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At least 1,315 records · Page 73Linked to original sources
PubMed2005

Krabbe disease treated with hematopoietic stem cell transplantation: serial assessment of anisotropy measurements--initial experience.

PURPOSE: To prospectively compare diffusion-tensor magnetic resonance (MR) imaging anisotropy measurements of white matter (WM) regions in early and late treatment groups of Krabbe disease patients treated with stem cell transplantation. MATERIALS AND METHODS: The study was approved by the Institutional Review Board and was compliant with Health Insurance Portability and Accountability Act; informed consent was obtained from the families of all patients. Patients with early-onset Krabbe disease (four girls and three boys) underwent diffusion-tensor MR imaging before and after stem cell transplantation. Fractional anisotropy (FA) values from serial studies were compared in patients who underwent transplantation at less than 1 month (early group, two girls and one boy) and those who underwent transplantation at 5-8 months (late group, two girls and two boys). FA values were measured in the genu and splenium of the corpus callosum, the frontal WM, and the internal capsule; were compared with those of five age-matched children in the comparison group (normal MR images and no proved neurologic disease); and were expressed as a ratio. Images obtained after transplantation were evaluated at approximately 1 (n = 7), 2 (n = 6), 3 (n = 1), and 4 (n = 1) years. RESULTS: Before transplantation, mean FA ratios in the early group for all four WM regions ranged between 97% and 117%. At 1 year, mean FA ratios at all locations were either 92% or 93%. At 2 years after transplantation, mean FA ratios were between 83% and 92%. In one patient imaged at 3 years, the mean FA ratio was 97%; in another patient imaged at 4 years, the mean FA ratio was 77%. Before transplantation, mean FA ratios in the late group ranged between 55% and 74%. Mean FA ratios were between 37% and 50% at 1 year after transplantation and between 36% and 39% at 2 years. CONCLUSION: All patients had decreases in FA ratios over time. The early group had higher initial FA ratios and lower subsequent decreases, which may indicate amelioration of the dysmyelinating process.

Anisotropy↗
PubMed1980

A new CT pattern in adrenoleukodystrophy.

A new CT pattern was observed in 2 patients with adrenoleukodystrophy (ALD). This pattern, which the authors call Type II, is characterized by the absence of posterior periventricular areas of decreased attenuation around the trigone on non-contrast scans: after contrast infusion, however, there is striking enhancement of various white-matter structures (tracts or fiber systems) such as the internal capsules, corpus callosum, corona radiata, forceps major, and cerebral peduncles. This is different from numerous previous descriptions of the CT pattern in ALD. Type II ALD does not appear to have been seen in any other leukoencephalopathy and is probably specific for a phenotypic variant or an evolving stage of ALD.

Child↗
PubMed1991

Portal-systemic encephalopathy: presence of basal ganglia lesions with high signal intensity on MR images.

Sixteen patients with cirrhosis of the liver underwent cranial magnetic resonance (MR) imaging and transarterial portography to evaluate the relationship between basal ganglia lesions and portal-systemic collateral vessels. No neuropsychiatric disturbance was observed in any of the patients at the time of the MR examination, but four patients with portal-systemic encephalopathy were included in the study. Basal ganglia lesions, characterized by increased signal intensity on T1-weighted MR images, were observed in nine of the 16 patients, including the four with portal-systemic encephalopathy. These nine patients had large portal-systemic collateral vessels that were more than 10 mm in diameter. These collateral vessels were receiving blood from the superior mesenteric vein (SMV) in all nine patients. The lesions involved the globus pallidus and portions of internal capsules in a bilateral and symmetric fashion and did not exhibit mass effect. The authors conclude that there may be a significant relationship between high-intensity basal ganglia lesions and large portal-systemic collateral vessels receiving blood from the SMV.

Aged↗
PubMed1995

Amyotrophic lateral sclerosis: correlation of clinical and MR imaging findings.

