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Biomedical subjects

Y Hiraki

Publications and source records attributed to Y Hiraki.

At least 19 recordsLinked to original sources
PubMed2006

Expression of the PTH/PTHrP receptor in chondrogenic cells during the repair of full-thickness defects of articular cartilage.

OBJECTIVE: We studied the accumulation of parathyroid hormone (PTH)/PTHrP receptor-positive mesenchymal cells using double immunohistochemistry and examined whether this correlated with the subsequent regeneration of 3-mm-diameter full-thickness defects of articular cartilage. MATERIALS AND METHODS: Cylindrical full-thickness articular cartilage defects (3 mm) were artificially created in the femoral trochlea of male adolescent Japanese white rabbits (n = 210) with a hand-drill. Recombinant human PTH(1-84) was then administered into the defect cavities with an osmotic pump for either 2 or 4 weeks post-injury. Following PTH treatment, the repair processes in the cartilage defects were histologically examined. Double immunostaining analyses for the PTH/PTH-related peptide (PTHrP) receptor and proliferating cell nuclear antigen (PCNA) in the regenerating tissues were then performed. RESULTS: Activation of PTH/PTHrP receptor signaling by hPTH(1-84) results in the inhibition of chondrogenic differentiation in full-thickness articular cartilage defects. At the conclusion of the 2-week PTH treatment, the defect cavities were filled with undifferentiated mesenchymal cells, which were similar to the controls. In addition, almost all of these cells localized at the center of the injuries were both PTH/PTHrP receptor- and PCNA-positive. In contrast, after prolonged PTH treatment for 4 weeks, there was no indication of a cartilaginous repair response and cells that had migrated to the defect cavities were found to have irreversibly lost expression of the PTH/PTHrP receptor. CONCLUSIONS: The chondrogenic capacity of cells that had migrated to the area of these defect cavities is closely associated with their ability to express the PTH/PTHrP receptor. Moreover, these cells maintain their chondrogenic potential within only a limited time-span of 2 weeks.

Animals↗
PubMed2005

Identification of clonally rearranged T-cell receptor beta chain genes in HTLV-I carriers as a potential instrument for early detection of neoplasia.

We analyzed the genetic recombination pattern of the T-cell receptor beta-chain gene (TCR-beta) in order to identify clonal expansion of T-lymphocytes in 17 human T-lymphotropic virus type I (HTLV-I)-positive healthy carriers, 7 of them with abnormal features in the peripheral blood lymphocytes. Monoclonal or oligoclonal expansion of T-cells was detected in 5 of 7 HTLV-I-positive patients with abnormal lymphocytes and unconfirmed diagnosis by using PCR amplification of segments of TCR-beta gene, in a set of reactions that target 102 different variable (V) segments, covering all members of the 24 V families available in the gene bank, including the more recently identified segments of the Vbeta-5 and Vbeta-8 family and the two diversity beta segments. Southern blots, the gold standard method to detect T-lymphocyte clonality, were negative for all of these 7 patients, what highlights the low sensitivity of this method that requires a large amount of very high quality DNA. To evaluate the performance of PCR in the detection of clonality we also analyzed 18 leukemia patients, all of whom tested positive. Clonal expansion was not detected in any of the negative controls or healthy carriers without abnormal lymphocytes. In conclusion, PCR amplification of segments of rearranged TCR-beta is reliable and highly suitable for the detection of small populations of clonal T-cells in asymptomatic HTLV-I carriers who present abnormal peripheral blood lymphocytes providing an additional instrument for following up these patients with potentially higher risk of leukemia.

Adult↗
PubMed2004

Preoperative concurrent chemoradiotherapy with cisplatin and docetaxel in patients with locally advanced non-small-cell lung cancer.

The objective of this study was to assess the feasibility and effectiveness of an induction chemoradiotherapy regimen followed by surgery in patients with locally advanced non-small-cell lung cancer (LA-NSCLC). A total of 22 patients with LA-NSCLC were treated with induction chemoradiotherapy consisting of cisplatin (40 mg m(-2)) and docetaxel (40 mg m(-2)) given on days 1, 8, 29 and 36 plus concurrent thoracic irradiation at a dose of 40-60 Gy (2 Gy fraction(-1) day(-1)). Surgical resection was performed within 6 weeks after completion of induction therapy. Objective response to the induction therapy was obtained in 16 patients (73%). In all, 20 patients (91%) underwent surgery and complete resection was achieved in 19 patients (86%). Pathological downstaging and pathological complete response were obtained in 14 (64%) and five (23%) patients, respectively. With a median follow-up period of 32 months, the calculated 3-year overall and progression-free survival rates were 66 and 61%, respectively. It is noteworthy that the 3-year overall survival rate in 14 patients achieving pathological downstaging was extremely high (93%). Toxicity was manageable with standard approaches. No treatment-related deaths occurred. This combined modality treatment is feasible and highly effective in patients with LA-NSCLC. The results warrant further large-scale study to confirm the effectiveness of this regimen.

