Search results for "Central Nervous System Disease"
showing 10 items of 199 documents
Abnormal Somatosensory Evoked Potentials Indicate Compressive Cervical Myelopathy in Mucopolysaccharidoses
2000
Compressive myelopathy at the cranio-cervical junction is a complication of mucopolysaccharidoses (MPS). To detect cervical myelopathy we recorded median and posterior tibial nerve SEPs in 15 patients aged 2.4 - 33.4 years (median 8.8 years) with MPS I-S (n = 3), MPS IVA (n = 8) and MPS VI (n = 4). In addition to the cortical waveforms we recorded the subcortical median nerve SEPs N13b and P13 generated near the cranio-cervical junction and the lemniscal P30 after posterior tibial nerve stimulation. MRI studies in 13 subjects revealed spinal cord compression at the cranio-cervical junction in 10 patients; 5 patients had an increased signal intensity on the T2-weighted initial MRI indicating…
Outcome of type III Gaucher disease on enzyme replacement therapy: review of 55 cases.
2007
The European Task Force for Neuronopathic Gaucher Disease (NGD) met in 2006 to review its 2001 guidelines. Fifty-five patients from five European countries were reviewed; 29 were male and 26 female. The majority of the patients were homozygous for the L444P mutation. All had been on enzyme replacement therapy (ERT). However, there was considerable variation in the dose of ERT, as well as an uneven distribution of risk factors. Thus, the oldest patients were on the lowest doses, and several had had a total splenectomy, while the youngest patients had a high proportion of compound heterozygosity and were on the highest doses, and very few had had a splenectomy. This heterogeneity rendered ana…
Auditory event-related potentials (P300) in epileptic patients.
2001
Auditory event-related potentials (AERPs) were recorded during an auditory oddball paradigm in 108 epileptics and in 32 healthy controls. P300 latency varied in relationship with age only in controls. Symptomatic epileptics had significantly prolonged P300 mean latency compared to those without detectable brain lesion(s) on MR scan. Moreover, these latter patients were compared on the basis of epilepsy duration, type of seizure, seizure frequency and antiepileptic treatment; the application of a multiple regression model showed a significant relationship between P300 latency prolongation and epilepsy duration, seizure frequency and polytherapy.
Red Ear Syndrome and Migraine: Report of Eight Cases
2002
We describe eight idiopathic cases of red ear syndrome in seven children and one adult. All were migraineurs with a history of paroxysmally painful and red ear, unilateral or alternating, in isolation or associated with migraine attacks. The reported duration of these episodes varied from 30 minutes to 1 hour. Neurologic examination, brain MRI and CT scans, and x-rays of the cervical spine were normal. The close temporal relationship between the "red ear episodes" and migraine attacks suggests an association between the two conditions.
Arachnoid cysts: does surgery improve epileptic seizures and headaches?
1995
During the period from 1985 to 1992 we treated 43 patients with intracranial arachnoid cysts (ACs). In this retrospective study we assessed the outcome of these patients with regard to non-specific symptoms such as headaches and epileptic seizures. Twelve patients had headaches of obscure origin and a mostly temporal located AC. Six of these 12 underwent surgery. After the operation 4 patients (4/6) had no further headaches, two remained unchanged. The other 6 conservatively treated patients (6/12) had further headaches. Ten of the 43 patients had epileptic seizures. One patient dropped out of the survey. Six of the 9 remaining patients had a temporal AC. Four of these 6 underwent surgery; …
Arachnoid cysts: How do postsurgical cyst size and seizure outcome correlate?
1998
Arachnoid cysts (ACs) are congenital cystic brain malformations associated with epilepsy. The purpose of this study was to determine the effect of surgical intervention of ACs on cyst size and seizure outcome. We reviewed the world's medical literature dealing with surgically treated ACs in epilepsy patients. Our study included only cases, in which the relationship between pre- and post-operative CT-size of the AC and seizure outcome was described. We also included six patients with ACs and epilepsy treated surgically at the University of Mainz. We analyzed postoperative AC size and seizure outcome with respect to mode of operation, cyst location, and patients' age. A total of 76 patients w…
Frame-based and frameless stereotaxy in the localization of cavernous angiomas
2001
Cavernous angiomas are mostly small intracranial vascular lesions which can be intraoperatively localized by sonography or stereotactic methods. This paper compares the results of cavernous angioma surgery localized by frame-based vs frameless techniques. Thirty-nine patients with cortical or subcortical lobar cavernoma were operated on by a microsurgical trans-sulcal approach. The localization of the lesion was performed in 19 (49%) patients by frame-based technique and in 20 (51%) patients by frameless navigation. In 22 (56%) of the patients, the cavernomas were located in an eloquent cortical area. Ten of 22 of these lesions were localized by frame-based stereotaxy and 12/22 by frameless…
Bovine pericardium for duraplasty: clinical results in 32 patients.
2001
Bovine pericardium has been widely used for grafts in cardiac surgery and seems to have suitable properties for use as a dural graft. We report on the use of solvent-preserved, gamma-sterilized Tutoplast bovine pericardium for dural grafts in 32 patients undergoing cranial and spinal operations with the objective of clinically assessing this material and technique by a retrospective analysis. All available records were reviewed and information regarding the indication for grafting, complications, and outcome were collected and analyzed for all patients. Indications for grafting included tethered cord myelolysis, closure of lumbosacral myeloceles, Chiari decompression, posterior fossa cranio…
Hypo-excitability of cortical areas in patients affected by Friedreich ataxia: A TMS study
2005
The aim of the study was to explore excitability of a motor and a non-motor (visual) area in patients affected by Friedreich ataxia and to correlate neurophysiological data with clinical parameters. Seven patients (3M/4F) and ten healthy controls (5M/5F) participated in the study. The hot-spot for activation of right abductor pollicis brevis was checked by means of a figure-of-eight coil and the motor threshold (MT) on this point was recorded. The phosphene threshold (PT) was measured by means of a focal coil over the occipital cortex as the lower intensity of magnetic stimulation able to induce the perception of phosphenes. The patients showed a significantly higher mean PT (p<.03) and MT …
Intravenous immunoglobulin in primary and secondary chronic progressive multiple sclerosis: a randomized placebo controlled multicentre study
2007
In patients with relapsing-remitting multiple sclerosis (MS), IVIG was shown to reduce the relapse rate and progression of disability. In patients with chronic progressive MS, a beneficial effect of IVIG was not documented in placebo controlled studies. This trial investigated the influence of IVIG in primary (PPMS) and secondary (SPMS) chronic progressive MS. Two-hundred and thirty-one patients stratified for PPMS ( n = 34) and SPMS ( n = 197) were randomly assigned to IVIG 0.4 g/kg per month or to placebo for 24 months. Primary endpoints were 1) the time to sustained progression of disease identified as worsening of the expanded disability status scale (EDSS) sustained for 3 months, and …