Progressive multifocal leukoencephalopathy: Clinical and radiographic features

Abstract

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.

Viral variant nucleotide sequences help expose leukocytic positioning in the JC virus pathway to the CNS

“…With the outbreak of the AIDS pandemic the number of PML cases began to rise with reports demonstrating that approximately 4% of AIDS patients developed PML [30,31]. In general, AIDS as well as non-AIDS patients diagnosed with PML demonstrated stable levels of preexisting immunoglobulin G (IgG) antibodies to JCV [5].…”

Progressive multifocal leukoencephalopathy in rheumatic diseases: Evolving clinical and pathologic patterns of disease

“…PML has been described mainly in patients infected with the human immunodeficiency virus (HIV). However, other immunosuppressed patient populations, including those with malignancies, organ transplants, and systemic lupus erythematosus (SLE) (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19)(20) and other rheumatic diseases (22)(23)(24)(25)(26)(27)(28)(29)(30)(31)(32), are at risk of developing this complication.…”

Detecção do DNA do Poliomavírus Humano JC em amostras de líquido cefalorraquidiano de pacientes com AIDS e lesões não expansivas de substância branca do sistema nervoso central

“…No início do quadro da LEMP, as imagens cerebrais obtidas através da tomografia computadorizada revelam alterações sutis, desproporcionais aos sinais e sintomas clínicos, caracterizando a dissociação clinicoradiológica (KRUPP et al, 1985). A alteração clinico-radiológica é uma característica não específica da apresentação da LEMP.…”

“…A alteração clinico-radiológica é uma característica não específica da apresentação da LEMP. Talvez isso ocorra porque pequenos focos de desmielinização produzem sintomas clínicos precoces, aparecendo na tomografia computadorizada de crânio apenas quando se tornam confluentes e mais intensos já com comprometimento axonal (KRUPP et al, 1985).…”

“…Na maioria dos casos ocorrem múltiplas lesões no sistema nervoso central, sendo que 10% dos casos apresentam uma única lesão cerebelar (KRUPP et al, 1985).…”