1/12. hemolytic-uremic syndrome with involvement of basal ganglia and cerebellum.hemolytic-uremic syndrome is a microangiopathy often associated with neurologic symptoms. Several patients with persistent lesions in cerebrum and basal ganglia have been reported. We present two children with bilateral basal ganglia and additional unilateral cerebellar lesions in magnetic resonance imaging. These resolved completely in one child. In the other child there were still residuals after 11 weeks. The neurologic symptoms of both improved after several therapeutic plasma exchanges and disappeared after months.- - - - - - - - - - ranking = 1keywords = cerebrum (Clic here for more details about this article) |
2/12. Auditory brainstem response and temporal bone pathology findings in a brain-dead infant.The criteria for assessing adult brain death have been already established, but those for infant brain death have not been yet established in japan. We report auditory brainstem response (ABR) and postmortem pathology of the temporal bone and brain of a brain-dead 9-month-old female. During the comatose state, her ABR showed only waves I and II bilaterally. autopsy revealed the presence of a left cerebellar astrocytoma, herniation and anoxic encephalopathy. The pathological examination of the temporal bone revealed the destruction of the inner ear particularly on the left side. In the auditory pathway of brain-dead patients, degeneration occurs first in the cerebrum, followed by the cochlear nerve. Thus, ABR is one of the useful means to assess brain death even in infants.- - - - - - - - - - ranking = 1keywords = cerebrum (Clic here for more details about this article) |
3/12. Crossed cerebral - cerebellar diaschisis: MRI evaluation.MRI, done later in life, in two patients with infantile hemiplegia syndrome showed significant volume loss in the cerebellar hemisphere contralateral to the side of the affected cerebrum. The cerebellar volume loss seemed to correlate with the degree of volume loss in the contralateral cerebral hemisphere. These observations provide morphological evidence of the phenomenon of crossed cerebral-cerebellar diaschisis (CCD). functional neuroimaging studies in support of the concept of CCD has been critically reviewed.- - - - - - - - - - ranking = 1keywords = cerebrum (Clic here for more details about this article) |
4/12. MR evaluation of crossed and uncrossed cerebral-cerebellar diaschisis.In three patients with infantile hemiplegia syndrome, MR imaging done later in life showed significant volume loss in the cerebellar hemisphere contralateral to the side of the affected cerebrum in two and ipsilateral in one. By comparison, the cerebellar volume loss seemed to correlate with the degree of volume loss in the contralateral cerebral hemispheres in two patients. These observations provide morphological evidence of the phenomenon of crossed and uncrossed/ipsilateral cerebral cerebellar diaschisis (CCD and ICD). functional neuroimaging studies in support of the concept of CCD and ICD have been critically reviewed in the light of the morphological changes demonstrated in the cases cited herein.- - - - - - - - - - ranking = 1keywords = cerebrum (Clic here for more details about this article) |
5/12. Molecular characterization of a jc virus (Sap-1) clone derived from a cerebellar form of progressive multifocal leukoencephalopathy.Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease caused by polyomavirus JC (JCV). In the majority of cases of PML the cerebrum is mainly affected (cerebral PML) but on rare occasions lesions are restricted to the cerebellum and brain stem (cerebellar PML). We report a rare cerebellar PML case which occurred in a Japanese patient undergoing prolonged hemodialysis treatment. To understand the molecular basis of the viral tissue tropism, we molecularly cloned JCV dna and compared it with those of cerebral PML. Of ten clones analyzed nine showed identical fragment patterns after digestion with various restriction endonucleases, and we designated these clones Sap-1. It could be shown that the basic structures of the regulatory regions are similar between Sap-1 and isolates from cerebral PML. Restriction endonuclease mapping analysis was used to examine the genetic relationship between Sap-1 and urine-derived isolates containing the archetypal regulatory sequence. We found that Sap-1 was genetically related to an archetypal JCV isolate in japan.- - - - - - - - - - ranking = 1keywords = cerebrum (Clic here for more details about this article) |
