Association between a polymorphism of brain-derived neurotrophic factor gene and sporadic Parkinson's disease.
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Aged↗
Biomedical subjects
Shinji Ohara
Publications and source records attributed to Shinji Ohara.
Motor-related functional subdivisions of human lateral premotor cortex: epicortical recording in conditional visuomotor task.
OBJECTIVE: To clarify the functional subdivisions of the human lateral premotor cortex (PM) in the visuomotor control. METHODS: Event-related potentials (ERPs) were epicortically recorded from PM in 5 epilepsy patients. S1-Go/NoGo choice delayed reaction time (RT), S1-warned S2-Go simple RT and control fixation paradigms were compared using paired visual stimuli (S1, S2). RESULTS: Signal-related activity peaked at 176-194 ms after S1 in the ventrorostral PM (PMvr) in all 3 paradigms, indicating its role in signal perception. Early set-related activity was recorded with its peak <810 ms after S1 in the dorsorostral PM (PMdr) and was larger in the choice than in the simple RT paradigm, suggesting its role in signal selection. Its cognitive component was recorded as surface-positive transients at PMdr, while its motoric aspect, seen as negative transients, extended to the caudal PM. Late sustained set-related activity was observed in preparation for hand movement in the caudal PM at the hand and face positive motor areas. After presentation of S2, movement-related activity was observed at the hand sensorimotor area for motor execution, following the signal-related activity at PMvr. CONCLUSIONS: The present ERP study suggests the temporally sequential representation of predominantly 'cognitive' function in the rostral PM and 'motor' function in the caudal PM. SIGNIFICANCE: The rostrocaudal cognitive-motor gradient was demonstrated in the lateral premotor cortex in humans by means of an epicortical ERP approach.
Adolescent↗
Usefulness of [123I]metaiodobenzylguanidine ([123I]MIBG) myocardial scintigraphy in differentiating between Alzheimer's disease and dementia with Lewy bodies.
OBJECTIVE: To confirm the clinical usefulness of [123I] metaiodobenzylguanidine ([123I]MIBG) myocardial scintigraphy in the antemortem differential diagnosis between patients with Alzheimer's disease (AD) and those with dementia with Lewy bodies (DLB). PATIENTS AND METHODS: We compared cardiac [123I] MIBG uptake in 10 patients with AD with that in 10 patients with DLB. We selected the patients with AD or DLB by using stringent diagnostic criteria that combined commonly used clinical criteria with tau protein levels in cerebrospinal fluid and radiographical examinations. RESULTS: The heart to mediastinum ratio of [123I]MIBG uptake in all the patients with DLB was significantly lower than that in the patients with AD (p<0.01). CONCLUSION: This study confirms that [123I]MIBG myocardial scintigraphy is useful in the antemortem differential diagnosis of AD and DLB.
3-Iodobenzylguanidine↗
Attenuation of cerebellar tremor with implantation of an intrathecal baclofen pump: the role of gamma-aminobutyric acidergic pathways. Case report.
The authors present the case of a 49-year-old woman with disabling bilateral upper-extremity cerebellar tremor that resolved unexpectedly after placement of an intrathecal baclofen pump for lower-extremity spasticity. The tremor amplitude decreased nearly linearly with increasing intrathecal baclofen dosage, and disappeared completely at a dose of 250 microg/day. In this report the authors demonstrate the role of the gamma-aminobutyric acidergic system in the pathogenesis of cerebellar tremor, and these findings may lead to a new treatment modality for patients disabled by this manifestation of their disease.
Baclofen↗
"Supplementary motor area (SMA) seizure" rather than "SMA epilepsy" in optimal surgical candidates: a document of subdural mapping.
