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Biomedical subjects

Mamiko Ishitobi

Publications and source records attributed to Mamiko Ishitobi.

9 recordsLinked to original sources

Spike orientation may predict epileptogenic side across cerebral sulci containing the estimated equivalent dipole.

OBJECTIVE: To evaluate whether the orientation of interictal spikes, localized in major sulci by magnetoencephalography (MEG), predicts the epileptogenic side of the sulcal wall. METHODS: Sixteen epilepsy patients were analyzed in whom equivalent current dipoles (ECDs) of MEG spikes were localized on the central (four patients), interhemispheric (4), or sylvian fissure (8); and the epileptogenic side across the sulci had been confirmed by seizure semiology, structural lesions, or intracranial electroencephalography (EEG). ECD was classified as epileptogenic side or normal side oriented and correlated to the scalp EEG map. RESULTS: All central (n=50) and interhemispheric (n=83) spike ECDs were oriented toward the epileptogenic side at peak latency. In scalp EEG, 91% of the spikes showed radial pattern of broad negativity above the sulcus whereas 9% showed tangential pattern with positive maximum above the epileptogenic side. Sylvian spikes were only found in patients with temporal lobe epilepsy (TLE). In sylvian spikes (n=220), 73% of ECDs were oriented toward the epileptogenic side, whereas 27% were oriented toward the normal side. CONCLUSIONS: In central and interhemispheric spikes, epileptogenic side cortex may be gross surface negative through the sulcal wall to the adjacent gyrus. Inconsistent orientation of the sylvian spikes suggests a complex pattern of spike propagation in TLE. SIGNIFICANCE: ECD orientation of central and interhemispheric spikes in MEG may predict the epileptogenic side.

Brain↗

Successful treatment of a 2-year-old girl with intractable myasthenia gravis using tacrolimus.

We used tacrolimus to successfully treat a patient with childhood-onset oropharyngeal myasthenia gravis (MG). A girl (2 years, 5 months old) with oropharyngeal MG responded partially to treatment including pyridostigmine bromide, intravenous immunoglobulin, and prednisolone (2 mg/kg/day) for 7 weeks, but this resulted in worsening of her eye symptoms. By contrast, tacrolimus at 2 mg/day resulted in complete remission of the MG, which made it possible to reduce the dose of prednisolone. This is a rare report of the use of tacrolimus as an effective treatment for patients with intractable childhood-onset MG.

Child, Preschool↗

Effective treatment with levodopa and carbidopa for hypomyelination with atrophy of the basal ganglia and cerebellum.

Hypomyelination with atrophy of the basal ganglia and cerebellum (H-ABC) is a rare leukoencephalopathy presenting in the infantile period and characterized by diffuse cerebral hypomyelination, and atrophy of the basal ganglia and cerebellum. As patients with H-ABC lack remarkable laboratory findings, the diagnosis is based on brain magnetic resonance imaging findings alone. Only eight cases have been reported in the literature, and thus the natural course and treatment of this disease are not fully understood. We report a 35-month-old boy with H-ABC who had hemidystonia, hypomyelination, and cerebellar ataxia. We diagnosed H-ABC after considering a thorough differential diagnosis, excluding other diseases involving hemidystonia, hypomyelination, and cerebellar ataxia. Furthermore, technetium-99m ethyl cysteinate dimmer-single-photon emission computerized tomography (Tc-ECD-SPECT) and positron emission tomography with fluorodeoxyglucose (18)F (FDG-PET) revealed decreased blood flow and glucose metabolism in the bilateral lenticular nucleus, thalamus, and cerebellum. A peroral levodopa preparation containing carbidopa (levodopa-carbidopa) was effective at ameliorating and stopping the progression of the patient's dystonia (final effective doses: levodopa, 200 mg/day and carbidopa, 20 mg/day). This is the first case report of a Japanese patient with H-ABC and treatment for this disease. Levodopa-carbidopa may be an effective treatment for H-ABC.

Administration, Oral↗

Evaluation of interhemispheric time difference by magnetoencephalography before and after total callosotomy. Two case reports.

Interhemispheric time difference (ITD) measured by electroencephalography (EEG) and magnetoencephalography (MEG) was compared to seizure outcome after callosotomy. Two patients with frequent drop attacks underwent simultaneous EEG and MEG before and after total callosotomy. ITDs in 30 bilateral synchronized (BS) discharges were calculated independently by EEG and MEG. As minimum transcallosal conduction time was suggested to be approximately 20 msec, BS discharges were classified into five categories according to ITD and side: left- or right-leading long (300 to 80 msec), left- or right-leading moderate (80 to 20 msec), and negligible (<20 msec). In Case 1 before callosotomy, EEG detected 77% negligible and 23% right-leading moderate BS discharges, whereas MEG detected 30% and 63%, respectively. After callosotomy, drop attacks reduced remarkably and EEG and MEG detected no BS discharges. In Case 2 before callosotomy, EEG detected 77% negligible and 23% moderate BS discharges, whereas MEG detected 80% and 20%, respectively. After callosotomy, drop attacks recurred 2 months later and EEG and MEG detected left- and right-leading long BS discharges (63% by EEG and 56% by MEG). MEG detected a large number of BS discharges with moderate ITD before surgery in Case 1, suggesting that the transcallosal pathway was the main pathway for the synchronization, whereas the negligible ITD in Case 2 excludes transcallosal propagation. BS discharges with longer ITD after surgery in Case 2 suggest a persistent poly-synaptic non-transcallosal pathway. MEG with higher spatial resolution than EEG may provide surgical indications for callosotomy.

Adolescent↗

Opercular to interhemispheric source distribution of benign rolandic spikes of childhood.