PURPOSE: To determine the magnetic resonance (MR) imaging characteristics of amyotrophic lateral sclerosis (ALS) and to evaluate possible correlations between the disease severity and the MR imaging findings. MATERIALS AND METHODS: The authors retrospectively reviewed MR images of the head in 17 patients with ALS (14 men and three women) and 17 age-matched control subjects. The corticospinal tract (CST) was analyzed for the following features: size, signal intensity, and number of sections and regions in which the CST could be seen. RESULTS: Patients with ALS demonstrated sharp, well-defined, round, symmetric lesions that were hyperintense to gray matter within the CST. The lesions were best seen at the level of the middle or lower internal capsule on T2-weighted images. Visualization of the CST on proton-density-weighted images (which occurred in eight ALS patients) is the most reliable MR finding. Low signal intensity was identified within the motor cortex in six patients. Positive MR findings correlated with average or rapid progression of the disease. CONCLUSION: Increased signal intensity in the CST on proton-density-weighted MR images is diagnostic for motor neuron disease and correlates with the rate of disease progression. Subtle ALS changes can be differentiated from the normal CST. Low signal intensity in the motor cortex on T2-weighted images is a useful finding.

Adult↗
PubMed1995

Neurofibromatosis type 1: pathologic substrate of high-signal-intensity foci in the brain.

PURPOSE: To investigate a correlation between pathologic and radiologic findings with regard to the characteristic high-signal-intensity foci seen on long repetition time (TR) magnetic resonance (MR) images of the brain in patients with neurofibromatosis type 1 (NF-1). MATERIALS AND METHODS: Three girls with NF-1 and abnormal hyperintensities on long TR images of the brain underwent pathologic examination at autopsy. RESULTS: Two 10-year-old girls had classic, focal hyperintensities in the internal capsules and globus pallidus regions, which have been associated with NF-1. The third patient, a neonate, had diffuse hyperintensity of the supratentorial and infratentorial white matter on T2-weighted MR images. Findings at histopathologic examination revealed spongiotic change in the tissue sections that correspond to the high-signal-intensity foci demonstrated on T2-weighted images. CONCLUSION: Hyperintense foci seen on T2-weighted MR images appear to correspond to pathologic findings of areas of vacuolar or spongiotic change. The resultant fluid-filled vacuoles explain the occurrence of high signal intensity demonstrated on T2-weighted images.

Brain↗
PubMed1995

Microscopic disease in normal-appearing white matter on conventional MR images in patients with multiple sclerosis: assessment with magnetization-transfer measurements.

PURPOSE: To assess for the presence of microscopic abnormalities in otherwise normal-appearing white matter on T2-weighted images in patients with multiple sclerosis (MS) by using magnetization-transfer (MT) measurements. MATERIALS AND METHODS: Twenty-three patients with MS and nine healthy control subjects underwent magnetic resonance (MR) imaging with a 1.5-T system. MT ratios (MTRs) were measured in 16 areas of normal-appearing white matter identified on conventional T2-weighted images in patients with MS. MTRs in the same white-matter locations in control subjects were determined. The two groups were compared. RESULTS: The mean whole-brain MTR of normal-appearing white matter in patients was 40.13% +/- 1.37 (standard deviation; range, 36.31%-42.09%); in control subjects, 42.93% +/- 0.95 (range, 41.62%-44.50%). The difference was statistically significant (P < or = .001). Except for the internal capsules, the difference in mean MTRs in each sublocation of the brain between patients and control subjects was statistically significant (P < or = .05). CONCLUSION: MT measurements are more sensitive than conventional MR imaging in the detection of abnormalities beyond the resolution of T2-weighted imaging.

Adult↗
PubMed1998

Congenital muscular dystrophy: use of brain MR imaging findings to predict merosin deficiency.

PURPOSE: To evaluate the brain magnetic resonance (MR) imaging findings in patients with the "classic" form of congenital muscular dystrophy (patients with normal intelligence) in relation to the absence of merosin, a recently identified molecular component in the basement membrane of muscle fiber. MATERIALS AND METHODS: Brain MR images in 15 patients (13 children, two adults) were reviewed and correlated with the patient's merosin status. Merosin was evaluated by means of immunocytochemical study of specimens from muscle biopsy. RESULTS: Nine patients had merosin deficiency. All patients had diffuse white matter alterations similar to those seen in cases of leukodystrophy. Periventricular and subcortical white matter were involved. The corpus callosum and internal capsule were spared. Follow-up MR images were available in two patients; changes were nonprogressive. White matter signal intensity was normal in the six patients with normal uniform labeling against merosin. Ventricular dilatation and cortical atrophy were observed in both groups. CONCLUSION: Diffuse white matter changes resembling those seen with leukodystrophy may be a valuable criterion for diagnosis of merosin deficiency in patients with classic congenital muscular dystrophy.

