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Biomedical subjects

J Llena

Publications and source records attributed to J Llena.

26 records · Page 2Linked to original sources

Central nervous system pathology in pediatric AIDS: an autopsy study.

The neuropathologic findings of brains and spinal cords removed at autopsy from 26 infants and children with AIDS is described; in two cases, only the spinal cords were available. The most common finding in the brains was dystrophic calcification of blood vessels of all calibers in the basal ganglia and deep cerebral white matter (21 og 24 cases). The next most frequent finding was subacute encephalitis (SE) (15 of 24 cases) with microglial nodules and multinulceated giant cells. Immunocytochemical and in situ hybridization studies showed HIV antigen or genetic sequences only in the brains of cases with SE. Multinucleated giants cells (MGC) were the most frequent cells with reaction products. MGC were labeled with ricinus lectin (RCA), but not with leukocyte common antigen (LCA) or glial fibrillary acidic protein. Many cells in microglial nodules were labeled with RCA, but not LCA; cells in the perivascular compartment were labeled with LCA, but not RCA. Corticospinal tract degeneration was noted in 15 of 20 spinal cords. In six cases tract degeneration was consistent with delayed myelination, and the remaining cases had axonal injury consistent with Wallerian degeneration. Opportunistic infections were rare (three cases). Central nervous system lymphoma occurred in three children and was the most common mass lesion. In two cases lymphoma occurred in the setting of a systemic polyclonal immunoproliferation possibly related to Epstein-Barr virus infection. Cerebrovascular accidents were noted in seven cases. Two cases had hemorrhage associated with immune thrombocytopenia; one hemorrhage was catastrophic. Two children had large vessel arteriopathy with multiple encephalomalacias. Two children had a necrotizing encephalopathy with encephalomalacia and vascular changes suggestive of a mitochondrial cytopathy.(ABSTRACT TRUNCATED AT 250 WORDS)

Acquired Immunodeficiency Syndrome↗

Pediatric acquired immunodeficiency syndrome. Neurologic syndromes.

Central nervous system (CNS) dysfunction was documented in 61 of 68 infants and children with symptomatic human immunodeficiency virus infection. The most frequent manifestations included acquired microcephaly, cognitive deficits, and bilateral pyramidal tract signs. Lymphoma of the CNS, cerebrovascular accidents, and CNS infection caused by conventional pathogens were documented in only ten children (15%). Neurologic deterioration in 11 children was subacute but steadily progressive; in 31 the course was more indolent and began with a plateau. Of these 31 children, 13 had further neurologic deterioration and the conditions of three improved. Seventeen children had a static course with cognitive deficits (seven children) or cognitive plus neurologic impairment (ten children). Neuroradiologic studies in the children with a subacute progressive or plateau course disclosed cerebral atrophy, white matter abnormalities, and calcification of the basal ganglia. Postmortem findings included variable degrees of inflammatory response, multinucleated cells, calcific vasculopathy, and pyramidal tract degeneration. Computed tomographic studies of the children with a static course were normal or showed mild atrophy, but poor brain growth was documented by serial head circumference measurements.

Acquired Immunodeficiency Syndrome↗

Multifocal cytomegalovirus encephalitis in AIDS.

A 43-year-old man with the acquired immunodeficiency syndrome had clinical evidence of multifocal disease of the brain, but computed tomography was negative. Magnetic resonance imaging revealed multifocal lesions, histologically proven to be caused by cytomegalovirus. Therapy with 9[2-hydroxy-1-(hydroxymethyl) ethoxymethyl] guanine (BW B759U) resulted in stabilization of the patient's clinical disease and radiographic improvement of the lesions.

Acquired Immunodeficiency Syndrome↗

Venous malformation mimicking a posterior fossa tumor in a young child.

Symptomatic venous malformations are uncommon at any age. They are extremely rare in the first decade of life. We describe a 3.5-year-old child with a cerebellar venous malformation presenting with a subacute course mimicking the more common posterior fossa tumors. The child's symptoms were due to a posterior fossa hemorrhage; she subsequently did well. The clinical, CT, angiographic, and pathologic features are described. Venous malformations should be included in the differential diagnosis of young children with evidence of posterior fossa disease.

Cerebellar Neoplasms↗

Progressive multifocal leukoencephalopathy: clinical and radiographic features.

Between April 1982 and March 1984 7 pathologically confirmed cases of progressive multifocal leukoencephalopathy (PML) were diagnosed at our institution. Only 1 case had been seen in the preceding twenty years. Four patients had acquired immunodeficiency syndrome (AIDS). The others had chronic lymphocytic leukemia, Hodgkin's lymphoma, and systemic lupus erythematosus. All patients presented with progressive neurological deficits. In most, the initial computed tomographic (CT) scan was disproportionately less abnormal than the clinical findings. In 5 patients the first CT scan revealed hypodensities of the cerebral white matter which lacked mass effect and did not enhance with contrast agent. The lesions were observed to enlarge progressively on CT scans but often lagged behind the rate of clinical evolution. Although 3 patients were treated with cytosine arabinoside, none improved. PML had similar clinical, radiographic, and pathological features in the AIDS and non-AIDs patients. Of 79 AIDS patients cared for at our institution between December 1979 and December 1983, 3.8% had PML. PML should be suspected in AIDS patients in the presence of the characteristic CT features, especially when CT-clinical dissociation occurs.

Acquired Immunodeficiency Syndrome↗

Ultrastructural study of early stage of calcification in herpes simplex encephalitis.

A case of herpes simplex encephalitis was investigated at the ultrastructural level. Herpes simplex virions were observed, both in nuclei and perikarya of degenerated cells in the cerebrum. Needle-shaped mineralized precipitates were also present in the disintegrated cells. These mineralized depositions were confined to mitochondria. This finding suggests that mitochondria serve as initial nidus of calcification in herpes simplex encephalitis.

Adult↗

Sleep apnea studies in an infant with congenital primary hypoventilation ("Ondine's curse").

Recurrent apneic episodes were typically associated with sleep, not wakefulness, in an infant with congenital primary hypoventilation ("Ondine's Curse"). Quiet sleep (SLQ) was shown to constitute a higher risk condition than active sleep (SLA) at the ages she was recorded polygraphically (2-4 months old). This infant's respiratory disorder was complicated by recurrent pneumonia, seizures and deficient growth which resulted in death at the age of eight months. Necropsy revealed bronchopulmonary dysplastic fibrosis and cor pulmonale. Neuropathologic examination failed to reveal pathologic changes in the brainstem.

Autonomic Nervous System Diseases↗

Cavernous haemangioma in the pineal region.

The authors report a case of a cavernous haemangioma in the pineal region which presented as a progressively enlarging mass lesion. Clinical, radiographic and pathologic features are briefly described. Total removal was achieved using the supracerebellar, infratentorial approach.

Brain↗