Pitavastatin attenuates the upregulation of tissue factor in restraint-stressed mice.
Explore the source record for details and available documents.
Animals↗
Biomedical subjects
K Takeshita
Publications and source records attributed to K Takeshita.
Fatal thrombosis of antithrombin-deficient mice is rescued differently in the heart and liver by intercrossing with low tissue factor mice.
BACKGROUND: We previously reported that the targeted disruption of murine antithrombin (AT) gene resulted in embryonic lethality before 16.5 gestational days (gd) because of severe cardiac and hepatic thrombosis. OBJECTIVE AND METHODS: To investigate the influences of lowered tissue factor (TF) activity upon hypercoagulation of AT-/- embryos, we crossed AT+/- with low TF (mTF-/- hTF+) mice to yield homozygous AT-deficient mice with the extremely low TF activity, that is expressed from the inserted human TF mini gene. RESULTS: AT-/- embryos either with 50% TF (AT-/- mTF+/- hTF+) or with low (approximately 1% TF, AT-/- mTF-/- hTF+) were not born, although the survival was prolonged until 18.5 gd. In both genotypes, histological examination showed disseminated thrombosis in hepatic sinusoidal space or in the portal veins, suggesting that the thrombogenesis caused loss of hepatic blood flow. As in original AT-/-, AT-/- mTF+/- hTF+ showed subcutaneous (s.c.) bleeding and also suffered from the myocardial degeneration apparently because of coronary thrombus formation. However, AT-/- mTF-/- hTF+ had no skin hemorrhage and the thrombosis and degeneration were completely abolished in the heart. Myocardium of adult low TF mice had exhibited fibrosis secondary to hemorrhage; however, it was significantly decreased in low TF mice with AT+/-. CONCLUSIONS: Our current model suggests that, in the heart, TF plays an important role in the thrombogenesis and it counterbalances AT-dependent anticoagulation. AT may be a potent anticoagulant during mice development and the activation and subsequent regulation of TF-procoagulant activity take place differently between the liver and the heart. These differences appear to point to local regulatory mechanisms in murine hemostasis.
Animals↗
New and reliable MRI diagnosis for progressive supranuclear palsy.
OBJECTIVE: To evaluate the area of the midbrain and pons on mid-sagittal MRI in patients with progressive supranuclear palsy (PSP), Parkinson disease (PD), and multiple-system atrophy of the Parkinson type (MSA-P), compare these appearances and values with those of normal control subjects, and establish diagnostic MRI criteria for the diagnosis of PSP. METHODS: The authors prospectively studied MRI of 21 patients with PSP, 23 patients with PD, 25 patients with MSA-P, and 31 age-matched normal control subjects. The areas of the midbrain tegmentum and the pons were measured on mid-sagittal MRI using the display tools of a workstation. The ratio of the area of the midbrain to the area of the pons was also evaluated in all subjects. RESULTS: The average midbrain area of the patients with PSP (56.0 mm2) was significantly smaller than that of the patients with PD (103.0 mm2) and MSA-P (97.2 mm2) and that of the age-matched control group (117.7 mm2). The values of the area of the midbrain showed no overlap between patients with PSP and patients with PD or normal control subjects. However, patients with MSA-P showed some overlap of the values of individual areas with values from patients with PSP. The ratio of the area of the midbrain to the area of pons in the patients with PSP (0.124) was significantly smaller than that in those with PD (0.208) and MSA-P (0.266) and in normal control subjects (0.237). Use of the ratio allowed differentiation between the PSP group and the MSA-P group. CONCLUSION: The area of the midbrain on mid-sagittal MRI can differentiate PSP from PD, MSA-P, and normal aging.
Aged↗
Unusual imaging appearances of pancreatic serous cystadenoma: correlation with surgery and pathologic analysis.
