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1.
Scand J Rheumatol ; 51(4): 259-267, 2022 07.
Article in English | MEDLINE | ID: mdl-34474646

ABSTRACT

OBJECTIVE: This study investigated the effectiveness of treatment with Janus kinase (JAK) inhibitors in rheumatoid arthritis (RA) assessed by ultrasonography (US) activity, and the influence of patient characteristics and previous treatments. METHOD: This prospective study assessed 60 treatment initiations among 53 Japanese patients diagnosed with RA who underwent treatment with JAK inhibitors during June 2013 to February 2020. Of the 53 patients, seven patients were enrolled in duplicate because they were treated with two different JAK inhibitors at different periods. For each case, the improvement rate on the power Doppler (PD) score was assessed at 6 month follow-up. Median improvement rate of PD score was used to classify cases as either US responders or non-responders, and patient characteristics were compared between the two groups. RESULTS: All indicators of clinical disease activity and US activity showed a significant improvement at 3 months compared with baseline. Although the JAK inhibitor-cycler group and the interleukin-6 (IL-6) inhibitor inadequate response (IR) group tended to show a later improvement for US activity, all indicators of clinical disease activity and US activity showed a significant improvement at 6 months compared with baseline for both groups. Multivariate analysis showed that concomitant methotrexate use and an IR to the previous biologic or targeted-synthetic disease-modifying anti-rheumatic drug (b/tsDMARD) treatment were independently and significantly associated with US responders. CONCLUSION: Use of a JAK inhibitor in combination with methotrexate and an absence of IR to any previous b/tsDMARDs demonstrated superior effectiveness for patients with RA.


Subject(s)
Antirheumatic Agents , Arthritis, Rheumatoid , Janus Kinase Inhibitors , Antirheumatic Agents/therapeutic use , Arthritis, Rheumatoid/diagnostic imaging , Arthritis, Rheumatoid/drug therapy , Humans , Janus Kinase Inhibitors/therapeutic use , Japan , Methotrexate/therapeutic use , Prospective Studies , Treatment Outcome , Ultrasonography
2.
Scand J Rheumatol ; 50(6): 436-441, 2021 Nov.
Article in English | MEDLINE | ID: mdl-33719841

ABSTRACT

Objectives: Using multicentre ultrasound (US) cohort data among patients with rheumatoid arthritis (RA), we aimed to identify baseline factors that permit differentiation between two patient cohorts achieving US remission and clinical remission, and to determine the factors contributing to the discrepancy.Method: We reviewed 248 Japanese patients diagnosed with RA who underwent treatment with biological disease-modifying anti-rheumatic drugs at 13 centres. We performed US assessments of the synovia of 22 joints. We assessed the percentages of patients with clinical remission and US remission, defined as total power Doppler scores of 0 at 12 months.Results: The 87 patients who achieved US remission were divided into a group that achieved both clinical and US remission (n = 53) and a group that achieved US remission only (n = 34). Baseline factors that were significantly and independently associated with clinical remission at 12 months among patients who also achieved US remission included short disease duration, the presence of concomitant methotrexate use, and low patient global assessment score (p < 0.05, p < 0.05, and p < 0.005, respectively).Conclusions: RA patients with baseline high patient global assessment scores and long disease duration at baseline were unlikely to achieve clinical remission even after achieving US remission. Objective joint assessments using US provide additional information of potential importance for the management of RA.


Subject(s)
Arthritis, Rheumatoid , Antirheumatic Agents/therapeutic use , Arthritis, Rheumatoid/diagnostic imaging , Arthritis, Rheumatoid/drug therapy , Cohort Studies , Humans , Japan , Remission Induction , Treatment Outcome , Ultrasonography
3.
Clin Anat ; 30(2): 276-283, 2017 Mar.
Article in English | MEDLINE | ID: mdl-27888604

ABSTRACT

The anterior cruciate ligament (ACL) is divided into three fiber bundles (AM-M: anteromedial-medial, AM-L: anteromedial-lateral, PL: posterolateral). We attempted to depict the three bundles of the human ACL on MRI images and to obtain 3-dimensional visualization of them. Twenty-four knees of healthy volunteers (14 males, 10 females) were scanned by 3T-MRI using the fat suppression 3D coherent oscillatory state acquisition for the manipulation of imaging contrast (FS 3D-COSMIC). The scanned images were reconstructed after the isotropic voxel data, which allows the images to be reconstructed in any plane, was acquired. We conducted statistical examination on the identification rate of the three ACL bundles by 2D planes. Segmentation and 3D visualization of the fiber bundles using volume rendering were performed. The triple-bundle ACL was best depicted in the oblique axial plane. While the AM-M and AM-L bundles were clearly depicted in all cases, the PL bundle was not clearly visualized in two knees (8%). Therefore, the three ACL bundles were depicted in 22 knees (92%). The results of 3D visualization of the fiber arrangement agreed well with macroscopic findings of previous anatomical studies. 3T-MRI and the isotropic voxel data from FS 3D-COSMIC made it possible to demonstrate the identifiable depiction of three ACL bundles in nearly all cases. 3D visualization of the bundles could be a useful tool to understand the ACL fiber arrangement. Clin. Anat. 30:276-283, 2017. 2016 The Authors. Clinical Anatomy published by Wiley Periodicals, Inc. on behalf of American Association of Clinical Anatomists.


