1/20. [11C](R)-PK11195 positron emission tomography imaging of activated microglia in vivo in Rasmussen's encephalitis.This study was designed to explore the feasibility of PET using [11C](R)-PK11195 as an in vivo marker of activated microglia/brain macrophages for the assessment of neuroinflammation in Rasmussen's encephalitis (RE). [11C](R)-PK11195 PET was carried out in four normal subjects, two patients with histologically confirmed RE, and three patients with clinically stable hippocampal sclerosis and low seizure frequency. Binding potential maps showing specific binding of [11C](R)-PK11195 were generated for each subject. Regional binding potential values were calculated for anatomically defined regions of interest after coregistration to and spatial transformation into the subjects' own MRI. In one patient with RE who underwent hemispherectomy, the resected, paraffin-embedded brain tissue was stained with an antibody (CR3/43) that labels activated human microglia. Whereas specific binding of [11C](R)-PK11195 in clinically stable hippocampal sclerosis was similar to that in normal brain, patients with RE showed a focal and diffuse increase in binding throughout the affected hemisphere. In RE, [11C](R)-PK11195 PET can reveal in vivo the characteristic, unilateral pattern known from postmortem neuropathologic study. PET imaging of activated microglia/brain macrophages offers a tool for investigation of a range of brain diseases where neuroinflammation is a component and in which conventional MRI does not unequivocally indicate an inflammatory tissue reaction. [11C](R)-PK11195 PET may help in the choice of appropriate biopsy sites and, further, may allow assessment of the efficacy of antiinflammatory disease-modifying treatment.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
2/20. Encephalopathy, hearing loss and retinal occlusions (Susac's syndrome): a new case.We describe a young woman with retinal arteriolar occlusions, encephalopathy, and hearing loss. At present this disorder is known as Susac's syndrome, a microangiopathy of the brain and retina whose pathogenesis is, however, unknown. The article reviews the clinical features of this rare, but not unusual, syndrome that can easily be misdiagnosed as multiple sclerosis.- - - - - - - - - - ranking = 5.681670796877keywords = multiple sclerosis, sclerosis (Clic here for more details about this article) |
3/20. Rasmussen's syndrome associated with chronic brain stem encephalitis.We report a 3-year-old boy with a clinical picture of Rasmussen's encephalitis who had clinical, radiological and pathological evidence of brain stem involvement resulting in death. In addition, there were unexpected neuropathological findings of severe bilateral mesial temporal sclerosis. We discuss the novel finding of brain stem involvement in this condition and the association with mesial temporal sclerosis.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
4/20. An autopsied case of interferon encephalopathy.A 78-year-old male with renal carcinoma was treated with a high dose infusion of interferon-alpha (IFN-alpha) for eight months. The patient had evidence of organic brain syndrome such as dysfunction of memory, slowing of behavior, and development of mental confusion that appeared eight months after the treatment. MRI at the time of mental confusion revealed diffuse white matter lesions. Neuropathologic findings were compatible to Binswanger's disease and Senile dementia of Alzheimer Type (SDAT), Preexisting neurologic abnormalities including intracerebral arteriosclerosis and cerebral atrophy may increase susceptibility to unacceptably severe IFN neurotoxicity.- - - - - - - - - - ranking = 0.5keywords = sclerosis (Clic here for more details about this article) |
5/20. headache and inflammatory disorders of the central nervous system.The subcommittee of the International headache Society for headache classification (ICHD-II) has recently recognised that secondary headaches may occur in patients affected by inflammatory diseases (ID) of the central nervous system (CNS), classifying them among the headaches attributed to non-vascular intracranial disorders. The aim of the study was to verify the association between headache and inflammatory non-infectious diseases of the CNS, by a review of the literature data on the topic, integrated by personal cases and data. Secondary headaches may occur in four main disorders: neurosarcoidosis (sec 7.3.1), aseptic (non-infectious) meningitis (7.3.2), other non-infectious ID (7.3.3) and lymphocytic hypophysitis (7.3.4). headache and/or primary headaches are frequently reported in patients with neurosarcoidosis (30%), Behcet's syndrome (BS) (55%) and acute disseminated encephalomyelitis (45-58%). Recent data show a high incidence of headache also in multiple sclerosis (MS) (58%) (not mentioned in ICHD-II). The association between headache and inflammatory dysimmune diseases of the CNS, in particular BS and MS, might suggest a pathogenetic relationship.- - - - - - - - - - ranking = 5.681670796877keywords = multiple sclerosis, sclerosis (Clic here for more details about this article) |
6/20. Binswanger's disease in the absence of chronic arterial hypertension. A case report with clinical, radiological and immunohistochemical observations on intracerebral blood vessels.The cerebral changes are described in a woman of 54 who suffered from Binswanger's encephalopathy: there were no signs or symptoms of chronic arterial hypertension. The disease presented as dementia of about 3 years duration. Computed tomography of the brain 2.5 years before her death showed bilateral widespread hypodense lesions in the cerebral white matter. She died of an asthmatic attack. autopsy disclosed extensive bilateral degeneration of the central white matter, lacunes and gliosis. Severe obliterative arteriolosclerosis occurred in the meningeal vessels and those supplying the affected parts of the brain. light microscopy showed that the most severe lesions occurred in the arterioles. immunohistochemistry demonstrated profound extravasation of plasma proteins chiefly albumin, indicating dysfunction of the blood-brain barrier. Thus, the lesions characteristic of Binswanger's encephalopathy may develop in the absence of chronic arterial hypertension. Additional pathogenic factors, possibly genetic predisposition to vascular injury may play a role in the development of this condition.- - - - - - - - - - ranking = 0.5keywords = sclerosis (Clic here for more details about this article) |
