1/53. Central nervous system infection caused by borrelia burgdorferi. Clinico-pathological correlation of three post-mortem cases.The spirochete borrelia burgdorferi (B. burgdorferi) may cause severe meningoencephalomyelitis as the sole manifestation of Lyme borreliosis. We would like to present three such cases, where definite neuroborreliosis was clinically diagnosed in two cases and possible neuroborreliosis was recognized in one case. Alive spirochetes were isolated and cultured from blood and cerebrospinal fluid (CSF) in both definite cases. B. burgdorferi as the causative agent of the infection was confirmed in CSF by polymerase chain reaction (PCR) in one definite case. In the possible case spirochetes were cultured from blood and CSF. Alive spirochetes were not isolated, however anti-B. burgdorferi antibody value in serum was significantly elevated. On necropsy gross examination brain edema without focal changes was detected in two cases. Cerebral atrophy was seen in Case 3. Microscopically, lymphocytic infiltrates, microglial diffuse and nodular activation, spongiform changes, diffuse demyelination of the cerebral and cerebellar white matter, and diffuse astrocytosis, were characteristic pathological features in all presented cases. Multifocal, perivascular degenerative changes in the cerebral and cerebellar white matter were observed in the first case. Inflammatory changes in the nuclei and roots of cranial nerves were present in the third case.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
2/53. Demyelination in primate autoimmune encephalomyelitis and acute multiple sclerosis lesions: a case for antigen-specific antibody mediation.Neuropathological and ultrastructural features of central nervous system demyelination were compared in marmoset experimental autoimmune encephalomyelitis (EAE) induced with myelin/oligodendrocyte glycoprotein (MOG), and in 3 cases of multiple sclerosis (MS) displaying recent lesions. At the edges of EAE and MS lesions, a zone of myelin vacuolation was common, whereas in the lesion proper, myelin sheaths were consistently transformed into vesiculated membranous networks. These networks became dissociated from axons by cell processes from macrophages. Oligodendrocytes were remarkably spared and evidence of myelin repair was present but not prominent. Axonal pathology was more common in the MS material than in marmoset EAE. Immunocytochemistry, using gold-labeled encephalitogenic peptides of MOG and silver enhancement to detect MOG autoantibodies, revealed the presence of MOG-specific autoantibodies over vesiculated myelin networks. gold-labeled antibody to IgG also gave a positive reaction. gold-labeled peptide of myelin basic protein did not react with MOG/EAE tissue, but the same conjugate gave positive staining in MS (and in marmoset EAE induced by whole white matter), perhaps indicating broader spectrum immunoreactivity or sensitization to myelin antigens. Thus, vesicular disruption of myelin was a constant feature in these evolving, highly active lesions in primate EAE and MS and appeared causally related to the deposition of antigen-specific autoantibodies.- - - - - - - - - - ranking = 0.758500890729keywords = central nervous system, nervous system (Clic here for more details about this article) |
3/53. Echovirus 7 associated encephalomyelitis.BACKGROUND: hand, foot, and mouth disease (HFMD) is endemic in malaysia. In 1997, a large outbreak of enterovirus 71 (EV-71) associated HFMD resulted in 41 deaths due to severe left ventricular dysfunction and central nervous system infection with extensive damage to the medulla and pons. The clinical presentation in all these patients were rapid cardio-respiratory decompensation leading to cardiac arrest. Another large outbreak of HFMD with 55 fatal cases and a similar clinical picture was reported in taiwan in 1998. In 2000, an outbreak of HFMD resulted in the deaths of three children who had rapid cardio-respiratory decompensation and one child who survived a central nervous system infection. OBJECTIVES: We set out to study the etiologic agent and mechanism involved in three children who presented to our hospital, two of whom died and one survived a central nervous system infection. STUDY DESIGN: The clinical course of the disease was described. Throat, rectal swab and cerebrospinal fluid samples were subjected to viral isolation and viral isolates were identified by immunofluorescence, micro-neutralisation using human rhabdomyosarcoma (RD) cells, and reverse transcritpase polymerase chain reaction. magnetic resonance imaging was performed on two of the patients. RESULTS: Echovirus 7 was the sole pathogen isolated from three cases of acute encephalomyelitis, two of which were fatal due to severe left ventricular dysfunction resistant to inotropic support. The survivor had residual bulbar palsy, but is considered to have had a good neurological outcome. CONCLUSION: Echovirus 7 infection associated with encephalomyelitis could be fatal due to indirect involvement of the heart resulting in severe left ventricular dysfunction. In addition one of the children presented with hand, foot, and mouth disease, a syndrome that has not been previously associated with echovirus 7 infection.