Biomedical subjects

Santiago Fernández

Publications and source records attributed to Santiago Fernández.

7 recordsLinked to original sources

PubMed2006

Early mitochondrial dysfunction in an infant with Alexander disease.

Alexander disease is a neurodegenerative disorder characterized by macrocephaly and progressive demyelination with frontal lobe preponderance. The infantile form, the most frequent variant, appears between birth and 2 years of age and involves a severe course with a rapid neurologic deterioration. Although magnetic resonance imaging is useful for diagnosis, currently diagnosis is confirmed by the finding of missense mutation in the glial fibrillary acidic protein (GFAP) gene. This case reports a female who presented at the age of 5 months with refractory epilepsy and hypotonia. Laboratory examinations, muscle biopsy examination, and energetic metabolic study in muscle indicated increased concentrations of lactate, mitochondria with structural abnormalities, and decreased cytochrome-c oxidase activity respectively. Later, both clinical course and magnetic resonance findings were compatible with Alexander disease, which was confirmed by the finding of a novel glial fibrillary acidic protein gene mutation.

Alexander Disease↗

PubMed2005

Brain dynamics of Arabic reading: a magnetoencephalographic study.

Arabic writing differs greatly from western scripts. To evaluate the influence of written Arabic on the pattern of language-related brain activation, a group of native Arab speakers and a control group of native Spanish speakers were scanned with magnetoencephalography during a reading task. In both groups, brain activity was strongly left lateralized during the time window between 200 and 500 ms after stimulus onset. During late latencies (beyond 500 ms), however, the right and the left hemispheres reached a similar activation level in the Arabic but not in the Spanish group. This suggests a time-dependent role of both hemispheres during Arabic language reading.

Adult↗

PubMed2005

Shifting-related brain magnetic activity in attention-deficit/hyperactivity disorder.

BACKGROUND: Current theories suggest a role for frontal-striatal circuits in the pathogenesis of attention-deficit/hyperactivity disorder (ADHD). METHODS: We used magnetoencephalography (MEG) to measure event-related brain activity during a simplified version of the Wisconsin Card Sorting Test in children with DSM-IV combined type ADHD (ADHD-C) or predominantly inattentive type ADHD (ADHD-PI) and in age- and intelligence-matched control children. RESULTS: In control children, set-shifting cues evoked a higher degree of activation in the medial temporal lobe (MTL) between 200 and 300 msec than non-shifting cues, with MTL activation predicting later activity in left anterior cingulate cortex (ACC) (at 400-500 msec). This MTL-ACC response pattern was diminished in children with ADHD. By contrast, children with ADHD showed early activity in regions barely activated in control children, such as left inferior parietal lobe and posterior superior temporal gyrus. CONCLUSIONS: These preliminary data support theories of frontal dysfunction in ADHD but also suggest that deficits in higher-level functions might be secondary to disruptions in earlier limbic processes.

Adolescent↗

PubMed2005

Is medial temporal lobe activation specific for encoding long-term memories?

Several neuroimaging studies have consistently demonstrated the critical involvement of prefrontal cortices and medial temporal lobes during long-term encoding. While the contribution of prefrontal lobes to working memory is well established, the role of the MTL structures remains controversial. To address this issue, we registered the neuromagnetic brain patterns of eight adult volunteers while they performed two working memory tasks (verbal and spatial) using magnetoencephalography (MEG). MEG recordings can provide real-time measures of brain activity, thus allowing detailed tracking of the time-course of brain activation during the encoding phase. We detected sustained and material-specific activity on the MTLs during the encoding phase of a working memory task, based on verbal and spatial information. Two peaks of activation were noted in the left MTL during word encoding in two non-consecutive time periods (500-600 ms and 700-800 ms after stimulus onset). Right MTL laterality was found for encoding locations when we collapsed activity sources in a wider time period (400-800 ms). In addition, we provided the spatiotemporal profiles of what seems to be two different brain circuits specific for each type of material. Finally, following an emerging conceptualization of working memory, we hypothesized that encoding processes mediated by the MTL to long-term memory would also apply to working memory.

Adult↗

PubMed2005

Activity in human medial temporal lobe associated with encoding process in spatial working memory revealed by magnetoencephalography.

Animal studies have suggested that working memory may be affected after lesions in the medial temporal lobe, although this assumption has not been corroborated by neuropsychological studies in humans. However, very recently, several functional neuroimaging studies in humans have successfully observed activation of the medial temporal lobe during working memory tasks. The main aim of this study was to investigate the contribution of the medial temporal lobe to the encoding process in spatial working memory. To address this issue we registered the neuromagnetic brain patterns of eight adult volunteers while they performed a spatial working memory task and more perceptual task using identical stimuli. After a initial phase (between 200 and 400 ms) without differences in activation, the medial temporal lobe showed a sustained activity, more evident in the right hemisphere, lasting up to 800 ms during the encoding stage of the spatial working memory task, while the activation in the perceptual task terminated earlier (approximately 400 ms after stimulus onset). The finding of a continued activation of the medial temporal lobe strongly suggests the contribution of this brain region to encoding operations in working memory.

Adult↗

PubMed2004

Time-modulated enhancing of the fronto-parietal circuits in the very-old elders.

Several studies have shown that memory circuits can be reorganised as a function of age. Brain magnetic activity evoked by a memory task was recorded in 19 healthy elderly subjects divided into two groups, a young-elder group (mean age of 62) and senior-elder group (mean age of 76). The young-elder group showed greater activity over the left medial temporal lobe in the late latency windows (between 400 and 800 ms) than the senior-elder group. The senior-elder group showed an initial increased activity (between 150 and 400 ms) over the complex of motor areas, followed by an increased activity in the left temporo-parietal cortex at the late latency window. These results revealed a reorganization of brain networks supporting memory in the eldest subjects. Furthermore, these circuits are reorganised in a time-dependent manner, meaning the rehearsal articulatory process active in the early time window followed by a phonological storage and recognition process in the late latency window.

Aged↗

PubMed2002

Spanish language mapping using MEG: a validation study.

The purpose of the present study was to compare magnetoencephalography (MEG) data with the results of the intracarotid amytal procedure (IAP). Twenty-one native Spanish-speaking patients with intractable epilepsy underwent MEG language mapping. A subset of 8 patients also underwent an IAP. With the exception of 2 patients who showed right hemisphere dominance, all other patients showed left hemisphere dominance for language on the MEG recording. The IAP findings were consistent with MEG results in 7 patients. The eighth patient who, according to the MEG data, had probable right hemisphere dominance for language did not show clear hemispheric specialization for language on the IAP and suffered a transient global aphasia following a right temporal lobotomy. These results suggests that MEG-based language mapping can play an important role in presurgical clinical evaluation.

Adult↗