Adult↗
PubMed1998

Normal brain in human newborns: apparent diffusion coefficient and diffusion anisotropy measured by using diffusion tensor MR imaging.

PURPOSE: To establish quantitative standards for the directionally averaged water apparent diffusion coefficient (D) and quantitative diffusion anisotropy (A sigma) of normal brains in newborns by using diffusion tensor magnetic resonance (MR) imaging. MATERIALS AND METHODS: Diffusion tensor MR imaging was performed during the first 36 hours of life in 22 newborns (gestational age range, 31-41 weeks). Values of D and A sigma were measured in regions of interest chosen in the cortical gray matter, centrum semiovale, caudate nuclei, lentiform nuclei, thalami, internal capsules, and cerebellar hemispheres. RESULTS: The D values in the gray and white matter in newborns are considerably higher than those in adults. There is a striking correlation between gestational age and D, with D decreasing as gestational age increases. The A sigma values in the white matter in newborns are lower than those in adults. Values of A sigma show statistically significant correlations with gestational age only in the white matter of the centrum semiovale, in which A sigma values increase sharply near term. CONCLUSION: The D values primarily reflect overall brain water content. The A sigma values are more sensitive to tissue microstructure (e.g., white matter packing and myelination). The D and A sigma images reveal information and not apparent on T1- and T2-weighted images.

Adult↗
PubMed2001

Quantitative apparent diffusion coefficient measurements in term neonates for early detection of hypoxic-ischemic brain injury: initial experience.

PURPOSE: To determine the utility of using quantitative apparent diffusion coefficient (ADC) values as an objective means of early detection of brain injury caused by hypoxic-ischemic encephalopathy (HIE) in term neonates. MATERIALS AND METHODS: Conventional images, diffusion-weighted images, ADC maps, and clinical charts from 13 term neonates clinically suspected of having HIE were retrospectively reviewed. Four term neonates without HIE served as control subjects. ADC values were calculated in predefined regions in patients and compared with those in control subjects. A Student t test was performed for each region to compare patients and control subjects. RESULTS: Abnormalities were more easily detected on diffusion-weighted images and ADC maps, compared with conventional images. ADC values in patients with HIE were significantly different from those of control subjects in the posterior limb of the internal capsule, corona radiata, posterior frontal white matter, and parietal white matter bilaterally. CONCLUSION: Evaluation of ADC maps can improve conspicuity of hypoxic-ischemic injury in the acute and/or subacute setting (within 12 days of insult), and calculation of ADC values can provide an objective measure of hypoxic-ischemic injury.

Diffusion↗
PubMed1975

Separate lateral hypothalamic pathways for extracellular and intracellular thirst.

Small lesions of the midlateral zone of the lateral hypothalamus in rats attentuated water intake elicted by the central microinjection angiotensin or by the peripheral injection of isoproterenol or renin without attenuating drinking to peripherally administered hypertonic saline. Lesions placed further lateral in the hypothalamus, which destroyed the medial aspects of the internal capsule and globus pallidus, produced a marked decrease in water intake induced by hypertonic saline. Abaltion of the ventromedial nucleus of the hypothalamus increased drinking elicited by angiotension, isoproterenol, or renin. These results suggest that extracellular and intracellular thirst stimuli are mediated by separate neural pathways at the level of the lateral hypothalamus.

Angiotensin II↗
PubMed2006

Computational analysis of subthalamic nucleus and lenticular fasciculus activation during therapeutic deep brain stimulation.

