Cases reported "Disease Progression"

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1/80. SSPE following neonatal measles infection.

    The authors report a case of subacute sclerosing panencephalitis in a child who had measles during the neonatal period. At 3 years, 6 months of age, over a period of a few weeks, the patient lost the ability to sit unaided as a result of progressive truncal ataxia, without apparent cognitive changes, simulating acute cerebellar ataxia. His symptoms improved in 1 month, and he was able to walk again with support, but mental alteration and periodic mild head nodding on awakening followed. His illness was diagnosed as subacute sclerosing panencephalitis on the basis of the elevated titers of measles antibodies in the cerebrospinal fluid. measles infection before 1 year of age is a risk factor of subacute sclerosing panencephalitis, but reports about patients with neonatal measles infection are rare. Immaturity of the brain at the time of measles infection may not only be a risk factor but may also influence the clinical course of the disease.
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ranking = 1
keywords = encephalitis
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2/80. Subretinal lesions in subacute sclerosing panencephalitis.

    subacute sclerosing panencephalitis (SSPE) is a rare progressive neurologic disorder. A 9-year-old boy was seen who had progressive neurocognitive decline, myoclonic jerking of the extremities, and an abnormal result of an electroencephalogram (EEG). Ophthalmoscopic examination revealed multifocal subretinal lesions. The diagnosis of SSPE was made on the basis of the clinical examination and elevated serum and spinal fluid measles titer. We describe subretinal lesions in a patient with SSPE.
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ranking = 1.6666666666667
keywords = encephalitis
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3/80. A 4-year-old with pica, progressive incoordination, and decreased responsiveness.

    This article reports a typical case of subacute sclerosing panencephalitis (SSPE). The patient contracted measles as an infant during the 1989 to 1991 united states measles epidemic. At 4 1/2 years of age, he developed behavioral changes and quickly progressed through the typical clinical stages of SSPE. His EEG was characteristic. serum and CSF measles immunoglobulin g were markedly elevated. He remains alive but is vegetative. To our knowledge, this is the first case of SSPE stemming from the 1989 to 1991 measles epidemic. Because infants--the group at highest risk to develop SSPE--were most severely affected by this measles outbreak, other cases of SSPE stemming from this epidemic may occur.
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ranking = 0.33333333333333
keywords = encephalitis
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4/80. Migratory basal ganglia lesions in subacute sclerosing panencephalitis (SSPE): clinical implications of axonal spread.

    We report a boy with subacute sclerosing panencephalitis (SSPE) who exhibited parkinsonian symptoms four months after onset. The symptoms improved after administration of levodopa. One year after onset, bilateral symmetric lesions appeared in the substantia nigra and the putamen, as observed using magnetic resonance imaging. After a one-year interval, the lesions migrated to the bilateral caudate and the cerebellar dentate nuclei. The series of migratory legions, each of which was connected by axonal pathways originating from the substantia nigra, suggests axonal spread of the sspe virus.
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ranking = 1.6666666666667
keywords = encephalitis
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5/80. The value of serial auditory brainstem response in patients with subacute sclerosing panencephalitis.

    A total of 98 serial auditory brainstem responses from 17 patients (11 boys and 6 girls) with subacute sclerosing panencephalitis were compared with their clinical course and stages. These patients were exposed to measles early in life (at 1.8 /- 1.4 years old) with the average onset of the disease at 8.8 /- 0.7 years of age. The main abnormalities of their auditory brainstem responses were a prolongation of waves I, III, and V, and of the I to V interpeak interval. Wave V thresholds were also elevated. These effects on the auditory brainstem responses started 1 to 2 years after onset of neurologic signs. I to V interpeak latency became prolonged with the progress of clinical stages, especially in Jabbour's clinically advanced stage IV. In two patients with an acute progressive type of subacute sclerosing panencephalitis, very rapid deterioration with a distorted wave pattern of later components was observed. These findings suggest rostrocaudal progression in the central nervous system of both long-term and fulminant cases.
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ranking = 2
keywords = encephalitis
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6/80. Acute relapsing encephalopathy mimicking acute necrotizing encephalopathy in a 4-year-old boy.

    A 4-year-old boy showed two episodes of encephalitis/encephalopathy involving disturbed consciousness, convulsion, and paresis associated with the elevated levels of protein and myelin basic protein of the cerebrospinal fluid. MRI studies of the brain revealed symmetrical lesions in the brain stem and thalami at the first episode, and additional lesions were found in the cerebellum involving both the gray and white matter in the second episode. The intensities of MRI lesions were low in T I and high in T2. These episodes were followed by an elevation of the anti-viral antibody titers, for influenza a virus during the first episode and for adenovirus during the second. In the second episode, intravenous methylprednisolone therapy resulted in rapid improvement of his neurological signs.
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ranking = 0.33333333333333
keywords = encephalitis
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7/80. subacute sclerosing panencephalitis : CT and MR imaging in a rapidly progressive case.

    We report the findings on CT and MR imaging in a patient with rapidly progressive subacute sclerosing panencephalitis (SSPE), which correlated with the clinical progression of the disease. In view of the rapid neurological deterioration and CSF pleocytosis, a brain biopsy was done and this confirmed the diagnosis.
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ranking = 1.6666666666667
keywords = encephalitis
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8/80. Benign cephalic histiocytosis progressing into juvenile xanthogranuloma: a non-Langerhans cell histiocytosis transforming under the influence of a virus?

    Benign cephalic histiocytosis (BCH) is best understood as a form of non-Langerhans cell histiocytosis, specifically as an early mononuclear variant of juvenile xanthogranuloma (JXG). However, the progression of BCH into JXG in the same patient has only been reported once before. We describe the case of a 2-year-old girl with asymptomatic, large, ill-defined infiltrated flat plaques over both cheeks, in addition to isolated papules. A punch biopsy of a plaque revealed dermal infiltration by vacuolated and scalloped histiocytes positive for CD68 KP-1, and that lacked expression of CD1a and S-100 protein, favoring macrophages over langerhans cells. Electron microscopy study showed comma-shaped intracytoplasmic bodies in the histiocytic cells leading to the diagnosis of BCH. One year later, after an episode of varicella-zoster infection, the flat plaques over the cheeks became large reddish-yellow nodules, and in a second biopsy appeared to progress to JXG. Virus-related mechanisms of progression are discussed.
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ranking = 0.0084109615385246
keywords = zoster, varicella
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9/80. Progressive outer retinal necrosis caused by herpes simplex virus type 1 in a patient with acquired immunodeficiency syndrome.

    OBJECTIVE/BACKGROUND: To identify the etiologic agent of rapidly progressive outer retinal necrosis (PORN) in a 32-year-old man with acquired immunodeficiency syndrome (AIDS), who had retinitis developed from cytomegalovirus (CMV). Multiple yellowish spots appeared in the deep retina without evidence of intraocular inflammation or retinal vasculitis, diagnosed clinically as PORN. death occurred after failure of multiple organs. DESIGN: Case report. methods: Both globes were taken at autopsy, fixed in formalin, and examined histopathologically and immunohistochemically to identify causative agents in the retinal lesions. MAIN OUTCOME MEASURE: immunohistochemistry. RESULTS: All layers of the retina were severely damaged and contained focal calcification. Cytomegalic inclusion bodies were found in cells in the damaged retina of the right eye. Immunohistochemical studies for herpesviruses revealed the presence of CMV antigens in the right retina at the posterior pole and herpes simplex virus type 1 (HSV-1)-specific antigen in the periphery of both retinas. No varicella-zoster virus (VZV) antigen was detected in either retina. CONCLUSIONS: PORN has been described as a variant of necrotizing herpetic retinopathy, occurring particularly in patients with AIDS. Although the etiologic agent has been reported to be VZV, HSV-1 can be an etiologic agent.
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ranking = 0.020790875238705
keywords = zoster, varicella, herpes
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10/80. Magnetic resonance and positron emission tomography changes during the clinical progression of Rasmussen encephalitis.

    The authors describe serial positron emission tomography (PET) and magnetic resonance imaging (MRI) studies in a patient with pathologically confirmed Rasmussen Encephalitis (RE). Results of initial PET and MRI studies were normal. Subsequent studies showed involvement of the percentral and postcentral gyri and the putamen on PET, and the precentral and postcentral gyri on MRI. Coregistration of PET and MR images showed good correlation between the precentral and postcentral gyri involvement. However, subcortical involvement occurred earlier on PET than on MRI. The authors demonstrate the evolution of changes on PET and MR images in a patient with RE. Despite early pathologic confirmation of RE, there were no definite structural or functional imaging changes on PET or MRI until 3 years after symptom onset. These findings demonstrate the variability of imaging changes in RE, and the need to carefully correlate electro-physiologic and clinical findings to confirm the diagnosis of RE.
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ranking = 1.3333333333333
keywords = encephalitis
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