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Biomedical subjects

J R Hesselink

Publications and source records attributed to J R Hesselink.

At least 19 recordsLinked to original sources

Callosal morphology concurs with neurobehavioral and neuropathological findings in two neurodevelopmental disorders.

To integrate neuroimaging, neuropathologic, and neuropsychological findings, computer-assisted morphometry was applied to magnetic resonance images of the corpus callosum in adolescents with Down and Williams syndromes and in control subjects. Callosa of subjects with Down syndrome were distinctively rounded in form, consistent with Down syndrome brachycephaly. These callosa also showed decreased widths throughout their rostral fifth, which serves frontal lobe projections. This finding correlates with the hypocellularity and hypofrontality of neocortex in subjects with Down syndrome and with their neuropsychological profile of frontal lobe dysfunction. Callosa of subjects with Williams syndrome generally resembled control specimens, in congruence with their frontal lobe structure and better preserved frontal lobe function. These results represent a convergence of findings across levels of neuroscientific investigation.

Adolescent

Fetal alcohol syndrome: a case report of neuropsychological, MRI and EEG assessment of two children.

Neuropsychological, neuroanatomical, and electrophysiological data are presented on two subjects with fetal alcohol syndrome (FAS). Both boys had intelligence quotients in the mentally deficient range and were found to have several other severe, specific deficits. Magnetic resonance imaging showed abnormalities of the corpus callosum, and reductions in the size of the basal ganglia and thalamic structures. No focal abnormalities were noted in the electroencephalogram records, although the electroencephalograms of both boys were moderately abnormal for their age group. A multidisciplinary approach to the study of FAS, hopefully will lead to a more unified concept of the disorder and perhaps indicate specific areas of vulnerability.

Adolescent

Incidence of AIDS dementia in a two-year follow-up of AIDS and ARC patients on an initial phase II AZT placebo-controlled study: San Diego cohort.

In a prospective study to determine the incidence of clinical dementia in patients with AIDS and ARC, 29 men and 3 women, 19 with ARC and 13 with AIDS, were examined neurologically and neuropsychologically every 6 months for 2 years during a placebo-controlled zidovudine (AZT) licensing trial. Most received two MRI brain scans. Although no patient was clinically demented at baseline, 9 (28%) developed dementia during the 2 years. Progression to dementia was associated with neuropsychological deterioration and with worsening on MRI during a preceding 6-month period, but not with baseline treatment group assignment. The results suggest that patients at CDC Stage IV who do not receive antiretroviral treatment earlier in their illness may develop clinical dementia at an annual rate of about 14%.

AIDS Dementia Complex

Specific neurobehavioral profile of Williams' syndrome is associated with neocerebellar hemispheric preservation.

Previous work demonstrated enlargement of the neocerebellar vermis in Williams' syndrome (WS), despite diminished volumes in the cerebral hemispheres. We present the first in vivo volumetric study of any structure within the cerebellar hemispheres. Using MRI, we identified and reliably measured the neocerebellar tonsils in WS subjects; Down's syndrome (DS) subjects matched for age, IQ, and cerebral volume; and age-matched normal controls. WS tonsils were equal in size to control tonsils and larger than DS tonsils. In proportion to the cerebrum, WS tonsils were larger than controls'. These results coincide with the remarkable neuropsychological preservation of language and affect in WS, despite general cognitive impairment. They contrast with the neocerebellar vermal hypoplasia seen in autism, with its communicative and affective deficits. Additionally, two WS subjects showed Chiari type I malformations, but the average tonsillar position in WS was not found to be different than in controls.

Adolescent

Cerebral structure on MRI, Part I: Localization of age-related changes.

In this report, earlier findings of age-related changes in brain morphology on magnetic resonance (MR) images are extended to include measurements of individual cerebral grey matter structures and an index of white matter degeneration. Volumes of caudate, lenticular, and diencephalic structures are estimated, as are grey matter volumes in eight separate cortical regions. Results suggest that between 30 and 79 years significant decreases occur in the volume of the caudate nucleus, in anterior diencephalic structures, and in the grey matter of most cortical regions. The data suggest that the volumes of the thalamus and the anterior cingulate cortex may be unchanged. Among those cortical regions found to be affected in aging, some evidence is present for greater change in association cortices and mesial temporal lobe structures. There are also dramatic age-related changes in the white matter, manifest as lengthened T2 values on MR images.

