Cases reported "Encephalomyelitis"

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1/206. 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. ( info)

2/206. Lethal encephalopathy complicating childhood shigellosis.

    A 6-year-old girl is described who died following rapid neurological deterioration, ending in lethal cerebral oedema. Despite the absence of severe intestinal and metabolic derangement, shigella was cultured from the stool. Toxic encephalopathy is responsible for death following this rare complication of childhood shigellosis in developed countries. The pathophysiology is unknown. CONCLUSION: Lethal toxic encephalopathy can be caused by shigella despite the absence of severe intestinal and metabolic derangement. If shigelllosis is suspected, headache may be a first significant sign for the development of toxic encephalopathy. Early recognition and rapid measures to prevent brain oedema may improve outcome. ( info)

3/206. 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. ( info)

4/206. Reversible paraneoplastic encephalomyelitis associated with a benign ovarian teratoma.

    BACKGROUND: Paraneoplastic encephalomyelitis (PEM) is a well characterized, and typically irreversible, paraneoplastic syndrome, usually associated with small cell lung cancer or other malignancy. We describe a case of a young woman with a benign ovarian teratoma who presented with a reversible PEM. CASE REPORT: A 24-year-old woman presented with a three week history of memory impairment, unusual behavior, personality changes, auditory hallucinations, hypersomnolence and binocular biplopia. On admission she was disoriented and inattentive with impaired short term memory. Small doses of lorazepam (1 mg), given for episodic agitation, repeatedly induced multidirectional bilateral nystagmus and a skew deviation, but her neurological examination was otherwise normal. A left-sided pelvic mass was palpable. Brain MRI pre- and post-gadolinium was normal. There was a mild CSF pleocytosis and an EEG showed minimal bilateral background activity irregularities. There were no other laboratory abnormalities. Two weeks after admission, she clinically deteriorated developing central respiratory failure and a flaccid paraplegia. Repeat MRI showed an area of increased T2 weighted signal in the medulla and three similar areas in the spinal cord. Following removal of her tumor, treatment with high dose corticosteroids and intravenous immunoglobulin, she ultimately made a full recovery. pathology revealed the tumor to be a benign ovarian cystic teratoma. CONCLUSIONS: This is the first report of a reversible PEM seen in association with a benign tumor, in this case a mature ovarian teratoma. Presumably, an immune response directed against neural clements of the teratoma cross-reacted with normal brain, brainstem and spinal cord antigens to cause neurologic symptoms. Tumor removal was followed by neurologic recovery. ( info)

5/206. Detection of anterior horn lesions by MRI in central European tick-borne encephalomyelitis.

    We report a case of central European tick-borne encephalitis with cervical myelitis presenting clinically as a lower motor neuron syndrome of the upper limbs with proximal asymmetrical pareses and atrophies. There were no sensory deficits nor signs of lesions of the spinal pathways or signs of encephalitis or meningitis. The affected motor fibers of the upper limbs were electrically inexcitable, but sensory findings were normal. electromyography of the paralyzed muscles revealed pathological denervation activity without voluntary activation. The initial magnetic resonance imaging (MRI) showed a large hyperdense lesion in the anterior part of the cervical cord from C3 to T1. Despite the fact that MRI changes disappeared completely within 6 weeks the patient showed only little improvement in the paralyzed muscles after 6 months. To our knowledge, these MRI changes in patients with tick-borne encephalitis, consistent with an isolated anterior horn lesion, have never been reported previously. The course may have been aggravated by an initial antibiotic treatment with cephalosporins. ( info)

6/206. Gabapentin-induced mood changes with hypomanic features in adults.

    We report two adults who received gabapentin (GBP) and subsequently developed behavioural side effects. Indications for GBP treatment were newly diagnosed epilepsy in one and painful paraesthesiae in the other. Both had no past history of psychiatric or behavioural disorder. Abnormal behaviour consisted of elevated mood, euphoria, and increased energy in both patients, and pressure of speech and decreased need for sleep in one of them. These symptoms were transient and fully reversible. One patient had to discontinue GBP. Behavioural changes were not related to seizure activity. They should be recognized as a possible side effect of GBP treatment in adults. ( info)

7/206. Acute hemorrhagic leukoencephalitis: report of three cases and review of the literature.

    attention is directed to the diagnosis of acute hemorrhagic leukoencephalitis and the possibility of its clinical recognition is discussed. Three case histories, two diagnosed at autopsy and one clinically, are presented. The latter was treated with very generous doses of steroids and recovered spectacularly. On omission of steroids, a mild, temporary clinical recurrence occurred. Clinical, clinico-pathologic, and pathologic aspects of the disease are discussed, and etiologic factors mentioned. Some thoughts on the relationship of this disease to experimental allergic encephalomyelitis are mentioned. Differential diagnosis is considered and the use of biopsy for confirmation of diagnosis especially from herpes encephalitis considered. A plea is made for the trial of treatment with large doses of steroids. ( info)

8/206. scrub typhus encephalomyelitis with prominent focal neurologic signs.

    BACKGROUND: encephalomyelitis with prominent focal neurologic signs and associated neuroradiologic abnormalities has not been previously described in scrub typhus. CASE DESCRIPTION: A 22-year-old woman was admitted because of fever and an altered mental state. neurologic examination revealed bilateral sixth and seventh nerve palsies, bilateral gaze evoked nystagmus, anarthria, dysphagia, quadriparesis, and sensory level at T1. serum and cerebrospinal fluid samples were positive for tsutsugamushi antibody. The patient's magnetic resonance images demonstrated the lesions responsible for the neurologic dysfunctions: in the lower brainstem, cerebellar peduncles, and spinal cord. It was interesting that the gray matter of the spinal cord was predominantly involved. CONCLUSIONS: The recognition of unusual manifestations and the clinical suspicion of this treatment-responsive disease may be important, particularly in the face of increasing international and intranational travel. ( info)

9/206. encephalomyelitis due to cryptococcus neoformans var gattii presenting as spinal tumour: case report and review of the literature.

    A 24 year old immunocompetent German resident is described who developed multifocal encephalomyelitis due to infection with cryptococcus neoformans var gatti, commonly considered a disease of tropical regions. In the light of current knowledge on the epidemiology of C neoformans var gatti and the travel history of the patient it is assumed that the infection was acquired outside europe. As exclusive intramedullary involvement is an outstandingly rare manifestation in spinal cryptococcosis, the particular diagnostic procedure and the therapeutic strategies are discussed ( info)

10/206. encephalomyelitis and bilateral optic perineuritis after influenza vaccination.

    We report the case of one patient suffering from headache, urinary retention, bilateral optic disc swelling and a mild bilateral visual defect after influenza vaccination. The presumptive diagnosis was encephalomyelitis with bilateral optic perineuritis caused by influenza vaccination. We stress on the interest to search for this aetiology in unexplained optic neuropathy. ( info)
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