[Progressive multifocal leukoencephalopathy with idiopathic CD4 positive T-lymphocytepenia mimicking a low grade glioma on proton MR spectroscopy. A case report].

A 61-year-old man with no history of HIV infection developed a subacutely progressive dementia and left hemiparesis. Brain MRI showed a high intensity lesion in the right frontal lobe on T2 weighted image. There was no contrast enhancement after gadolinium-DTPA administration. 1H MRS revealed a marked decrease in the n-acetyl aspartate/creatine ratios and an increase in the choline/creatine ratio. A lactate peak also was present. A low-grade glioma was suspected and he was admitted to our hospital. On examination, there was a mild dementia and left hemiparesis. A peripheral blood count revealed lymphocytopenia (426/mm3) with a CD4/CD8 ratio of 0.28. No evidence of HIV infection, malignancies or collagen disease was found. A brain biopsy revealed no tumor cells but instead demyelinated brain tissue with large nucleated cells. JC virus antigen was detected in the cells of the demyelinated lesions. A diagnosis of PML associated with idiopathic CD4 positive lymphocytopenia was made. There are only a few reports concerning 1H-MRS findings in patients with PML and the present case illustrates the difficulty of making a differential diagnosis between PML and glioma.

[Significance of shunt efficacy decision of SPECT on idiopathic normal pressure hydrocephalus].

PURPOSE: The purpose of this study was to investigate the significance of the shunt-effect evaluation of SPECT in idiopathic normal pressure hydrocephalus (iNPH). SUBJECTS AND METHODS: The subjects were 15 patients with possible iNPH, aged 62-83 (mean 75.3, the ratio of males to females to 6:9), who were treated at our department during the period from June to September, 2004. All patients received the lumbar tap test (LTT) at the outpatient section before surgery. An L-P shunt was conducted on patients whose the LTT positive or negative with cerebrospinal fluid outflow resistance value (Ro) was 10 mmHg/ml/min. or higher patients. As for SPECT, a 3D-SSP Z-score, as well as an mCBF, was conducted before and after the LTT and within one month after surgery. Comparisons were made for (1) the shunt effect, (2) mCBF before and after the LTT and after surgery, (3) mean cerebral blood flow increase rate (mIR) after the LTT, and (4) 3D-SSP before and after surgery. RESULTS: (1) The shunt was effective for all the patients. (2) The mCBF levels was 30.8 +/- 4.02 ml/100 g/min. before the LTT, 37.1 +/- 100 g/min. after the LTT, and 38.6 +/- 3.4 ml/100 g/min. after surgery. A significant increase in mCBF was observed both after the LTT and after surgery (p < 0.05). (3) The mean mIR after the LTT was 21.2 +/- 8.01%, with all the patients showing 10% or higher. (4) The ischemic patterns in the SD-SSP Z-score before surgery were the frontal type (F: 10 cases, 66.7%), the occipitotemporal type (OT: 3 cases, 20%), and the mixed type (M: 2 cases, 13.3%), but not the parietal localized type. The post-operative course showed no-change in 4 cases, disappearance-reduction in 9 cases, and shift to OT in 2 cases. CONCLUSION: The evaluation factors in the measurement of the cerebral blood flow for evaluation of the shunt effect were the following two items. (1) The mIR of mCBF after the LIT was 10% or higher. (2) As for the preoperative cerebral ischemic patterns, there were many F cases and no parietial localized types found.

Early diagnosis of vertebral dissecting aneurysm: a magnetic resonance angiography study.

We report a patient with dissecting aneurysm who presented with a sudden severe headache without any neurological symptoms. Although brain computed tomography (CT) scan and MRI were negative, magnetic resonance angiography (MRA) showed a pseudocavity in a segment of the left vertebral artery. In addition, the dissecting wall of the left vertebral artery was clearly visualized in the original images of MRA. Our findings indicate that brain CT, MRI or cerebral angiography alone are sometimes inadequate for the diagnosis of vertebral dissecting aneurysm, and that MRA and its original images are necessary to establish the correct diagnosis.

