Abstract
A 56-year-old man noticed discomfort in his left lower limb, followed by convulsion and numbness in the same area. Magnetic resonance imaging (MRI) showed white matter lesions in the right parietal lobe accompanied by leptomeningeal or leptomeningeal and cortical post-contrast enhancement along the parietal sulci. The patient also exhibited higher brain dysfunction corresponding with the lesions on MRI. Histological pathology disclosed β-amyloid in the blood vessels and perivascular inflammation, which highlights the diagnosis of cerebral amyloid angiopathy (CAA)-related inflammation. Pulse steroid therapy was so effective that clinical and radiological findings immediately improved.CAA-related inflammation is a rare disease, defined by the deposition of amyloid proteins within the leptomeningeal and cortical arteries associated with vasculitis or perivasculitis. Here we report a patient with CAA-related inflammation who showed higher brain dysfunction that improved with steroid therapy. In cases with atypical radiological lesions like our case, cerebral biopsy with histological confirmation remains necessary for an accurate diagnosis.
Highlights
Cerebral amyloid angiopathy (CAA) is a common pathology in the elderly characterized by the deposition of amyloid proteins within the leptomeningeal and cortical arteries [1]
A subset of patients who presented with seizures, subacute cognitive decline, or headaches with hyperintensities on T2-weighted or fluid attenuated inversion-recovery (FLAIR) Magnetic resonance imaging (MRI) images with microhemorrhages were described as having CAA-related inflammation [2,3]
A possible explanation is that the inflammation caused by the immunoreactivity to amyloid might precede the vascular change of cerebellar amyloid angiopathy in some cases, such that microhemorrhages were not observed in radiological exams
Summary
Cerebral amyloid angiopathy (CAA) is a common pathology in the elderly characterized by the deposition of amyloid proteins within the leptomeningeal and cortical arteries [1]. We report a patient with CAA-related inflammation who showed convulsion in the left lower extremity and higher brain. Because a follow-up MRI revealed progression of the white matter lesions and parenchymal enhanced lesions without microhemorrhages (GRE-T2* imaging; 3T) (Figure 1C-G), a brain biopsy was performed in March 2010. Histological pathology showed nonspecific meningoencephalitis involving perivasculitis of the leptomeninges and cortical gray matter (Figure 2A-D). Neuropsychological tests of higher brain functions revealed mild constructional apraxia, line imbalance for words and numbers, difficulty drawing a figure following oral instructions, and problems with visual reproduction. 2 weeks later, the lesions had relapsed on a follow-up MRI, no clinical signs were observed. We performed pulse steroid therapy again, followed by oral methylprednisolone therapy (70 mg/day). After the oral steroid therapy was initiated, no relapses were observed either clinically or radiologically. The oral steroid was tapered at a rate of 5 mg/week, and he was discharged on a regimen of methylprednisolone 30 mg/day
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