The frequencies of neurologic diseases and various corresponding causes were analyzed in 4,319 Norman horses examined by autopsy between 1986 and 2006. Five hundred forty-three cases of nervous system disorders were detected, or a total prevalence of 12.6 % . One hundred eighty-seven of the 543 horses (34.4 % ) exhibited lesions of a traumatic origin affecting the skull or the vertebral column at different levels. Their frequency was less in foals younger than 1 month of age and higher in horses aged 6 months to 2 years. Eighty-three cases of cervical vertebral compressive myelopathy (CVCM) (15.3 % ) were identified. Males and geldings were more often affected than females. CVCM was diagnosed mostly in animals aged 6 months to 2 years. Horses affected with lesions in the mid-cervical region (region C3–C5) were significantly younger than those exhibiting more caudal lesions (region C5–C7). Neonatal maladjustment syndrome was diagnosed in 62 foals younger than 3.5 days (4.8 % of the causes of death between birth and 1 month) by using clinical or lesional criteria. The prevalence was higher in Thoroughbreds as well as in the miscellaneous breed category and less in French Standardbreds. Bacterial meningoencephalomyelitis and meningitis represented 9.6 % of nervous system disorders (52 cases) and most often affected foals aged 1 to 6 months. The main bacteria identified were beta-hemolytic Streptococci , Escherichia coli, Listeria monocytogenes, Staphylococcus aureus, and Actinobacillus sp. The other causes diagnosed were, in decreasing order of frequency: grass sickness or equine dysautonomia (6.3 % of neurologic diseases), hepatic encephalopathy syndrome (5.2 % ), septic vertebral arthritis or osteitis mainly caused by Rhodococcus equi (2.6 % ), congenital defects of the nervous system (2.4 % ), equine herpesvirus 1 myeloencephalopathy (2.2 % ), botulism (2.2 % ), malformations of the vertebral column other than CVCM (2 % ), and neighboring polyneuritis associated with guttural pouch mycosis (1.5 % ).