Disappearing Leukoencephalopathy
Chan, Amy M; Huttner, Anita; Baehring, Joachim. Disappearing Leukoencephalopathy, Clinical Neuroradiology; Heidelberg (Dec 2017): 1-5. DOI:10.1007/s00062-017-0650-4
View Original Source →Case Details
Disease Location
Intravascular left frontal white matter
Personal Characteristics
74 -year-old male no significant family history geologist
Clinical Characteristics
Was abeing seen in neuro-oncological consultation for 2 years of episodic right hemiparesis, speech difficulties, and confusion the sentinel event was a short episode of right hand clumsiness, dysarthria and confusion. He could not understand a menu to order food he received a left carotid endarterectomy for a 70% carotid artery stenosis and presumed transient ischemic attack 1 year postop, he started to have recurrent fleeting episodes of right hand dysesthesia and spasm every few months, there were more extensive episodes of dysphasia and confusion lasting for days. Subtle cognitive impairment wavered. He periodically had difficulty aligning maps while working as a geologist MRI surrounding episodes of attack showed multifocal progressive fluid-attenuated inversion recovery t2-sequences hyperintensities mostly involving the left frontal subcortical white matter the largest lesion had subtle slowed diffusion and a small linear focus of enhancement there was mild elevated protein level in the csf there was then a 2 month MRI follow-up given his improvement, he was started on corticosteroids to abate a possible lymphoproliferative process. On steroid taper, he re-presented with epilepsia partialis continua, spastic right hemiparesis and episodes of unresponsiveness repeat flair imaging showed relapse of previous abnormalities and mulifocal new flair abnormalities with subtle enhancement and ischemic changes intravascular diffuse large b-cell lymphoma was finally diagnosed with a left frontal brain biopsy he then started r-mpv treatment
Remission Characteristics
The 2 months MRI follow-up during a period of good neurologic status showed marked regression of the multifocal areas of flair abnormality. There were no medication changes neurologic exam confirmed fluent language, intact delayed recall, serial-7 and complex pattern copying there was an asymptomatic year after the initial transient ischemic attack-like event, in the 2nd year there was clinical and significant radiographic regression in between episodic attacks without interventions
Treatment & Mechanisms
Proposed Remission Mechanisms
Possibly from host immunologic responses
Clinical Treatment
Corticosteroids r-mpv (rituximab, high-dose methotrexate-procarbazine-vincristine)
Non-Clinical Treatment
None reported