A four-year-old girl diagnosed with a rare autoimmune disorder, Rasmussen’s encephalitis, is being treated by neurologists at the Government General Hospital here.
Rasmussen, 92, a neurosurgeon had done extensive research on the disease, and identified the disease as having a poor prognosis. The symptoms of the disease, also known as chronic focal encephalitis, include seizures and paralysis of left upper and lower limb. The patient will continue to have seizures which is termed as epilepsia partialis continua despite using multiple anti-epileptic drugs.
After conducting a series of clinical tests and collaborating with imaging evidence, Head of Department of Neurology N.V. Sundarachary said that though the exact cause was not known, the disorder could be due to sub-acute chronic infection or autoimmune attack on Glutamate 3 receptors in the brain.
Clinical examination of the girl revealed that she was having left focal seizures and involuntary dance like movements (chorea) of right upper and lower limb and that she was completely paralysed on her left side.
Neurons on one side of brain are destroyed leading to weakness and seizures of contralateral limbs. Brain on the affected side gets shrunken called hemiatrophy. These seizures, which are lateral or focal, are resistant to conventional anti-epileptic drugs. These children may require removal of the damaged hemisphere which is called hemispherectomy.
Loss of speech
“The premorbid status of the disease is uneventful and the girl has become aphasic which means loss of language [speech],” Dr. Sundarachary said.
Fortunately, the girl has started responding to treatment, the doctor said.
