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Measuring outcomes for neurological disorders: a review of disease-specific health status instruments for three degenerative neurological conditions.

Health-related quality-of-life measures have been increasingly used in research into neurological disorders in recent years. The aim of this paper is to provide an objective appraisal of the evidence in regard to disease-specific quality-of-life measures used in research on health interventions for three degenerative neurological disorders: multiple sclerosis, motor neurone disease/amyotrophic lateral sclerosis and Parkinson's disease. A comprehensive search strategy was developed to include nine relevant electronic databases. Only studies pertaining to patient-based outcome measurements in multiple sclerosis, motor neurone disease and Parkinson's disease were included. We identified 76 eligible studies. As studies consisted of descriptive and cross-sectional survey study designs, results were reported qualitatively rather than in the form of a meta-analysis. Four disease-specific measures were found for Parkinson's disease, 11 for multiple sclerosis and one for motor neurone disease. We conclude that health-related quality-of-life measures are useful in assessing the impact of treatments and interventions for neurological disorders. However, further research is needed on the development of instruments using psychometric methods and on the validation, utilization and responsiveness of instruments to change.

Health Status↗

A review of neurological disorders presenting at a paediatric neurology clinic and response to anticonvulsant therapy in Gambian children.

A clinical review of 128 children presenting at the paediatric neurology clinic of the Royal Victoria Hospital, The Gambia was conducted between July 2001 and January 2002. The aim was to assess the spectrum and burden of neurological disease presenting at this tertiary-level clinic and to evaluate the effectiveness of anticonvulsant therapy. The principal clinical diagnoses most frequently made were seizures (57%), neuromotor problems (15%) and developmental delay (11%). Many children also had associated developmental delay or learning difficulties (55%), speech and language problems (42%) and physical disability (36%). Although 65% were considered to need assistance in the community, only 11% were receiving such help. There were 73 children with seizures, 80% of whom had a greater than 50% reduction in seizure frequency following the start of simple anticonvulsant medication. Of the children who had experienced more than 100 convulsions during the previous year, only 50% responded similarly to treatment. This review emphasises the need for improved community services and accessible medical care for children with neurological problems in West Africa.

Adolescent↗

Use of the Children's Orientation and Amnesia Test at hospital discharge for children with neurological and non-neurological traumatic injuries.

The measurement of post-traumatic amnesia in children is a common clinical index of the extent of brain injury. The only published paediatric test, the Children's Orientation and Amnesia Test (COAT), has been standardized on a sample of children in a school setting who had not been injured or hospitalized. Two questions arise in the use of this test: are the published norms valid for hospitalized children with non-neurological trauma? and does the COAT offer better discriminative power or clinical utility over the adult test, the Galveston Orientation and Amnesia Test (GOAT)? This paper describes scores on the COAT for 28 children hospitalized with non-neurological injuries and compares them with the published scores. The hospitalized children scored within normal limits when compared with the school sample. In addition, 23 children with neurological traumatic injuries were given both the COAT and the GOAT. Failure rate was higher for the COAT. Some implications for the use of the COAT in paediatric trauma are discussed.

Adolescent↗

[Neurological complications of non neurological disorders: from protagonism to comorbidity].

INTRODUCTION: The development of scientific advances, constantly opening new frontiers of knowledge, leads to increasing complexity and specialization. DEVELOPMENT: The conflict between the never ending medical specialization and the opposite tendency, which is based mostly on economic grounds, prevents the acquisition of new knowledge across different disorders and diseases. Considering the brain as a complex adaptative system, it follows that in order to understand its pathogenic mechanisms and to design new therapies, the study of the interrelation of all parts concerned must be undertaken. Such approach requires the use of increasingly complex degrees of knowledge, covering different fields and disciplines. A first step might be the study of neurological complications of non neurological disorders. The former should leave its principal status in order to be studied as a consequence of the latter by disconcerted neurologists. The neurological complications of systemic cancer, congenital cardiopathy and autoinmune diseases are good examples of the need for a multidisciplinary approach to achieve a satisfactory capacity of diagnosis and treatment.

Humans↗

[Intractable neurological diseases and neurology].

In intractable neurological diseases, there are often no appropriate treatment methods even after admission and the course is frequently chronic. Therefore treatment at home is a major choice. In particular, in Parkinson's disease and amyotrophic lateral sclerosis, care at home while symptoms are stable is appropriate in terms of extension of ADL and the QOL of the patient and family. We have performed continuous treatment at home according to our plan for the previous 9 years. This treatment was favorably accepted by the patient and family without major problems. It is important to organize network to support patient with intractable neurological diseases. By virtue of the care insurance system established in 2000, services of medical care, health and welfare are being collectively provided at present. But, there still remain many issues awaiting solution on the support to advanced intractable neurological diseases. A pending problem about suction of sputa for the ALS patients serving at home are being allowed to non-medical profession like home helper.

