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R Neshige

Publications and source records attributed to R Neshige.

At least 19 recordsLinked to original sources

An angiographically occult arteriovenous malformation in the medial parietal lobe presenting as seizures of medial temporal lobe origin.

We present an unusual case of a patient who was diagnosed with temporal lobe epilepsy and whose seizures were reduced markedly after excision of an angiographically occult arteriovenous malformation (AVM) located in the left medial parietal lobe. A 38-year-old man had complex partial seizures characterized by motionless staring with oroalimentary and behavioral automatisms since the age of 15 years. Magnetic resonance imaging (MRI) demonstrated a small lesion extending from the left posterior cingulate gyrus to the precuneus. There was no MRI evidence of mesial temporal sclerosis. Intracranial EEG recordings showed ictal onset from the left medial parietal lobe propagating to the medial temporal lobes. Clinical signs appeared when these discharges reached the temporal lobes. After excision of the lesion (which was histologically confirmed as an AVM), together with the marginal cortex, seizures were reduced significantly. Careful diagnostic evaluation of lesions such as the this one may reveal an epileptogenic lesion (zone) far from the region where scalp ictal discharges seem to arise. In our case, we hypothesize that false localization was due to propagation of ictal discharges from the parietal focus through the limbic system.

Adult

Genetic analysis enables definite and rapid diagnosis of cerebrotendinous xanthomatosis.

Mutations in the sterol 27-hydroxylase gene (CYP27) cause cerebrotendinous xanthomatosis (CTX). Early diagnosis of CTX is crucial because treatment with chenodeoxycholic acid can prevent or reverse some of the neurologic disability associated with the disease. We report the identification of three types of mutations (Arg441Trp, Arg372Gln, and Arg441Gln) in the CYP27 gene in five patients with suspected CTX from four unrelated families by restriction endonuclease analysis.

Adult

[A case of Prader-Willi syndrome with bilateral middle cerebral artery occlusion and moyamoya phenomenon].

We report a case of Prader-Willi syndrome (PWS) complicated with juvenile stroke. The patient is a 19-year-old man with right hemiplegia, who has had a history of non-insulin-dependent diabetes mellitus (NIDDM) for ten years. The diagnosis of PWS was confirmed genetically by the method of fluorescence in situ hybridization which showed the deletion of chromosome 15. His brain MRI revealed abnormal signal intensities in the left basal ganglia and around the right trigone of the lateral ventricle. Angiographic examination showed occlusions of bilateral proximal middle cerebral arteries with basal moyamoya vessels. The left vertebral artery was also occluded at its origin. Only a few cases of PWS complicated with stroke have been reported before and, to date, there has been no case with arterial occlusion similar to our case. Though the cause of these arterial occlusions is unknown, it may be related to arteriosclerosis following NIDDM.

Adult

Do patients with Down's syndrome recognize Mickey Mouse?

In 47 patients with Down's syndrome who were too retarded to understand a conventional oddball paradigm, we studied event-related potentials (ERPs) by using task-irrelevant visual stimuli. As the patients had expressed interest in Disney cartoons and books, five pictures of Disney characters such as Mickey Mouse or Peter Pan were employed as the rare stimuli, and nine ordinary scenes such as mountains as the frequent stimuli. Five sets were constructed by intermingling each rare picture with nine frequent pictures, and each picture was presented on a TV screen. ERPs to the rare stimuli (N270-P380), which were different from the averaged waveforms to frequent stimuli and electrooculograms (EOGs), were clearly recorded in 35 out of 47 patients. Only 24 of these patients had ERPs using a conventional auditory oddball paradigm. The peak latencies in patients were significantly prolonged relative to age-matched normal controls, probably due to a slowed neural processing. The amplitude in patients was slightly larger than normal controls, probably due to the patients' greater interest or surprise when confronted with the Disney characters.

Adolescent

Automatic EEG interpretation: a new computer-assisted system for the automatic integrative interpretation of awake background EEG.

A new computer-assisted system for automatic interpretation of the awake electroencephalogram (EEG) was developed. First, all the items necessary for EEG interpretation were determined in accordance with the procedure that a qualified electroencephalographer (EEGer) goes through for the visual inspection of the background EEG activity, and then each item was defined quantitatively. For the automatic interpretation, specific EEG parameters were determined for each item so that they could fit the graded judgement of the item by the qualified EEGer as closely as possible. These specific EEG parameters were actually calculated from periodograms obtained from the time series of EEG records of 14 patients with various neurological diseases. The automatic EEG interpretation system thus established was applied to the EEG data of these 14 subjects and to 3 additional EEGs, and the results were compared with those obtained through the visual interpretation by the EEGer. This automatic EEG interpretation was found to be in good agreement with the visual interpretation by the EEGer in most EEG records. In contrast with the previous automatic analyses of EEG which were focussed on certain aspects of EEG such as the dominant rhythm, the present system is unique in its capability of providing an integrative interpretation of the spontaneous awake EEG by taking into account all its features except for paroxysmal abnormalities.

