Once a rare disease,LGBS now fast affecting kids

Seven-year-old Punit (name changed) was suffering from viral fever for about a week and was on the road to recovery when he suddenly complained of pain in his legs and was almost immobilised below the waist.

Written by Smriti Sharma Vasudeva | Chandigarh | Published:September 19, 2012 1:32 am

Seven-year-old Punit (name changed) was suffering from viral fever for about a week and was on the road to recovery when he suddenly complained of pain in his legs and was almost immobilised below the waist. Initially,his parents thought that it might be weakness which is usually an after-effect of viral fever. But with Punit not responding to massage on his legs,he was rushed to the the Advanced Paediatric Centre at PGIMER,where doctors diagnosed him with what is called the Landry Guillain-Barre Syndrome (LGBS),an uncommon syndrome that can be potentially life-threatening and affects one in 20,000 children. The fact that Punit’s parents lost no time in reaching PGI ensured that treatment began in time and he could be cured.

Once known as a rare disorder,off late,LGBS or Landry Guillain-Barre Syndrome,a very rapidly progressive disease of the nerves of the legs,arms etc,there has been an increase in the number of cases being reported especially among children.

At the Advanced Pediatric Centre,of the PGIMER,during the last two years,42 children have been admitted and all were admitted to the Paediatric ICU indicating that the syndrome had already progressed to an advanced level. Says Dr Pratibha Singhi,Head of the Department of Paediatric Neurology,PGIMER,“ It can be triggered by a viral infection,after certain vaccinations wherein an attack is carried out by abnormally reacting proteins of the human body called “antibodies.” In all humans antibodies are normally protective against infections but in LGBS while attacking the infection the antibodies damage the nerves also. The illness is rapidly progressive and goes up from the legs to arms,respiratory tract etc.”

“It starts as weakness and numbness of the legs followed by similar symptoms in the arms”,she added. “There are no symptoms for the syndrome and it is not a genetic disorder and the only signs could be weakness in legs,pain tingling in legs. So the only warning signals could be early treatment. The parents should get their children immediately to the hospital”,she added. “Though it is a non-seasonal syndrome but it has been seen that during monsoon,there is an increase in cases of viral fevers and thus the cases of LGBS also see an increase,” she said.

On an average,the departments is getting three to five cases per month from the city as well the neighbouring states and mostly these children are brought at the advanced stages. Adds Dr Deepak Gupta,senior consultant,Max Super Specialty Hospital,Mohali,“In the syndrome,the patient over a matter of hours-days develop weakness of legs,arms,swallowing and breathing. The nerves at all these sites come under an “immune attack” most commonly because of a preceding infection. Along with leg and arm weakness the patients develop difficulty in swallowing and lastly difficulty in breathing necessitating artificial ventilatory support. GBS has been noted to have a seasonal occurrence. In a scientific study published from Kerala the disease incidence was more in the rainy season.

There are two scientifically proven treatments,IVIG: Intravenous Immuneglobulin and plasma exchange”. “In the first treatment,patient is given IVIG injections in a dose according to patient’s body weight (0.4mg/kg/day) for 5 days. IVIG consists of normal antibodies which neutralize the damaging harmful antibodies causing the disease. In the second treatment,Plasma exchange,blood is filtered off the harmful antibodies by using a special machine. It is also carried out in 5 sessions,commonly on alternate days. Overall this is more tedious though equally effective as IVIG and also IVIG is expensive as compared to plasma exchange,” said Dr Gupta.

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