Shervin Taslimi1, Jerry C Ku1, Amirhossein Modabbernia2, R Loch Macdonald3. 1. Division of Neurosurgery, Department of Surgery, University of Toronto, Toronto, Ontario, Canada. 2. Department of Psychiatry, Icahn School of Medicine at Mount Sinai Hospital, New York, New York, USA. 3. Division of Neurosurgery, Department of Surgery, University of Toronto, Toronto, Ontario, Canada; Division of Neurosurgery, Labatt Family Centre of Excellence in Brain Injury and Trauma Research, Keenan Research Centre for Biomedical Science, and Li Ka Shing Knowledge Institute, St. Michael's Hospital, Toronto, Ontario, Canada. Electronic address: macdonaldlo@smh.ca.
Abstract
BACKGROUND: Cerebral cavernous malformations (CCMs) may be familial or nonfamilial. This systematic review compared the natural history of CCMs in familial compared with nonfamilial cases. METHODS: We searched MEDLINE, Web of Science, and EMBASE for natural history studies on CCMs up to September 2018. We included studies that followed at least 20 untreated patients. Primary outcomes were hemorrhage, seizures, and neuroimaging changes in familial and nonfamilial cases. Incidence rate per person-year (PY) or lesion-year (LY) of follow-up were used to pool the data using fixed-effects or random-effects models. We used the incidence rate ratio for comparison. RESULTS: We could not compare hemorrhage rates between familial and nonfamilial cases mainly owing to mixtures of subgroups of patients. The seizure rate was similar in familial and nonfamilial cases with pooled incidence rate of 1.5%/PY (95% confidence interval 1.1%-2.2%). The reseizure rate was higher than the seizure rate (P < 0.001). New lesion development was higher in familial cases (32.1%/PY vs. 0.7%/PY, P < 0.001). Signal change on neuroimaging ranged from 0.2%/LY to 2.4%/LY in familial cases. In familial cases, incidence rate of size change was 8%/PY (95% confidence interval 5.2%-12.2%) and 1.1%/LY (95% confidence interval 0.6%-1.6%). CONCLUSIONS: Familial CCMs show higher dynamic changes than nonfamilial cases. However, the presence of actual dynamic changes needs further assessment in nonfamilial cases. CCMs demonstrate a low incidence of seizure. First-time seizure increases the chance of recurrent seizure. Seizure rate based on the location and type of the lesion should be investigated further.
BACKGROUND:Cerebral cavernous malformations (CCMs) may be familial or nonfamilial. This systematic review compared the natural history of CCMs in familial compared with nonfamilial cases. METHODS: We searched MEDLINE, Web of Science, and EMBASE for natural history studies on CCMs up to September 2018. We included studies that followed at least 20 untreated patients. Primary outcomes were hemorrhage, seizures, and neuroimaging changes in familial and nonfamilial cases. Incidence rate per person-year (PY) or lesion-year (LY) of follow-up were used to pool the data using fixed-effects or random-effects models. We used the incidence rate ratio for comparison. RESULTS: We could not compare hemorrhage rates between familial and nonfamilial cases mainly owing to mixtures of subgroups of patients. The seizure rate was similar in familial and nonfamilial cases with pooled incidence rate of 1.5%/PY (95% confidence interval 1.1%-2.2%). The reseizure rate was higher than the seizure rate (P < 0.001). New lesion development was higher in familial cases (32.1%/PY vs. 0.7%/PY, P < 0.001). Signal change on neuroimaging ranged from 0.2%/LY to 2.4%/LY in familial cases. In familial cases, incidence rate of size change was 8%/PY (95% confidence interval 5.2%-12.2%) and 1.1%/LY (95% confidence interval 0.6%-1.6%). CONCLUSIONS: Familial CCMs show higher dynamic changes than nonfamilial cases. However, the presence of actual dynamic changes needs further assessment in nonfamilial cases. CCMs demonstrate a low incidence of seizure. First-time seizure increases the chance of recurrent seizure. Seizure rate based on the location and type of the lesion should be investigated further.
Authors: Ana Filipa Geraldo; Cesar Augusto P F Alves; Aysha Luis; Domenico Tortora; Joana Guimarães; Daisy Abreu; Sofia Reimão; Marco Pavanello; Patrizia de Marco; Marcello Scala; Valeria Capra; Rui Vaz; Andrea Rossi; Erin Simon Schwartz; Kshitij Mankad; Mariasavina Severino Journal: Neuroradiology Date: 2022-10-06 Impact factor: 2.995
Authors: Christine K Fox; Jeffrey Nelson; Charles E McCulloch; Shantel Weinsheimer; Ludmila Pawlikowska; Blaine Hart; Marc Mabray; Atif Zafar; Leslie Morrison; Joseph M Zabramski; Amy Akers; Helen Kim Journal: Neurology Date: 2021-08-13 Impact factor: 11.800