Cases reported "Nervous System Diseases"

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11/27. HTLV-1-associated myeloneuropathy in an Italian.

    A 58-year old man presented with slowly progressive spastic paraparesis, ataxia, absent ankle jerks, bladder disturbances, impairment of vibration sense and mental deterioration. Electrophysiological studies documented axonal sensory neuropathy, posterior column and optic nerve involvement. serum tests for anti-HTLV-1 antibodies were negative but HTLV-1 proviral sequences were consistently demonstrated in white blood cell genomic dna using the polymerase chain reaction technique. Western blot and polymerase chain reaction assays of sera and dna from family members were negative for HTLV-1. The most likely cause of infection in this patient was a blood transfusion received 2 years before onset of symptoms. This is the second Italian case of HTLV-1 associated myelopathy and the fourth reported in white subjects living in europe.
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ranking = 1
keywords = lyme
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12/27. stroke in neuroborreliosis.

    A 20-year-old man suffered two thalamic infarctions during the course of neuroborreliosis and was successfully treated with intravenous ceftriaxone. review of 11 additional cases of stroke and cerebral vasculitis in neuroborreliosis suggests that there is a meningovascular form of the infection with predilection for the posterior circulation and an association with the European strains of borrelia burgdorferi.
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ranking = 30.703203716883
keywords = burgdorferi
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13/27. Cultivation and characterization of spirochetes from cerebrospinal fluid of patients with Lyme borreliosis.

    Attempts were made to culture spirochetes from cerebrospinal fluid samples of 105 patients suspected of having Lyme borreliosis with neurological complications. At the final evaluation, only 38 patients fulfilled the criteria of neuroborreliosis. Spirochetes were cultured from cerebrospinal fluid samples of four of these patients. All four patients had pleocytosis in their cerebrospinal fluid and a history of neurological symptoms of only 4 to 10 days in duration. Two of them had no detectable antibodies against any of the isolated spirochetes in their cerebrospinal fluid, both when tested with an enzyme-linked immunosorbent assay and when tested by immunoblotting. An antibody reaction against the homologous isolate that was distinctly stronger than that against the heterologous isolates was found in the serum and cerebrospinal fluid samples from one patient. The cells of the isolates were morphologically similar and showed a very similar protein pattern when analyzed by sodium dodecyl sulfate-polyacrylamide gel electrophoresis. cells of all isolates reacted with the monoclonal antibodies H5332 and H9724, which also react with borrelia burgdorferi B31, the type strain. One isolate lost a major protein of 23 kilodaltons after subcultivation for 4 months. We conclude that isolation of spirochetes from cerebrospinal fluid might prove successful in clinically selected cases of Lyme borreliosis.
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ranking = 30.703203716883
keywords = burgdorferi
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14/27. Immunologic reactivity against borrelia burgdorferi in patients with motor neuron disease.

    Of 19 unselected patients with the diagnosis of amyotrophic lateral sclerosis (ALS) living in Suffolk County, new york (an area of high lyme disease prevalence), 9 had serologic evidence of exposure to borrelia burgdorferi; 4 of 38 matched controls were seropositive. Eight of 9 seropositive patients were male (8 of 12 male patients vs 2 of 24 controls). Rates of seropositivity were lower among patients with ALS from nonendemic areas. All patients had typical ALS; none had typical lyme disease. cerebrospinal fluid was examined in 24 ALS patients--3 (all with severe bulbar involvement) appeared to have intrathecal synthesis of anti-B burgdorferi antibody. Following therapy with antibiotics, 3 patients with predominantly lower motor neuron abnormalities appeared to improve, 3 with severe bulbar dysfunction deteriorated rapidly, and all others appeared unaffected. There appears to be a statistically significant association between ALS and immunoreactivity to B burgdorferi, at least among men living in hyperendemic areas.
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ranking = 214.92242601818
keywords = burgdorferi
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15/27. Histopathological findings in the central and peripheral nervous systems in neuroborreliosis. A report of three cases.

    Very little is known about tissue changes and pathophysiological mechanisms in Garin-Boujadoux-Bannwarth syndrome. We report histopathological findings in the central and peripheral nervous systems of three typical cases. In the acute stage of the disease mononuclear perivascular infiltrations with mainly T-helper cells were the prominent finding, whereas after treatment there was no vessel involvement. The fibre changes in the peripheral nervous system consisted of axonal degeneration. No borrelia burgdorferi-specific antigen could be detected by immunohistochemical methods. Thus vasculitis might be one of the primary pathophysiological mechanisms for the involvement of the nervous system.
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ranking = 30.703203716883
keywords = burgdorferi
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16/27. Two novel variants of transthyretin identified in Japanese cases with familial amyloidotic polyneuropathy: transthyretin (Glu42 to Gly) and transthyretin (Ser50 to Arg).

