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
- Asymptomatic
Primary infection occurs in childhood and it is asymptomatic in most patients. [semanticscholar.org]
Radiol 2017 Jun;90(1074):20160721 • Sarbu N, Shih RY, Jones RV, Horkayne Szakaly I, Oleaga L, Smirniotopoulos JG White Matter Diseases with Radiologic Pathologic Correlation Radiographics 2016 Sep Oct;36(5):1426-47 • Hodel J, Outteryck O, Dubron C, et al Asymptomatic [piper.espacio-seram.com]
On day +20 from the end of treatment, the patient developed an asymptomatic CMV infection, documented by a positive pp65 assay (3 and 4 positive nuclei/200 000 cells), and was treated with gancyclovir in our outpatient department until negativization [bloodjournal.org]
The patient with diffuse large B cell lymphoma was asymptomatic. Neither had imaging results suggestive of a demyelinating process. Detection of JCV DNA by PCR demonstrated only low titers, and results of repeat testing within 1 month were negative. [bloodadvances.org]
- Epilepsy
Lipoic acid synthetase deficiency causes neonatal-onset epilepsy, defective mitochondrial energy metabolism, and glycine elevation. Am. J. Hum. [frontiersin.org]
Respiratoric
- Cough
The patient was admitted with a 7-day history of fever, productive cough, anorexia and right hand altered mobility and sensibility. [scielo.mec.pt]
Neurologic
- Confusion
CONCLUSIONS: HSLE is usually underdiagnosed since it is often confused with a neuropsychiatric or behavioral disorder associated with the consumption of psychoactive substances (PAS). [pesquisa.bvsalud.org]
Second, this syndrome may present with rather nonspecific but severe symptoms and can be confused clinically with other often irreversible syndromes (e.g., HIE); hence, the window for effective treatment may be missed. [ajronline.org]
- Psychomotor Regression
Psychomotor development was referred normal until 7 months of age. During a mild febrile illness, he presented severe and rapid psychomotor regression, loosing sitting position and smiling, and developed spastic tetraparesis. [frontiersin.org]
- Aura
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]
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]