Tuesday, March 21, 2017

WHEN SLEEPING BEAUTY IS NOT JUST A FAIRY TALE!

Mr. Naresh Kumar Sagar interacting with the panelists during Press MeetYoungest child in the world diagnosed with ultra-rare sleeping disorder at Aster Medcity
March 21, 2017: New Delhi / Kochi: Everyone has read the classic fairy tale, Sleeping Beauty, about a princess who falls into a deep sleep for hundreds of years. Doctors at Aster Medcity in Kochi found a real life ‘Sleeping Beauty’ when a 4 year old girl was diagnosed with an ultra rare sleeping disorder known as Kleine-Levin syndrome. She is the youngest patient in the world to be diagnosed with this disorder which is also known as ‘Sleeping Beauty’ syndrome. This is an extraordinarily rare neurological disorder with only around 1-2 cases per million people.
Liya was born after six years of marriage through in-vitro fertilization. Much loved by her parents, the only concern in an otherwise normal childhood was a delay in her speech which started occurring as she turned three. In October 2016, she started crying and getting irritable without any reason. This was followed by an episode of unconsciousness where she was unresponsive for more than 24 hours. After regaining consciousness, she was confused and aloof. Suspecting epilepsy, the doctors started anti-convulsive medication. But despite this, she had eight episodes in four months. She was hospitalized at other hospitals thrice but the problem was not diagnosed.
Dr. _Akbar Mohammad Chettali, Paediatric Neurologist at Aster Medcity said, “Liya was brought to the emergency in Aster Medcity for the first time on 16th February after a sudden episode of unconsciousness, where she was found to be deeply comatose and having very low heart rate and breathing. Continuous EEG monitoring ruled out non-convulsive epileptic state. After investigations that ruled out other suspected conditions that can cause prolonged unconsiousness, the child was found to be in deep sleep with poor responsiveness lasting almost for 5 days. This was confirmed by Polysomnography, a sleep study that records brain waves, the oxygen level in blood, heart rate and breathing, as well as eye and leg movements and is used to diagnose sleep disorders. The results of the sleep study suggested prolonged REM (rapid eye movement) sleep. After further investigations and discussions across specialities, the condition was diagnosed as the rare disease ‘Kleine Levin syndrome’ or ‘Sleeping Beauty disorder’.”
Two-thirds of the affected patients are male. Patients with this disorder are usually teenagers with symptoms starting during adolescence. Liya is the youngest known case of the Sleeping Beauty syndrome. In the Kleine Levin syndrome, the patient goes into deep sleep that can last from many hours to many days at a time. In Liya’s case, she used to fall asleep for five days at a time. As in Liya’s case, the sleep state is usually preceded by a period of irritable and abnormal behavior.
Dr. Akbar Mohammad Chettali, Paediatric Neurologist said, “An interesting finding from the patient’s history was unexplained psychiatric illness with prolonged coma and unexplained death in maternal grandmother at an young age, which could have been from the same Kleine Levin syndrome. The child psychiatry consultation also confirmed the diagnosis of Kleine Levin syndrome. Subsequently the child was started on medication that included neuroleptics, mood stabilizers and stimulants. She showed good response to treatment, staying awake and feeding well.”
While the frequency of the sleep attacks are known to decrease with increasing age, the parents were counseled regarding the recurring nature of illness and the need to be compliant with the medication over a long duration. Sleep hygiene, that is, habit of sleeping well on a regular basis was emphasized.
Mr. _Denny,  Liya’s father, said, “We are happy that the doctors at Aster Medcity were able to diagnose this rare condition. Thanks to them, this real life fairy tale of our Sleeping Beauty has a happy ending just like in the story. Liya is now awake and is playing with her little sister.”

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