PURPOSE: To clarify the relationship between epileptogenic zone and supplementary motor area (SMA) in patients who were regarded as the optimal surgical candidates for their intractable SMA seizures. METHODS: We analyzed the epileptogenic zone at/or adjacent to the SMA in four patients with clinical SMA seizures. All four patients had noninvasive presurgical evaluations (long-term video/EEG monitoring, MRI, and neuroimaging with radioisotopes), which provided convergent results between ictal semiology and the epileptogenic area, and thus, they had chronically implanted subdural electrodes, and finally had focus resection with a follow-up period of more than 2 years. RESULTS: Three patients had lesions shown by MRI outside the SMA, and one patient had a lesion within the SMA. Interictal epileptiform discharges were seen at/or outside the SMA. Ictal EEG pattern originated from the SMA in one patient, from the high lateral frontal area in two patients, and from the precuneus in one patient. In the latter three patients, the ictal EEG pattern immediately spread to the SMA. Those ictal onset zones were consistently localized within/or just adjacent to the lesions revealed by MRI. Only one patient had SMA resection, and three had the resection of epileptogenic zone by preserving the SMA. No neurological deficits developed and good seizure control was achieved. CONCLUSION: Among surgical candidates for intractable SMA seizures, frontal cortex other than SMA or even parietal cortex can be epileptogenic, and thus, the SMA itself may not necessarily have to be resected. This notion is clinically important when selecting surgical candidates as well as when planning presurgical invasive evaluation in patients with intractable SMA seizures.
Adolescent↗
A clinical and neuropathological study of an unusual case of sporadic tauopathy. A variant of corticobasal degeneration?
We report a sporadic case of tauopathy with unusual clinical and neuropathological features. The patient presented with progressive symmetric rigid-akinetic parkinsonism and dementia of the subcortical type. Magnetic resonance imaging of the brain revealed atrophy resembling multiple system atrophy. The level of cerebrospinal fluid tau protein phosphorylated at serine 199 was markedly elevated. The autopsy revealed more glial than neuronal tauopathy, with much heavier involvement of subcortical white matter and the brainstem than of the cerebral cortex. Analysis of dephosphorylated tau revealed that hyperphosphorylated four-repeat tau isoforms were deposited in the brain of the patient. Despite morphological and biochemical resemblance to a certain form of familial fronto-temporal dementia, no mutation of the tau gene including exon 10 could be found. Our findings, taken together with those in previous similar case reports, indicate that the case represents an atypical form of corticobasal degeneration or a new variant of sporadic tauopathy.
Aged↗
Progressive supranuclear palsy with asymmetric tau pathology presenting with unilateral limb dystonia.
We report an autopsy case of a 77-year-old Japanese man with a 7-year history of progressive unilateral left limb dystonia and arm levitation. Brain computed tomography showed fronto-temporal atrophy. The patient was diagnosed as having corticobasal degeneration. Histopathologically, the cerebral cortices, especially of the parasagittal region, and subcortical nuclei revealed numerous Gallyas/tau-positive cytoplasmic inclusions characteristic of progressive supranuclear palsy (PSP). Grumose degeneration was evident in the dentate nucleus. Astrocytic plaques were not present, but a small number of ballooned neurons were found in the fronto-temporal regions. The involvement by the PSP lesions was quite asymmetric in the affected areas, including the frontal cortices, basal ganglia, red nuclei, and inferior olivary nuclei, being more prominent on the side contralateral to the side of limb dystonia. The apparent unilateral dominance of PSP pathology may be relevant to the asymmetric clinical presentation of this patient.
Aged↗
Intraoperative microelectrode and semi-microelectrode recording during the physiological localization of the thalamic nucleus ventral intermediate.
We review the techniques of physiological localization of the site for ventralis intermedius (Vim) thalamotomy or implantation of Vim-deep brain stimulation (DBS) for treatment of parkinsonian, essential, and intention tremor. Both microelectrode and semi-microelectrode techniques are reviewed. We believe the use of microelectrode and semi-microelectrode recordings in combination with Radiological landmarks provide the most accurate localization of the target. In addition to recording, microstimulation of subcortical structures such as Vim and thalamic nucleus ventralis caudal through the microelectrode may improve physiological identification by altering the tremor and evoking somatic sensations, respectively. Microelectrode recording provides the highest resolution picture of the target site at a cost of increased time to locate the target. We also review the relationship between thalamic neuronal firing and electromyographic activity during tremor. Implications of these results for the mechanisms for parkinsonian, essential, and intention tremors are discussed.
Brain Mapping↗
Spinocerebellar ataxia type 6 with motor neuron loss: a follow-up autopsy report.
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Amyotrophic Lateral Sclerosis↗
Low-frequency electric cortical stimulation has an inhibitory effect on epileptic focus in mesial temporal lobe epilepsy.
