Spontaneous progressive multifocal leukoencephalopathy

Fermaglich, Joseph; Hardman, John; Earle, Kenneth M.

doi:10.1212/wnl.20.5.479

Cited by 46 publications

(15 citation statements)

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“…PML without associated immunosuppression is rare. Although there have been some cases reported as early as the 1970s, it is unclear whether those patients underwent a thorough workup for immunosuppression (Bolton and Rozdilsky 1971;Fermaglich et al 1970). In addition, there is a greater recognition of novel clinical presentations of JCV infection, such as JCV encephalopathy, JCV granule cell neuronopathy, and JCV meningitis (Tan and Koralnik 2010), which makes the diagnosis of neurological syndromes associated with JC virus more plausible in patients, regardless of the immune status.…”

Section: Introductionmentioning

confidence: 99%

Progressive multifocal leukoencephalopathy in a patient without apparent immunosuppression

et al. 2016

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Progressive multifocal leukoencephalopathy (PML) is a viral demyelinating disease due to the reactivation of the JC virus (JCV), which usually occurs in the context of immunosuppression in HIV infection, malignancy, or in patients on disease modifying therapy for autoimmune diseases, such as multiple sclerosis (MS) and Crohn's disease. Notably, there is growing recognition that PML can occur in patients with transient immune dysfunction. Here, we present a case of a 55-year-old man without history of immunosuppression or evidence of ICL who was diagnosed with PML on brain biopsy. We will discuss the potential etiologies of mild and transient immunosuppression that can lead to PML with non-apparent immunosuppression.

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Section: Introductionmentioning

confidence: 99%

Progressive multifocal leukoencephalopathy in a patient without apparent immunosuppression

et al. 2016

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“…However, there have been only a very small number of PML cases in patients without underlying disease or remarkable immunodeficiency 1,16, 18,24–26 . Such PML cases have been reported as ‘primary PML’ 18,25, 26 or ‘spontaneous PML’ 24 . In the present case there was no underlying disease to cause immunodeficiency such as lymphoproliferative disease, organ transplantation, chemotherapy for malignacy or HIV infection.…”

Section: Discussionmentioning

confidence: 54%

Autopsy case of the cerebellar form of progressive multifocal leukoencephalopathy without immunodeficiency

Arai¹,

Tsutsui²,

Nagashima³

et al. 2002

Neuropathology

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A case of the cerebellar form of progressive multifocal leukoencephalopathy (PML) without remarkable immune depression or immune deficiency is reported here. The patient was a 74-year-old-woman who had complications of chronic renal failure and renal anemia for several years. Seven months before her death she had symptoms of general fatigue, gait disturbance and articulation disorder. During her hospitalization period her neurological disorder gradually progressed irreversibly with failure of consciousness and she died of respiratory failure. She did not have remarkable clinical signs of immunodeficiency nor did she receive immunosuppressive therapy. Clinically she had not been diagnosed with PML. At the post-mortem examination different degrees of demyelination were observed in the brain white matter: diffuse and severe in the cerebellum, moderate and coalescent in the brainstem, and light and patchy in the cerebrum. JC virus antigen-positive cells were frequently observed in the demyelinated lesions in the cerebrum and sometimes observed in the brainstem, but were rarely found in the cerebellum. These findings suggest that PML lesions may be present with different degrees of demyelination that are inversely correlated with the number of JC virus-infected cells. This fact should be considered when evaluating the brain biopsies of PML patients.

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“…Although rare, there have been a few reported cases in which PML has occurred in patients without an immunosuppressive state. Such PML cases have been reported as ‘primary PML’ 1,2 or ‘spontaneous PML’ 3 . The present case belongs to this rare group.…”

Section: Discussionmentioning

confidence: 63%

“…Such PML cases have been reported as 'primary PML' 1,2 or 'spontaneous PML'. 3 The present case belongs to this rare group.…”

Section: Discussionmentioning

confidence: 67%

A 64‐year‐old man presenting MRIs of expansive multiple high intensity areas in the cerebral white matter, cerebellum and brainstem

Takeda

Yamazaki²,

Miyakawa³

et al. 2007

Neuropathology

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A 64-year-old man, who had been suspected as a hepatitis carrier 25 years ago and also was detected with a decrease of leukocytes and lymphocytes in the previous year, visited our hospital on 31 January, because of vertigo. Neurological status revealed normal findings. Blood cell count disclosed 4200 WBCs with a differential count of 66% segmented neutrophils, 8% non-segmented neutrophils, 10% lymphocytes, 14% monocytes, 1% eosinophiles and 1% basophiles. HBs Ag and HCV Ag were negative. T2-weighted brain MRI showed small high intensity areas (HIAs) in the left occipital white matter (Fig. 1a) and the midbrain tegmentum. He was admitted for 20 days as having cerebral infarcts. Anticoagulant therapy was effective. He was readmitted because of paralysis of the left leg 2 months after discharge. MRI showed a new HIA in the convolutional white matter of the right motor area. Anticoagulant therapy and rehabilitation were effective. He was admitted at the 3rd time on 3 July, because of worsened weakness of the left leg. On MRI examination HIA in the motor area spread and a small HIA was revealed in the right frontal white matter. Left hemiparesis was progressive. Mental retardation, visual disturbance and emotional instability developed. In September he was suspected as having vascular dementia or some degenerative condition in the cerebral white matter as well as cerebral infarcts. MRI examination revealed widespread HIAs in the brainstem, cerebellar white matter, thalamus and cerebral white matter (Fig. 1b). He died in December. The duration of the present illness was 11 months after his first visit to our hospital. PATHOLOGICAL FINDINGSPostmortem examination, performed 2 h after death, revealed no malignancy, atrophy of the adrenal gland, nor thrombi on the aortic valves. Atherosclerosis was mild.The fresh brain weighed 1150 g. Sections of the fixed brain revealed subcortical grayish discoloration of the cerebral white matter (Fig. 2a). The cerebral arteries including Willis circle were delicate. KB preparation showed extensive loss of myelin sheaths in widespread areas in the bilateral cerebral hemispheres (Fig. 2b). The lateral angle of the lateral ventricle and optic tract were relatively or well preserved. In the extensive lesions of the cerebral white matter, macrophage infiltration and astrocytic gliosis were seen in variable degrees. In the margin of the diffusely affected white matter, multiple small lesions, which stained pale in KB preparation, were seen (Fig. 2b). These small lesions were confluent in part. The arcuate fibers were mostly involved. The intracortical myelinated fibers were also affected. In fresh lesions, the axons were well preserved in contrast to the marked loss of myelin sheaths (Fig. 2c,d). In older lesions, the myelin sheaths and axons disappeared. The cortical neurons were relatively preserved. The same demyelinated lesions were seen in the basal ganglia and thalamus.The extensive demyelinated lesions as seen in the cerebrum were also seen in the brainstem, cerebellar white matter an...

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Spontaneous progressive multifocal leukoencephalopathy

Cited by 46 publications

References 0 publications

Progressive multifocal leukoencephalopathy in a patient without apparent immunosuppression

Progressive multifocal leukoencephalopathy in a patient without apparent immunosuppression

Autopsy case of the cerebellar form of progressive multifocal leukoencephalopathy without immunodeficiency

A 64‐year‐old man presenting MRIs of expansive multiple high intensity areas in the cerebral white matter, cerebellum and brainstem

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