[Usefulness of carotid duplex ultrasonography in a patient with moyamoya disease].

The patient is a 35-year-old man who had a medical history of epilepsy in childhood. He came to our hospital because of transient disturbance of consciousness and left hemiplegia just after evacuation. At first, we thought that he had epilepsy with Todd's palsy. But we had to do a differential diagnosis for a transient ischemic attack such as paradoxical embolism, because his symptoms occurred just after evacuation. An electroencephalogram and brain computerized tomography were immediately performed, but no abnormality was detected. Hematologic studies were normal, and no deep vein thrombosis was detected in the veins of the lower extremities by duplex ultrasonography Doppler. But carotid duplex ultrasonography showed an increase in end-diastolic flow velocity and a decrease in vascular resistance in both external carotid arteries. These findings indicated that there was arteriovenous malformation such as moyamoya disease. Brain magnetic resonance imaging showed spotty high signal lesions in the subcortical areas on a fluid-attenuated inversion-recovery(FLAIR) image, and the middle cerebral artery was not visualized on magnetic resonance angiography (MRA). Cerebral angiography demonstrated moyamoya vessels in the brain and collateral circulation from the external carotid artery. Therefore, we diagnosed him as having moyamoya disease. Duplex ultrasonography of the common and, internal carotid, and vertebral arteries is a widely-used technique. Recently, cerebral angiography, MRA and transcranial Doppler have been applied to detect intracranial vascular malformation. But these results suggested that moyamoya disease could be detected by means of carotid duplex ultrasonography. Finally, we considered that carotid duplex ultrasonography was not only a noninvasive screening method but also a useful for the diagnosis of moyamoya disease.

Diabetic striatal disease: clinical presentation, neuroimaging, and pathology.

BACKGROUND: Unilateral movement disorders and contralateral neuroimaging abnormalities of the striatum have been sporadically reported as a rare syndrome associated with diabetes mellitus. Despite characteristic imaging findings and clinical manifestations, the mechanism underlying this syndrome is still unclear. METHODS: Six patients with this syndrome were studied clinically and subjected to MRI neuroimaging; one underwent biopsy of the striatum, and another underwent additional MR spectroscopy at 3.0T and FDG-PET. RESULTS: Neuroimaging findings were characterized by a T1-hyperintense unilateral lesion restricted to the striatum, contralateral to the symptomatic limbs. The biopsied striatum contained patchy necrotic tissue, severe thickening of all layers of arterioles, and marked narrowing of vessel lumens. Hyaline degeneration of the arteriolar walls, extravasation of erythrocytes, and prominent capillary proliferation were also notable, together with lymphocytic infiltration and macrophage invasion. In one patient, PET examination revealed decreased accumulation of FDG in the lesion. The MR spectrum for the diseased striatum revealed a decrease in the NAA/Cr ratio (1.35), normal Cho/Cr ratio (1.22), and a peak for myoinositol, while the spectrum on the contralateral site revealed a decrease in the NAA/Cr ratio (1.48), increase in Cho/Cr (1.32), but no peak for myoinositol. CONCLUSION: The constellation of signs and symptoms and neuroimaging characteristics in previous reports and the six additional cases described here with neuropathological data and findings of MR spectroscopy appears unique enough to be termed "diabetic striatopathy." This syndrome appears in poorly controlled diabetics due to obliterative vasculopathy with prominent vascular proliferation, vulnerability to which is restricted to the striatum.

Stereoselective synthesis of tetrahydronaphthocyclobuta[1,2-d]pyrimidinediones from 5-fluoro-1,3-dimethyluracil and naphthalenes.

Upon UV-irradiation in the presence of piperylene, 5-fluoro-1,3-dimethyluracil (5-FDMU) couples with naphthalenes having either an electron-withdrawing group or an electron-donating group by way of 1,2-cycloaddition via mode selectivity to give the corresponding naphthocyclobutapyrimidines regio- and stereo-selectively.