Results specify cognitive capabilities in disorders of consciousness, and determine their prognostic value. Specifically the N400 ERP is suggested as an important tool to assess information-processing capacities that can predict the likelihood of recovery of patients in UWS or MCS.
ObjectiveLittle is known about the long-term outcome of patients with disorders of consciousness (DOCs) such as unresponsive wakefulness syndrome (UWS) or minimally conscious state (MCS). We describe the disease course of a large group of DOC patients 2–14 years after brain damage.MethodsIn 102 patients (59 UWS, 43 MCS), clinical and demographic variables from disorder onset were related to the patients' outcomes 2–14 years after discharge. Etiology, age at event, time since onset, gender, and home care versus institutional care were assessed as predictors and similarities and differences between UWS and MCS determined.ResultsSeventy-one percent of the patients had passed away or showed no improvement in condition. Twenty-nine percent regained consciousness and developed some communicative capacities. The time a syndrome persisted did not predict clinical outcome in either condition. Six patients regained consciousness after more than 3 years. Of these, five had been UWS (42% of recovered UWS, three traumatic origins, one tumor, one hypoxia) and one MCS (5% of recovered MCS, traumatic origin). In UWS, younger patients, those cared for at home, and in tendency those with traumatic origins, were more likely to recover. In MCS, no reliable outcome predictors were found.InterpretationCurrent predictors are too vague for single patient predictions. This study identifies a subgroup of late-recovering patients, casting doubt on the 12-month boundary, after which UWS is stated to be permanent. Routine reexamination, use of more reliable outcome predictors and research determining optimal care settings are needed to inform the crucial decisions made for these patients.
Opsoclonus-myoclonus syndrome (OMS) is a rare neurological disease in childhood which can be associated with neuroblastoma. Since autoantibodies have been detected in some patients with OMS, an autoimmune etiology is suspected. We compared the prevalence of autoimmune disorders and autoantibodies in parents of children with OMS and in a group of controls of same age and sex. Autoimmune diseases were found in 15.8% of the parents of OMS children, but only in 2.0% of the controls (p<0.001) There was also an increased prevalence of autoantibodies in the OMS parents (42.8% vs. 8.0%, p<0.001). Thyroid diseases were the most frequent autoimmune diseases found, followed by inflammatory rheumatic diseases. Interestingly, the OMS parents also had significantly more autoantibodies against CNS structures than the controls (p<0.01). These findings support the autoimmune hypothesis of childhood OMS and may also hint to a genetic susceptibility for OMS.
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