Adult

Physiological study of the spinothalamic tract conduction in multiple sclerosis.

This is the first paper to study the physiological function of the spinothalamic tract in multiple sclerosis (MS) using pain-related somatosensory evoked potentials (pain SEPs) following CO2 laser stimulation. Among 12 patients with MS, hand- and foot-stimulated pain SEPs were significantly delayed or absent in 3 and 7, respectively. These results were totally consistent with clinical impairment of pain-temperature sensation. In contrast, the results of conventional electrically-stimulated SEPs were compatible with impairment of vibration sensation. Therefore, the examination of both pain and electric SEPs is very useful to evaluate the physiological function of the ascending spinal tract in patients with MS.

Adult

Recording of event-related potentials (P300) from human cortex.

Auditory event-related potentials were recorded simultaneously from chronically implanted subdural electrodes and from scalp electrodes in three patients who were being evaluated for surgical treatment of epilepsy. These three cases showed clearly defined scalp-P300 and scalp-N300. A cortex-P300 was recorded from the midtemporal area, and a cortex-N300 was recorded from the inferior frontal area with some reflection at the basal temporal region. There were no potentials from an interhemispheric region. We could not observe any component from the cortex studied corresponding to scalp-recorded N200. Therefore, while the activity generated from the mesial temporal lobe may only make minor contributions to scalp-P300, that generated from the midtemporal area might make a major contribution to the scalp-P300. Additionally, generators of N200, P300, and N300 are different from each other. These findings, together with previous reports regarding the generator source of P300, also suggest that P300 is a complex arising from multifactorial generator sources, including the midtemporal and inferior frontal area.

Acoustic Stimulation

Pain-related somatosensory evoked potentials following CO2 laser stimulation in peripheral neuropathies.

Pain-related somatosensory evoked potentials (pain SEPs) following CO2 laser stimulation were examined in 30 patients with peripheral neuropathies, and the results were compared with clinical sensory findings. Pain SEP findings showed a significant correlation with the clinical impairment of pain sensation, but not with the impairment of deep sensations. In contrast, conventional electrically-stimulated SEPs (electric SEPs) showed a significant correlation with deep sensations, but not with the impairment of pain sensation. Examinations of both pain SEPs and electric SEPs, therefore, are considered to be very useful to evaluate physiological functions of sensory nerves in patients with peripheral neuropathies.

Adult

Systemic interferon-alpha in the treatment of HTLV-I-associated myelopathy.

Treatment with interferon-alpha (IFN-alpha) was undertaken in 16 patients with human T-lymphotropic virus type I-associated myelopathy (HAM). All patients had progressive spastic paraparesis before treatment. Twelve patients were enrolled in an open therapeutic trial with a dose of 3.0 x 10(6) IU/day of IFN-alpha and 4 in a randomized, double-blind, multidose (3.0 x 10(6), 1.0 x 10(6) or 0.3 x 10(6) IU/day) trial. IFN-alpha was injected intramuscularly for 28 days. Eight of 12 patients enrolled in an open trial and 2 patients receiving a dose of 3.0 x 10(6) IU/day of IFN-alpha in a randomized trial showed clinical improvements during and after the treatment. The results showed that, although not for all patients, systemic IFN-alpha with a dose of 3.0 x 10(6) IU/day is effective in the treatment of HAM.

Adult

Accelerated aging of the brain in Werner's syndrome.

We report the electrophysiologic examination of a 55-year-old woman with Werner's syndrome. Needle EMG and peripheral conduction studies were normal. In contrast, EEG was moderately abnormal, the N20 and P25 potentials of SEPs were delayed and enlarged, and the event-related potential, P300, was delayed. These characteristic findings indicated accelerated aging of the brain, as in the other organs, in Werner's syndrome.

Brain

CO2 laser-induced pain-related somatosensory evoked potentials in peripheral neuropathies: correlation between electrophysiological and histopathological findings.

Pain-related somatosensory evoked potentials (pain SEPs) following CO2 laser stimulation as well as conventional electrically stimulated SEPs (electric SEPs) were examined in 10 patients with peripheral neuropathies in whom the histopathological examination of the sural nerve was done. Results of pain SEPs showed a positive relationship with clinical impairment of pain sensation and densities of small myelinated fibers of the sural nerve. In contrast, results of electric SEPs showed a positive relationship with clinical impairment of deep and tactile sensations and with densities of large myelinated fibers of the sural nerve. Therefore, pain SEPs are considered to be generated by ascending signals mediated through nociceptive receptors and A delta fibers. The pain SEP is only one noninvasive and objective method currently available to investigate a physiological condition of the sensory pathway responsible for pain sense, and is especially useful when combined with the conventional electric SEPs.

Adolescent

Estimation of conduction velocity of A delta fibers in humans.