Adult

Cerebral structure on MRI, Part II: Specific changes in Alzheimer's and Huntington's diseases.

Using magnetic resonance (MR) imaging and morphometric techniques, groups of patients with Alzheimer's disease (AD) and Huntington's disease (HD) were compared with a large group of normal control subjects. Measures of volume loss in specific subcortical nuclei and eight cortical regions as well as an index of white matter abnormality were obtained. Results indicated expected widespread cortical volume reductions in AD, which were especially severe in mesial cortices; but comparable reductions were present in subcortical structures, particularly the thalamus. In HD, the greatest reductions were in striatal structures, but significant abnormalities were also detected in the thalamus and inferior cortical areas, especially in mesial temporal lobe structures. Significant degeneration in white matter was present in both groups, but was more dramatic in the HD patients. The significant diencephalic reduction in AD may make an important contribution to early memory deficits in the disorder, which are usually attributed to hippocampal damage. Similarly, damage to both the thalamus and mesial temporal lobe structures may play a role in the memory deficits of HD.

Aged

Cerebral structure on magnetic resonance imaging in language- and learning-impaired children.

Using magnetic resonance imaging 20 language- and learning-impaired children were compared with 12 normal control subjects. Gross brain structure was remarkably normal in the language- and learning-impaired group. Semiautomated morphometry was used to measure hemispheric volumes and cerebral asymmetries in six cerebral regions. The volume of the left posterior perisylvian region was significantly reduced in language- and learning-impaired children. Asymmetries in inferoanterior and superoposterior cerebral regions were also significantly different in this group. Results of descriptive group comparisons of estimated volumes of other cerebral gray-matter structures raise the possibility that some language- and learning-impaired children may have additional volume reductions in cortical and subcortical structures. The results suggest that hemispheric specialization of function may be anomalous in this population.

Brain

Magnetic resonance imaging abnormalities in lenticular nuclei and cerebral cortex in schizophrenia.

Neuropathologic and brain imaging studies have produced evidence of brain abnormalities in schizophrenic patients, often within the cerebrum's limbic lobe, and, less frequently, within basal ganglia. In the present study we used magnetic resonance imaging morphometric techniques to estimate volumes of specific cerebral structures in schizophrenic patients and age- and sex-matched normal controls. Estimates of the volume of mesial temporal lobe structures were reduced and estimates of the volume of the lenticular nucleus were increased in the schizophrenic patients. There was also evidence of reduced cranial volume in some schizophrenics. The magnitude of the lenticular abnormality, but not the temporal lobe abnormality, was associated with age at first psychiatric contact; earlier onset was associated with larger lenticular nuclei. The possible relevance of these results to neurodevelopmental hypotheses about the pathogenesis of schizophrenia is discussed.

Adolescent

Maturation of human cerebrum observed in vivo during adolescence.

In the present study using magnetic resonance imaging (MRI), age changes in the morphology of the cerebral cortex, greatest in the frontal and parietal convexities, were observed during adolescence. Results suggest that increases in cerebrospinal fluid (CSF) within the sulci of these cortical regions accompany grey matter decreases. Smaller reductions in volume are also observed in subcortical grey matter nuclei. These apparent grey matter volume reductions presumably reflect processes of late brain maturation. The changes may be related to decreasing neural plasticity.

Adolescent

Neuroradiology.

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Brain Diseases

Magnetic resonance imaging of AIDS-related polyradiculopathy.

AIDS-related polyradiculopathy is a syndrome associated with cytomegalovirus infection. We report two cases of AIDS-related polyradiculopathy in which spinal T1-weighted MRI with gadolinium-DTPA showed enhancement of the pial lining of the conus medullaris, cauda equina, and lumbar nerve roots. Both patients clinically improved with ganciclovir treatment.

Acquired Immunodeficiency Syndrome

Magnetic resonance imaging in the evaluation of vitreoretinal disease in eyes with intraocular silicone oil.