BACKGROUND: We describe imaging and pathologic features of serous cystadenoma of the pancreas on multislice helical computed tomography CT (MS-CT) and surgical resection. METHODS: Radiologic and pathologic features were analyzed in five patients. All patients underwent MS-CT and digital subtraction angiography (DSA), and four patients underwent magnetic resonance (MR) imaging. Preoperatively, three cases showed radiologic evidence of mainly solid appearance on MS-CT, and the suspected diagnoses were solid pancreatic tumors (patients 1-3). The other two cases showed radiologic evidence of macrocystic tumor of the pancreas, and the suspected diagnoses were mucinous cystic tumors (cases 4 and 5). All patients underwent surgery, and the diagnosis of serous cystadenoma was confirmed on pathologic examination. RESULTS: In three cases that showed a solid appearance on MS-CT, a microcystic appearance was identified on microscopic examination, and the tumors were found to be hypervascular lesions on multiphasic contrast-enhanced CT and DSA. In cases 1 and 2, the lesions showed high intensity with internal septation on T2-weighted MR images. In two cases, the tumors were classified as a macrocystic variant of serous cystadenoma, and no mural nodules, papillary projections, or calcifications were seen in the tumors. CONCLUSION: Imaging appearance of serous cystadenoma on MS-CT is various and sometimes indistinguishable from that of solid tumor or mucinous cystic tumors of the pancreas. Imaging findings of hypervascularity and a well-marginated high-intensity lesion with internal septation on T2-weighted MR imaging may be crucial to identify serous cystadenoma that contains no visible cystic compartments on MS-CT.
Adult↗
Characterization of novel squamous cell carcinoma antigen-related molecules in mice.
The squamous cell carcinoma antigen 1 (SCCA1) and SCCA2 are unique serpins that can inhibit cysteine proteinases. SQN-5, their mouse ortholog, has already been identified, and its inhibitory property has been characterized; however, its biological role has remained undefined. Furthermore, no other mouse homolog of SQN-5 has been known. We characterize three mouse members of SCCA-related molecules including SQN-5 in this article. Serpinb3a (SQN-5) and Serpinb3b, but not Serpinb3c, were functional, inhibiting both serine and cysteine proteinases with different inhibitory profiles due to the difference of two amino acids in their reactive site loops. Serpinb3a was ubiquitously expressed in most tissues, whereas expression of Serpinb3b was limited to keratinocytes. Keratinocytes secreted both SCCA-related proteins, Serpinb3a and Serpinb3b. These results indicate that Serpinb3a and Serpinb3b may play different roles by inhibiting intrinsic or extrinsic proteinases with different expression distributions and different inhibitory profiles.
Amino Acid Sequence↗
A small chimerically bifunctional monomeric protein: Tapes japonica lysozyme.
The lysozyme of the marine bilave Tapes japonica (13.8 kDa) is a novel protein. The protein has 46% homology with the destabilase from medicinal leech that has isopeptidase activity. Based on these data, we confirmed hydrolysis activity of T. japonica lysozyme against three substrates: L-gamma-Glu-pNA, D-gamma-Glu-pNA, and epsilon-(gamma-Glu)-L-Lys. The optimal pH of chitinase and isopeptidase activity was 5.0 and 7.0, respectively. The isopeptidase activity was inhibited with serine protease inhibitor, but the lytic and chitinase activities were not. Moreover, only isopeptidase activity is decreased by lyophilization, but lytic and chitinase activities were not. We conclude that T. japonica lysozyme expresses isopeptidase and chitinase activity at different active sites.
Amino Acid Sequence↗
Maturational change of parallel auditory processing in school-aged children revealed by simultaneous recording of magnetic and electric cortical responses.
OBJECTIVES: To elucidate the maturational change of cortical auditory processing, we analyzed simultaneously recorded auditory evoked potentials (AEPs) and magnetic fields (AEFs) in school-aged children. METHODS: Simultaneous recording of AEP and AEF were performed in 32 healthy children of age ranging from 6 to 14 years and 10 adults. Tone bursts of 1 kHz were presented to the left and right ears alternately with 3 different within-ear stimulus onset asynchronies (SOAs) (1.6, 3.0 and 5.0 s for each ear) under attention-distracted condition. RESULTS: All subjects showed clear N100 and N100m peaks under the longest SOA condition (5.0 s). Under the shortest SOA condition (1.6 s), 4 out of 19 subjects under 12 years (21%) failed to show the N100m component. By contrast, N250 and N250m were observed in the majority of children (29/32: 91%) while those were detected in only 4 out of 10 adults (40%). The spatial distribution of N100 in children under 9 years differed from that in older subjects, whereas the dipole orientation of N100m was constant among age groups, suggesting that radially oriented sources might make additional contribution to the generation of N100 in early childhood. N250 was significantly larger in children than in adults. The strength of N250 was suppressed with longer SOAs, whereas that of N100 was enhanced. The dipole of N250m was located around Heschl's gyrus on the superior temporal plane which was significantly medial, anterior and inferior to that of N100m. CONCLUSIONS: Dissociation of maturational change between the tangential and radial components of N100 suggests that auditory processing at around 100 ms consists of multiple parallel pathways which mature independently. Furthermore, a negative peak at around 250 ms specifically seen in children has different generators from N100 and might represent a special auditory processing which takes an active part until acquisition of the efficient cortical networks of the adult brain.
