Cases reported "Encephalitis"

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1/15. Presence, distribution and spread of productive varicella zoster virus infection in nervous tissues.

    Nervous tissue lesions were retrospectively studied for detection of productive varicella zoster virus (VZV) infection in 33 autopsied cases, including 19 herpes zoster (HZ) (10 trigeminal, nine spinal) and 14 cases of nodular brainstem encephalitis without HZ. Immunocytochemistry for VZV antigens and in situ hybridization with a biotinylated VZV DNA probe were used on formol-fixed paraffin sections. Peripheral and central nervous system, skin and striated muscle were investigated in serial sections; available tissue blocks, however, varied between cases. Varicella zoster virus production (both antigen and DNA) in nervous tissue was found in HZ cases but only of short survival after a rash of up to 7 wks (eight out of 12 patients). Varicella zoster virus was visualized in nerve cells, glial cells, schwann cells and blood vessels. In the central nervous system (CNS), VZV was detected in trigeminal nuclei (one out of 10 brains) or disseminated nodular brainstem lesions (one out of 10 brains), in subependymal microvessels (one out of 10 brains) or vasculitic arteries (two out of 19 brains or spinal cords). In the peripheral nervous system (PNS), VZV (DNA and antigen) was found in neurons and satellite cells of sensory ganglia (four out of seven cases with sampling of ganglia), and in damaged nerve fibres including a muscle nerve in one case; myositis with VZV in affected muscle fibres was found in the latter case. In nodular brainstem encephalitis, one case contained VZV within nodular lesions. We conclude that (i) VZV neural spread is suggested by detectable virus in ganglia, nerve fibres and CNS target nuclei; (ii) haematogenous spread of VZV is suggested by detection of virus in CNS microvessels and in disseminated brainstem encephalitis; (iii) VZV myositis may occur in zosteric myotomes; and (iv) VZV is a possible agent in nodular brainstem encephalitis.
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2/15. Varicella-zoster virus encephalitis in acquired immunodeficiency syndrome: report of four cases.

    Four patients with acquired immunodeficiency syndrome, a 27-year-old female intravenous drug abuser and three males (two drug addicts aged 27 and 33 years and a 40-year-old homosexual) presented with a rapidly progressive encephalopathy. Two had generalized varicella-zoster virus skin infection, one had had a regressive thoracic zoster rash 7 months previously and one had no history of cutaneous eruption. Neuropathological examination revealed, in each case, multifocal necrotic changes with numerous, intranuclear Cowdry type A inclusion bodies in glial cells, endothelial cells, macrophages and neurons, within and around the lesions. These inclusion bodies were stained positively for varicella-zoster virus by immunocytochemistry and contained herpes virus nucleocapsids by electron microscopy. molecular biology using the polymerase-chain-reaction method demonstrated viral genome. In one case, zoster-induced non-inflammatory vasculopathy involved medium sized leptomeningeal vessels and was associated with circumscribed areas of cortico-subcortical infarction. In another case, varicella-zoster virus encephalitis was associated with human immunodeficiency virus encephalitis and a secondary cerebral lymphoma. Multinucleated giant cells expressing human immunodeficiency virus proteins in their cytoplasm, were found in the lymphomatous deposits and in the varicella-zoster virus necrotic lesions. In these latter lesions, Cowdry type A inclusion bodies could be seen in the nuclei of some multinucleated giant cells confirming previous observations of MGCs co-infected by hiv and CMV, and supporting the hypothesis that dna viruses interact with hiv, thus increasing its effect.
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3/15. Encephalitis and chorioretinitis associated with neurotropic African horsesickness virus infection in laboratory workers. Part II. Ophthalmological findings.

    Four laboratory workers developed uveitis-chorioretinitis, associated with encephalitis in 3 cases. The retinitis was characterised by haemorrhages and areas of retinal oedema, most marked over the posterior polar regions, and was associated with exudative retinal detachments. The lesions progressed over weeks and showed a severe retinal arterial vasculopathy with arteriolar narrowing, ghost vessel formation and the development of optic atrophy. The picture in 2 of the patients resembled that of the acute retinal necrosis syndrome (ARN). antibodies to African horsesickness (AHS) virus were detected. The serology for AHS virus was positive in all 4 patients as well as in 5 of 15 laboratory workers from the same facility who were clinically and ophthalmologically normal. This is to our knowledge the first description of subclinical and probable clinical neurotropic AHS virus infection in man. AHS is a hitherto-unrecognised possible cause of viral retinitis and the ARN syndrome.
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4/15. 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.
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5/15. Multiple subpial transection: a new approach to the surgical treatment of focal epilepsy.

