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Biomedical subjects

J M Stevens

Publications and source records attributed to J M Stevens.

At least 19 recordsLinked to original sources
PubMed1999

Disproportion in the distribution of gray and white matter: neuropsychological correlates.

OBJECTIVE: To examine the relationship between measures of disproportion in the regional distribution of gray and white matter and preoperative neuropsychological function in temporal lobe epilepsy patients with proved hippocampal sclerosis (HS). BACKGROUND: Subtle cerebral structural disruption, not evident on routine inspection of high-resolution MRI, is associated with poor surgical outcome in patients with histologically proved HS. Preoperative global memory dysfunction is also associated with poor postoperative seizure control. The authors hypothesize that patients with HS and abnormal regional distributions of gray and white matter would show more diffuse neuropsychological deficits preoperatively than patients with isolated HS alone. METHODS: A total of 28 adults with lateralized temporal lobe epilepsy and hippocampal volume loss measured on MRI were assessed preoperatively on neuropsychological tests of general intellect and the learning and recall of both verbal and nonverbal material. Quantitative MRI analysis of the regional distribution of gray and white matter was performed. Chi-square analyses were used to examine the relation between the presence or absence of cerebral abnormalities and preoperative performance on the neuropsychological tests. RESULTS: A total of 15 of 28 patients had extrahippocampal abnormalities on quantitative MRI analysis. Thirteen patients had global memory impairment. Bilateral memory deficits were significantly associated with both the presence of cerebral abnormalities (p < 0.02) and poor postoperative seizure control (p < 0.05). CONCLUSIONS: Disproportion in the regional distribution of gray and white matter in patients with HS may form the structural basis of global memory disturbance in a distinct group of patients with temporal lobe epilepsy.

Adult↗
PubMed1999

Presurgical evaluation of patients with epilepsy and normal MRI: role of scalp video-EEG telemetry.

When considering surgery for intractable partial seizures, even with high resolution MRI, some patients do not show structural abnormalities. The aim was to consider whether these patients were likely to proceed to surgical treatment after scalp video-EEG telemetry. All patients undergoing presurgical evaluation at the National Hospital for Neurology and Neurosurgery between 1995 and 1997 were reviewed and 40 were identified without definite MRI abnormalities. None of 40 disclosed a well localised epileptogenic zone concordant with other tests that would have allowed the patient to proceed directly to surgery. In five of the 40, evaluation led to a hypothesis that could be tested by intracranial studies; three proceeded to surgery. It is suggested that high quality MRI is performed first when surgical evaluation is undertaken and if negative the patient carefully counselled before proceeding with any investigations, as successful resective surgery is an unlikely outcome in such MRI negative cases.

Adolescent↗
PubMed1999

Supratentorial cavernous haemangiomas and epilepsy: a review of the literature and case series.

OBJECTIVES: To characterise the clinical features and response to treatment of supratentorial cavernomas associated with epilepsy. METHODS: A systematic review of the literature was carried out and a retrospective case series of patients with cavernoma diagnosed by MRI and/or histology was compiled. Patient selection biases in the literature review were reduced as far as possible by selection of unbiased publications. RESULTS: In the literature, cavernomas were relatively less common in the frontal lobes. There were multiple cavernomas in 23% of cases. The main clinical manifestations were seizures (79%) and haemorrhage (16%). The annual haemorrhage rate was 0.7%. The outcome after excision was good with improvement in seizures in 92% of patients. In the case series the surgical outcome was less favourable, reflecting inclusion of a higher proportion of patients with intractable epilepsy. In both the literature review and the case series, outcome was poorer in cases with a longer duration of seizures at the time of surgery. CONCLUSIONS: The good surgical results, particularly in cases treated earlier, and the significant cumulative haemorrhage rate, suggest that excision is the optimum treatment. However, these factors have not been examined prospectively and, despite the availability of several retrospective studies, the optimum treatment, particularly for non-intractable cases, will only be determined by a prospective study.

Adolescent↗
PubMed1999

Cortical dysplasia with angiodysgenesis and chronic inflammation in multifocal partial epilepsy.

A 25-year-old man with a long history of temporal lobe epilepsy developed right occipital lobe seizures and a progressive right homonymous hemianopia. MRI showed diffuse enhancement of the left temporoparieto-occipital white matter and cortical thickening of the left medial temporal lobe. The resected temporal lobe revealed cortical dysplasia and angiodysplasia with foci of more recent ischemic necrosis and chronic inflammation as an explanation for the clinical deterioration.