We evaluated the source distribution of benign rolandic spikes of childhood along and across the central sulcus in 15 patients, aged between 7 and 15 years, who suffered from seizure disorders. Previous routine EEG showed centrotemporal spikes, but none of them had major abnormalities on brain magnetic resonance imaging or neurological deficits. The equivalent current dipoles (ECDs) of the spikes measured by whole-head magnetoencephalography (MEG) were compared to the spike distributions detected by simultaneous scalp EEG according to the international 10-20 system. Locations and orientations of the MEG spikes corresponded to the EEG spike distribution as follows: superiorly oriented spike MEG dipoles in the opercular area corresponded to T3/4 negative peaks (8 spike groups in 6 patients); anteriorly oriented spike dipoles in the rolandic area corresponded to C3/4 or P3/4 negative peaks (17 spike groups in 13 patients); laterally oriented spike dipoles in the interhemispheric area corresponded to Cz/Pz negative peaks (4 spike groups in 3 patients); and others (4 spike groups in 4 patients). Rolandic spikes include three main types according to the ECD location from the opercular to the interhemispheric areas. The functional anatomy of benign rolandic spikes was correlated with partial seizure semiology. All three rolandic spike types can be explained by a precentral origin, assuming that the surface negative potential is continuous from the gyral to fissural cortices.

Adolescent↗

Abnormal primary somatosensory function in unilateral polymicrogyria: an MEG study.

The purpose of this study is to investigate the primary somatosensory function in patients with unilateral polymicrogyria. Somatosensory evoked fields (SEFs) due to median and posterior tibial nerve stimulation were compared in the normal and dysplastic cortices of five patients with unilateral polymicrogyria. SEFs were observed in all five normal hemispheres and three dysplastic hemispheres. Latencies of N20m and P38m, the first cortical components of and SEFs for median nerve and tibial nerve stimulation, were all within the normal range in both normal and dysplastic hemispheres. The amplitudes of the N20m and P38m in the dysplastic hemispheres were smaller in one patient and larger in two patients compared to the normal hemispheres. Equivalent current dipoles of N20m and P38m were localized on the anatomical central sulcus of the normal hemispheres and over the central area of the dysplastic hemispheres. P38m dipoles were localized medial and upward to the N20m dipole in both normal and dysplastic hemispheres. N20m dipole orientation was normal in all normal hemispheres and in one dysplastic hemisphere, but abnormally inferior in two dysplastic hemispheres. P38m dipole had normal medial orientation in all hemispheres except one dysplastic hemisphere. Abnormality of the primary somatosensory function in the dysplastic cortex of patients with unilateral polymicrogyria was clearly demonstrated by magnetoencephalography with high resolution in time and space. The normal somatotopic arrangement was preserved.

Adolescent↗

The effects of copper-histidine therapy on brain metabolism in a patient with Menkes disease: a proton magnetic resonance spectroscopic study.

We report on metabolic changes in the brain of a boy with Menkes disease. He was treated with parenteral copper (Cu)-histidine supplementation, from 5 months of age, and assessed with proton magnetic resonance spectroscopy ((1)H-MRS). The single-voxel (1)H-MRS before treatment revealed an accumulation of lactate and a reduced N-acetyl aspartate (NAA)/total creatine (tCr) ratio with a z-score of -3.0. During treatment, the lactate signal faded away, whereas the NAA signal gradually increased to a z-score of -1.5 at 120 days of treatment. The choline/tCr ratio did not deviate much initially (z-score +0.5), but the ratio increased markedly during treatment (z-score +4.8). Consequently, the Cu-histidine therapy initiated after the critical period still improved the neuronal metabolism, suggesting that some Cu was delivered to neurons. Nevertheless, the brain atrophy, impaired myelination, and severe neurological symptoms were not ameliorated.

Brain↗

Acute dysautonomia: complete recovery after two courses of IVIg.

An 11-year-old boy presented with acute dysautonomia manifesting as severe orthostatic hypotension following fever. Serial orthostatic tests with measurement of the coefficient of variation in the R-R intervals showed improvement after one course and complete recovery after two courses of intravenous high-dose immunoglobulin therapy (IVIg). Repeated courses of IVIg should be considered to treat this disorder if spontaneous remission does not occur.

Autoimmune Diseases of the Nervous System↗

Dynamic cortical activity during spasms in three patients with West syndrome: a multichannel near-infrared spectroscopic topography study.

PURPOSE: To investigate spatial and temporal cortical activity during clusters of naturally occurring epileptic spasms in patients with West syndrome (WS) by using multichannel near-infrared spectroscopy (mNIRS). METHODS: Conventional magnetic resonance imaging (MRI) and interictal and ictal single-photon emission computed tomography (SPECT) were carried out in three patients with WS. Thereafter, cortical hemodynamics during naturally occurring epileptic spasms were measured by mNIRS with simultaneous video/electroencephalographic (EEG) monitoring. RESULTS: Ictal SPECT revealed multiple hyperperfused areas within the cortex. With the use of mNIRS, an increase in regional cerebral blood volume (CBV) was observed in these areas, which is representative of cortical activation. The increase in CBV was accompanied by an increase in the concentrations of both oxy- and deoxyhemoglobin. The following heterogeneous regional changes in CBV during ictus were observed: (a) transient increases that were synchronized with spasms; (b) a gradual increase during an ictal event that fluctuated in synchrony with spasms; and (c) a combination of transient and gradual increases. An increase in regional CBV occurred in multiple areas that were activated either simultaneously or sequentially during an ictal event. Topographic changes in CBV were closely correlated with the phenotype of the spasm. CONCLUSIONS: During ictal events, multiple cortical areas were activated simultaneously or sequentially. The pattern of cortical activation closely affected the phenotype of the spasm, which suggested that the cortex was involved in the generation of spasms.

Brain Mapping↗