- - - - - - - - - - ranking = 2.275502672187keywords = central nervous system, nervous system (Clic here for more details about this article) |
4/53. A local outbreak of paralytic rabies in Surinam children.A rapidly fatal encephalomyelitis, which was in most cases characterized by ascending paralysis, developed in seven children of the age of 3 to 10 years in a bushnegro village in the interior of Surinam. rabies virus was recovered from the central nervous system of three autopsied children. Although the source of infection has not been detected, there is an indication that, at least in some cases, the disease has been transmitted by rat-bite rather than by vampire bats. During the same period a few cases of minor febrile illness occurred in the same community. Since virological and serological evidence of a wide-spread distribution of Coxsackie A virus type 4 was obtained, the latter illness may presumably be attributed to this virus.- - - - - - - - - - ranking = 0.758500890729keywords = central nervous system, nervous system (Clic here for more details about this article) |
5/53. Progressive encephalomyelitis with rigidity presenting as a stiff-person syndrome.diagnosis criteria of stiff-person syndrome (SPS) include progressive, fluctuating muscular rigidity and spasms with normal neurological examination. The presence of unusual features such as prominent limb rigidity with segmental signs and contracture, evidence of brainstem dysfunction, profound autonomic disturbances, CSF pleiocytosis or MRI abnormalities in patients with SPS presentation allows to classify these patients as progressive encephalomyelitis with rigidity (PER). We report a 50 year-old woman suffering from severe painful spasms of abdominal wall and limb muscles. Neurological examination showed pyramidal signs. EMG disclosed continuous muscle activity with superimposed discharges. Treatment with high doses of diazepam and baclofen led to moderate improvement of generalised stiffness. However, the right arm became more rigid with oedema and vasomotor changes. Subsequently, bilateral nystagmus and internuclear opthalmplegia appeared. There was mild CSF pleiocytosis. Associated auto-immune thyroiditis was found with positive anti-microsome antibodies and decreased thyroid hormones. Search for profound neoplasm was negative. The patient had three subacute bouts then she improved with methylprednisolone. The initial clinical presentation mimicking a SPS with subsequent diffuse involvement of the central nervous system and a striking localisation of a severe rigidity to one arm allowed to suspect the diagnosis of PER. The relationship between SPS and PER remains unclear because of the rarity of these disorders. The observation reported in this paper gives evidence that both the disorders are probably two clinical presentations of the same pathogenic process.- - - - - - - - - - ranking = 0.75917895697436keywords = central nervous system, nervous system, neoplasm (Clic here for more details about this article) |
6/53. encephalomyelitis due to human parechovirus type 1.OBJECTIVES: The implication of a viral agent in encephalomyelitis has been reported for several years. In the present study we wanted to demonstrate the presence of human parechovirus type 1 (HPEV1) in a patient diagnosed with encephalomyelitis. STUDY DESIGN: Clinical samples (throat and rectal swabs, acute and convalescent sera, cerebrospinal fluid) were collected from a 10-month-old boy diagnosed with encephalomyelitis. The appropriated samples were tested for cytomegalovirus, varicella zona virus, mumps virus and enteroviruses with specific culture, and serological and molecular biological techniques. RESULTS: HPEV1 was isolated from the throat and its genome was additional detectable in the cerebrospinal fluid, throat swab and acute serum. The samples were negative for all other tested viruses. CONCLUSION: To our knowledge, this is the first reported case of HPEV1 infection related to encephalomyelitis. This emphasis that human parechoviruses can be responsible for severe central nervous system infections in children.- - - - - - - - - - ranking = 0.758500890729keywords = central nervous system, nervous system (Clic here for more details about this article) |
