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Functional single-nucleotide polymorphisms (SNPs) in inositol 1,4,5-trisphosphate 3-kinase C (ITPKC) (rs28493229) and caspase-3 (CASP3) (rs113420705; formerly rs72689236) are associated with susceptibility to Kawasaki's disease (KD). To evaluate the involvement of these 2 SNPs in the risk for intravenous immunoglobulin (IVIG) unresponsiveness, we investigated 204 Japanese KD patients who received a single IVIG dose of 2 g kg(-1) (n=70) or 1 g kg(-1) daily for 2 days (n=134). The susceptibility allele of both SNPs showed a trend of overrepresentation in IVIG non-responders and, in combined analysis of these SNPs, patients with at least 1 susceptible allele at both loci had a higher risk for IVIG unresponsiveness (P=0.0014). In 335 prospectively collected KD patients who were treated with IVIG (2 g kg(-1)), this 2-locus model showed a more significant association with resistance to initial and additional IVIG (P=0.011) compared with individual SNPs. We observed a significant association when all KD patients with coronary artery lesions were analyzed with the 2-locus model (P=0.0031). Our findings strongly suggest the existence of genetic factors affecting patients' responses to treatment and the risk for cardiac complications, and provide clues toward understanding the pathophysiology of KD inflammation.

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SUMMARY: We report a case of bilateral common carotid artery dissection due to strangulation successfully treated by stent placement, with a review of the literature. A 61-year-old woman was strangled by an apron strap. She was admitted to our hospital with tetraparesis, because of spinal cord injury. On the next day, her left hemiparesis aggravated and left facial palsy newly appeared. Diffusion weighted magnetic resonance imaging (MRI) showed new ischemic lesions in the right cerebral hemisphere. Aortography revealed bilateral common carotid artery dissection. Moreover, thrombus or intimal flap was recognized in the right common carotid artery. The right common carotid dissection was fixed with deployment of self expanding stents to prevent the aggravation of ischemic stroke at that time. The contralateral lesion was also treated ten days later because small ischemic lesions were newly recognized in the left hemisphere on MRI. No new neurological deficit appeared after bilateral carotid artery stenting. Her paraparesis completely improved two months after the spinal cord injury. Carotid artery stenting using self expanding stents was especially effective as the treatment for bilateral carotid artery dissection.

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SUMMARY: We developed a rabbit saccular aneurysm model for coil embolization training. Elastaseinduced aneurysms were created successfully in about 80% of the rabbits. The aneurysms were usually broad in the neck and lengthy. At the 28(th) postoperative day, the aneurysms were about 1.5 times larger in both width and height than they had been at the 14(th) day. All aneurysms were successfully embolized with 18-sized electrically detachable (ED) platinum coils. After embolization, almost all aneurysms had a neck remnant. In conclusion, this model is useful not only for learning the technique of coil embolization but also for testing new embolic materials. The rabbit aneurysm model proved to be an efficacious training modality for endovascular coil embolization.

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SUMMARY: In this paper, we report five cases with acutely ruptured wide-necked aneurysms, which were treated with coil embolization using a balloon or stent-assisted technique. Balloon-assisted coil embolization using Equinox balloon, Commodore balloon, and Hyperform balloon were performed for four patients and stent-assisted coil embolization using BX velocity for one patient. We discuss problems of coil embolization for acutely ruptured wide-necked aneurysms with a balloon or stent-assisted technique.

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SUMMARY: We report a series of coil embolizations for small aneurysms solely using GDC ultrasoft coils and discuss the advantages of this method. Seven small aneurysms (< 4.0 mm) were embolized solely with ultrasoft coils. Ultrasoft coils were sequentially inserted into aneurysms. Immediately after embolization, five aneurysms were completely occluded, and two exhibited body filling. All cases were treated successfully without any complications. In conclusion, ultrasoft coils were found efficacious for the treatment of small, irregular-shaped, and ruptured aneurysms; their softness and malleability facilitated their compaction into an aneurysm.

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76 consecutive patients with 78 unruptured cerebral aneurysms underwent endovascular therapy from July 1999 to May 2004 in our institute. For the wide-necked aneurysms, the remodeling technique, double microcatheter technique, or stent-assisted coil embolization was used, while a parent artery occlusion or covered stent was applied for the giant or fusiform aneurysms. Immediate angiographical results demonstrated 33 complete occlusions, 26 neck remnants, and 14 dome fillings. Four cases were treated with parent occlusion or stenting only, and one case was not treated with embolization but with clipping due to the rupture of the aneurysm during coil embolization. Immediate angiographic findings demonstrated that in aneurysms between 5 to 10 mm, the rate of complete occlusion was 48%, that of neck remnants 33%, and that of dome fillings 27%. In aneurysms between 11 to 25 mm, the rate of complete occlusion was 14%, that of neck remnants 28%, and that of dome fillings was 58%. In the angiographic follow-up results, all aneurysms smaller than 5 mm showed complete occlusion. In aneurysms between 5 to 10 mm, 74% of the aneurysms showed complete occlusion, and 21% showed neck remnants, and 5% showed dome filling. In aneurysms between 10 to 24 mm, 25% showed complete occlusion, while 75% showed dome filling. The overall mortality rate was 0% and the morbidity rate was 3.7% (2 major strokes, 1 minor stroke) at 30-days after embolization. In the clinical follow-up study, one case of a large basilar tip aneurysm caused a fatal rupture 28 months after the initial embolization. Endovascular therapy was performed on the unruptured aneurysms and was found to be an acceptable treatment, except for durability in cases of large aneurysms.