7/20. The neurological complications of cardiac transplantation.review of the neurological complications encountered in 83 patients who received cardiac homografts over a seven-year period leads to the following conclusions: (1) Neurological disorders are common in transplant recipients, occurring in over 50 per cent of patients. (2) infection was the single most frequent cause of the neurological dysfunction, being responsible for one-third of all CNS complications. (3) The infective organisms were typically those considered to be usually of low pathogenicity: fungi, viruses, protozoa and an uncommon bacterial strain. (4) Other clinical neurological syndromes were related to vascular lesions, often apparently from cerebral ischaemia or infarction occurring during the surgical procedure, metabolic encephalopathies, cerebral microglioma, acute psychotic episodes and back pain from vertebral compression fractures. (5) The infectious complications and probably the development of neoplasms de novo, are related to immunosuppressive therapy which impairs virtually all host defence mechanisms and alters the nature of the host's response to infective agents or other foreign antigens. (6) Because neurological symptoms and signs were usually those of behavioural changes or deterioration in intellectual performance, the neurological examination was often of little value in diagnosing the nature or even the anatomical site of the neuropathological process. (7) The possibility of an infectious origin of the neurological manifestations must be aggressively pursued even in the absence of fever and a significantly abnormal spinal fluid examination. The diagnostic error made most frequently was to ascribe neurological symptoms erroneously to metabolic disturbances or to "intensive care unit psychosis" when they were in fact due to unrecognized CNS infection. (8) maintenance of mean cardiopulmonary bypass pressures above 70 mmHg, particularly in patients with known arteriosclerosis, may reduce operative morbidity. (9) Though increased diagnostic accuracy is possible with routine use of a variety of radiological and laboratory techniques, two further requirements probably must be met before a significant reduction in the frequency of neurological complications will occur: the advent of greater immunospecificity in suppressing rejection of the grafted organ while preserving defences against infection; and a more effective armamentarium of antiviral and antifungal drugs.- - - - - - - - - - ranking = 0.5keywords = sclerosis (Clic here for more details about this article) |
8/20. Psychiatric disorders in the encephalitic form of multiple sclerosis.multiple sclerosis lesions may occur predominantly in the hemispheric white matter and cause various psychiatric disorders such as remitting-relapsing endoform or exogenous psychosis, organic personality alterations and dementia. Nineteen patients suffering from this encephalitic form of multiple sclerosis as diagnosed by characteristic CSF immunoglobulin findings are analysed according to established psychopathological criteria. All cases began with psychiatric symptoms and neurological signs were either absent or overlooked. Several patients developed typical encephalomyelitic symptoms in successive relapses, but other remained with psychiatric disorders over many years. Only four patients had retrobulbar neuritis, but seven suffered from epileptic seizures. The humoral immune response was characterized by a strong dominance of IgG and a local synthesis of polyspecific antibodies against measles, rubella and varicella/zoster virus. The mononuclear CSF pleocytosis was comparatively marked with cell counts up to 180/microliter.- - - - - - - - - - ranking = 28.908353984385keywords = multiple sclerosis, sclerosis (Clic here for more details about this article) |
9/20. Postinfectious encephalitis with multifocal white matter lesions.Two cases of multifocal white matter lesions occurring after viral illness are reported. evoked potentials study and cranial magnetic resonance imaging (T2-weighted image) showed early abnormalities while CT scan was initially normal. patients improved dramatically with steroid therapy. It would seem that because of a considerable responsiveness to steroids this affection should be differentiated from other types of encephalitis. Relations with multiple sclerosis are discussed.- - - - - - - - - - ranking = 5.681670796877keywords = multiple sclerosis, sclerosis (Clic here for more details about this article) |
10/20. Relapsing central and peripheral demyelinating diseases. Unusual pathologic features.We treated a patient who had a demyelinating peripheral neuropathy and a central nervous system inflammatory demyelinating disease. The unusual pathologic feature of dense infiltrates of atypical macrophages was observed in many areas of the brain; otherwise the process had several features in common with either multiple sclerosis or chronic relapsing experimental allergic encephalomyelitis. The illness followed "swine-flu" inoculation; exacerbation followed pneumococcal vaccination.- - - - - - - - - - ranking = 0.5keywords = sclerosis (Clic here for more details about this article) |
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