Acoustic Stimulation↗
NOS and COX isoforms and abnormal microvessel responses to CO2 and H+ in hyperoxia-injured lungs.
The aim of the present study was to compare microvessel responses to hypercapnic and isocapnic acidosis in hyperoxia-injured lungs and to assess the role of constitutive and inducible forms of nitric oxide synthase (NOS) and cyclo-oxygenase (COX). Real-time confocal luminescence microscopy was used to measure changes in the diameter of acinar arterioles, venules and capillaries in response to stimulation with hypercapnic and isocapnic acidosis in isolated rat lungs injured by 90% oxygen exposure for 48 h. Observations were made with and without inhibition of constitutive (endothelial constitutive NOS (ecNOS) and COX-1) and inducible isoforms (iNOS and COX-2) of NOS and COX. Upregulation of NOS was assessed by measuring enzyme levels in lung homogenates by Western blot analysis and enhancement of the COX-related pathway was judged from perfusate concentrations of 6-ketoprostaglandin F1alpha. ecNOS and COX-1, but not iNOS and COX-2, were upregulated in hyperoxia-injured lungs. The nitric oxide produced by ecNOS attenuated COX-1 activity in injured arterioles and venules, but carbon dioxide enhanced it, leading to paradoxical dilatation of these microvessels under hypercapnic conditions with ecNOS inhibition. Although a high hydrogen ion concentration was unnecessary for excitation of COX-1, venule constriction in response to H+ was enhanced by COX-1 inhibition. Constitutive, but not inducible, isoforms of cyclo-oxygenase and nitric oxide synthase play an important role in abnormal microvessel responses to carbon dioxide and hydrogen ions in hyperoxia-injured lungs.
Acidosis, Respiratory↗
KE-758, an active metabolite of the new anti-rheumatic drug KE-298, suppresses production of tumor necrosis factor-alpha and interleukin-1 beta in THP-1, a human monocyte cell line.
KE-758, an active metabolite of KE-298, is a novel sulfhydryl antirheumatic drug. We analyzed the effect of KE-758 on tumor necrosis factor (TNF)-alpha and interleukin (IL)-1 beta production by a human monocytes cell line (THP-1 cells), stimulated with interferon-gamma (IFN-gamma) plus lipopolysaccharide (LPS). We compared the effects with other thiol-modifying antirheumatic drugs such as D-penicillamine, bucillamine and auranofin. THP-1 cells were treated with IFN-gamma for 16 h and were then exposed to LPS for an additional 6 h (for TNF-alpha detection) or 24 h (for IL-1 beta detection). The amounts of TNF-alpha and IL-1 beta in culture supernatants were measured using enzyme-linked immunosorbent assay. KE-758 and auranofin but not D-penicillamine and bucillamine significantly suppressed both TNF-alpha and IL-1 beta. Auranofin suppressed IL-1 beta production by reducing cellular viability. Reverse transcriptase-polymerase chain reaction analysis revealed that the suppressive effect of KE-758 is based on the inhibition of messenger ribonucleic acid expression of TNF-alpha and IL-1 beta. KE-758 had no effect on p75 and p55 soluble TNF receptor production in IFN-gamma and LPS-stimulated THP-1 cells. Thus, KE-758 inhibits both TNF-alpha and IL-1 beta production and its antirheumatic profile is apparently distinct from that of D-penicillamine, bucillamine and auranofin.
Antirheumatic Agents↗
Bile salt tauroursodeoxycholic acid modulation of Bax translocation to mitochondria protects the liver from warm ischemia-reperfusion injury in the rat.