    Two mutant genes coding for two different variants of transthyretin were identified in two independent kindreds with familial amyloidotic polyneuropathy. A single base change from A to G was identified in exon 2 of transthyretin gene in two brothers from the first kindred. This base change led to replacement of glutamate by glycine at position 42 of 127-residue molecule. In a patient from the second kindred, T to G transversion in exon 3 of transthyretin gene led to replacement of Ser by Arg at position 50. The two mutants were discovered by randomly sequencing recombinant clones containing the entire length of each one of the four exons selectively amplified by polymerase chain reaction. The base change produced a new restriction site for Hae III and Cfr 13 I in the exon 2 and for Mva I in the exon 3, respectively. Restriction fragment length polymorphisms and allele-specific oligonucleotide hybridizations confirmed the base changes. The accurate detection of the new mutant genes is hereafter possible by these procedures.
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ranking = 0.5
keywords = lyme
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17/27. The neurological complications of borrelia burgdorferi in the New Forest area of Hampshire.

    The neurological complications of borrelia burgdorferi infection have only recently been recognised in the United Kingdom. Eight cases are reported which were all contracted in the New Forest area of Hampshire. The majority of patients had Bannwarth's syndrome though meningism and parenchymal lesions also occurred. All patients made a virtually complete neurological recovery in contrast to some patients with lyme disease.
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ranking = 153.51601858441
keywords = burgdorferi
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18/27. Seronegative chronic relapsing neuroborreliosis.

    We report an unusual patient with evidence of borrelia burgdorferi infection who experienced repeated neurologic relapses despite aggressive antibiotic therapy. Each course of therapy was associated with a Jarisch-Herxheimer-like reaction. Although the patient never had detectable free antibodies to B. burgdorferi in serum or spinal fluid, the CSF was positive on multiple occasions for complexed anti-B. burgdorferi antibodies, B. burgdorferi nucleic acids and free antigen.
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ranking = 122.81281486753
keywords = burgdorferi
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19/27. lyme disease: a neuropsychiatric illness.

    OBJECTIVE: lyme disease is a multisystemic illness that can affect the central nervous system (CNS), causing neurologic and psychiatric symptoms. The goal of this article is to familiarize psychiatrists with this spirochetal illness. METHOD: Relevant books, articles, and abstracts from academic conferences were perused, and additional articles were located through computerized searches and reference sections from published articles. RESULTS: Up to 40% of patients with lyme disease develop neurologic involvement of either the peripheral or central nervous system. Dissemination to the CNS can occur within the first few weeks after skin infection. Like syphilis, lyme disease may have a latency period of months to years before symptoms of late infection emerge. Early signs include meningitis, encephalitis, cranial neuritis, and radiculoneuropathies. Later, encephalomyelitis and encephalopathy may occur. A broad range of psychiatric reactions have been associated with lyme disease including paranoia, dementia, schizophrenia, bipolar disorder, panic attacks, major depression, anorexia nervosa, and obsessive-compulsive disorder. Depressive states among patients with late lyme disease are fairly common, ranging across studies from 26% to 66%. The microbiology of borrelia burgdorferi sheds light on why lyme disease can be relapsing and remitting and why it can be refractory to normal immune surveillance and standard antibiotic regimens. CONCLUSIONS: Psychiatrists who work in endemic areas need to include lyme disease in the differential diagnosis of any atypical psychiatric disorder. Further research is needed to identify better laboratory tests and to determine the appropriate manner (intravenous or oral) and length (weeks or months) of treatment among patients with neuropsychiatric involvement.
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ranking = 30.703203716883
keywords = burgdorferi
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20/27. Severe neurological abnormalities associated with a mutation in the zinc-finger domain in a group A xeroderma pigmentosum patient.

    All the reported Japanese patients with group A xeroderma pigmentosum (XP) have two or three mutations at codon 116 in exon 3, codon 228 in exon 6, and the splicing acceptor site of intron 3 of XP group A complementing (XPAC) gene. A homozygote (XP39OS) with a nonsense mutation at codon 228 has less severe neurological abnormalities than patients with the splicing mutation at the acceptor site of intron 3. As homozygotes for the nonsense mutation at codon 116, which truncates a carboxyl-terminal site of XPAC protein at an early part of its zinc-finger domain, have not been reported previously, the possible severity of associated neurological abnormalities was not known. We report a group A XP patient, XP18OS, who had neurological abnormalities which were more severe than those in patients homozygous for the splicing mutation. The polymerase chain reaction product from exon 3 of the patient's XPAC gene was digested completely into three fragments by MseI restriction endonuclease. Thus, the patient was homozygous for the mutation at codon 116.
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ranking = 0.5
keywords = lyme
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