Adult↗
PubMed2004

One day exposure to FGF-2 was sufficient for the regenerative repair of full-thickness defects of articular cartilage in rabbits.

OBJECTIVES: Administration of fibroblast growth factor (FGF)-2 for 2 weeks induces a successful cartilaginous repair response in 5-mm full-thickness articular cartilage defects in rabbits. The purpose of this study was to investigate the effects of a short time exposure to FGF-2 on the repair of the defects. METHODS: Five-mm-diameter cylindrical defects, which do not repair spontaneously, were created in the femoral trochlea of the rabbit knees. The defects were administered sterile saline or FGF-2 (150pg/h) via an osmotic pump for the initial 1 day, 3 days, or 2 weeks, and we assessed the FGF-2 action on the proliferation and migration of mesenchymal cells in the reparative tissue. Using a total of 126 rabbits, we performed three sets of experiments. We also studied the effect of FGF-2 on migration of marrow-derived mesenchymal cells in vitro. RESULTS: FGF-2 treatment for 1 day or 3 days induced the sequential chondrogenic repair responses that led to successful cartilaginous resurfacing of defects within 8 weeks as well as the 2-week treatment did. We confirmed by a radioisotope study that FGF-2 injected was rapidly eliminated from the defects (a residual ratio of 50% within 30min). The effect of FGF-2 on cultured marrow-derived cells suggested that FGF-2 facilitated the mobilization and migration of replicating mesenchymal cells from bone marrow. CONCLUSIONS: Only 1 day exposure to FGF-2 is sufficient for induction of the chondrogenic repair response in 5-mm-diameter full-thickness defects of articular cartilage in rabbits. FGF-2 stimulated the recruitment of mesenchymal cells into the defects, which was a limiting step for the induction of cartilage.

Animals↗
PubMed2004

Development of a phantom compatible for MRI and hyperthermia using carrageenan gel-relationship between T1 and T2 values and NaCl concentration.

The authors developed a phantom, designated as the CAGN phantom, compatible for MRI and hyperthermia that is useful in the fundamental studies of non-invasive MR thermometry. The ingredients of this phantom are carrageenan, GdCl3 as a T1 modifier, agarose as a T2 modifier, NaCl as a conductivity modifier, NaN3 as an antiseptic and distilled water. Another phantom that was developed, the CAG phantom, has relaxation times that are adjustable to those of any human tissue. To use this phantom for electromagnetic heating, NaCl was added to change the conductivity of the phantom and clarified the relationship between the conductivity and NaCl concentration. This study examined the relationship between relaxation times and NaCl concentration of the CAGN phantom. The results showed that both T1 and T2 values were affected by NaCl and the experimental results led to the empirical formulae expressing the relationship between the relaxation rates (1/T1, 1/T2) and the concentrations of GdCl3, agarose and NaCl. The appropriate concentrations of T1 and T2 modifiers were calculated from these empirical formulae when making a specified phantom that has the required relaxation times and NaCl concentration.

Algorithms↗
PubMed2004

Development of a phantom compatible for MRI and hyperthermia using carrageenan gel--relationship between dielectric properties and NaCl concentration.