The subthalamic nucleus (STN) is the most common target for the treatment of Parkinson's disease (PD) with deep brain stimulation (DBS). DBS of the globus pallidus internus (GPi) is also effective in the treatment of PD. The output fibers of the GPi that form the lenticular fasciculus pass in close proximity to STN DBS electrodes. In turn, both STN projection neurons and GPi fibers of passage represent possible therapeutic targets of DBS in the STN region. We built a comprehensive computational model of STN DBS in parkinsonian macaques to study the effects of stimulation in a controlled environment. The model consisted of three fundamental components: 1) a three-dimensional (3D) anatomical model of the macaque basal ganglia, 2) a finite element model of the DBS electrode and electric field transmitted to the tissue medium, and 3) multicompartment biophysical models of STN projection neurons, GPi fibers of passage, and internal capsule fibers of passage. Populations of neurons were positioned within the 3D anatomical model. Neurons were stimulated with electrode positions and stimulation parameters defined as clinically effective in two parkinsonian monkeys. The model predicted axonal activation of STN neurons and GPi fibers during STN DBS. Model predictions regarding the degree of GPi fiber activation matched well with experimental recordings in both monkeys. Only axonal activation of the STN neurons showed a statistically significant increase in both monkeys when comparing clinically effective and ineffective stimulation. Nonetheless, both neural targets may play important roles in the therapeutic mechanisms of STN DBS.

Animals↗
PubMed2003

Effects of high-frequency stimulation in the internal globus pallidus on the activity of thalamic neurons in the awake monkey.

The reduction in symptoms of Parkinson's disease produced by high-frequency stimulation (HFS) in the internal globus pallidus (GPi) has been proposed to be due to stimulus-induced inactivation of pallidal neurons and resulting disinhibition of thalamic neurons. We tested this in awake Macaca fascicularis by stimulating between pairs of electrodes inserted into GPi under electrophysiological control and recording the responses evoked in thalamic neurons. HFS produced a reduction, not an increase, in discharge frequency during the stimulus train in 77% of the responsive thalamic neurons. Only 16% of the responsive cells showed an increase in discharge during stimulation and, for some of these, stimulation at a similar intensity produced contralateral muscle contraction, a probable sign of current spread to the internal capsule. The few thalamic neurons studied during bursting had a reduction in burst frequency and duration during HFS. We conclude that high-frequency stimulation within GPi does not necessarily facilitate thalamic discharge, and it may act, instead, to interrupt abnormal patterns of thalamic discharge associated with parkinsonian symptoms.

Action Potentials↗
PubMed2002

Regulation of the timing and pattern of action potential generation in rat subthalamic neurons in vitro by GABA-A IPSPs.

The regulation of activity in the subthalamic nucleus (STN) by GABAergic inhibition from the reciprocally connected globus pallidus (GP) plays an important role in normal movement and disorders of movement. To determine the precise manner in which GABAergic synaptic input, acting at A-type receptors, influences the firing of STN neurons, we recorded the response of STN neurons to GABA-A inhibitory postsynaptic potentials (IPSPs) that were evoked by supramaximal electrical stimulation of the internal capsule using the perforated-patch technique in slices at 37 degrees C. The mean equilibrium potential of the GABA-A IPSP (EGABA-A IPSP) was -79.4 +/- 7.0 mV. Single IPSPs disrupted the spontaneous oscillation that underlies rhythmic single-spike firing in STN neurons. As the magnitude of IPSPs increased, the effectiveness of prolonging the interspike interval was related more strongly to the phase of the oscillation at which the IPSP was evoked. Thus the largest IPSPs tended to reset the oscillatory cycle, whereas the smallest IPSPs tended to produce relatively phase-independent delays in firing. Multiple IPSPs were evoked at various frequencies and over different periods and their impact was studied on STN neurons held at different levels of polarization. Multiple IPSPs reduced and/or prevented action potential generation and/or produced sufficient hyperpolarization to activate a rebound depolarization, which generated a single spike or restored rhythmic spiking and/or generated a burst of activity. The pattern of IPSPs and the level of polarization of STN neurons were critical in determining the nature of the response. The duration of bursts varied from 20 ms to several hundred milliseconds, depending on the intrinsic rebound properties of the postsynaptic neuron. These data demonstrate that inhibitory input from the GP can produce a range of firing patterns in STN neurons, depending on the number and frequencies of IPSPs and the membrane properties and voltage of the postsynaptic neuron.