Subject(s)
Anterior Cruciate Ligament/diagnostic imaging , Magnetic Resonance Imaging/methods , Adult , Female , Humans , Imaging, Three-Dimensional , Male , Young Adult
4.
Knee Surg Sports Traumatol Arthrosc ; 19 Suppl 1: S47-53, 2011 Dec.
Article in English | MEDLINE | ID: mdl-21541712

ABSTRACT

PURPOSE: The reconstruction technique to individually reconstruct multi-bundles of the anterior cruciate ligament (ACL) has been improved in the last decade. For further improvement of the technique, the present study was conducted to determine the force sharing among the three bundles (the medial and lateral bundles (AMM and AML) of the anteromedial (AM) bundle and the posterlateral (PL) bundle) of the human ACL in response to hyperextension, passive flexion-extension and anterior force to the knee. METHODS: Using a 6-DOF robotic system, the human cadaveric knee specimens were subjected to hyperextension, passive flexion-extension and anterior-posterior tests, while recording the 6-DOF motion and force/moment of the knees. The intact knee motions recorded during the tests were reproduced after sequential bundle transection to determine the bundle forces. RESULTS: The bundle forces were around 10 N at 5 N-m of hyperextension and remained less than 5 N during passive flexion-extension. In response to 100 N of anterior force, the AMM and PL bundle forces were slightly higher than the AML bundle force at full extension. The AMM bundle force remained at a high level up to 90° of flexion, with significant differences versus the AML bundle force at 15°, 30° and 60° of flexion and the PL bundle force at 90° of flexion. CONCLUSION: The AMM bundle is the primary stabilizer to tibial anterior drawer through wide range of motion, while the AML bundle is the secondary stabilizer in deep flexion angles. The PL bundle is the crucial stabilizer to hyperextension as well as tibial anterior drawer at full extension.


Subject(s)
Anterior Cruciate Ligament Reconstruction/methods , Anterior Cruciate Ligament/physiology , Knee Joint/physiology , Robotics , Aged , Aged, 80 and over , Anterior Cruciate Ligament/surgery , Biomechanical Phenomena , Cadaver , Humans , Knee Joint/surgery , Middle Aged , Range of Motion, Articular/physiology , Reproducibility of Results , Stress, Mechanical
5.
Clin Neurophysiol ; 119(8): 1923-1933, 2008 Aug.
Article in English | MEDLINE | ID: mdl-18579439

ABSTRACT

OBJECTIVE: At present, whole-head MEG systems are designed to accommodate adult heads, thereby introducing a technical issue unique to pediatric MEG. It is known that magnetic field strength decreases as a function of 1/distance(2). For pediatric patients, we questioned whether re-positioning the head to minimize the distance between the expected source location and the MEG sensor array would significantly improve source measurement. METHODS: Somatosensory-evoked fields (SEFs) were recorded in 17 children (mean=4.96 years) with their head placed centrally in the MEG, and then re-positioned laterally to reduce the distance between the cortical source and sensors. Equivalent current dipole (ECD) source models were evaluated for changes in residual variance (RV), signal-to-noise ratio (SNR), moment (strength), and location. RESULTS: Re-positioning the head closer to the sensors resulted in a significant shift in the mediolateral dipole coordinate location, accompanied by a significant increase in the SNR, decrease in the dipole RV, and a reduction in size of ECD confidence volumes. CONCLUSIONS: We conclude that for clinical pediatric measurement of the SEF, repositioning of the head to minimize the distance between the expected SEF source location and the sensor array will significantly improve SEF source measurement and concomitant ECD source modeling. SIGNIFICANCE: These issues are relevant to all pediatric MEG settings involving healthy or clinical populations and underscores the need for future development of a MEG helmet specifically designed for pediatric populations.


