patients with ALS, because their appreciation of QoL mainly relies on psychological, supportive, and spiritual factors. Therapeutic interventions should consider the psychological needs of patients and pay greater attention to caregivers' issues.
Subject(s)
Motor Neuron Disease/psychology , Quality of Life/psychology , Sick Role , Adult , Aged , Aged, 80 and over , Caregivers/psychology , Comprehensive Health Care , Cross-Sectional Studies , Female , Health Surveys , Humans , Italy , Male , Middle Aged , Motor Neuron Disease/epidemiology , Needs Assessment , Sickness Impact Profile , Social SupportABSTRACT
BACKGROUND: Enteral nutrition may be required in amyotrophic lateral sclerosis (ALS), and is usually achieved by percutaneous endoscopic gastrostomy (PEG). As PEG is not indicated in patients with severe respiratory impairment, an alternative is percutaneous radiological gastrostomy (PRG), involving air insufflation into the stomach under fluoroscopic guidance for tube insertion. OBJECTIVE: To evaluate the safety of PRG and its effect on survival and respiratory function in ALS patients with respiratory failure. METHODS: 25 consecutive ALS patients with severe dysphagia and forced vital capacity (FVC) <50% underwent PRG after October 2000. They were compared with 25 consecutive ALS patients with FVC <50% who underwent PEG before October 2000. Respiratory function was evaluated before and after the procedure. RESULTS: The two groups were similar for all relevant characteristics. PRG was successful in all cases, PEG in 23/25. One patient in each group died after the procedure. The mean survival time after the procedure was 204 days in the PRG group and 85 days in the PEG group (p<0.004). Respiratory function decreased more in the PEG group than in the PRG group (p<0.02). CONCLUSIONS: PRG appears to be safer than PEG in ALS patients with moderate or severe respiratory impairment, and is followed by a longer survival.
Subject(s)
Enteral Nutrition/methods , Fluoroscopy/methods , Gastrostomy/methods , Motor Neuron Disease/therapy , Pneumoradiography/methods , Punctures , Respiratory Insufficiency/therapy , Aged , Aged, 80 and over , Deglutition Disorders/therapy , Female , Follow-Up Studies , Humans , Male , Middle Aged , Motor Neuron Disease/diagnostic imaging , Motor Neuron Disease/mortality , Respiratory Insufficiency/diagnostic imaging , Respiratory Insufficiency/mortality , Survival Analysis , Treatment Outcome , Vital Capacity/physiologySubject(s)
Amyotrophic Lateral Sclerosis/epidemiology , Environmental Exposure/adverse effects , Spouses , Amyotrophic Lateral Sclerosis/blood , Amyotrophic Lateral Sclerosis/urine , Female , Humans , Male , Metals/analysis , Middle Aged , Soil/analysis , Spouses/statistics & numerical data , Water/analysisABSTRACT
The recognition of the anaplastic variant of oligodendroglioma is difficult, since it is not easy to identify histological prognostic factors. Among the latter, vascular productive changes have been inconsistently put in relation with survival. In 95 cases of operated oligodendrogliomas, endothelial cell hyperplasia, microvascular proliferations and capillary density were studied by histological and immunohistochemical methods. Capillary density was evaluated on CD31-stained sections by a grid of 100 squares placed in the ocular of the microscope. Statistical analysis was performed in order to compare these parameters with survival. A nodular growth pattern was observed more frequently among tumor grades 3-4 than among tumor grades 1-2. Endothelial cell hyperplasia was more frequent in nodular growth pattern, but it did not correlate with survival. The highest capillary density was found in nodular growth pattern, but it did not correlate with survival as well. Microvascular proliferations correlated with survival only in univariate, but not in multivariate analysis. Age, extent of surgical removal, year of surgery, post-operative Karnofsky score and MIB-1 LI remained associated with survival, as observed in a previous study.