BACKGROUND: Tauroursodeoxycholic acid (TUDC) is a hydrophilic bile acid that has a cytoprotective effect in primary biliary cirrhosis and primary sclerosing cholangitis. TUDC also protects hepatocytes from hydrophobic bile acid-induced apoptosis. The aim of this study was to determine whether TUDC ameliorates hepatocyte apoptosis during ischemia-reperfusion injury. METHODS: We used a rat model of hepatic warm ischemia-reperfusion injury to assess the effects of TUDC. Male Sprague-Dawley rats were subjected to 1 or 2 hr of normothermic ischemia followed by 3 or 6 hr of reperfusion. The treatment group received TUDC (50 mg/kg) by bolus intravenous injection 30 min before initiation of ischemia, whereas the control group received saline only. Blood samples for biochemical analysis were obtained after 6 hr of reperfusion. Liver biopsies for histological assessment were obtained 3 and 6 hr after reperfusion. Hepatocyte apoptosis was determined by terminal dUTP nick-end labeling. The pro-apoptotic protein Bax was quantified at the mRNA and protein level. RESULTS: Treatment with TUDC significantly reduced serum transaminase levels. This was associated with a significant amelioration in the levels of hepatocyte apoptosis in the TUDC-treated group compared with control. Furthermore, Western blot analysis of Bax expression in liver tissue indicated that TUDC inhibited the translocation of Bax from the cytosol to the mitochondria. CONCLUSIONS: TUDC significantly reduced hepatic injury in this model. The beneficial effects of TUDC upon hepatocyte apoptosis were related to the modulation of Bax protein translocation.
Animals↗
Effect of highly active antiretroviral therapy on survival in patients with AIDS-associated pulmonary Kaposi's sarcoma treated with chemotherapy.
PURPOSE: Kaposi's sarcoma (KS) is the most common AIDS-related malignancy. Pulmonary involvement by KS (PKS) has carried a poor prognosis with median reported survival ranging from 3 to 10 months. We studied whether the introduction of highly active antiretroviral therapy (HAART; triple antiretroviral therapy including a protease inhibitor and two reverse transcriptase inhibitors) has been associated with improved survival for AIDS patients with PKS. PATIENTS AND METHODS: A retrospective study was performed of 37 consecutive patients with PKS and human immunodeficiency virus infection in the tumor registry at a large municipal hospital in New York City between 1994 to 1997. There were 16 patients from 1994 to 1995 (pre-HAART period) and 21 patients from 1996 to 1997 (post-HAART period). The primary end point was survival, which was defined as time from start of chemotherapy until death from any cause. RESULTS: Patients were analyzed by the date of diagnosis (pre- v post-HAART period) and whether or not they received HAART. Kaplan-Meier analysis showed significantly better survival in patients diagnosed in the post-HAART period (P =.0025). Additional Kaplan-Meier analysis indicated that patients on HAART had substantially better survival (P <.0001). Cox multivariate analyses showed that HAART therapy was associated with a reduced risk of death (hazard ratio = 0.09; 95% confidence interval, 0.03 to 0.69). CONCLUSION: In patients with AIDS-associated PKS and undergoing chemotherapy, administration of HAART was associated with increased survival.
Acquired Immunodeficiency Syndrome↗
Noninvasive detection of hydroxyl radical generation in lung by diesel exhaust particles.
Diesel exhaust particles (DEP) induce pulmonary tumors, asthma-like symptoms, and the like in experimental animals. The involvement of reactive oxygen species (ROS) is suggested in the injuries induced by DEP, though the generation of ROS has not been proven. The present study provided the first direct evidence of *OH generation in the lungs of living mice after intratracheal instillation of DEP, using noninvasive L-band ESR spectroscopy and a membrane-impermeable nitroxyl probe. *OH generation is confirmed with the enhancement of in vivo ESR signal decay rate of the probe. The decay rate at mid-thorax was significantly enhanced in DEP-treated mice compared to that in vehicle-treated mice. The enhancement was completely suppressed by the administration of either *OH scavengers, catalase, or desferrioxamine, while the administration of SOD further increased the rate. The administration of Fenton's reagents into the lung also enhanced the decay rate of the probe at mid-thorax of mice. These results clearly provided evidence that the intratracheal exposure to DEP in mice produced *OH in the lung through an iron-catalyzed reaction of superoxide/H(2)O(2). This first direct evidence of *OH generation in DEP-treated mice lung may be utilized to determine treatments for DEP-induced lung injury.
Animals↗
Long-term results of double-door laminoplasty for cervical stenotic myelopathy.