A phantom has previously been developed containing carrageenan, agarose and gadolinium chloride (called CAG phantom) for MRI with 1.5 T. T(1) and T(2) relaxation times of this phantom are independently changeable by varying concentrations of relaxation-time modifiers to simulate relaxation times of the various types of human tissues. The CAG phantom has a T(1) value of 202-1904 ms and a T(2) value of 38-423 ms, when the GdCl(3) concentration is varied from 0-140 micromol/kg and the agarose concentration is varied from 0-1.6%. A new phantom has now been developed (called CAGN phantom), made by adding an electric conductive agent, NaCl, to the CAG phantom for use in the areas of MRI and hyperthermia research. Dielectric properties of the CAGN phantom were measured and the results of experiments were expressed by the Cole-Cole equation in the frequency range of 5-130 MHz. The relationship between the conductivity of the CAGN phantom and the concentration of NaCl was expressed by a linear function in the frequency range of 1-130 MHz. The linear function involves a parameter of frequency and, when the frequency is 10 MHz, the conductivity of the CAGN phantom can be changed from 0.27-1.26 Sm(-1) by increasing the NaCl concentration from 0-0.7%. The CAGN phantom developed can be employed in basic experiments for non-invasive temperature measurement using MRI and as a loading phantom for MRI with up to 3 T.

Carrageenan↗
PubMed2004

[Report on the 89th Scientific Assembly and Annual Meeting of the Radiological Society of North America--analysis of radiation dose by a change of a scan parameter of multi-slice computed tomography by a film method].

PURPOSE: It is for a purpose of this study to measure radiation dose by analyzing a dose profile of multi-slice computed tomography varying with helical pitch and a row slice thickness difference complicatedly. MATERIALS AND METHODS: We used multi-slice computed tomography, and helical pitch and row slice thickness change and scanned the helical scan. I used CTDI phantom of a diameter of 25 cm and I inserted roentgen diagnosis use film UR-2(new) which I put between my own phantom in center and 1 cm away from the outer surface and scanned it. And the provided level profile was converted into a dose profile with the dose-density curve which I made beforehand. I analyzed radiation dose than the dose profile. RESULT: In multi-slice computed tomography, radiation dose varied with assembly of row slice thickness and helical pitch. The change of a dose profile changed in a phantom surface part complicatedly. The maximum dose by the measurement of this time was 29 mGy in row slice thickness 0.5 mm, assembly of helical pitch 2.5. In addition, the minimum dose was 6.8 mGy in row slice thickness 3.0 mm, assembly of helical pitch 5.5. And, as for the difference of maximum dose in the same dose profile and the smallest dose, there were about 20 % in row slice thickness 1.0 mm, assembly of helical pitch 5.5. CONCLUSION: The dosimetry of multi-slice computed tomography by a film method enabled it to measure a change of a dose profile by a difference of a scan parameter by high interest solution ability. In addition, it is a method more superior in dosimetry of multi-slice computed tomography spreading through a Z-axis direction broadly than determination by computed tomography use ionization chamber dosimeter. Because radiation dose increases by a scan in thin row slice thickness and small helical pitch, care is necessary.

Phantoms, Imaging↗
PubMed2003

Phase I/II study of docetaxel and cisplatin with concurrent thoracic radiation therapy for locally advanced non-small-cell lung cancer.

Recent studies have suggested the superiority of concomitant over sequential administration of chemotherapy and radiotherapy. Docetaxel and cisplatin have demonstrated efficacy in advanced non-small-cell lung cancer (NSCLC). This study evaluated the safety, toxicity, and antitumour activity of docetaxel/cisplatin with concurrent thoracic radiotherapy for patients with locally advanced NSCLC. Patients with locally advanced NSCLC (stage IIIA or IIIB), good performance status, age or=3 toxicities of 71, 60, 24, and 19%, respectively. Toxicity was significant, but manageable according to the dose and schedule modifications. Dose intensities of docetaxel and cisplatin were 86 and 87%, respectively. Radiotherapy was completed without a delay in 67% of 42 patients. The overall response rate was 79% (95% confidence interval (CI), 66-91%). The median survival time was 23.4+ months with an overall survival rate of 76% at 1 year and 54% at 2 years. In conclusion, chemotherapy with cisplatin plus docetaxel given on days 1, 8, 29, and 36 and concurrent thoracic radiotherapy is efficacious and tolerated in patients with locally advanced NSCLC and should be evaluated in a phase III study.

Adult↗
PubMed2003

Long-term survivors of advanced esophageal cancer without surgical treatment: a multicenter questionnaire survey in Kyushu, Japan.

