Seminar At Andheri, Mumbai on 26th June 2011
The other case was amazing...
Mast. B.k
A 6 year old boy came to me last September with the complaints of
Convulsions on a daily basis.
This boy experienced the attack of seizure for the first time at a very tender age of 1½ year while he was asleep. He was unconscious during attack, followed by a 2nd attack at the age of 2½ year.
The 3rd attack came at the age of 3½ year and this time it lasted for 7-8 days during which he was unconscious. Till then, he had normal milestones with good intellect, good memory and all normal curiosities which a child usually has, except that he had
bilateral Congenital cataract.
His anxious parents got his MRI done which suggested
RASMUSSEN’S ENCEPHALITIS. He started taking treatment with allopathic medicines. Then after 1 year, he started getting full fledged tonic clonic convulsions after every ½ hour daily. He developed stammering speech, all mental activities decreased and physical activities were disturbed.
This time Doctors took their hands-off the case, saying there is no treatment for this case. Parents returned hopeless from hospitals like Ruby Hall, in spite of being completely prepared for the expenditure.
A physician, who himself is a patient of mine, suggested consultation with me and they came to see me on 22.09.2007 with great hopes.
The boy entered my clinic and I saw that he was unable to sit without support, restless, appeared retarded mentally and physically.
Rasmussen’s Encephalitis : Rasmussen’s encephalitis (RE; also called Rasmussen’s syndrome) is a progressive inflammation of the part of the cerebral cortex, which is made up of a right and a left hemisphere. The disease starts at one site in one hemisphere and spreads to adjoining areas on the same side. Strangely, it does not spread to the other hemisphere. The inflammation leads to loss of nerve cells and scar formation and usually results in severe disability. Although RE is most often diagnosed in children under the age of 10 years, it can also start in adolescence and adulthood. It is a rare disorder and probably affects one person in every 500 000 to 1 000 000.
Clinical features : The clinical features in RE are determined by which areas of the hemisphere are affected: each area has different functions. As the disease spreads, more areas are damaged and the greater the severity and range of the disabilities. Typically, the disease progresses relentlessly until most of one hemisphere is affected. The inflammation burns out by itself only rarely before severe disability has occurred. However, the speed of the spread varies among patients. At one end of the spectrum, the disease advances rapidly over a few weeks or months. At the other end, progression occurs slowly over several years. This slower clinical variant seems to be more common in adolescents and adults than in children. It is possible that there are milder forms of RE that we fail to recognize.
The typical clinical features are :
Epilepsy : Usually, the first sign of the disease is focal epilepsy. If the motor cortex (the area that controls movement) is affected, the patients have motor seizures with jerking of one side of the body. Sometimes the motor seizures become continuous, and this state is called continuous partial epilepsy. Similarly, if the temporal cortex is affected, patients have temporal lobe seizures (complex partial seizures) with altered awareness of their surroundings.
Although the seizures start at one site, they can spread to the rest of the brain and cause generalized epilepsy with loss of consciousness. As the disease progresses, the seizures become more frequent, more severe, and more difficult to treat, with standard anti-epileptic drugs.
Neurological deficits :Hemiparesis. The wiring of the nervous system determines that a lesion of one side of the brain causes problems on the opposite side of the body. Thus, involvement of the motor cortex in one hemisphere causes weakness of the other side of the body. If the sensory cortex is affected the patient has numbness of the other side of the body
(hemianaesthesia).
Visual loss. Damage to the visual areas in one hemisphere causes loss of vision in the opposite direction
(hemianopia)