Media opacification in eyes filled with silicone oil makes the evaluation of recurrent retinal detachment difficult. Ultrasonography through silicone oil is subject to significant imaging artifacts. We performed magnetic resonance imaging on six patients with unilateral intravitreal silicone oil to determine if the technique would detect detached retina and subretinal oil. All patients had undergone pars plana vitrectomy with silicone oil injection for proliferative vitreoretinopathy; five patients had encircling solid silicone scleral buckles. In five patients the media were clear, and ophthalmoscopic findings were correlated with magnetic resonance findings. Four patients had recurrence of inferior retinal detachment; magnetic resonance imaging demonstrated subretinal oil in three of these patients. One patient had a concentric, shallow, anterior retinal detachment; magnetic resonance scanning demonstrated a globular hyperintensity suggestive of subretinal oil. In the sixth patient, who had an opaque cornea, magnetic resonance imaging suggested that the retina was attached preoperatively; this was confirmed at subsequent surgery. A chemical shift artifact was helpful in defining the contour of retinal detachments and the presence of subretinal oil by outlining the silicone oil within the eye.

Eye

Methods for measuring brain morphologic features on magnetic resonance images. Validation and normal aging.

In this article, methods for measuring brain morphologic features on magnetic resonance images are described and normative data are provided for six morphologic variables. An estimated function relating age (ranging from 8 to 79 years) to average values is given for each measure. A linear decrease over the age range is observed in the volume of the cerebrum. Linear increases are observed in measures of ventricular and sulcal fluid. A curvilinear decrease in cortical volume is found and is demonstrable even in young adults. Highly nonlinear increases in the volume of signal hyperintensities are observed in cortical and subcortical regions. The methods described may be used to provide an age-adjusted index of morphologic abnormality for each subject on any of the measures. They are currently in use in ongoing neurobehavioral studies of patients with nonfocal brain abnormalities and primary disorders of affect and cognition.

Adolescent

Subcortical abnormalities detected in bipolar affective disorder using magnetic resonance imaging. Clinical and neuropsychological significance.

Magnetic resonance imaging was utilized to determine the nature and rate of subcortical abnormalities in bipolar affective disorder. Nine of 19 bipolar patients and no controls demonstrated subcortical signal hyperintensities on blind evaluation of the images. There was no apparent change in the appearance of the hyperintensities in 7 of 7 subjects with abnormal magnetic resonance images who underwent repeated imaging at 1 year. Bipolar patients with abnormalities had a history of more hospitalizations and appeared more impaired on tests of fluency and recall when compared with bipolar patients without abnormalities or with controls. The possible etiology and significance of signal hyperintensities in bipolar affective disorder is discussed.

Adult

Prenatal diagnosis of a craniopharyngioma using ultrasonography and magnetic resonance imaging.

We report an infant with a craniopharyngioma which was detected in utero. Maternal uterine ultrasonography, done at 27 weeks because of polyhydramnios, revealed a 4 cm midline mass near the base of the fetal skull. At 31 weeks, magnetic resonance imaging of the maternal abdomen confirmed the presence of a mass in the region of the third ventricle and revealed hydrocephalus. Two days post-partum a computed tomography (CT)-guided needle biopsy of the mass was performed and recovered tissue which was histologically consistent with a craniopharyngioma. The infant's postnatal period was complicated by seizures, which were treated with phenobarbital, and by progressive hydrocephalus, necessitating placement of a ventriculo-peritoneal shunt. He also received therapy for central hypothyroidism and diabetes insipidus. The infant's parents refused permission for attempted resection of the tumour and he died at 8 weeks of age. This represents the second reported case of an antenatally detected craniopharyngioma. Four other cases of different intracranial tumours have been detected in utero using ultrasound, with no reported survivors past 3 days of age. There is a uniformly poor prognosis of such infants, but earlier diagnosis and intervention may change this result.

Craniopharyngioma

Fast imaging.

Gradient-echo pulse sequences are a valuable adjunct to the standard spin-echo sequences used for MR imaging of the brain and spine. Gradient-echo techniques enhance the signal of flowing fluids and increase the conspicuity of vascular lesions. They also increase the sensitivity of MR for chronic hemorrhage and calcification. Finally, steady-state techniques produce a myelographic effect to help evaluate degenerative diseases of the spine.

Arterial Occlusive Diseases

Primary myxoma of the posterior fossa.

A rare case of primary intracranial myxoma of the posterior fossa is described in a 32-year old woman. The patient presented with a history of headaches, disequilibrium, nausea and abnormal vision for several months. Computed tomography (CT) showed a well-demarcated hypodense mass. After contrast administration the mass appeared as an inhomogeneously enhancing lesion surrounded by a small rim of edema. The mass was surgically excised with histological examination revealing a true myxoma. Echocardiography revealed no evidence of a cardiac myxoma. The CT appearance of other case reports of non-cardiac myxomas in the literature is reviewed.

Adult