Subject(s)
Brain Mapping , Evoked Potentials, Somatosensory/physiology , Head/physiology , Magnetoencephalography , Pediatrics , Adolescent , Age Factors , Child , Child, Preschool , Electric Stimulation/methods , Electrodes , Electroencephalography/methods , Female , Humans , Infant , Male , Median Nerve , Models, Biological
7.
AJNR Am J Neuroradiol ; 39(12): 2371-2377, 2018 12.
Article in English | MEDLINE | ID: mdl-30442696

ABSTRACT

BACKGROUND AND PURPOSE: Abnormalities of oligodendrocytes have been reported in surgical specimens of patients with medically intractable epilepsy. The aim of this study was to compare the MR imaging, magnetoencephalography, and surgical outcome of children with oligodendrocytosis relative to focal cortical dysplasia I. MATERIALS AND METHODS: Oligodendrocytosis included oligodendroglial hyperplasia, oligodendrogliosis, and oligodendroglial-like cells in the white matter, gray matter, or both from children with medically intractable epilepsy. Focal cortical dysplasia I included radial and tangential cortical dyslamination. The MR imaging, magnetoencephalography, type of operation, location, and seizure outcome of oligodendrocytosis, focal cortical dysplasia I, and oligodendrocytosis + focal cortical dysplasia I were compared. RESULTS: Eighteen subjects (39.1%) had oligodendrocytosis, 21 (45.7%) had focal cortical dysplasia I, and 7 (15.2%) had oligodendrocytosis + focal cortical dysplasia I. There were no significant differences in the type of seizures, focal or nonfocal epileptiform discharges, magnetoencephalography, and MR imaging features, including high T1 signal in the cortex, high T2/FLAIR signal in the cortex or subcortical white matter, increased cortical thickness, blurring of the gray-white junction, or abnormal sulcation and gyration among those with oligodendrocytosis, focal cortical dysplasia I, or oligodendrocytosis + focal cortical dysplasia I (P > .01). There were no significant differences in the extent of resection (unilobar versus multilobar versus hemispherectomy), location of the operation (temporal versus extratemporal versus both), or seizure-free outcome of oligodendrocytosis, focal cortical dysplasia I, and oligodendrocytosis + focal cortical dysplasia I (P > .05). CONCLUSIONS: Oligodendrocytosis shared MR imaging and magnetoencephalography features with focal cortical dysplasia I, and multilobar resection was frequently required to achieve seizure freedom. In 15% of cases, concurrent oligodendrocytosis and focal cortical dysplasia I were identified. The findings suggest that oligodendrocytosis may represent a mild spectrum of malformations of cortical development.


Subject(s)
Drug Resistant Epilepsy/etiology , Malformations of Cortical Development/diagnostic imaging , Malformations of Cortical Development/surgery , Oligodendroglia/pathology , Adolescent , Brain/diagnostic imaging , Brain/pathology , Brain/surgery , Child , Drug Resistant Epilepsy/diagnostic imaging , Drug Resistant Epilepsy/surgery , Female , Humans , Magnetic Resonance Imaging/methods , Magnetoencephalography , Male , Malformations of Cortical Development/complications , Retrospective Studies , Treatment Outcome
8.
Acta Neurol Scand Suppl ; 186: 29-36, 2007.
Article in English | MEDLINE | ID: mdl-17784535

ABSTRACT

Magnetoencephalography (MEG) provides accurate localizing information of the epileptogenic zones in localization-related epilepsies. Refractory status epilepticus (RSE) is a life-threatening emergency that often requires prolonged high-dose suppressive therapy (HDST) to stop frequent and prolonged seizures. Surgical treatments for patients with RSE secondary to pre-existing epilepsy were reported. This article addresses the role of MEG in localizing the epileptogenic zone for the surgical treatment of patients with RSE. Five pediatric patients with RSE underwent epilepsy surgery using MEG, scalp video EEG and magnetic resonance imaging (MRI). Ictal MEG spike sources (MEGSSs) were localized in the clustered interictal MEGSSs in right Rolandic region (patient 3) and right temporal region (patient 5). Interictal MEG revealed unilateral clustered MEGSSs in four patients (patients 1, 2, 4, and 5) and bilateral (patient 3). Ictal-onset EEG findings were localized to one region in three patients (patients 1, 3, and 5) and two regions in the other two patients (patients 2 and 4). In all five patients, interictal discharges were widespread involving over two lobes (patients 2 and 4) or three lobes (patients 1, 3, and 5). Suppression burst pattern was obtained by HDST (patient 5). MRI showed cortical dysplasia in three patients (patients 1, 3, and 4). Patient 2 had a normal MRI. Patient 5 had normal MRI at the onset. Repeat MRI 5 days later showed diffusion restriction in the right hippocampus associated with increased signal intensity on T2 and FLAIR sequences. We performed cortical excision in two patients (patients 1 and 4), hemispherectotomy one (patient 3) and anterior temporal lobectomy two patients (patients 2 and 5). Two patients (patients 1 and 3) became seizure free, the other three patients experienced residual seizures. MEG showed clustered MEGSSs during the RSE in the pre-existing epilepsy patients and at an early time window in the acute symptomatic RSE patients. The complete resection of clustered MEGSSs can control RSE and possibly lead to a seizure free outcome.