Since the introduction of recent improvements in adjuvant therapy for esophageal cancer, some patients have demonstrated good prognosis. In the present study, we analyzed 3- and 5-year survivors of advanced esophageal cancer who did not undergo any surgical treatment. Between 1990 and 1998, 831 patients were admitted to 14 university hospitals and one cancer center associated with the membership of the Kyushu study group for adjuvant therapy of esophageal cancer. Twelve (1.4%) of the patients were 3-year survivors and 13 (1.6%) were 5-year survivors. The reasons for non-operation were refusal (eight patients), tumor-related factors (11 patients), and host-related factors (six patients). With a single exception, all patients had locally advanced tumors. Almost all long-term survivors had fewer than five lymph node metastases, in regions limited to the neck and/or mediastinum. Radiation therapy was combined with chemotherapy for 16 of the 25 patients, and chemotherapy-based cisplatin was used for 15 of these 16 patients. Fifteen of the patients remain alive; 10 died seven of them from esophageal cancer. Chemoradiation therapy was effective for some patients with locally advanced esophageal cancer, particularly in the absence of or with few lymph node metastases. To improve the prognosis of patients with advanced esophageal cancer who, for various causes, cannot undergo surgical treatment, a new protocol for adjuvant therapy is required.

Aged↗
PubMed2001

Molecular cloning of tenomodulin, a novel chondromodulin-I related gene.

Murine expressed sequence tags (EST) showing homology with chondromodulin-I (ChM-I) were identified. Cloning of the full-length cDNA revealed a novel protein (317 amino acid residues) having a domain homologous to ChM-I, and we termed it tenodmoulin (TeM). The predicted amino acid sequence revealed 33% overall identity with mouse ChM-I precursor. Overall structural features were conserved well in TeM, including a single transmembrane domain at the N-terminal region and the putative antiangiogenic domain with eight cysteine residues. However, TeM lacked a hormone-processing signal present in the ChM-I precursor, suggesting that it may function as a type II transmembrane protein on cell surface. TeM transcript (1.4 kb in size) was detected in skeletal muscle by Northern blot analysis. In situ hybridization analysis revealed that the expression of TeM mRNA was not associated with muscle fibers, but was tightly associated with epimysium and tendon, both of which are classified as dense connective tissue having little vascularity.

Amino Acid Sequence↗
PubMed2001

Detection of hepatic metastasis from medullary thyroid cancer with Tc-99m-MIBI scintigraphy in a patient with Sipple's syndrome.

We report a case of Sipple's syndrome with a hepatic tumor. A 28-year-old male with medullary thyroid cancer and bilateral pheochromocytomas was diagnosed as having Sipple's syndrome. The hepatic tumor was found to be hypervascular by means of a dynamic abdominal CT scan. An I-131-MIBG scan showed no increased accumulation in the tumor. A whole body Tc-99m-MIBI scan had shown, however, increased accumulation in the tumor by 10 minutes after i.v. Tc-99m-MIBI. The hepatic tumor was diagnosed histologically as a metastasis from the medullary thyroid cancer. To our knowledge, there have been no previous reports of increased Tc-99m-MIBI accumulation in hepatic metastases from medullary thyroid cancer.

Adrenal Gland Neoplasms↗
PubMed2001

Cartilage-specific matrix protein chondromodulin-I is associated with chondroid formation in salivary pleomorphic adenomas: immunohistochemical analysis.

Chondromodulin-I (ChM-I) is a novel cartilage-specific matrix protein. In the growth plates of the long bones, ChM-I was shown to be expressed in mature to upper hypertrophic chondrocytes, and to be deposited in the cartilage matrix. As ChM-I strongly inhibits angiogenesis, cartilage is avascular. Also, ChM-I has bifunctional activity against chondrocyte proliferation. On the other hand, pleomorphic adenomas of the salivary glands frequently have chondroid elements. To elucidate the relationship between chondroid formation and hypovascularity in salivary pleomorphic adenomas, we immunohistochemically examined the expression and localization of ChM-I in 35 cases of this tumor. ChM-I was immunolocalized to the lacunae in the chondroid elements of pleomorphic adenomas (100%). Type II collagen and aggrecan were immunolocalized throughout the matrix around lacuna cells of the chondroid element (100%, 91.7%), and ChM-I was infrequently immunolocalized to the spindle-shaped myoepithelial cells in the myxoid element (37.5%). Fibroblast growth factor-2 was strongly immunolocalized to the lacuna cells in the chondroid element (100%), among the neoplastic myoepithelial cells in the myxoid elements (96.9%), and on the basement membranes around the solid nests of neoplastic myoepithelial cells (71.4%). Although CD34 is a marker of endothelial cells, CD34 was expressed in the endothelial cells in only a few areas around the epithelial elements and in the fibrous element of pleomorphic adenomas. No signals for CD34 were observed in chondroid elements in pleomorphic adenomas (P < 0.001), but a few signals were seen in the myxoid elements (P < 0.05). These findings suggested that lacuna cells and neoplastic myoepithelial cells expressed ChM-I, and that this molecule may play an important role in hypovascularity and chondroid differentiation in pleomorphic adenoma. In conclusion, pleomorphic adenoma expressed ChM-I, which is involved in hypovascularity and chondroid formation in this type of tumor.

