Teaching NeuroImages: Steroid-responsive leukoencephalopathy in inflammatory cerebral amyloid angiopathy¶
Summary¶
- 60yo F
- HPI
- –2w headache
- –1mo memory loss
- Ex
- MRI
- DSA / VZV PCR / CSF IgG / HBV, HCV / cryptococcal Ag, HIV, Lyme / syphilis (–)
- DDx
- CNS vasculitis
- intracerebral lymphoma
- Dx
- inframmatory cerebral amyloid angiopathy
- amyloid-β-related antiitis subtype
- Tx
- streoid
- Prog
- MoCA 23/30(+2w) → 22/30(+5mo)
Further¶
Terminology¶
Original¶
An obtunded previously healthy 60-year-old woman was intubated with a 2-week history of headaches and 1-month history of memory loss. MRI was performed (figure 1). Digital subtraction angiography of the head, varicella-zoster virus PCR, immunoglobulin G in the CSF, hepatitis B/C, CSF cryptococcal antigen, HIV, Lyme serology, and syphilis screen were negative. The initial differential diagnosis included CNS vasculitis and intracerebral lymphoma. She was diagnosed with [inflammatory cerebral amyloid angiopathy] based on clinicoradiologic criteria (which has a sensitivity of 82% and specificity of 97%),1 with infarction suggesting amyloid-β-related angiitis subtype. Within a few days of steroids, she was fully alert, and repeat MRI 5 months later showed improvement of leukoencephalopathy (figure 2). Montreal Cognitive Assessment scores were 23/30 2 weeks after initiation of therapy and 22/30 5 months later.
Figure 1¶
Initial MRI
(A) T2-weighted confluent leukoencephalopathy extending to the subcortical white matter with mass effect.
(B) Diffusion-weighted imaging 10 days later with associated changes on the apparent diffusion coefficient sequence (not pictured) was consistent with infarction.
(C) Gradient echo sequence revealed microhemorrhages.
Figure 2¶
Radiologic improvement after steroids Comparison between initial imaging (A, D), around 3 weeks after initiation of steroid therapy (B, E), and after 5 months of corticosteroid therapy (C, F) showed improvement of leukoencephalopathy.
