Recurrent craniospinal subarachnoid hemorrhage in cerebral amyloid angiopathy
A 48-year-old female presented with sudden onset of loss of consciousness and left hemiplegia in July 2007. Computed tomography (CT) of the brain showed right frontotemporal ICH with SAH in the right sylvian fissure and temporal region []. The digital subtraction angiogram (DSA) was normal. Her sensorium continued to fluctuate. CT brain done after 10 days showed SAH in the parietal, frontal and temporal regions []. Magnetic resonance imaging (MRI) of the brain showed subacute right frontal hemorrhage extending from the sylvian fissure [] and sulcal SAH at the vertex bilaterally. Superficial cortical hemosiderosis was seen in the parasagittal region on both sides []. She gradually improved with residual deficits (word finding difficulty and left facial paresis). Repeat DSA after 6 weeks was unremarkable. In October 2007, she had sudden onset numbness/weakness in the right faciobrachial region lasting 15 min. MRI brain showed subacute hemorrhages in the right posterior frontal and left anterior frontal regions. In December 2007, she had an episode of giddiness lasting 10 min. CT brain showed persisting subacute blood in the old lesions and ventricular dilation. Since then, there was progressive slowing of psychomotor activity and cognitive decline. In December 2008, she presented with altered sensorium and parapresis. MRI spine showed subacute hemorrhages, intrathecal at the S1-2 level, along the anterior surface of the cord at the L1-L2 level and along the filum terminale at the L4-S1 level []. MRI brain showed bifrontal and right temporal gliotic areas with superficial hemosiderosis []. Nodular enhancement was seen along the surface of the brain stem, cerebellum and spinal cord. In May 2009, she came with progressive altered sensorium. MRI brain showed ventricular dilatation with IVH in the occipital horns []. During the course of her illness, she was evaluated for underlying bleeding diathesis, connective tissue disorders, malignancy and vascular lesions, which were negative. Subsequent brain CT/MRI scans showed evidence of recurrent convexity SAHs in different locations [] with progressive superficial hemosiderosis []. Right frontal lobe biopsy showed thickened small-/medium-sized meningeal vessels, containing Congo red positive, birefringent, immunofluorescent amyloid material within the tunica media and adventitia []. Many vessels showed concentric separation of their walls (lumen in a lumen appearance). There was reactive gliosis in the white matter with normal parenchymal vessels. Features were consistent with CAA. She continued to worsen with declining sensorium and recurrent infections, and expired in May 2009. The features were of probable CAA with supporting pathology.