Adenoma, Pleomorphic↗
PubMed2001

Sequence analysis of zebrafish chondromodulin-1 and expression profile in the notochord and chondrogenic regions during cartilage morphogenesis.

Chondromodulin-I (ChM-I) is suggested in higher vertebrate systems to function as a key regulatory protein for cartilage development. To further understand the process of chondrogenesis and the function of ChM-I, we have cloned the zebrafish cDNA for chondromodulin-1 (chm1) and have mapped the chm1 gene locus. The expression profile of chm1 was determined during zebrafish embryonic development and compared to that of type II collagen (col2a1). Maternal chm1 transcripts were detected before midblastula transition and zygotic expression of chm1 was first observed in the notochord at the 10-somite stage. At later developmental stages, chm1 expression was detected in areas surrounding the otic vesicles, in the developing craniofacial cartilage elements, and in the chondrogenic region of the pectoral fins.

Amino Acid Sequence↗
PubMed2001

Differentiation of chondrogenic precursor cells during the regeneration of articular cartilage.

OBJECTIVE: Full-thickness defects that penetrate articular cartilage are filled by fibrous, or fibrocartilaginous tissue and, to a very limited extent, also by hyaline cartilage. In rabbits, small full-thickness defects (to < or =3 mm in diameter) are capable of regenerating surfacing hyaline cartilage. However, chondrogenic differentiation does not occur in larger defects (> or =5 mm in diameter). We studied the involvement of fibroblast growth factor-2 (FGF-2) in the cartilaginous repair response in full-thickness defects of articular cartilage in vivo, and attempted to facilitate cartilaginous repair of the defects by the local administration of FGF-2. DESIGN: The right knee joint of male adolescent Japanese white rabbits was entered through a medial parapatellan approach, and the patella was dislocated laterally to expose the articular surface of the femoral trochlea. Full-thickness defects were created in the weight-bearing area of the femoral trochlea with a hand-drill (the 5-mm diameter defects in 80 rabbits and the 3-mm diameter defects in 40 rabbits). The animals were fitted with an osmotic pump connected to silastic medical grade tubing, and a length of the tubing about 5 mm long was introduced into the articular knee cavity. The 5-mm-diameter defects received FGF-2 (50 pg/h) or sterile saline via an osmotic pump for the initial 2 weeks. Five animals each were sacrificed after 1, 2, 4, 8, or 24 weeks after creation of defects. The 3-mm diameter defects received a neutralizing monoclonal antibody against FGF-2 (50 ng/h) or pre-immune mouse IgG (50 ng/h) for the initial 2 weeks. Five animals each were sacrificed after 2, 3, or 4 weeks after creation of defects. The distal portion of each femur was removed, fixed, decalcified, and embedded in paraffin for the subsequent histological analysis. Sections were cut in the transverse plane, and histologically examined. RESULTS: The administration of FGF-2 (50 pg/h) resulted in successful regeneration of articular cartilage and the subchondral bone within 8 weeks after creation of 5-mm diameter defects. In these defects, undifferentiated mesenchymal cells initiated chondrogenic differentiation coupled with replacement by subchondral bone, resulting in the resurfacing of the defects by hyaline cartilage and the recovery of subchondral bone up to the original bone-articular cartilage junction. In contrast, the administration of a neutralizing monoclonal antibody against FGF-2 clearly interfered with the action of endogenous FGF-2 in 3-mm diameter defects, which were filled with fibrous tissue. None of the antibody-treated defects were covered with cartilage. We then assessed the proliferative capacity of the undifferentiated mesenchymal cells in the defects by immunostaining the proliferating cell nuclear antigen (PCNA) at 1 week after creation of defects. The capacity of reparative tissue to form cartilage was well correlated with the occurrence in the defects of a cell population that was PCNA-positive, undifferentiated, and capable of self-renewal. CONCLUSIONS: The local administration of FGF-2 resulted in the successful resurfacing of large (5 mm in diameter) defects by hyaline cartilage. Prechondrogenic mesenchymal cells were the likely targets of FGF-2, which probably promoted the formation of cartilage by stimulating a selective expansion of chondroprogenitor cells. Thus, activation of FGF-2 signalling is critically important for the induction of cartilaginous repair response in full-thickness articular cartilage.