Subject(s)
Brain/pathology , Brain/surgery , Magnetoencephalography/methods , Preoperative Care/methods , Status Epilepticus/diagnosis , Status Epilepticus/surgery , Action Potentials , Adolescent , Brain/physiopathology , Brain Mapping/methods , Child , Child, Preschool , Electroencephalography , Female , Gadolinium , Humans , Magnetic Resonance Imaging , Male , Neurosurgical Procedures/methods , Predictive Value of Tests , Status Epilepticus/physiopathology , Temporal Lobe/pathology , Temporal Lobe/physiopathology , Temporal Lobe/surgery , Treatment Outcome
9.
J Neuroendocrinol ; 19(1): 54-65, 2007 Jan.
Article in English | MEDLINE | ID: mdl-17184486

ABSTRACT

The effects of intraperitoneal (i.p.) administration of 2-buten-4-olide (2-B4O), an endogenous sugar acid, on the hypothalamo-adenohypophysial system were examined in Lewis rats that were normal and in adjuvant-induced arthritic (AA) rats. In comparison with vehicle-treated rats, the plasma corticosterone and c-fos mRNA levels in the paraventricular nucleus (PVN) of normal rats increased significantly after i.p. administration of 2-B4O. Dual immunostaining revealed that almost all corticotrophin-releasing factor (CRF)-immunopositive neurones in the parvocellular division of the PVN exhibited Fos-like immunoreactivity (LI) 120 min after i.p. administration of 2-B4O (100 mg/kg). In the AA rats, repeated i.p. administration of 2-B4O (100 mg/kg) after immunisation significantly suppressed the expression of clinical symptoms and significantly increased plasma concentrations of corticosterone. Further, repeated i.p. administration of 2-B4O significantly increased CRF mRNA levels in the PVN and pro-opiomelanocortin mRNA levels in the anterior pituitary; however, they did not change arginine vasopressin mRNA levels in the parvocellular division of the PVN. These results suggest that i.p. administration of 2-B4O activates the hypothalamo-pituitary-adrenal (HPA) axis via the activation of CRF neurones in the PVN, and the activation of the HPA axis by i.p. administration of 2-B4O may be associated with the inhibition of AA in rats.


Subject(s)
4-Butyrolactone/analogs & derivatives , Arthritis, Experimental , Hypothalamo-Hypophyseal System/drug effects , Pituitary-Adrenal System/drug effects , 4-Butyrolactone/administration & dosage , 4-Butyrolactone/pharmacology , Adjuvants, Immunologic , Animals , Appetite Depressants/pharmacology , Arginine Vasopressin/metabolism , Arthritis, Experimental/blood , Arthritis, Experimental/metabolism , Corticosterone/blood , Corticotropin-Releasing Hormone/metabolism , Hypothalamo-Hypophyseal System/metabolism , Injections, Intraperitoneal , Male , Proto-Oncogene Proteins c-fos/metabolism , Rats , Rats, Inbred Lew
10.
Acta Neurol Scand ; 115(4 Suppl): 29-36, 2007 Apr.
Article in English | MEDLINE | ID: mdl-17362274

ABSTRACT

Magnetoencephalography (MEG) provides accurate localizing information of the epileptogenic zones in localization-related epilepsies. Refractory status epilepticus (RSE) is a life-threatening emergency that often requires prolonged high-dose suppressive therapy (HDST) to stop frequent and prolonged seizures. Surgical treatments for patients with RSE secondary to pre-existing epilepsy were reported. This article addresses the role of MEG in localizing the epileptogenic zone for the surgical treatment of patients with RSE. Five pediatric patients with RSE underwent epilepsy surgery using MEG, scalp video EEG and magnetic resonance imaging (MRI). Ictal MEG spike sources (MEGSSs) were localized in the clustered interictal MEGSSs in right Rolandic region (patient 3) and right temporal region (patient 5). Interictal MEG revealed unilateral clustered MEGSSs in four patients (patients 1, 2, 4, and 5) and bilateral (patient 3). Ictal-onset EEG findings were localized to one region in three patients (patients 1, 3, and 5) and two regions in the other two patients (patients 2 and 4). In all five patients, interictal discharges were widespread involving over two lobes (patients 2 and 4) or three lobes (patients 1, 3, and 5). Suppression burst pattern was obtained by HDST (patient 5). MRI showed cortical dysplasia in three patients (patients 1, 3, and 4). Patient 2 had a normal MRI. Patient 5 had normal MRI at the onset. Repeat MRI 5 days later showed diffusion restriction in the right hippocampus associated with increased signal intensity on T2 and FLAIR sequences. We performed cortical excision in two patients (patients 1 and 4), hemispherectotomy one (patient 3) and anterior temporal lobectomy two patients (patients 2 and 5). Two patients (patients 1 and 3) became seizure free, the other three patients experienced residual seizures. MEG showed clustered MEGSSs during the RSE in the pre-existing epilepsy patients and at an early time window in the acute symptomatic RSE patients. The complete resection of clustered MEGSSs can control RSE and possibly lead to a seizure free outcome.


