1/7. Familial intracranial haemorrhage due to factor v deficiency.Strokes may occur with a large number of genetic disorders. Natowicz and Kelley have reviewed the single gene disorders capable of causing familial haemorrhagic strokes. These may be classified into four groups: a) hereditary disorders affecting clotting factors or platelets; b) hereditary disease producing vascular anomaly; c) hereditary disease causing hypertension and d) miscellaneous group including neurofibromatosis, sickle cell disease and tuberous sclerosis.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
2/7. Supported employment and compensatory strategies for enhancing vocational outcome following traumatic brain injury.Epidemiological research clearly indicates that traumatic head injury has reached epidemic proportions. incidence rates for head injury are greater than those for cerebral palsy, multiple sclerosis, and spinal cord injury combined. Many victims suffer from long-term impairments in functional, neurological, medical, neuropsychological and linguistic status. Emotional and behavioural problems are common as well. Additionally, family problems often ensue as a consequence of the victim's dependency and concomitant emotional changes. Investigations of post-injury vocational status indicate that unemployment rates within the first 7 years post-injury range as high as 70% for those with moderate and severe injuries. Researchers have demonstrated that the emotional and neuropsychological changes arising from injury are the greatest contributors to reduced employability. Relatively high unemployment rates strongly suggest that traditional approaches to physical and vocational rehabilitation have been entirely inadequate. To complement existing services and enhance employment outcome, two approaches have been developed and refined for use with victims of head injury. Supported employment is a unique approach which assists the client to select, obtain and maintain suitable employment on the basis of his/her interests and abilities. Compensatory strategies have been developed to help the individual offset intellectual problems which would otherwise interfere with learning job skills and maintaining production levels. Often, compensatory strategies are used in the context of a comprehensive supported employment programme. The greater use of supported employment and compensatory strategies is likely to enhance employment outcomes for those with traumatic head injury. Nevertheless, additional research is needed to more clearly identify the types of techniques which work best for each unique set of problems.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
3/7. memory loss from a subcortical white matter infarct.Clinical disorders of memory are believed to occur from the dysfunction of either the mesial temporal lobe, the mesial thalamus, or the basal forebrain. Fibre tract damage at the level of the fornix has only inconsistently produced amnesia. A patient is reported who suffered a cerebrovascular accident involving the posterior limb of the left internal capsule that resulted in a persistent and severe disorder of verbal memory. The inferior extent of the lesion effectively disconnected the mesial thalamus from the amygdala and the frontal cortex by disrupting the ventral amygdalofugal and thalamic-frontal pathways as they course through the diencephalon. This case demonstrates that an isolated lesion may cause memory loss without involvement of traditional structures associated with memory and may explain memory disturbances in other white matter disease such as multiple sclerosis and lacunar state.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
4/7. The effect of intrathecal baclofen on electrical muscle activity in spasticity.The efficacy of intrathecally administered baclofen was demonstrated in three patients with different types of muscular hypertonia (supraspinal rigidity, spasms shortly after spinal trauma, spasms for many years induced by multiple sclerosis) using integrated electromyography. Reduction of muscular electrical activity was accompanied by clinical improvement during long-term infusion via an implanted pump. The three patients have been observed for more than 1 year, during which time the antispastic activity of intrathecally infused baclofen has remained stable. Intrathecal application of baclofen may be considered as a possible alternative to surgery.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
5/7. An autopsy case of Binswanger's disease without hypertension and associated with cerebral infarction in the terminal stage.We report here an autopsy case of Binswanger's disease (BD) without hypertension and associated with cerebral infarction in the terminal stage. The female patient, who was 74 years old at the time of death, had initially demonstrated manic-depressive disorder-like mental disorder, followed by dementia and neurological deficits. A brain CT scan showed white matter low attenuation bilaterally and symmetrically. BD was clinically diagnosed despite the lack of hypertension. In the terminal stage, she suffered an infarction in the left anterior cerebral artery region, and died of pneumonia. Neuropathologically, we found the infarction of the left anterior cerebral artery region, demyelination, fibrillary gliosis, lacunae and arteriosclerosis of the small arteries and arterioles in the white matter.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
6/7. Longstanding isolated cerebral systemic lupus erythematosus in an 8-year-old black girl. Resemblance with multiple sclerosis.We describe a 12-year-old girl with SLE who presented with longstanding isolated neurological symptoms resembling MS. The literature concerning the difficulties in diagnostic procedures in cerebral SLE are reviewed in comparison to MS.- - - - - - - - - - ranking = 4keywords = sclerosis (Clic here for more details about this article) |
7/7. Severe amnesia: an usual late complication after temporal lobectomy.A patient developed the severe amnesic syndrome 8 years after temporal lobe surgery for epilepsy. He underwent left temporal lobectomy (6 cm, 43.5 g; hippocampal sclerosis) aged 19, and remained seizure free for 8 years until a convulsion followed a head injury. He became severely amnesic after a fourth convulsion 16 months later. He was right-handed, pre-operative IQ was average, verbal memory poor and non-verbal memory normal. Post-operatively, these were unchanged. After the first post-operative seizure he began professional training. After onset of amnesia IQ was unchanged, anterograde memory severely impaired and retrograde amnesia dense for at least 16 months. He died 2 years later. Magnetic resonance imaging before amnesia showed absence of anterior left temporal lobe, atrophy of left fornix and mamillary body, and normal right temporal lobe. Four months after onset of amnesia, right hippocampal volume had reduced by 36%. autopsy showed: previous left temporal lobectomy with absence of left amygdala and hippocampus, atrophy of fornix and mamillary body; neuronal loss in the right hippocampus, severe in CA1 and CA4; intact right amygdala and parahippocampal gyrus; recent diffuse damage associated with cause of death. A convulsion can cause severe hippocampal damage in adult life. Hippocampal zones CA1 and/or CA4 are critical for maintaining memory and the amygdala and parahippocampal gyrus cortex alone cannot support acquisition of new memories.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |