Seven-year-old girl with rare epileptic disorder treated successfully at AIIMS

Shri Laxmi Goyal, 7, would burst into a bout of roaring laughter every 15 minutes. A happy child, you would say. Only there was never anything funny. Those were actually a rare type of epileptic attacks that she had been experiencing since age two. Rhythma Kaul reports.

Shri Laxmi Goyal, 7, would burst into a bout of roaring laughter every 15 minutes. A happy child, you would say. Only there was never anything funny. Those were actually a rare type of epileptic attacks that she had been experiencing since age two.

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Goyal suffered from a rare condition called hypothalamic hamartoma, wherein a lesion forms at the centre of the brain called hypothalamus. It starts affecting vital functions such as thirst, satiety and output of emotions such as laughter, grief, anger, etc.

Her family, however, initially thought their little girl had been possessed by an evil spirit and took her to people who practiced warding off the evil eye. “This is the commonest of family reactions in these cases that delays treatment,” said Dr Manjari Tripathi, additional professor, department of neurology, All India Institute of Medical Sciences (AIIMS).

“It is scary to even think about the experience. She would go out of control and one person had to be with her all the time,” said Shobha, Laxmi’s mother.

The lesion contains cells that are hyper-excitable and stimulate surrounding brain areas. The location of lesion usually manifests in two types of symptoms — laughing uncontrollably and premature onset of puberty.

“Since the symptoms are so rare, only an experienced doctor can diagnose it. The child was pointlessly put on several medicines all these years,” said Dr Sarat Chandra, professor, department of neurosurgery at AIIMS, who operated upon the girl on June 6.

Surgery is the only effective solution for this disease as it instantly stops the attacks. But the lesion can never be removed as it is located in a very sensitive part of the brain.

“We merely disconnect the lesion from the rest of the brain, which is akin to disconnecting wires from a fuse box to stop the flow of current,” added Chandra.

The procedure is performed either through an open surgery or endoscopically, wherein a 10mm hole in the skull makes way for the endoscope to pass into the brain and cut the lesion from the surface.

Out of five procedures that Chandra has performed in AIIMS in six years, three were done endoscopically on children aged between four and seven years.

Laxmi can now lead a perfectly normal life, but needs to continue with anti-epileptic medicines for about a year. “We need to give the brain time to stabilise,” said Tripathi.