Action Potentials↗
PubMed1985

Microstimulation of the primate neostriatum. I. Physiological properties of striatal microexcitable zones.

Microstimulation was carried out at over 1,250 sites in the putamen in four unanesthetized rhesus monkeys. At numerous sites, microstimulation resulted in movements of individual body parts including leg, arm, and face. Microstimulation-evoked limb movements were invariably contralateral to the stimulating electrode. In nearly all instances, the response at threshold was restricted to or maximal about a single joint. A small percentage of stimulation-evoked axial and orofacial movements were bilateral. The same motor response was frequently evoked over distances of up to 1,200 micron along a single penetration, suggesting that a relatively homogeneous motor-response zone underlies the observed micro-stimulation effects. We have designated these presumptive functional units striatal microexcitable zones (SMZ). The boundaries of adjacent SMZ involved in different movements frequently appeared to overlap. Amplitude, velocity, and acceleration of microstimulation-evoked elbow movements were assessed quantitatively. With increasing stimulus current, each of these parameters increased monotonically until saturation occurred. The spread of intrastriatal microstimulation currents was found to be comparable to that reported for motor cortex. The effective radius of 40-microA putamen microstimulation currents was estimated to be approximately 150 micron. This effectively rules out the possibility of current spread to the internal capsule. Microstimulation effects were abolished by fiber-sparing lesions produced by microinjections of the neurotoxin ibotenic acid. Moreover, chronaxie measurements in putamen (327 +/- 47 microseconds) were significantly higher than for capsular stimulation (150 +/- 32 microseconds). These observations are consistent with the proposal that movements evoked by putamen microstimulation resulted from activation of putamen output neurons. On the other hand, a possible contribution from the antidromic activation of corticostriate afferent terminals or axons cannot be excluded.

Animals↗
PubMed1992

Neuronal activity in monkey striatum related to the expectation of predictable environmental events.

1. This study investigated neuronal activity in the striatum preceding predictable environmental events and behavioral reactions. Monkeys performed in a delayed go-nogo task that included separate time periods during which animals expected signals of behavioral significance, prepared for execution or inhibition of arm reaching movements, and expected the delivery of reward. In the task, animals were instructed by a green light cue to perform an arm reaching movement when a trigger stimulus came on approximately 3 s later (go situation). Movement was withheld after the same trigger light when the instruction cue had been red (nogo situation). Liquid reward was delivered on correct performance in both situations. 2. A total of 1,173 neurons were studied in the striatum (caudate nucleus and putamen) of 3 animals, of which 615 (52%) showed some change in activity during task performance. This report describes how the activity of 193 task-related neurons increased in advance of at least 1 component of the task, namely the instruction cue, the trigger stimulus, or the delivery of liquid reward. These neurons were found in dorsal and anterior parts of caudate and putamen and were slightly more frequent in the proximity of the internal capsule. 3. The activity of 16 neurons increased in both go and nogo trials before the onset of the instruction and subsided shortly after this signal. These activations may be related to the expectation of the instruction as the first signal in each trial. 4. The activity of 15 neurons increased between the instruction and the trigger stimulus in both go and nogo trials. These activations may be related to the expectation of the trigger stimulus independent of an arm movement. Further 56 neurons showed sustained activations only when the instruction requested a movement reaction. Activations were absent in trials in which the movement was withheld. Twenty-one of these neurons were tested with 2 different movement targets, 5 of which showed activity related to the direction of movement. These activations may be related to the preparation of movement or expectation of the specific movement triggering signal. The activity of an additional 20 neurons was unmodulated before the trigger stimulus in movement trials but increased in the interval between the no-movement instruction and the trigger stimulus for withholding the movement. These activations may be related to the preparation of movement inhibition as specific nogo reaction.(ABSTRACT TRUNCATED AT 400 WORDS)

Animals↗
PubMed1994

Oscillatory synaptic interactions between ventroposterior and reticular neurons in mouse thalamus in vitro.