Adolescent↗
PubMed1998

Class sigma glutathione transferase unfolds via a dimeric and a monomeric intermediate: impact of subunit interface on conformational stability in the superfamily.

Solvent-induced equilibrium unfolding of a homodimeric class sigma glutathione transferase (GSTS1-1, EC 2.5.1.18) was characterized by tryptophan fluorescence, anisotropy, enzyme activity, 8-anilino-1-naphthalenesulfonate (ANS) binding, and circular dichroism. Urea induces a triphasic unfolding transition with evidence for two well-populated thermodynamically stable intermediate states of GSTS1-1. The first unfolding transition is protein concentration independent and involves a change in the subunit tertiary structure yielding a partially active dimeric intermediate (i.e., N2 left and right arrow I2). This is followed by a protein concentration dependent step in which I2 dissociates into compact inactive monomers (M) displaying enhanced hydrophobicity. The third unfolding transition, which is protein concentration independent, involves the complete unfolding of the monomeric state. Increasing NaCl concentrations destabilize N2 and appear to shift the equilibrium toward I2 whereas the stability of the monomeric intermediate M is enhanced. The binding of substrate or product analogue (i.e., glutathione or S-hexylglutathione) to the protein's active site stabilizes the native dimeric state (N2), causing the first two unfolding transitions to shift toward higher urea concentrations. The stability of M was not affected. The data implicate a region at/near the active site in domain I (most likely alpha-helix 2) as being highly unstable/flexible which undergoes local unfolding, resulting initially in I2 formation followed by a disruption in quaternary structure to a monomeric intermediate. The unfolding/refolding pathway is compared with those observed for other cytosolic GSTs and discussed in light of the different structural features at the subunit interfaces, as well as the evolutionary selection of this GST as a lens crystallin.

Animals↗
PubMed1998

Fast fluid-attenuated inversion-recovery imaging: first experience with a 3D version in epilepsy.

We developed a 3D version of fast fluid-attenuated inversion-recovery imaging (FLAIR) which provides images with a slice thickness of 1.5 mm. We present our initial experience with 3D fast FLAIR in patients with epilepsy. We compared 3D fast FLAIR (slice thickness 1.5 mm), 2D fast FLAIR (slice thickness 5 mm) and a 3D spoiled GRASS (IRSPGR) sequence (slice thickness 1.5 mm) in 10 patients with lesional epilepsy (head injury 1, hippocampal sclerosis 2, low-grade glioma 2, dysembryoplastic neuroepithelial tumour 2, polymicrogyria 1, perinatal infarct 1 and presumed thrombosed aneurysm 1). Both 2D and 3D fast FLAIR sequences yielded higher conspicuity for lesions than the T1-weighted IRSPGR sequence, except in the patient with polymicrogyria. The extent of the lesion, in particular that of low-grade tumours, was best assessed on 3D fast FLAIR images. 3D fast FLAIR may be a useful additional tool especially for imaging low-grade tumours.

Artifacts↗
PubMed1998

Image contrast and hippocampal volumetric measurements.

The objective was to assess the influence of image contrast on the repeatability of hippocampal measurements. We compared measurements on spoiled GRASS (SPGR) three-dimensional acquisition with measurements on inversion pulse prepared spoiled GRASS (IRSPGR) three-dimensional acquisition. IRSPGR images had a higher grey matter-white matter contrast than SPGR images. Repeated measurements of hippocampi of 13 subjects were performed on both SPGR and IRSPGR images and the standard deviation of the differences of repeated measurements calculated. SD was 239 mm3 for SPGR and 134 mm3 for IRSPGR. Our data suggest that higher image contrast improves the repeatability of hippocampal measurements.

Hippocampus↗
PubMed1998

Longitudinal quantitative hippocampal magnetic resonance imaging study of adults with newly diagnosed partial seizures: one-year follow-up results.