Subject(s)
Magnetoencephalography , Neurosurgery/methods , Status Epilepticus/physiopathology , Status Epilepticus/surgery , Adolescent , Brain Mapping , Child , Child, Preschool , Electroencephalography/methods , Female , Humans , Magnetic Resonance Imaging , Male , Time Factors , Treatment Outcome
11.
J Neurol Neurosurg Psychiatry ; 77(11): 1256-61, 2006 Nov.
Article in English | MEDLINE | ID: mdl-16891385

ABSTRACT

OBJECTIVE: To characterise magnetoencephalographic spike sources in paediatric patients with auditory auras and recurrent localisation-related epilepsy. METHODS: Six patients (four boys and two girls (ages 7-14 years) were retrospectively studied. All patients had auditory auras as part of their initial seizure manifestation, including four patients who underwent previous brain surgery. Scalp video electroencephalography and magnetoencephalography (MEG) were carried out in six patients, intraoperative electrocorticography in three patients and extraoperative intracranial video electroencephalography in one patient. MEG auditory-evoked fields (AEFs) were studied in four patients. RESULTS: Three patients had elementary auditory auras, one had complex auditory aura and two had both complex and elementary auras. All six patients had clustered MEG spike sources with coexisting scattered spike sources. MEG clusters were localised in the superior temporal gyrus with surrounding scatters in four patients (two left and two right); two patients had scattered spikes in the superior temporal gyrus in addition to clustered MEG spike sources in the left inferior and middle frontal gyri or parieto-occipital region. AEFs were located within an MEG cluster in one patient and within 3 cm of a cluster in two patients. Surgical resection, including the regions of MEG clusters, was carried out in four patients. Three of four patients who had previous surgeries were seizure free at 2 years after excision of the MEG cluster region. CONCLUSIONS: MEG spike sources clustered in the superior temporal gyrus in six patients with auditory auras. These spike sources were in close proximity or seemed to engulf the magnetic AEF. Areas with MEG spike sources contained the residual or recurrent epileptogenic zone after incomplete cortical excision for lesional epilepsy.


Subject(s)
Epilepsies, Partial/physiopathology , Temporal Lobe/physiopathology , Adolescent , Auditory Perception , Child , Female , Humans , Magnetoencephalography , Male , Retrospective Studies
12.
Exp Hematol ; 25(11): 1180-6, 1997 Oct.
Article in English | MEDLINE | ID: mdl-9328455

ABSTRACT

Vesnarinone is a positive inotropic agent used for treating congestive heart failure. We evaluated its ex vivo effects on myeloid leukemia cell lines and primary acute myelogenous leukemia cells. Vesnarinone inhibited the incorporation of radiolabeled thymidine by a myeloid cell line, HL60, in a dose-dependent manner at concentrations ranging from 0.1 to 30 microg/mL. A maximum 40% suppression was seen at a concentration of 10 microg/mL. Determination of viable cell counts by trypan blue dye exclusion method demonstrated vesnarinone to be cytocidal for HL60 cells. Vesnarinone induced DNA fragmentation as detected by electrophresis in HL60 cells after 72-hour culture; this effect was not inhibited by G-CSF. The apoptosis induced by vesnarinone was also detected by the in situ end-labeling method. Northern blot analysis showed a reduction of c-myc mRNA expression in HL60 cells by vesnarinone. However, immunostaining assay showed no change in the expression of Fas and Bcl-2 proteins. We next examined the effect of vesnarinone on primary myeloid leukemia cells derived from 10 patients: 3 cases of M1, 2 of M2, 3 of M3, 1 of M4, and 1 of M6, by the French-American-British classification. Vesnarinone inhibited the incorporation of thymidine in all cells, with a mean suppression of 58.1%. DNA electrophoresis showed induction of DNA fragmentation in cultured cells with vesnarinone for 72 hours in 8 of the 10 patients with primary leukemia. However, bone marrow mononuclear cells from healthy controls showed no growth suppression or DNA fragmentation in response to vesnarinone. These results suggest that vesnarinone may be useful in treating myeloid leukemia.