Activities of Daily Living↗

[The phlebologist confronted with the neurologic foot. Phlebologic aspects of the neurologic foot].

Vascular complications are frequent in paralyzing neurologic disorders, particularly in peripheral neurologic diseases: edema of the declive, cold feet, acrocyanosis, increased sudation. An enlarged leg in a paralyzed subject presenting frequently reduced sensitivity may indicate phlebitis, but also hematoma of an undetected fracture, or osteoarthropathy. Trophic disorders, such as edema, ulcerative hyperkeratosis and subcutaneous hematoma, are sometimes indicative of an unrecognized peripheral neuropathy, notably diabetic. Sclerous complications of venous impairment, or trophic disorders in algesic dystrophy, may result in incorrect diagnosis of a neurologic disorder. In practical therapeutic terms, it is necessary to emphasize the value of elastic retention, attentive pedicure and flexible and shock-absorbing footwear.

Cyanosis↗

[Stroke in the records of the Department of Neurology, Silesian Medical Academy in Katowice and Zabre as well as the Neurology Department of the 8th Municipal Hospital in Katowice in the years 1970-1974].

The authors analysed the clinical material of acute vascular diseases of the central nervous system treated at the Department of Neurology, Silesian Medical Academy and Neurological Department of the 8th Municipal Hospital providing in the years 1970-1974 medical care for the population of two greatest cities of the Upper Silesian Industrial Region in cases of sudden neurological diseases. Cerebrovascular strokes accounted for 14.7% of all hospitalized cases. The mean annual index of admissions for these departments was 52.5 per 100 000 population. Cerebral haemorrhages were diagnosed in 28% strokes, they were somewhat more frequent in women, especially in oldest age. Subarachnoideal haemorrhages were observed in 9.3% of strokes, more frequently in the age range from 41 to 60 years, and more frequently in middle aged men. Thrombotic brain softening accounted for 37.1% of cases, more frequently in men aged 51-70 years. Similarly, transient cerebral ischemia recognized in 13.9% of cases was more frequent in men. On the other hand, cerebral arterial embolism diagnosed in 11.7% of cases occurred usually in women aged over 60 years. In March and April the number of patients admitted with cerebral haemorrhages was slightly higher, while in April, May and June cases of thrombotic encephalomalacia were more frequent.

Cerebral Hemorrhage↗

The neurological information network of the National Institute of Neurological Diseases and Blindness.

The National Institute of Neurological Diseases and Blindness (NINDB) has the responsibility of trying to alleviate the morbid and mortal effects of all the diseases of the nervous system and the special senses including speech, hearing, and vision. The information problem facing the NINDB is only a fraction of the total information problem of the biomedical community, but it is probably representative. The NINDB information audience has a core of about 19,000 persons, 10 percent of whom are nonclinical research scientists; 10 percent of whom are engaged in patient care, teaching, and some research; 12 percent in training (post doctoral); and 68 percent chiefly involved in the care of patients. In addition, there is an unknown number of other interested scientists. Fifty to eighty thousand journal articles, an unknown number of technical reports, and other items produced each year are potentially valuable to this group. The exact needs of each subgroup must be identified and ways developed to deliver the required information quickly, accurately, and in an easily useable form. NINDB has undertaken a large information program as a service to the biomedical community to help cope with these information problems. This program is to serve the needs of the practicing physician, the research scientist, and NINDB program analysis and planning. The Neurological Information Network is the core effort of the program.The Neurological Information Network envisions large units incorporating both information storage and retrieval and information analysis. These will be in universities with strong biomedical libraries where there are ongoing research programs in some area relevant to NINDB responsibilities. These units are to be integrated and will interact with each other and with the National Library of Medicine to avoid duplication of work. In addition to their own information analysis activities, the major documentation centers will be able to support information analysis satellites at other research centers where specific categorical research programs are underway.

Blindness↗

[A nationwide survey on paraneoplastic neurological syndromes. Project group on paraneoplastic neurological syndromes, Neuroimmunological Disease Research Committee, the Ministry of Health and Welfare, Japan].

A nationwide survey on paraneoplastic neurological syndrome was carried out using enquiry sheets for neurologists in hospitals to examine the approximate number of patients, clinical features, characteristic anti-neuronal antibodies, accompanying malignancy and current therapies of these syndromes. In the present survey, 159 case records of this syndrome were accumulated. The major clinical variations were as follows: sensory neuropathy (54 cases; 34%), Lambert-Eaton myasthenic syndrome (45 cases; 28%) including 5 cases complicated with subacute cerebellar degeneration (PCD), PCD (40 cases; 25%) and limbic encephalitis (8 cases; 5%). Neurological symptoms preceded diagnosis of malignancy in over 83% of the total cases of these four major clinical variations in which tumors were verified. The detection of anti-neuronal antibodies, which are characteristic of some clinical types of this syndrome, is very important for early diagnosis and treatment for the neurological symptoms and underlying malignancy.

