Presentation
A typical bilateral asymmetric pattern in conventional magnetic resonance imaging is present in the majority of the patients. [ncbi.nlm.nih.gov]
43-year-old man who presented with tremors and dystonia. [ajronline.org]
Fluid-attenuated inversion recovery (FLAIR) signal abnormalities are not present elsewhere. [bloodadvances.org]
Main laboratory and serological tests The present report adds to the list of unusual viral infections in patients treated with high-dose chemotherapy and rituximab. [bloodjournal.org]
They presented with failure to thrive, pulmonary hypertension, and neurological regression. [frontiersin.org]
Entire Body System
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Falling
(A) Initial magnetic resonance imaging performed 2 months after the onset of headaches and balance disturbance (gait instability and repeated falls) depicting the asymmetric atrophy of the left cerebellum, which was not present in imaging performed 7 [bloodadvances.org]
Workup
Other Pathologies
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White Matter Lesions
Cerebral white matter lesions equal or over 3 mm in diameter were considered abnormal. The spinal cord size was evaluated using an index we have called "spinal cord index". [scielo.br]
Other clinical manifestations are visual impairment from vascular retinopathy and neurological decline and premature death due to progressive enhancing cerebral white matter lesions. [ichd-3.org]
matter lesions usually enhance CNS manifestations of AIDS [radiopaedia.org]
Etiology of cortical and white matter lesions in cyclosporin A and FK-506 neurotoxicity. Am J Neuroradiol 2001; 22:1901-1914 [Google Scholar] 48. Bartynski WS, Boardman JF. [ajronline.org]
Treatment
Median survival from PML diagnosis was 4.3 months for the 11 patients on treatment and 0.87 months for the 5 without treatment. PML still occurs in patients with hematologic malignancies in the absence of treatment. [bloodadvances.org]
Two additional weekly doses of rituximab, 375 mg/m2, ended the treatment. [bloodjournal.org]
Treatment with highly active antiretroviral therapy (HAART) may prolong survival, although in some instances this leads to PML-IRIS. High-dose glucocorticoid therapy is recommended for patients with PML-IRIS. [radiopaedia.org]
Progressive multifocal leukoencephalopathy-immune reconstitution inflammatory syndrome presents with different imaging characteristics from progressive multifocal leukoencephalopathy and treatment with steroids can improve survival. [ncbi.nlm.nih.gov]
Prognosis
Prognosis is generally poor with an inexorable neurological decline leading to coma and death occurring in the majority of patients with PML 9. [radiopaedia.org]
Its course may be rapidly progressive, although immunological responsiveness can be associated with an improved prognosis. [ncbi.nlm.nih.gov]
MR imaging and prognosis of hypoxic-ischemic leukoencephalopathy. Neurocrit Care 2006; 4:119-126 [Google Scholar] 17. Roychowdhury S, Maldjian JA, Galetta SL, et al. Postanoxic encephalopathy: diffusion MR findings. [ajronline.org]
Etiology
The precise pathogenesis of infection by JC virus, the etiologic human papovavirus, remains elusive, but much has been learned since the original description of the pathologic entity PML in 1958. [journals.lww.com]
Etiology PML lesions are secondary to progressive demyelination due to John Cunningham (JC) virus (usually reactivation) which infects oligodendrocytes. [radiopaedia.org]
The viral etiology of this condition was not recognized for many years, at which time the inherent immune dysfunction associated with these neoplasms was appreciated as a necessary prerequisite for the full-blown syndrome. [bloodadvances.org]
Etiology of cortical and white matter lesions in cyclosporin A and FK-506 neurotoxicity. Am J Neuroradiol 2001; 22:1901-1914 [Google Scholar] 48. Bartynski WS, Boardman JF. [ajronline.org]
Pathophysiology
Contribution to the pathophysiology of the disease. J. Inherit. Metab. Dis. 36, 841–847. doi: 10.1007/s10545-012-9565-z Pubmed Abstract Pubmed Full Text CrossRef Full Text Google Scholar Kashani, A. L., Thiffault, I., Dilenge, M. [frontiersin.org]
The basic pathophysiologic mechanisms leading to acute toxic leukoencephalopathy and reduced diffusion are unknown but are likely multifactorial. [ajronline.org]
Prevention
[…] to optimal prevention, care, and treatment, more so in middle and low income economies. [newdailyherald.com]
This warrants investigation in the area of hematologic cancers, as prevention is the only approach currently used to avoid the disability and mortality associated with reactivation of JCV in the nervous system. [bloodadvances.org]
CONCLUSION Maintenance immunosuppressive therapy is administered to all renal transplant recipients to help prevent acute rejection and the loss of the renal allograft. [scielo.mec.pt]
This observation underscores the importance of recognizing even mild or early cases of this disorder in order to prevent progression to a severe outcome. [ajronline.org]