Conduction velocity of A delta fibers of the human peripheral nerves was measured by using pain-related somatosensory evoked potentials following CO2 laser stimulation. It was found to be approximately 9 m/s in the forearm as well as in the lower leg. Because conventional conduction study using electric stimulation reflects only functions of large myelinated fibers related to deep proprioceptive and tactile sensations, the present noninvasive and simple, novel method is the only laboratory examination currently available to investigate physiological functions of the small diameter fibers mediating pain-temperature sensations.

Adult

Treatment of HTLV-I-associated myelopathy with high-dose intravenous gammaglobulin.

Fourteen patients with HTLV-1-associated myelopathy were treated with high-dose intravenous gammaglobulin (IVGG). Ten received 10 g/day of IVGG and 4 received 400 mg/kg of body-weight/day of IVGG for 5 consecutive days. Improvement of spastic paraparesis was observed in 10 within 7 days of the commencement of IVGG. The therapeutic effects were sustained for more than 3 weeks in some patients. There were no side effects. Analysis of factors of relevance to the clinical improvement with IVGG showed that the beneficial response was preferentially found in patients having a high CSF titre of anti-HTLV-I antibodies, a high CSF IgG level and a marked brain MRI abnormality.

Adult

Event-related brain potentials as indicators of visual recognition and detection of criminals by their use.

An event-related potential (ERP) was recorded, using photographs as stimuli, in 12 subjects for attended, 9 subjects for non-attended conditions and 14 subjects for a simulated criminal investigation. An ERP was detected only when a subject recognized a familiar image (target) mixed with other, unfamiliar images (non-target), regardless of whether he was asked to attend to or neglect the target image. ERPs in the subject who watched each picture but tried to ignore the relevant picture (non-attended) were more activated at the parietal region than at the central region, in contrast with ERPs in the subjects who paid attention to each picture without trying to ignore the relevant picture (attended). In the simulated criminal investigation, only a simulated thief, but not a simulated innocent subject elicited ERP only after the picture of a criminal site or thing was intermingled with pictures bearing no relationship to the crime. These findings indicate that the ERP using photographs as stimuli is useful as an objective indicator of crime-relevance.

Adult

Pain-related somatosensory evoked potentials in syringomyelia.

Pain-related somatosensory evoked potentials following CO2 laser stimulation (pain SEPs) and conventional electrically-stimulated SEPs (electric SEPs) were examined in 8 patients with syringomyelia who showed various forms of dissociated sensory loss. Unlike clinical examination using a pin or needle, pain SEP is considered to be an objective and quantitative test to investigate functions of peripheral and central sensory pathways responsible for pain-temperature sensation (A delta fibres and the spinothalamic tract). Pain SEPs were abnormal in all patients. The results were generally compatible with the degree of a clinical impairment of pain-temperature sensation. Subclinical abnormality was detected in 3 patients. Electric median nerve SEPs using the scalp reference (Fz) were normal in 6 out of 8 patients. However, anterior and posterior cervical responses using a noncephalic reference were absent or small in 7 patients. Electric SEPs following tibial nerve stimulation were normal in 7 patients. These findings suggest that the function of the ascending fibres through the dorsal columns is intact in most patients, whereas the dorsal horn, where a fixed cervical potential is generated, is impaired. Pain SEPs combined with electric SEPs therefore appear to be extremely useful for investigating physiological function in the sensory pathways in patients who show 'dissociated sensory loss' such as in syringomyelia.

Adult

Neuroepithelial cyst presenting as recurrent aseptic meningitis.

A 41-year-old man had three episodes of acute aseptic meningitis from the age of thirty-six. With each episode he had severe occipital headache associated with a mononuclear pleocytosis and an increase in CSF protein. He had a neuroepithelial cyst originating from the choroid plexus of the right cerebellomedullary cistern. Spontaneous ruptures of the cyst probably caused recurrent chemical meningitis. A neuroepithelial cyst must be considered in the differential diagnosis of intracranial cystic tumors presenting with aseptic meningitis.

Adult

Numb chin syndrome secondary to Burkitt's cell acute leukemia.

We describe a case of Burkitt's cell acute lymphoblastic leukemia presenting with the bilateral numb chin syndrome as the initial symptom of the disease. Postmortem study of the trigeminal nerve showed heavy infiltrations of leukemic cells and destruction of axon and myelin by leukemic cells in the mandibular nerve.

Burkitt Lymphoma

[A case of upper brainstem infarction developing symptomatic narcolepsy after the administration of anti-convulsant drugs].

A 52-year-old woman, who had ischemic infarction in the ventral upper brainstem due to subarachnoid hemorrhage in October 1986, had recurrent sleep and cataplexy attacks from May 1987. She was receiving valproate and phenytoin daily since 1986. The diagnosis of narcolepsy was made based on the clinical symptoms and EEG findings showing REM sleep during a sleep attack. Both sleep and cataplexy attack increased in parallel with an increase of the dose of anti-convulsant drugs and disappeared immediately after the discontinuation of the treatment. The findings that suggest that the administration of anti-convulsant drug as well as the brainstem vascular lesion was deeply involved in the development of narcolepsy in this case.

Brain Stem