Adolescent↗

[Item response theory and its application in neurology. Measurement of activity limitations in neurologic patients].

This article is concerned with the measurement of activity limitations in neurologic patients and with the application of the item response theory (IRT), especially the Rasch analysis, in analyzing activity ratings. Activity limitations of 166 patients with different neurologic disorders (e.g., stroke, traumatic brain injury) were assessed with the Functional Independence Measure (FIM) during their stay in a rehabilitation hospital. Data analysis was performed with the Rasch model, which allows testing the psychometric qualities of the FIM. Results indicate that the FIM has good psychometric qualities. However, results also show that the 18 FIM items define two statistically and clinically different indicators. Thirteen items define disability in motor functions. Five items define disability in cognitive functions. Separate analyses of the two scales help to improve the psychometric quality of the FIM.

Activities of Daily Living↗

Practice parameter: treatment of the child with a first unprovoked seizure: Report of the Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society.

The Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society develop practice parameters as strategies for patient management based on analysis of evidence regarding risks and benefits. This parameter reviews published literature relevant to the decision to begin treatment after a child or adolescent experiences a first unprovoked seizure and presents evidence-based practice recommendations. Reasons why treatment may be considered are discussed. Evidence is reviewed concerning risk of recurrence as well as effect of treatment on prevention of recurrence and development of chronic epilepsy. Studies of side effects of anticonvulsants commonly used to treat seizures in children are also reviewed. Relevant articles are classified according to the Quality Standards Subcommittee classification scheme. Treatment after a first unprovoked seizure appears to decrease the risk of a second seizure, but there are few data from studies involving only children. There appears to be no benefit of treatment with regard to the prognosis for long-term seizure remission. Antiepileptic drugs (AED) carry risks of side effects that are particularly important in children. The decision as to whether or not to treat children and adolescents who have experienced a first unprovoked seizure must be based on a risk-benefit assessment that weighs the risk of having another seizure against the risk of chronic AED therapy. The decision should be individualized and take into account both medical issues and patient and family preference.

Adolescent↗

Practice parameter: corticosteroid treatment of Duchenne dystrophy [RETIRED]: report of the Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society.

BACKGROUND: The Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society develop practice parameters as strategies for patient management based on analysis of evidence. OBJECTIVE: To review available evidence on corticosteroid treatment of boys with Duchenne dystrophy. METHODS: Relevant literature was reviewed, abstracted, and classified. Recommendations were based on a four-tiered scheme of evidence classification, and areas for future research are defined. RESULTS: Seven class I studies and numerous less rigorous trials all demonstrated that corticosteroid treatment for 6 months with prednisone (0.75 or 1.5 mg/kg/day) increased muscle strength, performance, and pulmonary function and significantly slowed the progression of weakness. Two class I trials examined the effect of lower dosage of prednisone (0.30 and 0.35 mg/kg/day), demonstrated lesser but similar benefits, and showed a lower frequency of side effects (e.g., weight gain). The only significant side effects in all class I trials were weight gain and development of a cushingoid facial appearance. One longer-term trial of daily prednisone (0.3 to 0.7 mg/kg/day), a class III study, showed prolongation of functional ability and slower progression of weakness in patients during 3 years of treatment. One class IV, open trial of alternate-day prednisone (2 mg/kg for 2 months, then two-thirds dose every other day) extended ambulation by approximately 2 years in treated compared with untreated patients. Deflazacort, a corticosteroid similar in structure to prednisone, produced similar improvement in muscle strength and function with a similar side effect profile. CONCLUSIONS: Prednisone has been demonstrated to have a beneficial effect on muscle strength and function in boys with Duchenne dystrophy and should be offered (at a dose of 0.75 mg/kg/day) as treatment. If side effects require a decrease in prednisone, tapering to dosages as low as 0.3 mg/kg/day gives less robust but significant improvement. Deflazacort (0.9 mg/kg/day) can also be used for the treatment of Duchenne dystrophy in countries in which it is available. Benefits and side effects of corticosteroid therapy need to be monitored. The offer of treatment with corticosteroids should include a balanced discussion of potential risks.

Adolescent↗

Validation and factorial structure of a standardized neurological examination assessing neurological soft signs in schizophrenia.

