Atypical acute hemorrhagic leukoencephalitis (Hurst's disease) presenting with focal hemorrhagic brainstem lesion.
BACKGROUND
Acute hemorrhagic leukoencephalitis (AHL; Hurst's disease) is a rare, severe, inflammatory CNS disease that is typically diffuse, multifocal and associated with petechial hemorrhage. The objective of this study is to report the clinical, radiologic, and pathologic findings in a fatal AHL case with focal brainstem involvement and gross hemorrhage.


METHODS
Patient evaluation in a tertiary neurointensive care unit with serial brain magnetic resonance imaging (MRI) and neuropathological examination on autopsy were performed.


RESULTS
The patient presented with mild, then rapidly worsening, brainstem impairment to a locked-in syndrome. Brain MRI demonstrated an isolated gadolinium enhancing brainstem lesion that enlarged dramatically over weeks and was associated with gross hemorrhage and necrosis. The patient died despite aggressive treatment with intravenous corticosteroids and plasma exchange. Autopsy demonstrated the isolated severe necrotic lesion consistent with AHL.


CONCLUSIONS
AHL may present as a solitary brainstem lesion with gross hemorrhage and should be considered in patients with isolated enhancing brainstem lesions. AHL may be fatal even despite early, aggressive immunomodulatory therapy.
Department of Neurology, Mayo Clinic College of Medicine, 200 First Street SW, Rochester, MN 55905, USA.
Nuhad E
NE
Abou Zeid
Joseph D
JD
Burns
Eelco F M
EF
Wijdicks
Caterina
C
Giannini
B Mark
BM
Keegan
D001929:Brain Edema / Q000175:diagnosis / Q000473:pathology; D001933:Brain Stem / Q000473:pathology*; D018450:Disease Progression; D017809:Fatal Outcome; D006801:Humans; D004684:Leukoencephalitis, Acute Hemorrhagic / Q000175:diagnosis* / Q000473:pathology; D008279:Magnetic Resonance Imaging; D008297:Male; D009336:Necrosis; D009460:Neurologic Examination; D011149:Pons / Q000473:pathology; D055815:Young Adult