STUDY DESIGN: A retrospective study of the long-term results from double-door laminoplasty (Kurokawa's method) for patients with myelopathy caused by ossification of the posterior longitudinal ligament and cervical spondylosis was performed. OBJECTIVE: To know whether the short-term results from double-door laminoplasty were maintained over a 10-year period and, if not, the cause of late deterioration. SUMMARY OF BACKGROUND DATA: There are few long-term follow-up studies on the outcome of laminoplasty for cervical stenotic myelopathy. METHODS: In this study, 35 patients with cervical myelopathy caused by ossification of the posterior longitudinal ligament in the cervical spine and 25 patients with cervical spondylotic myelopathy, including 5 patients with athetoid cerebral palsy, underwent double-door laminoplasty from 1980 through 1988 and were followed over the next 10 years. The average follow-up period was 153 months (range, 120-200 months) in patients with ossification of the posterior longitudinal ligament and 156 months (range, 121-218 months) in patients with cervical spondylotic myelopathy. Neurologic deficits before and after surgery were assessed using a scoring system proposed by the Japanese Orthopedic Association (JOA score). Patients who showed late deterioration received further examination including computed tomography scan and magnetic resonance imaging of the cervical spine. RESULTS: In 32 of the patients with ossification of the posterior longitudinal ligament and 23 of the patients with cervical spondylotic myelopathy, myelopathy improved after surgery. The improvement of Japanese Orthopedic Association scores was maintained up to the final follow-up assessment in 26 of the patients with ossification of the posterior longitudinal ligament and 21 of the patients with cervical spondylotic myelopathy. Late neurologic deterioration occurred in 10 of the patients with ossification of the posterior longitudinal ligament an average of 8 years after surgery, and in 4 of the patients with cervical spondylotic myelopathy, including the 3 patients with athetoid cerebral palsy, an average of 11 years after surgery. The main causes of deterioration in patients with ossification of the posterior longitudinal ligament were a minor trauma in patients with residual cervical cord compression caused by ossification of the posterior longitudinal ligament and thoracic myelopathy resulting from ossification of the yellow ligament in the thoracic spine. CONCLUSIONS: The short-term results of laminoplasty for cervical stenotic myelopathy were maintained over 10years in 78% of the patients with ossification of the posterior longitudinal ligament, and in most of the patients with cervical spondylotic myelopathy, except those with athetoid cerebral palsy. Double-door laminoplasty is a reliable procedure for individuals with cervical stenotic myelopathy.
Adult↗
Adequate endoscopic mucosal resection for early gastric cancer obtained from the dissecting microscopic features of the resected specimens.
BACKGROUND: We have employed endoscopic mucosal resection (EMR), using a cap-fitted panendoscope (EMRC), for early gastric cancer since 1992. The presence of an adequate surgical margin is a requirement because of the radicality of EMR, and dissecting microscopic examination is useful in regard to the diagnosis of spread of the disease. METHODS: To devise an adequate method of EMR that allows no lateral residue, we examined gastric mucosal specimens obtained by EMRC. One hundred and sixty-seven specimens from 97 lesions in 85 patients treated by EMRC were examined in regard to characteristic features, the recovery of marks made around the lesion, and the frequency of residue, and comparisons were made between the dissecting microscopic and histopathological findings. RESULTS: The first specimen obtained with a large cap under full suction was a circular shape measuring 21 x 19mm. The second specimen from fractionated resection was a half-moon or crescent shape, and the third specimen had a ginkgo leaflike or irregular shape. In the elevated lesions, coincidence regarding the spread, as determined by dissecting microscopy and histopathology, was present in 62 (93%) of the 67 lesions. In 16 (53%) of 30 flat or depressed lesions, there was a difference of 2 to 5 mm between the spread determined by these two examinations. CONCLUSION: It is important to place an adequate number of marks around the lesion and recover all marks by resection. When an elevated lesion measures 15mm or more, and a flat or depressed lesion is not clearly demarcated, aggressive use of planned fractionated resection seems to be the best way to prevent a lateral residue in EMR.
Aged↗
Relationship between cervical curvature index (Ishihara) and cervical spine angle (C2--7).