PURPOSE: We wished to establish whether hippocampal changes occur in 1 year in adults with newly diagnosed partial seizures and, if so, to identify possible causes and mechanisms. METHODS: Thirty-six adult patients with newly diagnosed partial seizures underwent a magnetic resonance imaging (MRI) scan of the brain including hippocampal volume and T2 relaxation time (HCT2) measurement and had a follow-up quantitative MRI scan approximately 1 year after the baseline MRI scan. RESULTS: At baseline, 4 patients (11%) had hippocampal sclerosis (HS), 4 (11%) had abnormalities other than HS, and 28 had a normal MRI scan (78%). Twenty-three patients (64%) had recurrent seizures in the period between the two MRI scans. One of the 4 patients with HS, who had daily seizures, had significantly increased HCT2 values on follow-up, possibly reflecting progressive hippocampal damage. None of the 32 patients with MRI findings other than HS at baseline progressed to HS on follow-up. However, 2 of the 32 patients had significant hippocampal changes, probably related to resolution of inflammatory swelling or edema after seizures were controlled. CONCLUSIONS: Subtle changes in hippocampi can occur in 1 year in adults with newly diagnosed partial seizures, which could be due to resolution of edema after seizure control or to hippocampal changes associated with frequent and daily seizures. Follow-up of the studied cohort for several years will be required to settle the question of whether progressive hippocampal damage occurs in temporal lobe epilepsy (TLE).

Adolescent↗
PubMed1997

Correlation of widespread preoperative magnetic resonance imaging changes with unsuccessful surgery for hippocampal sclerosis.

Despite meticulous preoperative assessment, about 30% of patients with refractory partial epilepsy due to hippocampal sclerosis fail to become seizure free after appropriate temporal lobe surgery. Perioperative complications, hippocampal remnants, and bitemporal disease do not account for all failures; extrahippocampal epileptogenic tissue must persist in some patients. Such dual pathology is detected on routine visual inspection of magnetic resonance images in about 15% of patients with hippocampal sclerosis, but most such patients are excluded from surgery. We postulated that some patients have occult extrahippocampal cerebral structural abnormalities (i.e., subtle dual pathology) and that the presence of these abnormalities would be associated with a poor surgical outcome. Quantitative postprocessing of preoperative magnetic resonance images from 27 patients subsequently proved to have hippocampal sclerosis demonstrated extrahippocampal structural abnormalities in 14, 10 of whom did not become seizure free, while 11 of 13 patients without such changes did become seizure free (chi2, p < 0.005). Such structural information may supplement clinical decision making in some patients being evaluated for epilepsy surgery and help to explain the biological basis of poor outcome from such surgery.

Adolescent↗
PubMed1997

Cerebellar axial postural tremor.

Three cases are presented with a predominantly axial postural tremor, without visible palatal tremor. Tremor varied in frequency between 3 and 10 Hz, often jumping from one frequency to another in this band. All three patients had evidence of cerebellar pathology. Cases 1 and 2 developed tremor in the setting of a late-onset cerebellar degeneration and after excision of a right cerebellar haemangioblastoma, respectively. Etiology was unclear in Case 3. Nevertheless, this patient had a cerebellar dysarthria. The tremor was similar to that sometimes seen in conjunction with palatal tremor, and EMG studies in Case 3 demonstrated a subclinical modulation of palatal muscle activity simultaneous with the truncal tremor. It is suggested that an axial postural tremor may be due to pathology of the cerebellum and its outflow pathways, despite the absence of clinically apparent palatal tremor.

Adult↗
PubMed1997

Somatosensory evoked potentials in adults with cortical dysgenesis and epilepsy.

Cortical dysgenesis (CD) is a well-recognised cause of epilepsy, but its functional anatomy is not fully understood. We recorded cortical somatosensory evoked potentials (SEPs) in 13 adult patients with epilepsy and various CDs excluding diffuse gyral malformations as diagnosed by MRI. The CD involved the perirolandic/perisylvian region in 7 patients. Six patients had neurological signs but only 3 had sensory dysfunction (astereognosis). As compared with 12 control subjects, SEPs were considered definitely abnormal in 7 patients (including the 6 with neurological signs) and equivocally abnormal in 2. The abnormalities ranged from defects affecting single components to absence of all potentials of cortical origin in one patient with hemiparesis and astereognosis. In this case it appears that gross sensory function must have been mediated by subcortical structures or through diffuse cortical projections. The initial cortical potentials (N20/P20) were absent in 6 patients, 5 of whom had CD in zones involving or bordering on the primary sensory cortex. Parietal potentials following N20 were absent or attenuated in 4 patients and of abnormally wide distribution, spreading to frontal, midline and ipsilateral electrodes, in 3 frontal components following P20 were absent, attenuated, delayed or distorted by abnormal spread of the parietal activity in 5 patients. Five patients with unilateral CD showed definite or equivocal SEP abnormalities to stimulation of both arms, suggesting there may be more widespread disturbance of cortical organisation and/or synaptogenesis, beyond the resolution of present day neuroimaging.