Subject(s)
Antineoplastic Agents/toxicity , Apoptosis/physiology , HL-60 Cells/drug effects , Leukemia, Myeloid, Acute/pathology , Quinolines/toxicity , Adult , Aged , Apoptosis/drug effects , Cell Division/drug effects , DNA Fragmentation , Dose-Response Relationship, Drug , Female , Humans , Leukemia, Myeloid, Acute/classification , Male , Middle Aged , Proto-Oncogene Proteins c-bcl-2/analysis , Pyrazines , Tumor Cells, Cultured , fas Receptor/analysis
13.
Neurology ; 45(1): 108-17, 1995 Jan.
Article in English | MEDLINE | ID: mdl-7824098

ABSTRACT

We made a pathologic diagnosis of chronic encephalitis on surgical resections or autopsy material in 10 patients with intractable seizures and studied the specimens by immunohistochemistry for herpes simplex virus (HSV) 1 and 2 and cytomegalovirus (CMV) as well as by the polymerase chain reaction (PCR) for viral DNA sequences (HSV1, HSV2, and CMV). We also assessed eight patients (nonepileptic) with pathologically documented or clinically suspected encephalitis and five resections from epileptics without encephalitis. Immunohistochemistry for viral antigens was negative in all cases. Using PCR assay, CMV was present in six and HSV1 in two of 10 epilepsy patients with chronic encephalitis. We demonstrated CMV by in situ hybridization in two of the six patients positive for CMV by PCR. We found no viral sequences by PCR in five epileptics without encephalitis. Of the eight patients (nonepileptic) with clinically suspected or pathologically confirmed encephalitis, two cases showed CMV sequences by PCR. These observations suggest that PCR allows detection of viral sequences in some cases of chronic encephalitis associated with epilepsy that may be missed by in situ hybridization.


Subject(s)
Cytomegalovirus/isolation & purification , Encephalitis/microbiology , Epilepsy/microbiology , Herpesvirus 1, Human/isolation & purification , In Situ Hybridization/methods , Polymerase Chain Reaction/methods , Adolescent , Age of Onset , Autopsy , Base Sequence , Child , Child, Preschool , DNA Primers , Encephalitis/complications , Encephalitis/pathology , Epilepsy/complications , Epilepsy/pathology , Humans , Infant , Magnetic Resonance Imaging , Molecular Sequence Data , Syndrome
14.
Neurology ; 57(4): 590-6, 2001 Aug 28.
Article in English | MEDLINE | ID: mdl-11524465

ABSTRACT

OBJECTIVE: To describe the diagnosis, treatment, and outcomes in children with malignant rolandic-sylvian epilepsy (MRSE), defined as a form of epilepsy characterized by sensorimotor seizures, medical refractoriness, normal MRI, frontocentrotemporal EEG spikes, rolandic-sylvian spike sources on magnetoencephalography (MEG), and cognitive problems. METHODS: A retrospective chart analysis of seven patients who had shown these characteristics and undergone extensive diagnostic testing, including MEG and intracranial video-EEG was performed. RESULTS: Interictal scalp EEG spikes were seen over the frontocentrotemporal regions bilaterally (6) and unilaterally (1). MEG showed spike sources in the perisylvian region in two patients (both bilateral) and in the perirolandic fissure in five (two bilateral). Three patients required bilateral subdural strips to lateralize seizures before electrocorticography. Final electrocorticograms showed an ictal onset zone around the rolandic (four cases) and rolandic-sylvian regions (three cases). Six patients showed neuropsychological deficits. After cortical excision and multiple subpial transection, three were seizure free and four had seizures rarely (30 months' mean follow-up). No child had a permanent deficit in sensorimotor or cognitive functions, although two showed exacerbation of preexisting attentional deficits. Tissue analysis showed definite evidence of neuronal migration disorders (3) and gliosis (2). CONCLUSIONS: MEG was helpful for localizing both malignant rolandic-sylvian neuronal activities and functional cortex. Successive neuropsychological assessments are necessary to detect cognitive deterioration and indicate remedial programming. If, after careful observation over at least 5 years, surgery is considered to control refractory seizures, intracranial video-EEG is needed to localize the epileptogenic zone.