Although neurological soft signs (NSS) have been consistently reported in patients with schizophrenia, their clinical relevance, the actual impact of treatment or their evolution during the disease are not well clarified, possibly because of methodological limitations of the available tools. We have developed a new standardized examination integrating the assessment of 23 NSS selected from the literature and the rating of well-validated scales for assessment of extra-pyramidal symptoms. We examined 161 subjects (controls, n=48; patients with schizophrenia, n=95; or recurrent mood disorder, n=18). Half of the patients were neuroleptic-free. Schizophrenic patients had significantly higher total score (14. 6+/-8) than mood disorder patients (12.0+/-7) and controls (5.0+/-2). Internal consistency (Cronbach's alpha=0.85) and inter-rater reliability were good. Principal component analysis found five consistent factors ('motor coordination', 'motor integrative function', 'sensory integration', 'involuntary movements or posture', 'quality of lateralization'). This scale thus confirmed a factorial structure in agreement with the conceptual areas of interest explored by NSS and should be a useful tool for assessment of the different dimensions of neurological dysfunction in schizophrenia.

Adult↗

Neurology in the United Kingdom. II: A study of current neurological services for adults.

Health care planning relies on accurate data, yet there are few published data on neurological services in the United Kingdom. This paper describes the number and distribution of consultant neurologists in the UK and is based on a questionnaire completed by Regional Health Authorities and their equivalents, by Special Health Authorities, and by regional representatives of the Association of British Neurologists. The data were published by the ABN in 1988 but have not previously been widely available. The study identified 190 consultant neurologists (152 whole time equivalents). Overall there was one whole time neurologist for 373,000 persons but the ratio varied by a factor of four in different regions. There was a wide variation in the distribution of consultant neurological sessions between Health Districts. One group of neurologists was based in centres. A second group spent most of their time within the Health District, but had an attachment to a Regional Centre.

Adult↗

[The most frequent neurologic disturbances. A contribution to the definition of topics for the programmatic content to the program of neurology in medical graduation].

INTRODUCTION: Knowledge accumulation is overfilling the thematic content of medical graduation. Medical Schools must be alert to define a minimal content related with the most frequent disturbs. We intent to suggest topics for the minimal content, to the program of Neurology in medical graduation. METHOD: 1. To identify the places where young doctors are working outside the School Hospital (SH): we analysed the answers of the letters sent to 6415 resident - doctors (RD) in São Paulo's State and we made personal interview with 201 RD. 2. To verify the most frequent neuropsychiatric disturbs (ND) in the population: we made the analysis of the patient's diagnostic records in emergency room of three institutions: Municipal of Taubaté-SP, Municipal of São José dos Campos-SP and Faculty of Medicine of São Paulo Santa Casa de Misericórdia. RESULTS: 1. The RD are young and, outside the SH, they work in other emergency services. 2. The most frequent diagnosis in institutions were listed: alcoholism, cerebrovascular disease, coma, cranial trauma, dementia, dizziness, epilepsy, facial paralysis, faint, headache, hemiplegia or paraplegia, meningitis, others paralysis, periferical neuropathy and psychiatric disorders. CONCLUSIONS: Those diagnostics most frequent are relevant topics of the programmatic content to the program of Neurology in medical graduation.

Brazil↗

[Clinico-occupational history in neurology and scope of occupational neurology].

INTRODUCTION: The occupational environment generates a large amount of illness which involves several specialities, including neurology. However, there is little awareness--or little training--in this field as in most medical specialities, since in the basic tool of a doctor's work, the clinical history, importance is sometimes given to aspects such as sex, race etc. and data referring to the patient's occupation and the risks this may imply for his health are considered to matter less. DEVELOPMENT: The creation of a new working party within the Sociedad Española de Neurología, that of Neurología del Trabajo (Occupational Neurology) aims to correct these deficiencies, and to make colleagues aware of the importance of assessing the occupational hazards of patients and attempt to relate them to the clinical picture which presents. In order to do this we must first mark out our field of action, the commonest diseases and the administrative route used for claiming for these conditions. Then we will have to evaluate our relationship with other professional colleagues in the field of occupational health and finally consider the tasks of training, investigation and promotion of occupational health.

Humans↗

[Neurologic manifestations of Behçet disease. Review of the caseload of the Neurology and Dermatology services at the Santa Maria Hospital].

Neurological involvement in a series of patients with Behçet's disease, evaluated at the Departments of Neurology and Dermatology, St. Maria Hospital is reported. Meningoencephalytic or encephalytic were the most common clinical forms, while headache, cerebellar and pyramidal signs were the most prevalent symptoms/signs. On follow-up (range 2-13 years) the majority of the patients had either a progressive or a remitting-progressive course. Magnetic ressonance imaging was the most valuable method of detecting central nervous system lesions.

Adult↗