Animals↗
PubMed2001

Role of cartilage-derived anti-angiogenic factor, chondromodulin-I, during endochondral bone formation.

OBJECTIVE: Cartilage is a typical avasclar tissue that exhibits powerful resistance to angiogenesis or vascular invasion. We previously identified a cartilage-specific 25 kDa glycosylated protein, chondromodulin-I (ChM-I), as anti-angiogenic factor. Taking advantage of ectopic bone formation and xenograft tumour model by human chondrosarcoma cell line OUMS-27, we examined how ChM-I is involved in switching of angiogenesis in cartilage. DESIGN: Gene expression pattern of ChM-I was examined in 4-week-old mice and mouse embryos by northern blot analysis and in situ hybridization. To evaluate the effect of ChM-I on ectopic bone formation, guanidine extracts of demineralized bone matrix were mixed with the ChM-I-bound heparin-Sepharose beads and were implanted onto the fasciae of back muscle of 6-week old nude mice. To analyse the effect of ChM-I on tumour angiogenesis, the level of ChM-I mRNA in cartilaginous tumours was assessed by competitive PCR, and compared with that of articular cartilage. Then, human chondrosarcoma OUMS-27 cells were inoculated into the back of nude mice to form a tumour about 45 mm3 in size. Recombinant ChM-I protein was administrated into OUMS-27 xenograft tumours for the initial 5 days to study its effect against tumour-angiogenesis. RESULTS: ChM-I gene was specifically expressed in cartilage of 4-week-old mice. Eye and thymus were also identified as minor expression sites. However, during endochondral bone development, cartilage changes its character from anti-angiogenic into angiogenic prior to the replacement of calcified cartilage by bone. In embryos, ChM-I mRNA was expressed in proliferative and upper hypertrophic cartilage zones in the developing cartilaginous bone rudiments, but completely abolished in lower hypertrophic and calcified cartilage zones. Purified ChM-I protein apparently inhibited vascular invasion into cartilage induced by the implantation of demineralized bone matrix in nude mice, leading to the inhibition of replacement of cartilage. The level of ChM-I transcripts in the lower-grade chondrosarcomas was substantially reduced to several hundreds or less in the lower-grade chondrosarcomas, compared with that of articular cartilage or other benign cartilage tumours. The local administration of recombinant human ChM-I almost completely blocked tumour angiogenesis and growth in the human chondrosarcoma xenografts in mice. CONCLUSIONS: ChM-I is involved in the anti-angiogenic property of cartilage and its absence creates a permissive microenvironment for vascular invasion into cartilage under physiological and pathological conditions.

Animals↗
PubMed2001

Clinical spectrum of epileptic spasms associated with cortical malformation.

The spectrum of clinico-electrical characteristics of epileptic spasms associated with cortical malformation was studied in detail. The subjects were 15 patients suffering from spasms and cortical malformation demonstrated by MRI. The types of cortical malformation causing spasms were various, including hemimegalencephaly, diffuse pachygyria, focal cortical dysplasia, and polymicrogyria. Ohtahara syndrome was diagnosed in 3 patients, and West syndrome in 8. Symptomatic localization-related epilepsy preceded West syndrome in 4 patients, and a transition from Ohtahara syndrome to West syndrome was observed in one. West syndrome was followed by symptomatic generalized epilepsy including Lennox-Gastaut syndrome in 4 patients. Nine patients showed a condition which was labeled "epilepsy with partial seizures and spasms" (EPS) and characterized by the coexistence of partial seizures and spasms, and multifocal epileptic discharges on EEG. Spasms occurred only as EPS in 5 patients. EPS appeared following Ohtahara syndrome or West syndrome in 4 patients, and showed a transition to symptomatic localization-related epilepsy in 4. However, EPS did not evolve into generalized epilepsy, and persisted until the time of last follow-up in 5 patients. Therefore, the clinico-electrical pictures of patients with spasms and cortical malformation were diverse and not always limited within those of typical generalized epilepsy.

Cerebral Cortex↗