1. The thalamic reticular nucleus (RTN) has reciprocal connections with relay neurons in the dorsal thalamus. We used whole cell recording in a mouse in vitro slice preparation maintained at room temperature to study the synaptic interactions between the RTN and the ventroposterior thalamic nucleus (VP) during evoked low-frequency oscillations. 2. After a single electrical stimulus of the internal capsule, postsynaptic potentials (PSPs) were recorded in all VP and RTN neurons. In 76% of slices, there was an initial response followed by recurrent PSPs lasting for up to 8 s and with a frequency of approximately 2 Hz in both the VP and RTN. 3. In RTN neurons the initial response consisted of a fast excitatory postsynaptic potential (EPSP) that generated a burst of action potentials. Recurrent PSPs consisted of barrages of EPSPs that often reached burst threshold. The structure of subthreshold EPSP barrages in RTN neurons suggested that they were generated by bursting VP neurons. 4. In VP neurons the stimulus usually evoked a small EPSP followed by a large inhibitory postsynaptic potential (IPSP) that was often followed by a rebound burst. This initial response was often followed by a series of recurrent IPSPs presumably generated by RTN bursts, because intrinsic inhibitory neurons are absent in rodent VP. 5. IPSPs in VP neurons and recurrent EPSPs in RTN neurons were completely abolished by application of a gamma-aminobutyric acid-A (GABAA) receptor antagonist. A GABAB receptor antagonist produced no or little change in either the initial or recurrent response. 6. Recurrent IPSPs in VP neurons were abolished by glutamate receptor antagonists before the initial IPSP, which always remained stimulus dependent. 7. The dependency of recurring IPSPs in VP and recurring EPSPs in RTN upon GABA-mediated inhibition and excitatory amino acid-mediated excitation, plus the character of recurring EPSPs in the RTN strongly suggest that the recurring events were generated through reverse-reciprocal synaptic interactions between VP and RTN neurons. These synaptic interactions most likely play an important role in thalamic oscillations in behavior.

Animals↗
PubMed1997

GABA(B)-receptor-mediated inhibition in developing mouse ventral posterior thalamic nucleus.

Inhibitory postsynaptic potentials (IPSPs) generated by activation of the thalamic reticular nucleus (RTN) were recorded in neurons of the ventral posterior nucleus (VP) in vitro in slices from mice aged postnatal day (P)1-P17. An early IPSP peaking 41 +/- 2.5 (SE) ms after electrical stimulation of the internal capsule or RTN was found in 96% of VP neurons. This early IPSP was blocked by bicuculline, showing its dependence on gamma-aminobutyric acid-A (GABA(A)) receptors. A late IPSP peaking 357 +/- 27 ms after the stimulus was observed in 22% of VP neurons in control medium but was uncovered in 38% of neurons when bicuculline was added. The late IPSP was blocked by addition of a GABA(B) antagonist, 2-hydroxysaclofen, to the medium (n = 7); it had a reversal potential of -98 +/- 1.3 mV, 14 mV negative to the early component. In contrast to the early IPSP, whose reversal potential became more negative during postnatal development, the reversal potential of the late IPSP remained constant throughout the postnatal period studied. The most significant change in the late IPSP was shortening in duration, with reduction in latency-to-peak by >400 ms, between P1 and P10. No changes of comparable magnitude were observed in the duration of the earlier GABA(A) response. These results show that both GABA(A) and GABA(B) IPSPs are present very early in the postnatal thalamus and that their characteristics evolve along independent paths during postnatal development.

Animals↗
PubMed1998

Focal stimulation of the thalamic reticular nucleus induces focal gamma waves in cortex.

Electrical stimulation of the thalamic reticular nucleus (TRN; 0.5-s trains of 500-Hz 0.5-ms pulses at 5-10 microA) evokes focal oscillations of cortical electrical potentials in the gamma frequency band ( approximately 35-55 Hz). These evoked oscillations are specific to either the somatosensory or auditory cortex and to subregions of the cortical receptotopic map, depending on what part of the TRN is stimulated. Focal stimulation of the internal capsule, however, evokes focal slow potentials, without gamma activity. Our results suggest that the TRN's role extends beyond that of general cortical arousal to include specific modality and submodality activation of the forebrain.

Acoustic Stimulation↗