The purpose of this study was to determine the relationship between the cervical curvature index (Ishihara) and the cervical spine angle (C2--7) from both cervical lateral radiographs and a geometrical model. The cervical lateral radiographs of 295 outpatients with no cervical symptoms were reviewed to measure the cervical curvature index (Ishihara) and the cervical spine angle (C2--7). The average cervical curvature index (Ishihara) was 10.9 (SD, 15.3) and the average cervical spine angle (C2--7) was 20.3 (SD, 14.3). A highly significant correlation (0.95; 95% confidence interval [CI], 0.94--0.96) was found between the cervical curvature index (Ishihara) and the cervical spine angle (C2--7). Their linear correlation was also validated in the geometrical model. Their correlation diminished, however, in subjects with an S-shaped cervical spine. The cervical curvature index (Ishihara) and the cervical spine angle (C2--7) were interchangeable, but this interchangeability was lost in the S-shaped cervical spines.
Adolescent↗
Diagnosis of Japanese patients with HHH syndrome by molecular genetic analysis: a common mutation, R179X.
Patients with mitochondrial ornithine transporter deficiency (or HHH syndrome) present with various neurological symptoms, including mental retardation, spastic paraparesis with pyramidal signs, cerebellar ataxia, and episodic disturbance of consciousness or coma due to hyperammonemia. We previously described three novel mutations in the ORNT1 gene in Japanese patients with HHH syndrome. In this article, we report a new patient with HHH syndrome, a 52-year-old woman, who had the typical clinical features, except for an absence of mental retardation. When we screened this patient, as well as a previously described Japanese patient, for mutations in the ORNT1 gene, we found that both were homozygous for a nonsense mutation (R179X). Furthermore, reverse transcription (RT)-polymerase chain reaction (PCR) of fibroblast RNA from one patient showed exon 4 skipping, as had been observed in a previously reported patient with R179X. These results, together with the findings in our previous report, show that, in three of our five reported Japanese HHH patients (six of ten alleles), R179X is present, suggesting that this is a common mutation in Japanese patients with HHH syndrome.
Amino Acid Metabolism, Inborn Errors↗
Adsorption mechanism of hexavalent chromium by redox within condensed-tannin gel.
We have proposed a new recovery system of hexavalent chromium Cr(VI) that is of great toxicity utilizing condensed-tannin gels derived from a natural polymer with many polyhydroxyphenyl groups. The adsorption mechanism of Cr(VI) to the tannin molecules was clarified. The adsorption mechanism consists of four reaction steps; the esterification of chromate with tannin molecules, the reduction of Cr(VI) to trivalent chromium Cr(III), the formation of carboxyl group by the oxidation of tannin molecules and the ion exchange of the reduced Cr(III) with the carboxyl and hydroxyl groups. It was found in this recovery system that a large amount of proton was consumed accompanied with the reduction of Cr(VI) so that the acidic solution containing Cr(VI) was transferred automatically to neutral one by choosing an appropriate initial pH. The carboxyl group which was created by the oxidation of tannin molecules parallel to the reduction of Cr(VI) to Cr(III) contributed to an increase in the ion-exchange sites of the reduced Cr(III). The maximum adsorption capacity of Cr(VI) reached 287 mg Cr/g dry tannin gel under the conditions of 0.77 water content of tannin gel and the initial pH = 2. This adsorption capacity was five to ten times higher than that obtained by the ion exchange between ordinary Cr(III) and tannin molecules for the tannin gels prepared under similar conditions. The system proposed here will provide an important information on a zero-emission-oriented process because it has such advantages as higher adsorption capacity of chromium and lower volume of secondary wastes compared with conventional process.
Adsorption↗
A Japanese girl with leukoencephalopathy with vanishing white matter.
A Japanese girl with peculiar leukoencephalopathy was reported. Following normal development until 1 year of age, she showed progressive neurological deterioration with ataxia, epilepsy, pyramidal tract signs and choreic movement. Serial brain computed tomographies (CTs) revealed markedly low density and progressive volume loss in whole white matter. In extensive laboratory investigations, the level of glycine in the urine was elevated. She died at the age of 4 years, and the neuropathological findings were comprised of severe extensive changes in cerebral and cerebellar white matter, such as marked rarefaction or cystic degeneration with axonal loss. The pontine central tegmental tracts were also affected. Neuronal loss was seen in the cerebellar cortex. These features were compatible with leukoencephalopathy with vanishing white matter, which was recently established as a clinical entity. To our knowledge, this is the first report of a non-Caucasian patient with this new type of leukoencephalopathy.
Brain↗