Hemispherotomy--only cure to Rasmussen’s syndrome

PRESS TRUST OF INDIA

New Delhi, July 4:  Imagine living only with half a brain?
 According to noted neurosurgeons, this is not as tragic as it might seem at first for patients suffering from epilepsy.
 Such persons can be totally relieved of the symptoms of a particular type of severe uncontrolled epilepsy by severing the connections between the two halves of the brain. The other half then picks up the missing functions, helping the body lead a normal life.
 Kirti (name changed), a 11-year-old studying in 5th grade is a bright and chirpy girl who participates in all of her school activities and ranks among the top ten in her class.
 However, she walks with a slight limp and has a mild weakness on the left side of her body but on seeing her no one can imagine she has only half of her brain functioning.
 The right cerebral hemisphere of her brain was disconnected from rest of the vital organ in a complex surgery called “hemispherotomy” four years ago at the All India Institute of Medical Sciences (AIIMS), New Delhi.
 When she was two years old, Kirti started having severe epilepsy fits and despite medication, her seizures worsened till she was totally debilitated.
 “At the time of admission, she was having as many as 100 attacks of seizures a day” said Dr Manjari Tripathi, senior neurologist, AIIMS.
 “After investigations we found that she was suffering from an uncommon condition called ‘Rasmussen’s syndrome’, where half the brain becomes diseased, leading to multiple epileptic attacks which may number up to few hundred a day.
 “In this condition, the neurons of the brain- due to severe inflammation- lose their protective insulation and continuously short circuit, causing epileptic seizures. While there are some medical modalities of treatment available, the only thing which finally works is surgery,” Dr Tripathi said.
 Fortunately for Kirti, this treatment was available at
 AIIMS. Dr P S Chandra, senior neurosurgeon who performed the surgery said, “Kirti was a difficult case as her epilepsy was badly neglected.
 “Following a eight-hour surgery her entire right brain was disconnected from the left side. Even though the diseased brain was retained within the skull, it was not electrically connected to the rest of the brain. The diseased portion gradually shrinks,” he explained.
 “It is like a defective electrical circuit. Instead of removing it, we simply cut the electrical connections so that the normal circuitry does not fuse anymore” said the doctor.
 “This type of surgical treatment is very unusual and I have performed it on just over 45 patients over the past 10 years,” Dr Chandra, who initiated the use of this technique at AIIMS, said.
 This kind of surgery is offered as a cure for a particular type of severe epilepsy, which cannot be controlled with drugs.
 “When a portion of the brain gets diseased, the functions of that area of brain get transferred to the other areas of the brain, a function called plasticity. Thus, even when a large diseased portion of the brain is removed, the person continues to carry on all of their activities because the disease has now been eliminated,” he said.
 Recently, a 9-month-old baby girl who was nearly 50 seizures a day, underwent a similar procedure and is now free from epilepsy.
 Prof A K Mahapatra, senior neurosurgeon, AIIMS remarked that surgery for persons suffering from uncontrolled epilepsy should not be an option of last resort, and should be performed when indicated as the risks are very low and chances of cure from epilepsy are high.

Lastupdate on : Sun, 4 Jul 2010 21:30:00 Mecca time
Lastupdate on : Sun, 4 Jul 2010 18:30:00 GMT
Lastupdate on : Mon, 5 Jul 2010 00:00:00 IST


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