title: The characteristics of superficial siderosis and convexity subarachnoid hemorrhage and clinical relevance in suspected cerebral amyloid angiopathy.
abstract: OBJECTIVE
  Systematic studies of superficial siderosis (SS) and convexity subarachnoid hemorrhage (cSAH) in patients with suspected cerebral amyloid angiopathy (CAA) without lobar intracerebral hemorrhage (ICH) are lacking. We sought to determine the potential anatomic correlation between SS/cSAH and transient focal neurological episodes (TFNE) and whether SS/cSAH is predictor of future cerebral hemorrhagic events in these patients.
  
  
  METHODS
  We enrolled 90 consecutive patients with suspected CAA (due to the presence of strictly lobar microbleeds (CMBs) and/or SS/cSAH) but without the history of symptomatic lobar ICH who underwent brain MRI including T2*-weighted, diffusion-weighted imaging and fluid-attenuated inversion recovery sequences from an ongoing single center CAA cohort from 1998 to 2012. Evaluation of SS, cSAH and CMBs was performed. Medical records and follow-up information were obtained from prospective databases and medical charts. TFNE was defined according to published criteria and electroencephalogram reports were reviewed.
  
  
  RESULTS
  Forty-one patients (46%) presented with SS and/or cSAH. The prevalence of TFNE was significantly higher in those with SS/cSAH (61 vs. 10%; p < 0.001) and anatomically correlated with the location of cSAH, but not SS. The majority of TFNE in patients with SS/cSAH presented with spreading sensory symptoms. Intermittent focal slowing on electroencephalogram was present in the same area as SS/cSAH in 6 patients, but no epileptiform activity was found in any patients. Among those with available clinical follow-up (76/90 patients, 84%), ten patients with SS/cSAH (29%, median time from the scan for all patients with SS/cSAH: 21 months) had a symptomatic cerebral bleeding event on follow up (average time to events: 34 months) compared with only 1 event (2.4%, 25 months from the scan) in patients without SS/​cSAH (time to event: 25 months) (p = 0.001). The location of hemorrhages on follow-up scan was not in the same location of previously noted SS/cSAH in 9 of 10 patients. Follow-up imaging was obtained in 9 of 17 patients with cSAH and showed evidence of SS in the same location as initial cSAH in all these 9 cases.
  
  
  CONCLUSIONS
  SS/cSAH is common in patients with suspected CAA without lobar intracerebral hemorrhage and may have a significantly higher risk of future cerebral bleeding events, regardless of the severity of the baseline CMB burden. The findings further highlight a precise anatomical correlation between TFNE and cSAH, but not SS. Distinct from transient ischemic attack or seizure, the majority of TFNE caused by SS/cSAH appear to present with spreading sensory symptoms.
authors: The Department of Neurology, Peking Union Medical College Hospital, Peking, China.
authors: Jun
authors: J
authors: Ni
authors: Eitan
authors: E
authors: Auriel
authors: Jenelle
authors: J
authors: Jindal
authors: Alison
authors: A
authors: Ayres
authors: Kristin M
authors: KM
authors: Schwab
authors: Sergi
authors: S
authors: Martinez-Ramirez
authors: Edip M
authors: EM
authors: Gurol
authors: Steven M
authors: SM
authors: Greenberg
authors: Anand
authors: A
authors: Viswanathan
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D016657:Cerebral Amyloid Angiopathy / Q000150:complications / Q000453:epidemiology*; D002543:Cerebral Hemorrhage / Q000150:complications; D005260:Female; D006801:Humans; D002546:Ischemic Attack, Transient / Q000150:complications / Q000453:epidemiology*; D008279:Magnetic Resonance Imaging / Q000009:adverse effects; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D011446:Prospective Studies; D012306:Risk; D012806:Siderosis / Q000150:complications / Q000453:epidemiology*; D013345:Subarachnoid Hemorrhage / Q000150:complications / Q000453:epidemiology*