1/3. Conceptual dilemmas in evaluating individuals with severely impaired consciousness.States of severely impaired consciousness (SIC) are characterized by cognitive and motor limitations. This case report describes a 45-year-old female with impaired consciousness who began to 'walk'. She initially presented to the hospital unresponsive and was subsequently diagnosed with metabolic encephalopathy due to severe hypoglycaemia. Traditional indices of consciousness indicated a low level of responsiveness; however, during physical therapy, she displayed reciprocal walking movements when lifted to a standing position by two therapists. Despite her ability to walk increased distances during and after neurorehabilitation, she was unable to consistently demonstrate responses indicative of higher levels of consciousness. This case illustrates the challenge of rating patients with limited behavioural repertoire using established measures of impaired consciousness.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
2/3. Cerebral MRI in two brothers with mucopolysaccharidosis type I and different clinical phenotypes.We describe a cerebral MRI study of two brothers with mucopolysaccharidosis type I. They are of similar physical appearance, but the younger is severely mentally retarded while the elder is of normal intelligence. MRI shows characteristic abnormalities in the CNS, but it is not yet possible to establish a correlation between them and the mental retardation.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
3/3. Neuronal and vascular disorders of the brain and spinal cord in Menkes kinky hair disease.A 3 2/12-year-old boy had recurrent seizures, chronic respiratory infection, and delayed physical and mental development. He also had low plasma copper content typical of Menkes syndrome. autopsy showed marked neuronal loss and gliosis in most areas of the cerebral and cerebellar cortices, midbrain, pons, and medulla. The spinal cord showed severe demyelination in both ascending (spinocerebellar) and descending (lateral corticospinal) tracts from the cervical to the sacral level. In addition to these neuronal lesions, both the meningeal and parenchymal arterial and venous branches were remarkably dilated in the brain and spinal cord. Our previous study of this case showed abnormal perivascular innervation and abnormal axonal swelling of the postganglionic adrenergic fibers elsewhere in the body. The metabolic disorder caused by copper deficiency induces severe neuronal degeneration that is apparently exaggerated by extensive and progressive vascular abnormality.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |