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Biomedical subjects

J Viaño

Publications and source records attributed to J Viaño.

At least 19 recordsLinked to original sources
PubMed2006

[Moyamoya disease: follow-up of 12 patients].

INTRODUCTION: We present 12 patients with moyamoya disease and their evolution from an early age to adulthood in some cases. MATERIAL AND METHODS: The patients (nine females and three males) were first studied before 10 years of age because of neurological disease. Bilateral carotid and vertebrobasilar arteriographies were performed during initial evaluation. In the patients who were followed-up during several or many years (in one, during 33 years and, in two, during 25 years), neuroimaging studies were done by magnetic resonance angiography (MRA). One patient also had neurofibromatosis type 1 (NF1). RESULTS: All patients showed bilateral occlusion of the supraclinoid portion of the internal carotid arteries. The basilar artery was markedly narrowed in one patient. One patient with very early onset of symptoms also showed collateral vascularization through the Bernasconi- Cassinari artery. Cerebral multifocal cortical and subcortical lesions were seen in the patients who had very long follow-up, although these already had appeared in the first MR studies. One patient showed arterial moyamoya 31 images and fibromuscular dysplasia. She was the only patient treated surgically during childhood with death after a short time. Another patient had a pregnancy and vaginal delivery at 26 years of age without complications. Images of moyamoya did not disappear in any of the patients in this series. Seizures were controlled with antiepileptic medication in all patients except in one who had NF1 and severe cerebral lesions. Although it is difficult to demonstrate the efficacy of calcium-antagonist medication (nicardipine), we found that it improved the neurological symptoms in most of our patients. CONCLUSIONS: Long-term follow-up of the patients in this series showed some neurological problems, although most of them did well, including those cases with early onset of symptoms. All adult patients in this series were working, although some of them in low qualified jobs. Collateral vascularization was very marked in all patients except in one who also showed fibromuscular dysplasia. Moyamoya images did not disappear in any patient. Ischemic cerebral lesions seem to be underlying the cause of the neurological sequelae. Seizures responded well to antiepileptic medication.

Child↗
PubMed2005

Evidence of biochemical and biomagnetic interactions in Alzheimer's disease: an MEG and MR spectroscopy study.

BACKGROUND: Several neuroimaging studies have shown reliable differences between Alzheimer's disease (AD) patients and age-matched controls. However, few studies have demonstrated the interactions between neuroimaging methods for the diagnoses of AD. OBJECTIVE: In this study, we try to elucidate the complementary nature of magnetoencephalography (MEG) and magnetic resonance spectroscopy (MRS) examinations in the assessmentof AD. METHODS: Ten patients fulfilling the NINCDS-ADRDA criteria of probable AD, and 10 elderly individuals with no history of neurological or psychiatric illness serving as age-matched controls participated in the study. All patients and controls received an MRS, MEG and neuropsychological assessment. MEG data were obtained in the context of a working memory task, previously utilized in a similar sample of patients. RESULTS: The AD group showed a reduced number of activity sources over left temporoparietal areas during the late portion of the evoked magnetic field (between 400-800 ms), as well as a bilateral temporoparietal increase in creatine and myoinositol concentrations, and in the myoinositol/N-acetyl-aspartate ratio. The combination of the variables 'number of dipoles during the late portion of the evoked magnetic field' and 'myoinositol/N-acetyl-aspartate ratio' accounted for 65% of the variance of the Mini Mental State Examination scores. CONCLUSIONS: These results highlight the importance of assessing the complex brain pathology underlying AD by utilizing multiple brain examination modalities in a coordinate approach.

Aged↗
PubMed2005

[Glutaric aciduria type 1 with normal evolution: follow-up of one case until adult age].

We present a patient of 20 years of age with glutaric aciduria type 1 (GA1) and normal psychomotor development. Her symptoms consisted of a few convulsions between 2.5 and 4.5 years of age. She was diagnosed at 9 years of age because of the typical alterations of GA1 that appeared in computed tomography and magnetic resonance (MR) imaging studies. Enzymatic activity in fibroblasts culture was nonexistent and glutarate excretion was elevated in the annual controls where this was investigated from the diagnosis of the disease so far. MR studies showed hyposignal in T1 of the subcortical white matter, severe dilatation of the Sylvian region and temporal fossa subarachnoid spaces, and hypoplasia of the subjacent cerebral parenchyma and of both temporal lobes. The corpus callosum and the surrounding zones appeared very enlarged and with signal changes. Spectroscopic MR showed signs of membrane instability and cellular impoverishment in subcortical white matter and basal ganglia and presence of lactic acid. Macrocephaly always maintained centiles over 98. The patient has no abnormal movements or motor disturbances, her behavior and intelligence being normal and she is able to follow studies of middle level.

Basal Ganglia↗
PubMed2003

[Extracranial and intracranial vascular abnormalities and nevus of Ota in the same family].

We present a 17 year old patient suffering from hemangioma on the left side of his face and neck. The evolution of his disease has been followed-up since he was 8 1/2 months old. His mother has nevus of Ota in the sclera and adjacent zones in both sides. At the age of 8 1/2 months, the patient had an ischemic stroke in the territory of the left middle cerebral artery during the surgical correction of the aortic arch coarctation. The conventional arteriogram performed at 10 months of life showed several malformations of the intracranial and extracranial arteries. At the age of 16 years the cutaneous hemangioma was atrophic and a new angiographic study by magnetic resonance arteriography (MRA) showed all the vascular anomalies with higher objectivity than the conventional arteriography, especially the absence of some arteries and the abnormal position of some others. Moreover it could detect the decrease of the caliber of the intracranial arteries and the disappearance of hemangiomatous malformations even though the patient had not received any treatment for the cutaneous hemangioma. We think that this syndrome, which still has no definite name, has a parallel spontaneous biological process with progressing vascular size during the first months or years of life and, later, the vessels regress slowly but progressively, leading to a small caliber or even the occlusion of the vessels of the cutaneous hemangioma and of the abnormal extracranial and intracranial arteries. At the same time, there is a slow but progressive presence of transdural collateral vascularization of the brain. We do not find any relationship between the hemangiomatous syndrome of the patient and the nevus of Ota of his mother.

Adolescent↗
PubMed2002

Proton magnetic resonance spectroscopy in dementia with Lewy bodies.

Proton magnetic resonance spectroscopy ((1)H-MRS) provides a non-invasive, in vivo insight into the brain metabolism, and has been successfully used in several neurological conditions. Our objective was to characterise the cerebral metabolic changes in dementia with Lewy bodies (DLB) patients using (1)H-MRS. Single Voxel (1)H-MRS was performed in 12 DLB patients with mild to moderate symptoms and 11 age-matched healthy controls. Volumes of interest (VOI) were selected, including white matter (WM) in the centrum semiovale and grey matter (GM) in the parasagittal parietal cortex. Main metabolic peaks corresponding to N-acetylaspartate (NAA), glutamate/glutamine (Glx), choline-containing compounds (Cho), myo-inositol (Ins), and creatine plus phosphocreatine (Cr) were identified. These areas were measured and referred to that of the water. Metabolic ratios among the different peak areas were also calculated. In comparison with the control group, DLB patients showed significantly lower mean NAA/Cr, Glx1/Cr and Cho/Cr ratios in the WM, while their Ins/Cr and Ins/NAA ratios did not differ from those of the control group. In the GM, no significant differences were found between both groups. Correlations between age at onset, disease duration, Mini-Mental State Examination, the motor section of the Unified Parkinson's Disease Rating Scale, Hoehn and Yahr staging and metabolic ratios, both for WM and GM, were not significant in DLB patients. Our spectroscopy data show WM involvement, along with GM preservation, in DLB patients with early or intermediate stages. Hence, (1)H-MRS may provide adjunctive information in the ante-mortem diagnosis of DLB.

Aged↗
PubMed2001

Unilateral polymicrogyria: a common cause of hemiplegia of prenatal origin.

We describe a series of 13 patients, ten males and three females, with ages ranging between three and fourteen years, who show unilateral polymicrogyria (in the left cerebral hemisphere in four cases and in the right in nine). The first and the main clinical alteration in all 13 cases was hemiparesis that did not change along the years. Ten patients had seizures that were completely controlled in seven. Three cases never presented seizures. Spontaneous threat of abortion or arterial hypertension during the first five months of pregnancy occurred in five cases. Weight less than 3 kg at birth was observed in six cases. The only patient who shows severe mental retardation associated with deafness is a girl who had infantile spasms at 20 days of age and presents duplication of a short arm of the chromosome X. Conventional magnetic resonance (MR) studies, performed in four cases did not disclose polymicrogyria but only an enlarged cortex that was diagnosed as cortical dysplasia. Three-dimensional MR (3DMR) images are very important not only to see the polymicrogyria, but also its extension and severity, especially if, moreover the axial, coronal and sagittal views, oblique, frontal, occipital, basal and superior images of the hemispheric cortical surface are performed.

Adolescent↗
PubMed2000

Generalized nerve sheath tumors in neurofibromatosis type 1 (NF1). A case report.

We describe a case of chronic distal sensorimotor neuropathy associated with neurofibromatosis type 1 (NF1) in a 15-year old girl. The patient showed a striking clinical picture consisting of diffuse nodular enlargements of peripheral nerves. Motor conduction velocities were decreased and sensory responses were absent after maximal stimulation. Magnetic resonance imaging (MRI) was performed throughout the body and disclosed generalized nerve sheath neurofibromas affecting all peripheral nerves. Intracranially, the patient had a glioma of the left optic nerve, but no other cranial nerve involvement. These results demonstrate the value of MRI for visualization of the peripheral nervous system in neurofibromatosis.

Child, Preschool↗
PubMed2000

[Posttraumatic intention tremor. Report of a case with a lesion in the mesencephalic area].

We present a child who suffered severe coma because of an automovile accident remaining two months in coma with brain stem dysfunction and one and a half additional months until he was able to recognize people. After recovering, he showed severe kinetic tremor in the upper right extremity and discrete right hemiparesis as well as a moderate clinical cerebellar disfunction. Magnetic resonance (MR) study, performed 1 and 3 years after the coma, showed normal images of the cerebellum and thalami, while it disclosed signal changes in the mesencephalic region next to both red nuclei, being more severe in the right side. It is suggested that the kinetic tremor can be related with location of the lesion near red nucleus.

Brain Injuries↗
PubMed1999

Clinical correlation of striatal 1H MRS changes in Huntington's disease.

OBJECTIVE: To study the clinical significance of metabolic alterations as measured in vivo with proton MRS in the striatum of patients with Huntington's disease (HD). METHODS: Localized, single-voxel MRS was performed on the basal ganglia of 10 HD patients (4 presymptomatic gene carriers and 6 akinetic patients) and 5 age-matched healthy individuals. Metabolite quantification was performed by referring the areas of the respective spectral peaks to that of water in the analyzed voxel. The spectroscopic findings were correlated with motor and cognitive performance in several specific tests and with the length of the CAG repeat expansion normalized for age. RESULTS: N-acetylaspartate (NAA) and creatine were reduced markedly in both groups of patients, particularly in the advanced group (approximately 60%), but the decrease was also significant in presymptomatic patients (approximately 30%) whose motor and cognitive performances were within the normal range. Both metabolites correlated highly with the motor score of the Unified Huntington's Disease Rating Scale and with computed measurements of saccadic and tapping speed. Creatine reduction was also well correlated with performance in cognitive timed tasks and with the length of CAG expansion (r = -0.81). CONCLUSION: The creatine signal appears to be an interesting marker for progression in HD and could be useful in assessing therapeutic outcome, particularly during the initial stages when most clinical indices are still within the normal range.

Adult↗
PubMed1999

In vivo proton magnetic resonance spectroscopy of diseased prostate: spectroscopic features of malignant versus benign pathology.

In vivo Proton Magnetic Resonance Spectroscopy appears potentially useful for non-invasive discrimination between benign prostatic hyperplasia (BPH) and prostate carcinoma (PC). Aiming to delimit the range within which spectra from one or the other pathology should occur, and establish extreme spectroscopic features of malignant versus benign prostate disease, we performed endorectal proton MR spectroscopy on 20 patients severely affected of either benign prostatic hyperplasia (BPH) (n = 10) or prostate cancer (PC) (n = 10). They were selected on the basis of the large volume and homogeneity of their lesions, which were histologically confirmed after spectroscopy. Consequently, high-quality short-TE proton spectra with well-resolved metabolite signals, and practically free of volume averaging issues were obtained in all cases. Apart from the typical citrate, creatine, and choline signals of prostate spectra, both BPH and PC spectra showed a peak centered at 3.6 ppm which was assigned to myo-inositol. The intensity of this contribution was found significantly increased in PC cases compared to BPH. Possible relationships between neoplastic transformation and the metabolic pathways in which myo-inositol participates are discussed. Average spectroscopic profiles were calculated for both advanced pathologies, and showed obvious differentiated features. In quantitative terms, the ratio of citrate to choline peak areas as well as that of creatine to myo-inositol appeared as the most convenient to discriminate between advanced PC cases (both ratios below 1.0) and advanced BPH cases (both ratios above 1.0).

Aged↗
PubMed1998

[The value of images in diagnosis of neuron migration disorders].

OBJECTIVE: To present the fitest classification and the imaging peculiarities of the malformations of cortical development, most of which have been related with the epilepsy origin. METHODS: The study is based on an anatomical-histological classification scheme that shows three great groups of malformations of cortical development: 1. Malformations due to abnormal neuronal and glial proliferation. 2. Malformations due to abnormal neuronal migration. 3. Malformations due to abnormal cortical organization. RESULTS: The result of these abnormalities of the cortical development is the presence of several anatomical histological entities, actually perfectly identified by the magnetic resonance (MR), especially with the new high resolution methods. The most frequent entities, such as polymicrogyria, lissencephaly, pachygyria, schizencephaly, cerebral heterotopia (cortical, subcortical or subependymal), and other rarer types are reviewed according with the numerous references of the literature and the findings observed in the cases of our series of about one hundred patients which includes cases of every type of malformation. CONCLUSION: MR is a conclusive study in order to identify and classify the malformations of cortical development, most of which are associated with neurological disturbances: epilepsy, mental retardation, language and/or behavioral problems or motor dysfunction.

Brain↗
PubMed1998

[The Leydig-cell testicular tumor. A case report with magnetic resonance images].

OBJECTIVE: An additional case of testicular Leydig cell tumor in a young man is reported. The clinical presentation included gynecomastia and a testicular mass. METHODS/RESULTS: Hormonal studies, testicular US and high resolution MRI were performed before orchidectomy. The histopathological findings were consistent with Leydig cell tumor. A CT scan revealed no metastasis. The patient is clinically disease-free 18 months after orchidectomy. CONCLUSION: High resolution MRI must be performed only when clinic and US findings are discordant.

Adult↗
PubMed1997

Unilateral somatic and intracranial hypoplasia.

We report on clinical and MR findings in a woman with a peculiar disease. She was studied from childhood to adulthood. She showed several anomalies affecting structures of ectodermal and mesodermal origin. Specific defects included right cerebral and cerebellar hypoplasia, right cerebral cortical polymicrogyria, agenesis of the corpus callosum, right micro-ophthalmia and cataract, right breast hypoplasia, right upper and lower extremity hypoplasia, bilateral acral alterations affecting especially the middle phalanges, left hemifacial hypoplasia (probably secondary to the severe contralateral cerebral hemisphere lesion), mental retardation and partial epilepsy. MR disclosed right cerebral and cerebellar hypoplasia, right cerebral cortical polymicrogyria, agenesis of corpus callosum and right micro-ophthalmia. She did not exhibit either orbital cysts or cutaneous abnormalities. The complex symptomatology of unknown origin presented by this patient suggests a new developmental malformation consisting in hypoplasia of a complete hemibody.

Abnormalities, Multiple↗
PubMed1997

Comparison of digital rectal examination, transrectal ultrasonography, and multicoil magnetic resonance imaging for preoperative evaluation of prostate cancer.

OBJECTIVE: A prospective study was designed to compare the potentials of digital rectal examination (DRE), transrectal ultrasound (TRUS), and magnetic resonance imaging (MRI) using integrated endorectal and pelvic phased-array coils for preoperative estimation of tumor volume and local extent of prostate cancer. METHODS: Evaluation of 20 consecutive patients undergoing radical retropubic prostatectomy included DRE, TRUS with a 7.5-MHz transducer, and MRI on a 1.5-tesla GE Signa system. Step sections (5 mm) of the entire specimen were performed, and tumor volume and percentage of gland involved were calculated. RESULTS: DRE, TRUS, and endorectal and pelvic phased-array MRI showed 50, 75, and 95% of the cancers, respectively. There was a linear correlation on MRI between predicted tumor volume and pathological tumor volume (r = 0.82, p < 0.0001), but not between predicted volume on DRE or TRUS and real volume. The accuracy for detecting extracapsular penetration was 60% for DRE and TRUS and 79% for MRI. The accuracy for detecting seminal vesicle invasion was 60% for DRE, 66 for TRUS, and 89% for MRI. The negative predictive value for extracapsular and seminal vesicle extension was highest for MRI (85 and 93%, respectively). The accuracy for tumor location in the apex of the prostate was 30% for DRE, 47 for TRUS, and 89% for MRI. CONCLUSIONS: MRI with integrated endorectal and pelvic phased-array coils satisfactorily predicted tumor volume and tumor extent preoperatively. Multicoil MRI can assist in decision making as it is valuable in the definition of patients that may benefit from surgery and can be of help for evaluating the risk of a positive margin, especially in the apical resection.

Aged↗
PubMed1997

[Unilateral agenesis of the internal carotid artery in childhood: description of a case].

INTRODUCTION: Unilateral or bilateral agenesis of the internal carotid artery has not often been described in children since it is usually asymptomatic. CLINICAL CASE: We describe a case of isolated unilateral agenesis of the internal carotid artery in a child of 20 months old who presented with two episodes of status convulsives, the first after a head injury and the second when he was febrile. The anomaly was a casual finding, diagnosed on cerebral magnetic resonance when there was no signal corresponding to carotid blood flow. It was confirmed by echography-doppler and angiographic magnetic resonance. We also describe the neuro-radiological findings which led to the diagnosis of this anomaly. CONCLUSIONS: We consider that unilateral agenesis of the carotid artery was a casual finding in this case. The provisional diagnosis may be made when there is absence of vascular signals on conventional magnetic resonance.

Carotid Artery, Internal↗