1/10. blastomyces dermatitidis occurring as an isolated cerebellar mass.Although abundant in nature, fungi are infrequently pathogenic in humans. CNS fungal infections in non-immunocompromised individuals are uncommon. We discuss here the case of an otherwise healthy black woman with an isolated mass of the cerebellopontine angle identified as blastomyces dermatitidis, successfully treated with surgical resection of the mass and intravenous amphotericin b therapy.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
2/10. hemifacial spasm or subcortical epilepsy?A child with Goldenhar's syndrome presented at about one week of age with stereotyped, repetitive paroxysmal episodes of hemifacial contraction, suggestive of partial seizures or hemifacial spasm. Later in life he also developed independent, permanent abnormal eye and chin movements identical in rhythm to those seen in myorhythmias, suggesting involvement of the dentato-olivary pathway. MRI demonstrated a hamartomatous lesion at the level of the pontomedullary junction. We speculate that the nature of the lesion could be responsible for the partial seizures mimicking hemifacial spasm and that because of its location, this same lesion could also be implicated in the genesis of myorhythmias. The presence of a hamartomatous lesion in a region affected by the abnormal development of the first and second branchial arches is not fortuitous.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
3/10. The functional nature of cerebellar diaschisis.We report a patient who presented with transient clumsiness of his right hand due to a small hemorrhage in the left globus pallidus. Ten days later, positron emission tomography performed at rest showed decreased oxygen metabolism and blood flow at the site of the anatomic lesion and in remote areas such as the ipsilateral frontotemporoparietal cortex and the contralateral cerebellar hemisphere. Cerebellar hypometabolism has been ascribed to functional disconnection of the contralateral hemisphere from the cerebral cortex and has been termed crossed cerebellar diaschisis. One month later, positron emission tomography performed during unilateral motor activation (finger opposition) showed increased blood flow in the sensorimotor and supplementary motor areas contralateral to the hand engaged in the motor task. An at-rest study at this time showed resolution of the crossed cerebellar diaschisis observed acutely, but cerebellar asymmetry was demonstrated during performance of the motor task with the normal as well as with the previously paretic hand. Our activation study demonstrated cerebellar asymmetry in the chronic phase during a motor task, even though resting cerebellar blood flow was symmetrical. This observation reveals the dynamic, functional nature of crossed cerebellar diaschisis and may partially explain the lack of any clinical counterpart in functional studies of the cerebellum performed with the patient at rest.- - - - - - - - - - ranking = 5keywords = nature (Clic here for more details about this article) |
4/10. Giant invasive cholesteatoma. Report of a case with cerebellar invasion.We present a case of epidermoid cholesteatoma that killed the patient by relentless invasion, despite radical surgery. An autopsy disclosed a focus of dural and cerebellar invasion, which dramatized the aggressive nature of this disease. In fact, the epithelial cells of this disease are not thought to possess the ability to invade. Instead, inflammation is believed to both stimulate the squamous epithelial cells to proliferate and destroy adjacent tissues, thereby providing a pathway for epithelial cell migration. Epidermoid cholesteatoma, then, is a disease in which the processes of inflammation and hyperplasia combine to mimic a neoplasm.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
5/10. Cystic cavernous angiomas of the posterior fossa. Report of three cases.Three histologically verified cases of cystic cavernous angioma of the posterior fossa are reported. The clinical history of these patients is presented, and the diagnostic and surgical problems arising from the extreme rarity of the combination of cerebellar site and cystic nature of the lesion are discussed.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
6/10. Fenestration and duplicate origin of the left vertebral artery in angiography. Report of three cases.Three cases are shown; 2 of angiographically proven distal fenestration of the left vertebral artery and one of duplicate origin. Both anomalies are classified as of inhibitory type. Their incidence in the literature is reviewed. Underlined is the fact that variations of this nature frequently occur in combination with other inhibitory malformations particularly in the vascular and skeletal systems.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
7/10. Autoimmune paraneoplastic cerebellar degeneration: immunohistological localization of antibody-binding sites.Sera from 3 patients with breast or ovarian tumors and paraneoplastic cerebellar degeneration (PCD) contained anti-Purkinje cell antibodies (PCAbs) which also bind to other neurons on frozen sections of adult rodent brain. PCAbs tested on new-born rodent (rabbit, rat, mouse) brain tissue detected only oligodendrocyte-like cells (ODLC) in the white matter and allowed us to speculate on the nature of the antigenic structure in the neuronal cytoplasm. All these PCAbs appear histochemically identical and recognize antigens which belong to so-called "Yo" proteins.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
8/10. Aprosencephaly and cerebellar dysgenesis in sibs.Aprosencephaly is a rare, lethal malformation sequence of the central nervous system that has been attributed to a postneuralation encephaloclastic process. We describe autopsy findings consistent with aprosencephaly in 2 fetuses conceived from a consanguineous mating (first cousins). Both showed anencephalic manifestations; however, the crania were intact, with fused sutures. The neuropathologic findings were essentially identical. Each fetus had complete absence of the telecephalon and pyramidal tracts, rudimentary diencephalic and mesencephalic structures, primitive cerebellar hemispheres, posterolateral clusters of primitive neural cells in the medullas suggesting an abnormality of neural migration, a normally-formed spinal cord, and retinal dysplasia within normally-formed globes. In addition, both fetuses manifested a peculiar perivascular mesenchymal proliferation seen only within the central nervous system. The similarity of these cases, coupled with parental consanguinity, suggests a primary malformation in brain development due to the homozygous representation of a mutant allele. We hypothesize that these patients may represent a defect in a gene important in brain development, the nature of which has yet to be elucidated.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
9/10. Lhermitte-Duclos disease: literature review and novel treatment strategy.BACKGROUND: Lhermitte-Duclos disease (LDD) is a rare pathologic entity involving the cerebellum. The fundamental nature of the entity and its pathogenesis remain unknown, and considerable debate has centered on whether it represents a neoplastic, malformative or hamartomatous lesion. The cell or cells of origin remain incompletely defined. Previous reports of cases in the English literature have dealt predominantly with the clinical and pathological aspects yet few address issues of treatment. methods: A case of Lhermitte-Duclos disease (LDD) in a 54-year-old female leading to local compressive symptoms and obstructive hydrocephalus is presented. A craniectomy, in addition to a C1 laminectomy followed by a decompressive duroplasty (using autologous fascia lata graft) was performed. RESULTS: The patient clinically improved and follow-up MRI 11 months post-operatively revealed improvement in hydrocephalus. CONCLUSION: The histological and immunohistochemical features of the lesion are described, emphasizing the role of an abnormal dysplastic granule cell layer. The evidence in favor of each of the major theories of pathogenesis, malformative and neoplastic is discussed. Based on these facts a form of surgical intervention involving decompressive duroplasty is proposed.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
10/10. magnetic resonance imaging findings in acute cerebellitis.Cerebellitis, also known as acute cerebellar ataxia, is an inflammatory syndrome of cerebellar dysfunction that may reflect an infectious, post-infectious, or post-vaccination disorder. We present serial magnetic resonance imaging (MRI) findings in a partially reversible, idiopathic cerebellitis. Bilateral cerebellar parenchymal abnormalities were noted, including hyperintensities on T2-weighted images and cerebellar swelling. After contrast administration, the cerebellum showed abnormal bilateral enhancement. The authors state this represents the first report of abnormal contrast enhancement in this condition. The MRI lesions most likely reflect the reversible, inflammatory nature of the syndrome.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
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