7/53. Unusual encephalopathy after acute chest syndrome in sickle cell disease: acute necrotizing encephalitis.stroke is the most common neurologic complication of sickle cell disease. acute chest syndrome (ACS) is a known risk factor for stroke in this population. Two patients (a 12-year-old boy and a 6-year-old girl) developed acute change of mental status and focal neurologic signs during episodes of ACS. The clinical and radiologic findings were compatible with acute necrotizing encephalitis, a variant of acute demyelinating encephalomyelitis. patients with acute neurologic deterioration in conjunction with ACS should be evaluated thoroughly for other causes of central nervous system disease including infectious/parainfectious processes as well as stroke.- - - - - - - - - - ranking = 0.758500890729keywords = central nervous system, nervous system (Clic here for more details about this article) |
8/53. Childhood chronic inflammatory demyelinating polyneuropathy with central nervous system demyelination resembling multiple sclerosis.Central nervous system demyelination has been described in adults but not in children with chronic inflammatory demyelinating polyneuropathy. We describe a patient with clinical and electrophysiological features consistent with chronic inflammatory demyelinating polyneuropathy who presented at age 5 with an intramedullary spinal cord tumor-like lesion and at age 8, represented with cerebral and spinal demyelinating lesions. Her clinical course and magnetic resonance imaging features were atypical for multiphasic disseminated encephalomyelitis and indistinguishable from multiple sclerosis. To our knowledge, this association has not been previously described in the English literature in childhood.- - - - - - - - - - ranking = 0.25keywords = nervous system (Clic here for more details about this article) |
9/53. Pathological and virological assessment of acute HTLV-I-associated myelopathy complicated with encephalopathy and systemic inflammation.HTLV-I-associated myelopathy, also known as tropical spastic paraparesis (HAM/TSP), is a chronic inflammatory disease of the spinal cord. Acute cases are uncommon. We report the case of a 41-year-old woman with acute HAM/TSP complicated with encephalitis, an intense inflammatory reaction of the nervous system and lymphocytic infiltration of skeletal muscles, liver, salivary, adrenal and pituitary glands. The immunohistochemical studies of the lymphocytes surrounding blood vessels showed both B- and t-lymphocytes, in similar proportion, with both CD4- and CD8-positive cells. In addition, many perivascular and scattered macrophages were observed. adult T-cell leukemia/lymphoma (ATL) was ruled out. The marrow aspirate was normal. Serial cerebrospinal fluid (CSF) analysis showed presence of htlv-i antibodies, but without intrathecal synthesis of specific antibodies. Determination of HTLV-I viral loads demonstrated increased levels in the CSF relative to the peripheral blood and may be associated with widespread inflammation. The pathological and immunological findings may help understand the role of immune-reactive cells in the pathogenesis of HTLV-I-associated myelopathy.- - - - - - - - - - ranking = 0.25keywords = nervous system (Clic here for more details about this article) |
10/53. role of plasmapheresis in acute disseminated (postinfectious) encephalomyelitis.Acute disseminated encephalomyelitis (ADEM) is a demyelinating central nervous system disease that is associated with high morbidity and mortality. Although the recognition of ADEM may be facilitated by newer imaging techniques, the optimal treatment of this disease remains uncertain. We describe 4 patients with severe ADEM who responded to treatment that included intensive plasmapheresis. Two of the patients were in coma at the time that plasmapheresis was instituted, and all 4 patients made an excellent recovery. Immunologic studies revealed increased serum IgA levels, increased circulating immune complex levels as measured by the Raji cell assay, and decreased numbers of T and B cells prior to treatment of ADEM. These abnormalities improved following plasma exchange. plasmapheresis appears to be effective in reversing the neuropathologic process in ADEM. The role of this treatment modality in ADEM requires further evaluation in controlled clinical trials.- - - - - - - - - - ranking = 0.758500890729keywords = central nervous system, nervous system (Clic here for more details about this article) |
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