Adolescent↗
PubMed1997

Clinicopathological features of familial Alzheimer's disease associated with the M139V mutation in the presenilin 1 gene. Pedigree but not mutation specific age at onset provides evidence for a further genetic factor.

Sixteen affected individuals are described from two families with early onset autosomal dominant familial Alzheimer's disease. A mutation at codon 139 in the presenilin 1 gene on chromosome 14 results in a methionine to valine substitution which cosegregates with the disease in these families. Onset of dementia was before the age of 50 years in all individuals. The ages at onset within each family were tightly clustered but were significantly different between the families; this difference could not be accounted for by apolipoprotein E status and suggests the existence of a further genetic factor that modifies age at disease onset. The pattern of cognitive decline was similar in both families: early memory loss (initially selective for verbal memory in some individuals) was followed soon after by loss of arithmetic skills while naming and object perception skills were relatively preserved. A speech production deficit was observed in three members of one family but not in the other. Seizures were common and usually predated by myoclonic jerks by a number of years. Serial MRIs showed progressive cortical atrophy with periventricular white matter change appearing 3-4 years into the disease. PET revealed parieto-temporal hypometabolism in all individuals scanned. The diagnosis of Alzheimer's disease was confirmed with typical histopathology in one individual from each family.

Adult↗
PubMed1997

The one bone spine: a failure of notochord/sclerotome signalling?

Three cases of extensive vertebral fusions with absent clivo-axial angle are presented. The 'bone-within-bone' appearance in two patients with almost complete fusion of the spine suggested ossification of the notochord and perinotochordal sheath. On the basis of the radiological appearances and the results of recent molecular genetic studies on vertebrate embryos, the suggested time of segmentation failure along the axis of the craniovertebral junction and between vertebrae is the third to fifth week of gestation. The possible roles of the Pax-1 gene and of signalling between notochord and sclerotome are discussed, concluding that an early defect of the notochord may be responsible for this type of failure of segmentation. Indications for surgery in these cases included cord compression with brachialgia and 'chin-on-chest' deformity causing severely restricted visual fields. A critical review of clinical lessons learned in the operative treatment is presented.

Adult↗
PubMed1997

Development of hippocampal atrophy: a serial magnetic resonance imaging study in a patient who developed epilepsy after generalized status epilepticus.

PURPOSE: To investigate changes in hippocampal volume. METHODS: We used serial magnetic resonance imaging (MRI) in a patient who developed chronic epilepsy after having generalized tonic-clonic status epilepticus (SE). Five MRI investigations were performed during SE and a 58-month follow-up period. Hippocampal volumetric measurements and coregistration of scans were performed to detect hippocampal atrophy. RESULTS: During status both mesiotemporal regions returned a high signal on T2-weighted images. Two months after the onset of SE, bilateral hippocampal atrophy was detected. Further progressive hippocampal atrophy was detected in the subsequent 58 months by both hippocampal volumetric measurements and coregistration of scans. CONCLUSIONS: Our findings suggest that hippocampal atrophy is a process that may continue after the end of the SE.

Adult↗
PubMed1997

Widespread cerebral structural changes in two patients with gelastic seizures and hypothalamic hamartomata.

PURPOSE: We tested the hypothesis that widespread extralesional abnormalities of cerebral structure exist in association with apparently isolated hypothalamic hamartomata, providing a structural basis for the poor response of seizures to removal of the hamartoma or other apparently focal epileptogenic zones present. METHODS: High-resolution magnetic resonance imaging (MRI) brain scans of 2 patients with hypothalamic hamartomata were quantified by determination of regional distribution and symmetry of distribution of cortical gray matter and subcortical matter volumes. The results were compared with normal ranges for the distribution of such tissues in 33 controls. RESULTS: Both patients had abnormalities of distribution of gray and subcortical matter, whereas control subjects did not. These abnormalities were beyond the hamartoma itself, in areas of cerebrum that on visual inspection alone appeared completely normal. CONCLUSIONS: Extralesional abnormalities of cerebral structure are present in the cerebrum of patients with hypothalamic hamartoma, as in most patients with other dysgeneses. These abnormalities may explain the poor outcome of epilepsy surgery in patients with this form of dysgenesis. These preliminary findings require further investigation.

Adult↗