1/59. Ethical perspectives in neuroscience nursing practice.The role of neuroscience nurses in relation to ethical issues has become increasingly complex. knowledge of ethical principles and theories assists the nurse in the development of a theoretical basis for resolution of ethical issues or concerns. Additionally, the nurse must possess information regarding practice codes or standards as well as legislative requirements. The nurse must act as an advocate for the patient and society through active participation in institutional ethics committees and legislative forums.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
2/59. Neurologic and gastrointestinal dysfunction in cardio-facio-cutaneous syndrome: identification of a severe phenotype.Controversy exists over the distinction between cardio-facio-cutaneous (CFC) syndrome and noonan syndrome (NS). Several authors have suggested that they are different phenotypes of the same condition. We present the cases of two patients with CFC syndrome to show that it is a distinct condition with a unique combination of findings and a more complex natural history. These patients, both girls, were born with signs of fetal edema following pregnancies complicated by polyhydramnios. Each has short stature with relative macrocephaly; fuzzy, sparse hair; and the typical craniofacial features, including a square forehead. Both have heart abnormalities, failure to thrive, and severe feeding problems requiring gastrostomy. They are markedly hypotonic and developmentally delayed. They show signs of frequent eyelid fluttering and have oral aversion, tactile hypersensitivity, and sensory integration abnormalities. keratosis pilaris, the characteristic skin symptom, is also present in both patients. In a review we identified 56 cases of CFC syndrome. We scored these cases by 10 clinical criteria and identified a subset with a specific, severe phenotype distinct from that of NS. The serious neurologic and gastrointestinal complications, in addition to the skin abnormalities and characteristic facies in this group, clearly separate these patients from the mildly affected ones, most of whom appear to have NS or another syndrome. We discuss the differences between the severe CFC phenotype and those of overlapping conditions. We set forth stringent diagnostic criteria for CFC syndrome, the initial step toward identifying a molecular basis for this condition.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
3/59. Familial dysautonomia: a diagnostic dilemma. chronic lung disease with signs of an autoimmune disease.We present an 11-year-old girl with sensory and autonomic neurological dysfunction, and respiratory insufficiency caused by recurrent aspiration. The diagnosis of familial dysautonomia (FD) was confirmed by a missing axonal flare to histamine, miosis in response to conjunctival methacholine and homozygous polymorphic linked markers DS58(18) and DS159(7) on chromosome 9. Ashkenazi Jewish descent could not be ascertained by history. A variety of positive tests for autoantibodies were initially interpreted as evidence for systemic lupus erythematosus vs. overlap syndrome with pulmonary, cerebral, skin, and ocular involvement. The diagnosis of FD was delayed because of the rarity of this disorder in germany (second case reported). We discuss possible explanations for the misleading immunological findings, including interference by antibodies binding to milk proteins used as blocking reagents in enzyme-linked immunoassays and circulating immune-complexes due to chronic aspiration pneumonitis.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
4/59. Concepts and data model for a co-operative neurovascular database.BACKGROUND: Problems of clinical management of neurovascular diseases are very complex. This is caused by the chronic character of the diseases, a long history of symptoms and diverse treatments. If patients are to benefit from treatment, then treatment decisions have to rely on reliable and accurate knowledge of the natural history of the disease and the various treatments. methods: Recent developments in statistical methodology and experience from electronic patient records are used to establish an information infrastructure based on a centralized register. RESULTS: A protocol to collect data on neurovascular diseases with technical as well as logistical aspects of implementing a database for neurovascular diseases are described. The database is designed as a co-operative tool of audit and research available to co-operating centres. CONCLUSION: When a database is linked to a systematic patient follow-up, it can be used to study prognosis. Careful analysis of patient outcome is valuable for decision-making.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
5/59. Complex neurologic syndrome associated with the G1606A mutation of mitochondrial dna.OBJECTIVES: To confirm the pathogenicity of the G-to-A substitution at nucleotide 1606 (G1606A) mutation in the mitochondrial dna (mtDNA) tRNA(Val) gene, and to characterize genotype-phenotype correlation. PATIENT AND methods: A 37-year-old man since childhood developed a complex clinical picture characterized by hearing loss, migraine, ataxia, seizures, cataracts, retinitis pigmentosa, mental deterioration, and hypothyroidism. magnetic resonance imaging revealed diffuse calcification of the basal ganglia and cerebral cortical atrophy. Morphologic and biochemical studies of respiratory chain complexes were performed in skeletal muscle. All 22 mitochondrial tRNA genes were screened for mutations by direct sequencing. RESULTS: Biochemical analysis showed normal activities of respiratory chain enzymes and citrate synthase; morphologic examination showed scattered ragged-red fibers and poor or absent cytochrome c oxidase staining in 10% of the fibers. A heteroplasmic G1606A transition in the mtDNA tRNA(Val) gene was found. Mutant dna was 70% of the total in the proband's muscle. The mutation was absent in blood samples and urinary sediment from his healthy brother and mother. CONCLUSION: This second patient with the G1606A mutation confirms both the pathogenicity of the mutation and its association with a characteristic complex neurologic phenotype.- - - - - - - - - - ranking = 3keywords = complex (Clic here for more details about this article) |
