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
- 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]
- Epilepsy
Lipoic acid synthetase deficiency causes neonatal-onset epilepsy, defective mitochondrial energy metabolism, and glycine elevation. Am. J. Hum. [frontiersin.org]
Cardiovascular
- Hypertension
Hypertensive leukoencephalopathy The classification of leukoencephalopathies is a matter of debate. Some authors divide leukoencephalopathies into hereditary disorders and acquired disorders. [en.wikipedia.org]
The clinical spectrum also includes impaired liver and kidney function, anaemia sometimes associated with gastrointestinal bleeding and hypertension. [ichd-3.org]
They presented with failure to thrive, pulmonary hypertension, and neurological regression. [frontiersin.org]
HIV/AIDS encephalopathy: often diffuse white matter disease, with atrophy posterior reversible encephalopathy syndrome (PRES) differing clinical history (e.g. hypertension) can involve both grey and white matter acute disseminated encephalomyelitis (ADEM [radiopaedia.org]
[…] of periventricular white matter could have an appearance shared by both hypertension and immunosuppressive medications. [ajronline.org]
Eyes
- Visual Impairment
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]
Neurologic
- Encephalopathy
Several factors enable distinction of this entity from posterior reversible encephalopathy syndrome—most notably, posterior reversible encephalopathy syndrome typically affects the cortex or subcortical white matter on FLAIR; the periventricular white [ajronline.org]
HIV/AIDS encephalopathy: often diffuse white matter disease, with atrophy posterior reversible encephalopathy syndrome (PRES) differing clinical history (e.g. hypertension) can involve both grey and white matter acute disseminated encephalomyelitis (ADEM [radiopaedia.org]
Key-Words: Immunosuppressants; kidney transplant; posterior reversible encephalopathy syndrome. [scielo.mec.pt]
Mutation of the iron-sulfur cluster assembly gene IBA57 causes severe myopathy and encephalopathy. Hum. Mol. [frontiersin.org]
The earliest onset cases are associated with oligohydramnios, IUGR, microcephaly, contractures and severe encephalopathy. [preventiongenetics.com]
- Headache
Description: Headache recurring as migraine-like attacks, mainly without aura, caused by the syndrome of retinal vasculopathy with cerebral leukoencephalopathy and systemic manifestations (RVCLSM). [ichd-3.org]
(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]
The clinical syndrome is characterized by headaches, altered consciousness, visual disturbances and seizures, the latter often being the presenting manifestation in a generalized tonic clonic way. [scielo.mec.pt]
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]
Citas • C McNamara G Sugrue B Murray, and P J MacMahon Current and Emerging Therapies in Multiple Sclerosis Implications for the Radiologist Part 2 Surveillance for Treatment Complications and Disease Progression American Journal of Neuroradiology Sep [piper.espacio-seram.com]
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]
Statistics on heroin use must be considered when approaching a patient with a history of PAS use and neurological signs, to relate them to this etiology and provide comprehensive management to these patients. [pesquisa.bvsalud.org]
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]