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SUMMARY: Eighteen patients with intracranial vertebrobasilar stenosis and occlusion were treated by PTA or stenting. In 11 of 18 cases, only PTA was performed and in seven of 18 cases, we used stents. The mean stenosis before and after PTA/stenting was 82.8% and 22.3%, respectively. In 11 cases of PTA only, the stenotic rate decreased from 81.8% to 29.6%, while 85.0% of the stenotic rate remarkably reduced to 6.0% in seven cases of stenting. The 30 days morbidity and 30 days mortality rate were 5.5% and 5.5%, respectively. There was only one haemorrhagic complication (cerebellar haemorrhage) in cases of stenting, and no ischemic events during or after the procedures. Restenosis (more than 50% stenosis) occurred in four of 18 cases(22.2%) during mean followup period of 12 months. Two patients with VA occlusion before treatment, developed restenosis and reocclusion. Complete total occlusion seems to be a high-risk lesion and strict follow-up is required. In this study, PTA/stenting for intracranial vertebrobasilar artery stenosis or occlusion is an effective treatment, but strict indications may be required because procedure-related 30 days morbidity rate was 5.5% in addition to unclear natural history.

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SUMMARY: We perfomed carotid artery stenting(CAS) in 215 patients from August 1997 to October 2003 mainly using the distal protection technique. Our technique and clinical results are described in this paper.

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SUMMARY: The number of successful case reports with percutaneous transluminal angioplasty (PTA) / stenting for intracranial atherosclerotic stenoses is recently increasing with the advent of flexible coronary stents. However, it is not well known whether the perforating artery is occluded or not after stent placement in the atherosclerotic stenotic vessels. We investigated this issue using five New Zealand white rabbits. We deployed stainless steel stents in the atherosclerosis-induced abdominal aorta across the lumbar artery in which the diameters of the abdominal arteries were similar to those of human intracranial arteries. We evaluated the patency of lumbar artery by angiography and scanning electron microscopy three months after stent placement. The lumbar arteries were patent in four out of five rabbits. However, SEM findings demonstrated stent struts were covered with thick neointima and the ostia between stent struts were partially occluded. It is possible that stent placement in the atherosclerotic arteries can cause the obliteration of the perforating arteries.

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SUMMARY: In this paper, we reviewed our cases of dural arteriovenous fistulae (dural AVFs) and analyzed periprocedural complications. In 157 procedures, we encountered 14 complications. Overall, complication rate of 9% was seen.We divided these complications into five subgroups such as cranial nerve palsy, coil-related trouble, thromboembolic complication, vessel perforation, and radiation-related trouble. There were five transient abducent nerve palsies in cases with cavernous sinus dural AVFs. There were two cases of coil unraveling and two cases of coil migration. In two cases, direct puncture of the internal jugular vein was performed to retrieve the unraveled coil by using dual microcatheter and guidewire snare technique. We encountered two thromboembolic complications. In one case, venous infarction was recognized after polyvinyl alcohol particle embolization. In two cases of vessel perforations, there were no new neurological deficits except one case with transient Gerstmann syndrome. In endovascular treatment of dural AVF, serious complications are rare and can be prevented if maximum attention is paid during the procedure.

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SUMMARY: PTA/stenting for the intracranial arteriosclerotic lesion is effective and novel treatment. Our standard technique to avoid serious complications, such as vessel rupture or acute occlusion was introduced in this paper.

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Mitochondrial acetoacetyl-CoA thiolase (T2) deficiency is an inborn error of metabolism affecting isoleucine and ketone bodies in the catabolic process. Mutation analysis and expression analysis of mutant cDNAs have facilitated the division of T2-deficient patients into two groups: those with null mutations in either allele (group 1) and those with mutation(s) retaining some residual T2 activity in at least one of two mutant alleles (group II). Among 5 Japanese T2-deficient patients, GK01 belonged to group I and the other patients (GK19, GK19B, GK30 and GK31) to group II. As we have suggested previously, the severity of ketoacidotic episodes in the group II patients was similar to that in the group I patient. However, the urinary organic acid and blood spot acylcarnitine profiles under stable conditions differed between the two groups. The group I patient had typical profiles for the T2 deficiency. In contrast, in all four patients in group II, tiglylglycine was not or was only faintly detected and the 2-methyl-3-hydroxybutyrate levels were less than the cutoff value. Their tiglylcarnitine levels were within the normal range and 2-methyl-3-hydroxy-, butyrylcarnitine was detected just around the cutoff value in our newborn screening pilot test. Hence, these analyses under stable conditions are not reliable for diagnosing the T2 deficiency in the group II patients. The T2 deficiency (group II) can be misdiagnosed as normal if these analyses are performed under nonepisodic conditions and possibly during the newborn screening for inborn errors of metabolism.

