Background: Encephalitis is a severe neurological syndrome with devastating consequences. Despite extensive testing, the etiology often remains unknown. Involvement of the thalamus or basal ganglia (T/BG) occurs in a subset of patients with encephalitis and may be an important etiological clue. In order to improve diagnosis of T/BG patients, we reviewed this subgroup within the California Encephalitis Project (CEP).
Methods: Data from T/BG cases enrolled in CEP were retrospectively reviewed. Cases were stratified by age and grouped by etiological classification: infectious, postinfectious, and noninfectious. Neuroimaging reports were examined and compared between etiologies.
Results: T/BG neuroimaging abnormalities were reported in 6% of 3236 CEP cases. An etiology was found in 76%: 37% infectious, 16% postinfectious, and 23% noninfectious. The most frequently identified infectious agents were respiratory viruses, accounting for 31%, predominantly in children. Other infections more common in the T/BG group included Creutzfeldt-Jakob disease, arbovirus, and Mycobacterium tuberculosis. Infectious and postinfectious cases had higher median cerebrospinal fluid white blood cell count than noninfectious etiologies. Notably, T/BG neuroimaging characteristics were associated with distinct etiologies. In particular, symmetric hemorrhagic abnormalities involving the thalamus were most frequently found within the respiratory virus group.
Conclusions: T/BG involvement in patients with suspected encephalitis was associated with specific etiologies. In addition to agents with established predilection for the T/BG such as M. tuberculosis and arboviruses, a surprisingly high number of cases were associated with respiratory viruses, especially in children. Neuroimaging abnormalities in such patients can aid clinicians in narrowing the etiological scope and in guiding testing.