Sudden death in rheumatoid arthritis with atlanto-axial dislocation

A post mortem material of 11 consecutive cases of severe atlanto-axial dislocation (a.a.d.) with cord compression is reported. The total number of deaths from rheumatoid arthritis (RA) during the period of 5 years was 104, and all were autopsied. Neurological symptoms correlated poorly to fatal a.a.d. Hemiplegia was found in three cases, one of which, however, was caused by thrombosis cerebri. Spastic signs were transiently recorded in two patients and dysphagia in a further two. Five patients had a history of recent vomiting. A.a.d. was the sole or main cause of death in 8 cases and contributory in 2. Sudden death occurred in 7 of the cases. Only 2 cases had obtained a correct diagnosis intra vitam. The CNS findings at autopsy consisted of cord compression (11/11 cases), cord malacia (2/11) and cerebral oedema (3/11). One case had polyarteritis and renal amyloidosis and one pulmonary carcinoma with metastatic spread. Signs of active inflammation in the axial joints were present in 4 cases. This study, based on systematic post mortem examinations, revealed an unexpectedly high and not previously reported incidence of fatal medulla compression in RA patients with a.a.d. (10%).