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SUMMARY: We have experienced total 116 stenting for 102 of cranio-cephalic arteries and 14 of intracranial arteries including occlusive cerebrovascular diseases, aneurysms, and fistulas.Ten complications were encountered. Three were ischemic complication, four stent migration, two restenosis, and one aneurysmal perforation during coiling across the stent strut. The mechanism and preventive method of these complications were discussed in this paper.

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We present a case of transcortical mixed aphasia caused by a cerebral embolism. A 77-year-old right-handed man was admitted to our hospital with speech disturbance and a right hemianopia. His spontaneous speech was remarkably reduced, and object naming, word fluency, comprehension, reading and writing were all severely disturbed. However, repetition of phonemes and sentences and reading aloud were fully preserved. Although magnetic resonance imaging (MRI) showed cerebral infarcts in the left frontal and parieto-occipital lobe which included the inferior frontal gyrus and angular gyrus, single photon emission CT revealed a wider area of low perfusion over the entire left hemisphere except for part of the left perisylvian language areas. The amytal (Wada) test, which was performed via the left internal carotid artery, revealed that the left hemisphere was dominant for language. Hence, it appears that transcortical mixed aphasia may be caused by the isolation of perisylvian speech areas, even if there is a lesion in the inferior frontal gyrus, due to disconnection from surrounding areas.

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We sought to clarify whether apparently silent cerebral infarcts and periventricular hyperintensities are associated with depressed cognitive function in middle-aged subjects. Subjects were 84 middle-aged neurologically normal adults who wished to undergo a screening examination of the brain. We performed magnetic resonance imaging (MRI) of the brain and neuropsychologic tests in all subjects. Silent cerebral infarcts and periventricular hyperintensities, respectively, were detected in 21 and 14 of 84 subjects. Mini-mental state (MMS) and Raven's colored progressive matrices (RCPM) scores were significantly lower in subjects with than without silent cerebral infarcts. By two-factor analysis of variance, MMS score was affected by silent cerebral infarcts or periventricular hyperintensities, with interactions between the two lesion types (P < 0.05). Silent cerebral infarcts may be an independent factor in the pathogenesis of intellectual dysfunction, but truly independent analysis is difficult because many subjects with silent cerebral infarcts also have periventricular hyperintensities.

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A 45-year-old right-handed woman suffered transient aphasia and persistent amnesia after a right thalamic haemorrhage. This patient appeared to have crossed aphasia, although it disappeared within 8 weeks. It is noteworthy that the patient had a unilateral right thalamic lesion but exhibited both verbal and non-verbal memory impairment. Computed tomography and magnetic resonance imaging revealed cerebral haemorrhage in the right thalamus involving the ventral anterior nucleus, medioventral nucleus, mamillothalamic tract, internal medullary lamina, and mediodorsal nucleus. An amytal test was performed and suggested that the right hemisphere was dominant for language functions and the left hemisphere was dominant for visuospatial functions. Single photon emission CT revealed a low perfusion area only in the right thalamus. These findings suggest that the right hemisphere might be dominant for both verbal and non-verbal memory function in this patient, although visuospatial function was lateralized in the left hemisphere.

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We treated a patient in whom a left retrosplenial lesion resulted in memory impairment and spatial disorientation. A 31 year old, right handed man was admitted to our hospital after the sudden onset of headache. He was alert, attentive and cooperative, and showed no motor or sensory deficits. Although intelligence was preserved, memory was obviously deficient. The patient proceeded in wrong directions after he left his hospital room and subsequently his home. Neuroimaging revealed a subcortical hematoma in the left cingulate isthmus, while single-photon emission computed tomography demonstrated decreased perfusion in the splenium and left parietal lobe.

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The efficacy of repeated percutaneous transluminal angioplasty (PTA) and carotid endarterectomy (CEA) was examined in patients with restenosis after PTA for carotid stenosis. After percutaneous transluminal angioplasty (PTA) for 63 cases of internal carotid stenoses 13 cases of restenosis appeared. They were treated by PTA or carotid endarterectomy. The treatment was chosen by the patient after explanation of each treatment. We initially treated seven patients by repeat PTA and six by carotid endarterectomy. The degree of stenosis improved from 82% to 30% on average after repeated PTA. However, one patient in the PTA group had restenosis, and carotid endarterectomy was then performed. The other cases also had restenosis and were treated by PTA. The six cases treated by carotid endarterectomy were successfully treated without difficulty. The success rate of PTA was 5/7 (71%) in the restenosis cases. Patients with a greater residual stenosis after initial PTA had significantly more frequent restenosis. Repeat PTA and CEA both appeared effective treatment for restenosis after initial PTA, although PTA had a restenosis rate similar to that of initial PTA.

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