Subject(s)
Electroencephalography , Epilepsy, Rolandic/diagnosis , Magnetoencephalography , Adolescent , Child , Electroencephalography/methods , Epilepsy, Rolandic/psychology , Epilepsy, Rolandic/surgery , Evoked Potentials, Somatosensory/physiology , Female , Humans , Magnetoencephalography/methods , Male , Neuropsychological Tests , Retrospective Studies , Telemetry/methods , Video Recording/methods
15.
Neurology ; 51(5): 1263-9, 1998 Nov.
Article in English | MEDLINE | ID: mdl-9818843

ABSTRACT

BACKGROUND: Seizures in patients with tuberous sclerosis complex (TSC) are often intractable to antiepileptic medications and searching investigation may provide evidence that surgical treatment can be considered. OBJECTIVE: To review the results of investigation and surgical therapy, a treatment modality not generally considered in patients with medically refractory seizures and TSC. METHODS: We report 18 patients (9 male) with TSC who underwent surgical treatment of medically refractory epilepsy. Twelve patients had a well-localized epileptogenic lesion and were treated by lesionectomy or focal resection. Resections were: 7 frontal, 4 temporal, 1 frontotemporal, 1 occipital, and 1 frontoparietal. Four patients underwent more than one operation. Six patients had corpus callosotomy (CC). RESULTS: Follow-up ranged from 1 month to 47 years. Outcome of the patients treated by resection was excellent in 7 (5 were seizure-free and 2 had auras only), good in 1, fair in 3, and 1 was lost to follow-up. Best outcome was obtained in patients who had focal seizures and good imaging and EEG correlation, although they might have multiple seizure types, other imaging abnormalities, and multifocal or generalized EEG findings. When there was no such correlation, CC was found to be an option as five patients had at least some improvement and only one showed no change. CONCLUSION: Surgical treatment of patients with TSC and intractable epilepsy is most effective when a single tuber or epileptogenic area can be identified as the source of seizures and resected. This may be possible even when other tubers or diffuse EEG abnormalities are present. In patients with unlocalizable epileptic abnormalities, palliation may be obtained by CC.


Subject(s)
Epilepsy/complications , Epilepsy/surgery , Tuberous Sclerosis/complications , Tuberous Sclerosis/surgery , Adolescent , Adult , Age of Onset , Brain/pathology , Child , Child, Preschool , Electroencephalography , Female , Follow-Up Studies , Humans , Infant , Magnetic Resonance Imaging , Male , Middle Aged , Reoperation , Retrospective Studies , Time Factors , Treatment Outcome
16.
Neuroreport ; 12(18): 3923-7, 2001 Dec 21.
Article in English | MEDLINE | ID: mdl-11742212

ABSTRACT

Neuromagnetic spectral distributions when silently viewing words and non-words were investigated using a 151-channel whole head MEG system. The data were analyzed with synthetic aperture magnetometry (SAM). The dominant changes of spectral power were identified in the parietal and occipital cortices in 15-30 Hz, 30-60 Hz and 60-125 Hz bands following both word and non-word stimulations. The changes in Broca's and Wernicke's areas were consistently observed in 60-125 Hz bands, mainly following word stimulation. The results indicated that the occipital and parietal cortices were most probably involved in visuospatial processing of words and non-words. The language areas, particularly Broca's and Wernickes's areas, might be involved in implicit word processing such as verbal searching and/or internal speech.


Subject(s)
Cerebral Cortex/physiology , Evoked Potentials, Visual/physiology , Magnetoencephalography , Reading , Adult , Female , Frontal Lobe/physiology , Humans , Male , Occipital Lobe/physiology , Parietal Lobe/physiology , Temporal Lobe/physiology
17.
Int J Hematol ; 68(4): 445-8, 1998 Dec.
Article in English | MEDLINE | ID: mdl-9885444

ABSTRACT

A 20-year-old male with ulcerative colitis complicated by mesalazine-associated severe aplastic anemia is described. The patient developed aplastic anemia four months after the start of mesalazine therapy. He was treated with antithymocyte globulin, cyclosporine, and granulocyte colony-stimulating factor (G-CSF) and responded well. Hematological complications of mesalazine are rare, but if bone marrow suppression is detected, immediate cessation of the drug and intensive immunosuppressive treatment with G-CSF should be considered.


