We recorded potentials evoked by specific somatosensory stimuli over peripheral nerve, spinal cord, and cerebral cortex. Vibration attenuated spinal and cerebral potentials evoked by mixed nerve and ...muscle spindle stimulation; in one subject that was tested, there was no effect on cutaneous input. Presynaptic inhibition of Ia input in the spinal cord and muscle spindle receptor occupancy are probably the responsible mechanisms. In contrast, muscle contraction attenuated cerebral potentials to both cutaneous and muscle spindle afferent volleys; central mechanisms modulating neurons in the dorsal columns nuclei, thalamus, or cerebral cortex are probably responsible.
We describe a 72-year old man with clinical features suggestive of dementia with Lewy bodies (DLB) who proved neuropathologically to have degeneration induced by relapsing polychondritis (RP), an ...autoimmune inflammatory disorder of cartilaginous tissues. There was lymphocytic infiltration of the leptomeninges, perivascular cuffing, reactive astrocytosis, and activation of microglia in multiple brain areas all consistent with an immunologically mediated process. There was widespread neuronal loss within the hippocampus, entorhinal cortex, and amygdala as well as diffuse myelin pallor of cortical pathways. Elevated levels of complement proteins and endothelial markers of inflammation were observed, which are similar to previous reports in DLB. This study demonstrates that qualitatively similar inflammation-associated neurodegeneration is present in widespread regions of the brain in a RP case presenting clinically as DLB.
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EMUNI, FIS, FZAB, GEOZS, GIS, IJS, IMTLJ, KILJ, KISLJ, MFDPS, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, SBMB, SBNM, UKNU, UL, UM, UPUK, VKSCE, ZAGLJ
Members of a Roma (Gypsy) family with hereditary motor and sensory peripheral neuropathy (HMSN) and concomitant auditory and vestibular cranial neuropathies were identified in Kocevje, Slovenia. The ...illness begins in childhood with a severe and progressive motor disability and the deafness is delayed until the second decade. There are no symptoms of vestibular dysfunction. The family structure is consistent with an autosomal recessive pattern of inheritance and the genetic locus for the disorder is linked to the same region of chromosome 8q24 as other Roma families with HMSN and deafness from Lom, Bulgaria (HMSN‐Lom). The present study shows that the deafness is caused by a neuropathy of the auditory nerve with preserved measures of cochlear outer hair cell function (otoacoustic emissions and cochlear microphonics) but absent neural components of auditory brainstem potentials. The hearing loss affects speech comprehension out of proportion to the pure tone loss. Vestibular testing showed absence of caloric responses. Physiological and neuropathological studies of peripheral nerves were compatible with the nerve disorder contemporaneously affecting Schwann cells and axons resulting in both slowed nerve conduction and axonal loss. Genetic linkage studies suggest a refinement of the 8q24 critical region containing the HMSN‐Lom locus that affects peripheral motor and sensory nerves as well as the cranial auditory and vestibular nerves. Ann Neurol 1999;46:36–44
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BFBNIB, FZAB, GIS, IJS, KILJ, NLZOH, NUK, OILJ, SBCE, SBMB, UL, UM, UPUK
Progressive declines in memory function accompany normal aging, mild cognitive impairment (MCI), and Alzheimer's disease (AD). Neuropathological studies suggest that damage to neurons providing ...connections between cortical areas may contribute to memory impairments in AD. Because AD develops slowly, similar neuropathological changes, to a lesser degree, may be present in MCI and some asymptomatic elderly subjects. In this study we tested the hypothesis that corticocortical interactions between sensory regions are impaired in aging, MCI, and AD, as compared with young subjects. When sensory cortical evoked potentials are elicited by pairs of stimuli the amplitudes of potentials to the second stimulus are attenuated. Corticocortical interactions were assessed by presenting stimulus pairs in different modalities (auditory/visual). There were significant group differences in the degree that a visual stimulus attenuated subsequent auditory potentials (young > healthy elderly > MCI > AD). Control experiments indicated equivalent amplitude reductions for all groups to the second stimulus for stimulus pairs having the same modality. These findings are compatible with progressive declines in corticocortical processing in aging, MCI, and AD.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UL, UM, UPCLJ, UPUK
A long-latency (300-millisecond), vertex-positive component of the event-related potential recorded from monkeys was present only when the eliciting stimulus was relevant to the task. The amplitude ...of this component varied inversely with stimulus probability and was dissociable from motor responses.
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BFBNIB, NMLJ, NUK, PNG, SAZU, UL, UM, UPUK
Previous working memory studies using auditory stimuli at both encoding and retrieval show amplitude decreases in event‐related potentials (N100 and late positive wave, LPW) at retrieval as a ...function of memory load. This study tested if these effects are associated with phonological or semantic coding by presenting visual stimuli at encoding and auditory stimuli at retrieval. We hypothesized that event‐related potentials associated with phonological but not semantic coding would be affected by modality differences at encoding and retrieval. Memory sets having one, three, or five visual digits were followed by auditory probes that subjects classified as present or absent from the set. Reaction time increased and LPW amplitudes decreased with increases in memory load, but there were no significant effects of memory load on N100 amplitude. Results suggest that with respect to brain activity that covaries with memory load, probe N100 amplitude is associated with phonological coding and LPW amplitude is associated with semantic coding.
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BFBNIB, DOBA, FSPLJ, FZAB, GIS, IJS, IZUM, KILJ, NLZOH, NUK, OILJ, PILJ, PNG, SAZU, SBCE, SBMB, UILJ, UKNU, UL, UM, UPUK
The effects of target tone frequency, presence of a masking stimulus, and subject sex on the auditory ERP were studied with an 'oddball' paradigm. P300 latency became shorter (about 15 msec) as the ...difference between the standard (1000 Hz) and target tone frequency increased (1500, 2000, 4000 Hz) but became longer (about 10 msec) with the presence of a white noise masking stimulus. Similar results were obtained for both the P3a and P3b subcomponents of the P300 potential. No significant differences between the adult male and female subjects were observed. The role of stimulus parameters in applied testing situations is discussed.
Somatosensory cerebral evoked potentials were recorded in man to natural forms of somatosensory stimulation of the lower extremity including stretching of the muscle tendons, tapping on muscle ...bellies and tapping on cutaneous surfaces. These potentials were compared with those evoked by electrical stimulation of peripheral nerves measuring the amplitudes and latencies of the evoked potential components and defining the effects of stimulus variables on these parameters. Spinal cord potentials could only be detected to electrical stimuli. Mechanical stimulation of tendons and muscle bellies evoked scalp potentials at latencies earlier than those evoked by electrical stimulation of the peripheral nerve and by cutaneous stimulation at the same level of the leg. Muscle receptors, most probably muscle spindles, are the source of the short latency components obtained by the stretching of tendons and tapping on muscle bellies. The proximal location of these receptors as well as very rapid spinal conduction account for the latency difference. The potentials were larger to electrical stimulation of nerve trunks than to mechanical stimulation of tendons or skin, suggesting the asynchronous activation of a smaller number of fibres by the latter. Individuals with the largest potentials to one form of stimulation usually had the largest potentials to the other modes of stimulation. The use of physiological stimuli such as muscle stretch to test the transmission in specific neural pathways might be useful in investigating the processing of relatively selective afferent volleys using noninvasive evoked potential recordings.
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