    A new operative approach has been designed for the relief of medically intractable focal epilepsy. It is intended particularly to be used in those cases where the epileptogenic lesion lies in "unresectable" cortex; that is, those cerebral regions subserving speech, memory, and primary motor and sensory function. The procedure is based upon experimental evidence indicating 1) that epileptogenic discharge requires substantial side-to-side or horizontal interaction of cortical neurons, and 2) that the major functional properties of cortical tissue depend upon the vertical fiber connections of the columnar units. The technique requires severing of tangential intracortical fibers while preserving the vertical fiber connections of both incoming and outgoing nerve pathways and of the penetrating blood vessels which also have a vertical orientation. In this study, the effect of multiple subpial transection was assessed on both function and seizure control. The effect on function was reviewed in 32 cases; only 20 cases were evaluated with respect to seizure control, since a follow-up period of 5 years or more (5 to 22 years) is required before conclusions can be drawn. Multiple subpial transection was applied to the precentral gyrus in 16 cases, the postcentral gyrus in six, Broca's area in five, and Wernicke's area in five. With respect to function, the major finding was that none of the 32 patients has suffered a clinically significant behavioral deficit (although subtle deficits could be detected by careful neurological examination). Complete control of seizures has been obtained in 11 (55%) of the 20 cases evaluated. Nine patients developed recurrent seizures consequent to progressive disease unsuspected before operation (Rasmussen's encephalitis in five, tumor in three, and subacute sclerosing panencephalitis in one). In none of these cases, however, did the recurrent seizures arise in the transected zone. Thus, the results indicate that multiple subpial transection is about as effective as standard excisional therapy, and can be successfully employed when epileptogenic lesions encroach upon cortical territories, the removal of which would be functionally incapacitating.
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6/15. Echogenic vasculature in the basal ganglia of neonates: a sonographic sign of vasculopathy.

    The vessels that supply the basal ganglia and thalami are not normally conspicuous on the cranial sonograms of neonates. Twelve neonates with abnormally echogenic or "bright" vessels on cranial sonograms were studied. Records of these 12 patients were reviewed and were correlated with the neuropathologic findings available in four. The clinical diagnoses were cytomegalovirus infection (five patients), rubella (two patients), congenital syphilis (one patient), and trisomy 13 syndrome (three patients). No diagnosis was made in one infant. At neuropathologic examination, perforating medium-sized arteries to the basal ganglia and thalami had thickened hypercellular walls, with deposits of amorphous basophilic material in three infants. Results of computed tomography and radiography of brain sections were normal in these areas. Sonography is helpful in detecting early noncalcific inflammation and mineralization in vasculitis. Although nonspecific, these findings should alert the physician to the possibility of congenital infection or chromosomal abnormality.
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7/15. Focal perivascular alterations of white matter in herpes simplex encephalitis--a histological and immunocytochemical study.

    Several cases of human herpes simplex encephalitis treated with Vidarabin have been investigated with the histological and immunocytochemical techniques. Cases with a subacute evolution revealed areas of focal perivascular myelin destruction in the white matter. The distribution of herpes simplex antigen did not show any preferential localization of the virus in perivascular oligoglial cells. In contrast, a spatial and temporal relationship has been found between the appearance of immunoglobulin-bearing cells around the vessels and that of areas of focal perivascular myelin damage. Therefore, it is postulated that the areas of focal destruction of myelin are not related to the cytotoxic effect of the virus but are rather dependent on the immune response of the host.
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8/15. Varicella-zoster virus leukoencephalitis and cerebral vasculopathy.

    Two patients with varicella-zoster virus leukoencephalitis and acquired immunodeficiency syndrome are described. Neither patient had cutaneous or disseminated varicella-zoster virus infection within the last six months of life. Demyelinated lesions resembling those of progressive multifocal leukoencephalopathy were seen in their brains at autopsy. Numerous cells with Cowdry type A intranuclear inclusions surrounded the lesions; these cells stained positively for varicella-zoster virus with immunohistochemistry and contained herpesvirus nucleocapsids by electron microscopy. Leptomeningeal vessels accompanying the lesions displayed a zoster-induced vasculopathy in one of the two patients. Vascular and parenchymal central nervous system infections with varicella-zoster virus are rare in the absence of cutaneous lesions, and to our knowledge, the presence of both in one patient has not yet been described.
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9/15. Trauma, sport, and malignant cerebral edema.

    Sudden cerebral swelling and death secondary to craniocerebral trauma has been noted in children and young adults. This is due to an increase in intracerebral blood, either secondary to an increase in cerebral blood volume or a redistribution of intracranial blood from the pial to the intraparenchymal vessels. Sequential craniocerebral trauma has been associated with the syndrome of "malignant cerebral edema"; the possibility of a "compliance compromised brain" has been suggested as the cause. The additional possibility of asymptomatic encephalitis leading to a compliance compromised brain and malignant cerebral edema is discussed.
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10/15. Arterial bolus contrast medium enhancement for computed tomographically guided stereotactic biopsy.

    Stereotactic biopsy of deep-seated brain lesions by computed tomography guidance with intravenous contrast medium enhancement is now a well-recognized technique. However, arteriography continues to be the best method for studying the vasculature of such lesions. A method is described here in which a limited arteriography can be incorporated into the computed tomographic technique especially for the purpose of avoiding injury to vessels in stereotactic biopsy procedures. The technique requires that a single 4-mL bolus of contrast medium be injected intraarterially as the computed tomography scan for stereotactic localization is performed. The vessels are thereby seen in the same computed tomographic image as that used for stereotactic localization. The method has these additional advantages: it provides more pronounced contrast medium enhancement; it requires only 4 mL of contrast medium and can therefore be used in patients with renal failure; it can often distinguish crude arterial and venous phases; and lastly, it can be performed with routinely available equipment.
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