Subject(s)
Anemia, Aplastic/chemically induced , Anemia, Aplastic/drug therapy , Anti-Inflammatory Agents, Non-Steroidal/adverse effects , Antilymphocyte Serum/therapeutic use , Cyclosporine/therapeutic use , Granulocyte Colony-Stimulating Factor/therapeutic use , Mesalamine/adverse effects , Adult , Drug Therapy, Combination , Humans , Male
18.
Int J Hematol ; 72(4): 463-5, 2000 Dec.
Article in English | MEDLINE | ID: mdl-11197212

ABSTRACT

Chronic natural killer (NK) lymphocytosis involves a persistent increase in CD56+ large granular lymphocytes (LGLs) that is sometimes associated with immune-mediated complications, such as anemia and neutropenia. However, aplastic anemia (AA) is a rare complication. Here we describe 2 patients with severe AA who presented with persistent increases in NK cells. Their LGLs were positive for CD56, CD16, and intracellular interferon (IFN)-gamma but negative for CD3, Fas-ligand, and T-cell receptor rearrangement, findings that are compatible with NK cells. Not only the number of NK cells, but NK activity as well, was increased in both patients. The number of NK cells changed according to hematologic recovery and relapse in 1 case. Thus, there seemed to be a close relationship between NK cells and the progression of AA, at least in this instance. Further investigation of the clinical course of similar cases and the characteristics of NK cells is necessary.


Subject(s)
Anemia, Aplastic/complications , Killer Cells, Natural , Lymphocytosis/etiology , Aged , Anemia, Aplastic/blood , CD56 Antigen/blood , CD56 Antigen/drug effects , Chronic Disease , Humans , Immunosuppressive Agents/pharmacology , Immunosuppressive Agents/therapeutic use , Killer Cells, Natural/pathology , Lymphocytosis/blood , Male , Middle Aged , Platelet Count
19.
Clin Neurophysiol ; 110(2): 374-7, 1999 Feb.
Article in English | MEDLINE | ID: mdl-10210627

ABSTRACT

OBJECTIVE: We used coherence analysis to test for leading discharges on an ipsilateral right mesial temporal lesion in a 5 year old boy with flexor spasms. METHOD: Digital EEG analysis with video-EEG telemetry was performed preparatory to epilepsy surgery. RESULTS: Study of 10 spasms with head drop and subsequent flexion of both arms demonstrated an interhemispheric time lag with secondary bilateral synchrony, with a mean difference of 17 ms. The right hemisphere led. After a lesionectomy with resection of epileptic regions (performed with electrocorticographical guidance), the patient has been seizure-free for 4 years. Pathology confirmed a low-grade mixed glioma and cortical dysgenesis. CONCLUSION: The coherence analysis demonstrated a pathway of secondary generalization, confirming that the lesional side was leading during ictal generalized discharges in flexor spasms.


Subject(s)
Brain/physiopathology , Electroencephalography/methods , Spasms, Infantile/physiopathology , Child, Preschool , Humans , Male , Monitoring, Physiologic , Time Factors
20.
Clin Neurophysiol ; 111(1): 161-8, 2000 Jan.
Article in English | MEDLINE | ID: mdl-10656524

ABSTRACT

OBJECTIVES: To assess the reliability of dipole localization based on residual variances (RV), using equivalent current dipole analysis of interictal EEG spikes in children with extratemporal lobe epilepsy. METHODS: Four pediatric patients with extratemporal lobe epilepsy were studied. Digital EEG was recorded from 19 scalp electrodes. Computer programs for spike detection and clustering analysis were used to select spikes. Dipoles were calculated 5 times for each spike using different initial guesses by the moving dipole model. Standard deviation (SD) of the dipole positions was calculated at each time point in the 5 trials. RESULTS: We analyzed the dipoles at 1097 time points from 4 patients. Among 106 time points with RV < 2%, the SD was < 1 mm in 78 (74%), while in those with SD > 1 mm the dipole positions varied between 2.8 and 52.6 mm. Of dipoles with RV < 1%, 26 of 27 (96%) had an SD < 1 mm; the one dipole with SD > 1 mm varied within 2.5 mm. The dipole localizations with RV < 2% corresponded to the epileptogenic zones identified on intracranial invasive video EEG and intraoperative ECoG. CONCLUSIONS: The systematic approach of equivalent current dipole analysis using spike detection, clustering analysis, and an RV < 2% as a standard is useful for identifying extratemporal epileptic regions.


Subject(s)
Electroencephalography , Epilepsies, Partial/diagnosis , Epilepsies, Partial/physiopathology , Epilepsy, Frontal Lobe/physiopathology , Adolescent , Brain/physiopathology , Brain Diseases/complications , Child , Cysts/complications , Epilepsies, Partial/etiology , Epilepsy, Frontal Lobe/diagnosis , Epilepsy, Rolandic/diagnosis , Epilepsy, Rolandic/physiopathology , Female , Humans , Magnetic Resonance Imaging , Male , Reproducibility of Results , Scalp , Software
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