6/59. Managing faecal retention and incontinence in neurodisability.The large number of patients with faecal retention and/or incontinence in continuing care wards and rehabilitation units presents a considerable challenge. In order to maintain dignity and minimize the unpleasant odour so commonly associated with these wards and units, effective bowel management should be planned for individual patients. For an effective bowel management regime a team approach should be adopted, involving, where possible, the patient and carer as well as all the health professionals administering the care. Two case studies illustrate the use of assessment and management of bowel problems in patients with severe complex neurodisability. Bowel dysfunction in this patient population, in general, is poorly covered in the literature. The present article, by relating theory to practice, offers information and guidance for nurses working with patients who have bowel-related problems.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
7/59. Geriatric Treatment Center: a contemporary model for collaboration between psychiatry and neurology.The diagnosis and treatment of individuals with problems involving both psychiatry and neurology have become more sophisticated in recent years, but these advances may be difficult to implement in the modern health care environment. For 16 years, an inpatient Geriatric Treatment Center within a state mental hospital has been used to diagnose and treat older persons with complex neuropsychiatric disorders. Eight illustrative cases are presented of patients with major behavioral dysfunction that could not be managed effectively in other health care facilities. After neuropsychiatric evaluation and behavioral neurology consultation, all had neurologic diagnoses established as the cause of their presentation. Seven improved with appropriate treatment, of whom one could return to independent living, and the eighth died and had an autopsy diagnosis of his disease at a nearby academic medical center. This series highlights the value of collaboration between psychiatry and neurology for evaluation and treatment of older patients with neuropsychiatric problems not easily accommodated by many existing health care settings.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
8/59. The reach of neurology.Neurologists experienced in the interpretation of disease in terms of disordered action of the nervous system should be well suited to extend their field of interest to the more complex disorders of human behavior, including hysteria, delirium, ill-defined pain syndromes, unexplained fatigue, disorders of thought, atypical depression, and delusions. To illustrate the potential of neurology in approaching the more complex disorders of behavior, several examples from clinical neurology are presented in which phenomena calling for inquiry and analysis in neurological terms are described. The categories are temporal lobe epilepsy, delirium, drug toxicity, disease processes of the cerebrum, obscure pain, dyslexia, and hysteria. Inquiry into complex disorders of behavior is inseparable from the broad subject of normal mental activity, the neural organization subserving all human thought, emotion, and action. Because of this close association, the comment on hysteria includes an introduction to the important question of whether we humans possess a free will to choose our course of behavior.- - - - - - - - - - ranking = 3keywords = complex (Clic here for more details about this article) |
9/59. Neurologic and cardiac progression of glycogenosis type VII over an eight-year period.Little is known about the progression of phosphofructokinase deficiency (glycogenosis type VII, Tarui's disease). We describe a 66-year-old woman who had this disease diagnosed in 1997. Initial manifestations had included simple partial seizures since 1977, anginal chest pain since 1982, and muscle cramps since 1983. To prevent recurrent myocardial infarction, anticoagulation therapy with phenprocumon was initiated. Cardiac involvement progressed over an 8-year period, manifesting as low-voltage electrocardiogram (ECG), ectopic supraventricular tachycardia, thickened mitral valve, mitral valve insufficiency, enlarged left atrium, left ventricular hypertrophy, and diastolic dysfunction. Progression of neurologic involvement manifested as complex partial seizures, double vision, reduced tendon reflexes, central facial palsy, bradydiadochokinesia, and distal weakness of the upper extremities. Discontinuance of oral anticoagulation after 19 years, initiation of enalapril therapy, and administration of carbamazepine markedly improved the patient's condition.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
10/59. Transient neurologic deficits associated with carbamazepine-induced hypertension.carbamazepine is a well-established, effective treatment of complex partial seizures and is well tolerated in most patients. The adverse effects of carbamazepine include aplastic anemia, agranulocytosis, pancytopenia, bone marrow depression, thrombocytopenia, cardiac conduction abnormalities, congestive heart failure, and peripheral edema. hypertension or hypotension has also rarely been documented in patients with either therapeutic or toxic blood levels of carbamazepine. It is possible that carbamazepine-induced hypertension in those with therapeutic blood levels is rarely seen because most of the patients who begin treatment are young and do not have baseline hypertension. The authors describe a patient of African-American descent with a history of controlled essential hypertension who developed severe uncontrolled hypertension when started on carbamazepine. Treatment with additional antihypertensive medications did not reduce his blood pressure. In addition, he developed two episodes of transient neurologic deficits, the symptoms of which consisted of dysarthria, vertigo, and unstable gait. A substantial reduction of his carbamazepine dose resulted in the control of his blood pressure and no recurrence of his symptoms.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
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