Kenji Mitsumori

Acetazolamide Test in Detecting Reduced Cerebral Perfusion Reserve and Predicting Long-Term Prognosis in Patients with Internal Carotid Artery Occlusion

In a series of 32 patients with internal carotid artery occlusion, regional cerebral blood flow (rCBF) and regional cerebral vasoreactivity (rCVR) were measured by xenon-133 single photon emission computed tomography and the acetazolamide test. We evaluated its usefulness in detecting the reduced cerebral perfusion reserve and predicting long-term prognosis. All Type 1 patients (normal rCBF and rCVR) were medically treated and experienced no recurrent ischemic attack. Cerebral hemodynamics remained unchanged. Type 2, 3, and 4 patients underwent superficial temporal artery-middle cerebral artery double anastomosis, if they consented to surgery. All Type 2 (normal rCBF and reduced rCVR) and Type 3 (reduced rCBF and rCVR) patients, who underwent surgery, showed no further ischemic attacks, as well as long-term normalization of rCVR, although long-term rCBF normalization was obtained in only three of seven Type 3 patients. Cerebral hemodynamics remained unchanged in Type 4 patients after surgery. In follow-up periods, major completed stroke occurred in all 3 Type 2 and Type 3 patients who were medically treated. These results suggest that the acetazolamide test is valuable in assessing the cerebral perfusion reserve and predicting long-term prognosis in patients with internal carotid artery occlusion, although further long-term or randomized studies are needed.

Primary extranodal marginal zone B-cell lymphoma of the mucosa-associated lymphoid tissue type in the CNS.

Extranodal marginal zone B‐cell lymphoma of the mucosa‐associated lymphoid tissue (MALT) type has been reported in various internal organs. Here a case is reported of MALT lymphoma developing in the cerebellopontine (CP) angle in a patient with Sjögren syndrome, and the concept of MALT lymphoma of the CNS is introduced. Pathologically, the tumor showed inflammatory features of reactive lymphocytic infiltration with follicle formation, but there were slightly atypical lymphocytes and plasmacytes with B‐cell markers. These cells invaded reactive follicles, showing follicular colonization, and showed aberrant expression indicating their neoplastic nature. A review of the literature revealed eight cases of MALT lymphoma originating from the dura mater and one from the CP angle. The average age of patients was 50 years (range 28–66 years), and all patients were female. The tumors were slow to develop and the patients were cured after surgical removal and/or additional therapies. It is proposed that MALT lymphoma should be considered as a differential diagnosis of inflammatory pseudotumor of the CNS.

Cerebral hemodynamics and 're-build-up' phenomenon on electroencephalogram in children with moyamoya disease

Abstract“Re-build-up” phenomenon, induced by hyperventilation, is a characteristic finding on electroencephalogram (EEG) in children with moyamoya disease, and suggests close correlation with cerebral ischemic attack. Its mechanism, however, remains obscure. In this study, we examined the relationship between the cerebral hemodynamics and this phenomenon before and after vascular reconstructive surgery. Regional cerebral blood flow and its reactivity to acetazolamide were analyzed using the xenon-133 inhalation method and single photon emission computed tomography in six children with moyamoya disease. These results were compared with the EEG. Regional vasoreactivity to acetazolamide was significantly lower in “re-build-up”-positive regions than in “re-build-up”-negative regions. Postoperative studies revealed a significant improvement of reactivity in the regions where the “re-build-up” phenomenon disappeared after surgery. These results suggest that the “re-build-up” phenomenon represents a focal reduction of the cerebral perfusion reserve in moyamoya disease.

Dysplastic gangliocytoma (Lhermitte-Duclos disease) associated with Cowden disease: report of a case and review of the literature for the genetic relationship between the two diseases.

We report a case of dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease, LDD). The patient also had cutaneous and mucosal hamartomas, adenomatous goiter, bilateral breast tumors, and gastrointestinal polyposis, indicating the diagnosis of Cowden disease (CD), the familial hamartoma syndrome. This was a rare sporadic case without any family history of CD, though CD is considered to be an autosomal dominant hereditary disease. Based on a thorough review of the previously reported cases, it is reasonable to consider that CD is inherited in autosomal dominant fashion through a CD gene (PTEN) containing a germline mutation, and that the occurrence of LDD is predicated on an additional somatic hit on the remaining normal CD allele or another unknown gene.

Immunohistochemical analysis of giant cell glioblastoma.

Giant cell glioblastoma (GCG) is one of a group of rare tumors in which the cell population is abnormally large and includes multinucleated cells of gigantic sizes. Immunohistochemical studies were performed on four GCG cases and found that all giant cells and/or tumor cells were positive for glial fibrillary acidic protein (GFAP), S‐100 protein, and vimentin, thus verifying the tumors glial origin. The nuclei of multinucleated giant cells of three adult cases were frequently immunostained for proteins expressed during the cell cycle (proliferating cell nuclear antigen (PCNA) and Ki‐67), thereby demonstrating the proliferative capacity of these cells. By contrast, those of a 12 year old girl expressed these cell cycle markers rather infrequently. Alpha I‐anti‐trypsin was detected with relatively high frequency in the giant cells, and its presence may explain their bizarre sizes and pericellular reticulin fiber formation. A literature review of 32 cases revealed that the GCG that occurs preferentially in young girls is a type of pleomorphic xanthoastrocytoma. By contrast, GCG in adult males has the same age incidence as ordinary glioblastomas and, as these, expresses high levels of cell cycle‐related proteins. Thus, GCG, which is subclassified morphologically as ordinary glioblastoma, has distinct biological and clinical characteristics, with that in children requiring re‐evaluation because of its similarities to pleomorphic xanthoastrocytoma.

Regional cerebral blood flow in pediatric moyamoya disease: age-dependent decline in specific regions.

Abstract Thirteen pediatric patients (ages 4–13 years) who underwent surgical treatment were examined regarding their rCBF in the preoperative periods. The postoperative rCBF was measured 39 times in these 13 patients. Thirteen healthy normal subjects (ages from 6 to 21 years) were also examined. The rCBF in the operculum and in the frontal, parietal, and occipital lobes was measured with 133Xe inhalation method and single photon emission computed tomography. In the parietal and occipital lobes, the preoperative rCBF had a negative and significant correlation with their ages, but not in the operculum or frontal lobe. However, subsequent to the surgical treatment, the rCBF increased significantly in the patients 5 years old or less, and then post-operative rCBF values had significant negative correlations with age in each region.

Transient ipsilateral blood flow reduction in the thalamus and cerebral cortex after pontine infarction

Remote cerebral hypoperfusion associated with pontine lesions is rare. We describe a patient who showed transient ipsilateral hypoperfusion in the thalamus and cerebral cortex after a pontine infarct. This resolved within 8 days after onset. Anatomical considerations strongly suggested involvement of the cerebellothalamocortical pathway in this case.