PURPOSE: This study was conducted to investigate the effect of low-frequency electric cortical stimulation on epileptic focus in humans. METHODS: We stimulated the epileptic focus in a patient with medically intractable mesial temporal lobe epilepsy (MTLE) by means of subdural electrodes and evaluated the change in the number of interictal epileptiform discharges. We used biphasic electric current of 0.3-ms duration presented at 0.9-Hz frequency for 250 s, comparing stimulus intensity of 7.5, 2, and 0.5 mA. RESULTS: Interictal epileptiform discharges at the ictal focus occurred less frequently after the stimulation with the intensity of 0.5 mA. With the intensity of 7.5 mA and 2.0 mA, however, habitual auras were elicited by the stimulation, and afterdischarges were seen on the cortical EEG. CONCLUSIONS: Low-frequency, low-intensity electric cortical stimulation could produce inhibitory effects on epileptic activity. At the same time, however, a caution for possible induction of EEG seizures is needed, even when applying low-frequency electric stimulation.
Adolescent↗
Partial epilepsy manifesting atonic seizure: report of two cases.
PURPOSE: Atonic seizures are commonly seen in patients with generalized epilepsy but only infrequently in patients with partial epilepsy. Clinically generalized atonic seizures as a partial epilepsy have not been studied in detail with video/EEG monitoring. Here we describe the clinical and physiologic characteristics of atonic seizures due to partial epilepsy and discuss the underlying mechanism. METHODS: Two patients with partial epilepsy manifesting atonic seizures, one with frontal lobe epilepsy (FLE) and the other with parietal lobe epilepsy (PLE), were reported. The long-term video/EEG monitoring, magnetic resonance imaging (MRI), and interictal fluorodeoxyglucose-positron emission tomography (FDG-PET) were investigated in each patient. RESULTS: Paroxysmal diminution of muscle tone mainly involved the axial muscles in both patients. In contrast with the abrupt falls seen in patients with Lennox-Gastaut syndrome, the falls in these patients were slow, taking 2-5 s to fall down. Ictal EEG records showed low-voltage fast activity in the frontocentral area followed by repetitive spikes at the midline frontocentral area in the patient with FLE, and rhythmic spikes in the left central area in the patient with PLE. Interictal FDG-PET disclosed hypometabolic regions consistent with the clinical and EEG findings. CONCLUSIONS: Slow falls might be a feature of atonic seizures in partial epilepsy. Long-lasting atonia in partial epilepsy could be due to either one of the following two possible mechanisms: (a) epileptic activities arising from the negative motor area, of which 50-Hz electric stimulation causes motor inhibition, or (b) sustained atonia with successive electromyogram (EMG) silent periods caused by epileptic discharges arising from the inhibitory area of the primary sensorimotor area.
Adult↗
Astrocytic deformity and globular structures are characteristic of the brains of patients with aceruloplasminemia.
Aceruloplasminemia is an interesting disease, the study of which helps elucidate how iron-induced oxidative stress is involved in neuronal cell death. In order to study the neuropathological characteristics associated with oxidative stress, we scrutinized the brains of 5 patients with aceruloplasminemia histopathologically and immunohistochemically. The pathological findings were essentially similar in all patients. In the frontal cortices, iron deposition and neuronal cell loss were trivial, but in the basal ganglia (especially in the caudate nucleus and putamen), severe iron overload and extensive neuronal loss were noted. Iron deposition was more prominent in the astrocytes than in the neurons in both regions. 4-hydroxynonenal (HNE), one of the most physiologically active lipid peroxides, was strongly detected on neurons and astrocytes by immunostaining. Markedly deformed astrocytes were observed in the striatum. These astrocytes were similar to Alzheimer type 1 astrocytes. Globular structures were seen in proportion to the degree of iron deposition. They clearly reacted with anti-glial fibrillary acidic protein (GFAP) and anti-S-100 antibodies and contained glial fibril-like filaments, but showed no or only faint immunoreactivity to antibodies for neuronal marker proteins, such as neurofilament and synaptophysin. Therefore, the globular structures presumably originated from astrocytes. The structures also reacted positively to anti-HNE and anti-ubiquitin antibodies. We conclude that astrocytic deformities and globular structures are characteristic neuropathological features of aceruloplasminemia and are closely linked to iron overload and subsequent oxidative stress.
Aldehydes↗