Confusional arousals are classified as “partial awakenings in which the state of consciousness remains impaired for several minutes without any accompanying major behavioural disorders or severe autonomic responses”. Complete or partial amnesia of the episodes may be present. Signs and symptoms
Confusional arousals are accompanied by mental confusion and disorientation, relative lack of response to environmental stimuli, and difficulty of awakening the subject. Vocalisation accompanied with coherent speech is common. Patients may appear upset, and some of them become aggressive or agitated. As well as for children, attempting to awaken or console an adult patient may increase agitation. Confusional arousals can occur during or following an arousal of deep sleep (see slow-wave sleep) and upon an attempt of awakening the subject from sleep in the morning.In children, confusional arousals can often be reproduced artificially by awakening the child during deep sleep. However, it doesn’t have any clinical significance without deeper investigation. Children living an episode of confusional arousal typically sit up in bed, whimper, cry, moan, and may utter words like “no” or “go away”. They remain distressed and inconsolable despite all parental efforts. Paradoxically, parental efforts can rather increase agitation of the child. The onset of symptoms is usually within 2 and 3 hours of sleep onset (at the time of transition from slow-wave sleep to a lighter sleep stage) and those events can last from 10 to 30 minutes. Patients generally wake up without any recollection of the event. It is necessary to distinguish confusional arousals in adults from children.
Graft versus leukemia: Graft versus leukemia has been shown many times to play an important role in curing JMML, and it is usually evidenced in a child after bone marrow transplant through some amount of acute or chronic Graft Versus Host Disease (GVHD). Evidence of either acute or chronic GVHD is linked to a lower relapse rate in JMML. Careful management of immunosuppressant drugs for control of GVHD is essential in JMML; importantly, children who receive less of this prophylaxis have a lower relapse rate. After bone marrow transplant, reducing ongoing immunosuppressive therapy has worked successfully to reverse the course of a bone marrow with a dropping donor percentage and to prevent a relapse. Donor lymphocyte infusion (DLI), on the other hand, does not frequently work to bring children with JMML back into remission. Prognosis
Prognosis refers to how well a patient is expected to respond to treatment based on their individual characteristics at time of diagnosis. In JMML, three characteristic areas have been identified as significant in the prognosis of patients:
Without treatment, the survival [5 years?] of children with JMML is approximately 5%. Only Hematopoietic Stem Cell Transplantation (HSCT), commonly referred to as a bone marrow or (umbilical) cord blood transplant, has been shown to be successful in curing a child of JMML. With HSCT, recent research studies have found the survival rate to be approximately 50%. Relapse is a significant risk after HSCT for children with JMML. It is the greatest cause of death in JMML children who have had stem cell transplants.