6/12. Cerebellar atrophy attributed to cerebellitis in two patients.In a review of magnetic resonance (MR) images from patients with spinocerebellar degeneration (SCD), we found 2 rare patients considered to be in late-stage cerebellitis who showed isolated cerebellar atrophy. The patients were negative for the spinocerebellar ataxia (SCA) genes and had no symptoms of hypothyroidism, history of malignant tumors, or history of alcohol and drug (phenytoin) abuse, which may cause cerebellar atrophy. MR images demonstrated generalized atrophy of the cerebellum, excluding the brainstem or cerebrum. In these cases, moreover, slightly high intensities were noted in the affected cerebellar cortices on fluid-attenuated inversion recovery (FLAIR) images. The distribution of widening of the folia and cortical high intensities on FLAIR images might be important clues with which to diagnose late-stage cerebellitis.- - - - - - - - - - ranking = 1keywords = cerebrum (Clic here for more details about this article) |
7/12. F-18 FDG PET demonstrates crossed cerebellar diaschisis 20 years after stroke.stroke produces an area of focal damage and distant areas of reduced blood blow and metabolism termed diaschisis. Tc-99m ECD and HMPAO brain SPECT have demonstrated crossed cerebellar diaschisis (CCD) in patients with cerebral cortical infarct. SPECT findings reflect abnormal cerebral blood flow. CCD as shown on F-18 FDG PET reflects abnormal reflects glucose metabolism. We present the case of a patient with laryngeal cancer who also had a stroke in the left cerebral hemisphere involving the territory of the middle cerebral artery 20 years ago. This patient underwent PET, including the head and neck. A current brain F-18 FDG PET exhibited hypometabolism in the contralateral cerebellum (CCD) as well as hypometabolism of the primary insult in the left cerebral hemisphere. These findings reflect partial impairment or diminished glucose metabolism in the primary insult to the cerebrum and contralateral cerebellum. In addition, this patient illustrates that on PET imaging, CCD could be demonstrated 20 years after a stroke.- - - - - - - - - - ranking = 1keywords = cerebrum (Clic here for more details about this article) |
8/12. Crossed cerebellar diaschisis in the sturge-weber syndrome.Crossed cerebellar diaschisis (CCD) is known as a cerebellar hemispheric hypometabolism due to contralateral supratentorial infarction or tumor. We report a case with the sturge-weber syndrome (SWS), whose cerebral blood flow was reduced in the anatomically affected lesion of cerebrum and in the contralateral cerebellar hemisphere. Not only acquired diaschisis, but congenital ischemic disorders such as SWS also showed CCD.- - - - - - - - - - ranking = 1keywords = cerebrum (Clic here for more details about this article) |
9/12. tuberous sclerosis: unusual associations in four cases.tuberous sclerosis is a rare disease with classic primary or secondary changes affecting mainly the cerebrum, skin, kidneys, and heart. Such lesions are generally hamartomatous and thus display malignant features only in rare cases. This paper describes four cases of tuberous sclerosis which were unique in their association with certain unusual congenital, metabolic, and tumorous conditions.- - - - - - - - - - ranking = 1keywords = cerebrum (Clic here for more details about this article) |
10/12. antibodies to cerebellar purkinje cells in patients with paraneoplastic cerebellar degeneration and ovarian carcinoma.Sera from 2 patients with ovarian carcinoma and paraneoplastic cerebellar degeneration confirmed postmortem were reacted with frozen sections of human cerebellum and stained using indirect immunofluorescence methods. Both sera produced bright cytoplasmic staining of purkinje cells and of neurons within deep cerebellar nuclei. Titration of these sera to end point revealed staining at final dilutions of 1:640 and 1:2,560, respectively. Neither of these sera reacted with sections of human cerebrum, basal ganglia, spinal cord, peripheral nerve, lung, liver, kidney, or ovary. Staining of purkinje cells was not obtained with sera from 34 normal, healthy controls, 5 patients with oat cell carcinoma of the lung, 6 patients with inflammatory central nervous system disorders, or 12 of 14 neurologically normal patients with ovarian carcinoma. Sera from 2 neurologically normal patients with ovarian carcinoma, however, produced staining of purkinje cells and deep nuclei similar to that obtained with sera from patients with paraneoplastic cerebellar degeneration. The present study documents the presence of antibodies to purkinje cells in patients with ovarian carcinoma and cerebellar degeneration and demonstrates that development of these antibodies may antedate the onset of clinically evident cerebellar degeneration.- - - - - - - - - - ranking = 1keywords = cerebrum (Clic here for more details about this article) |
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