Intractable Epilepsy due to multilobar cortical dysplasia

Posted by Dr. Thamburaj Dr. Thamburaj
By Dr. Manish Kumar

This 8-year-old  baby girl was brought to us with the history of fits since the age of 2 days of her life.
The father noticed the fits when there was abnormal movement of the left eyeball towards left side.
On the third day she had clonic seizure.
She had different types of seizures over last 8 years.
It starts with the abnormal movement of the left eyeball towards left then progresses to involve left eyelids,
left side of the face, neck turning towards left, and jerking of the left hand and leg (adversive attacks).
There  was associated with minimal frothing, but no tongue biting or urinary incontinence.
Descending down from left eye and face to the left leg, the seizure used to involve different parts at times.
At the age of two years she used to suddenly fall down forward which subsided with treatment.
Sometimes she had generalized tonic clonic seizures also.
She usually becomes conscious immediately after the fits and run out pushing the people around.
Following generalized fits, she used to take rest, sleep for a long time but still used to be responsive.
She used to have severe long drawn fits at around 9.30 PM few days.
Usually the episodes occur when she is normal but at times she used to become dull, sometimes she used to run
to her room from outside, lie down on her bed and the she used to have fits,
long drawn and usually more aggressive.
The seizures were very frequent, as frequent as every half an hour.
The best controlled day had seen 10 fits; a maximum of 60 episodes a day has been counted.
Usually she didn't have fits during the sleep.
There had been altered sleep rhythm, used to sleep from 6 AM to 4 PM and be active during night.
She prefers to use the right hand until unless compelled to use the left hand.
According to the parents there has been no weakness of any of the hands or legs.
It was a full term hospital delivery; mother was treated for post partum hypertension with cocktail therapy.
The baby cried after birth, APGAR score is not available. But for next 12 hours or more the baby did not cry
 or show any movement and mother did not give milk. Between the two babies the mother used oral
contraceptive pills for 3 years and copper-T for 2 years. Mother has had two miscarriages.
Usually she has been a restless baby more so over last 6 months or little more than that.
Over the last 6 months she has not slept well.
She is highly irritable. She cries or shouts or does the things she likes and stops others from doing
the things which she dislikes. She has been trained well for social habits.
Speech has not developed at all. Other milestones had been normal.
She can understand the speech and work accordingly. She was admitted in a school meant for the disabled and
she was able to make few figures but only when she liked.
There was no history suggestive of infectious child hood diseases, skin rashes, fever and febrile fits or trauma.
Her father is a bank clerk. Her elder sister is 7 years elder to her and doing well in her academic carrier.
There is no history of familial tumors or congenital abnormalities.
She was treated with Phenytoin from the 3rd day of her life. All along she has been treated with different
permutations and combination of the drugs. At present she is on carbamazepine 300 mg daily,
Lamotrigine 75mg daily and phenobarbitone 30 mg daily under supervision of a senior neurologist.
Surgical treatment was advised 4 years ago; but the parents were unwilling.
She is a hyperactive baby, moderately nourished, physical development not adequate for the age,
mentally subnormal, does not obey easily. Can perform simple commands. Can’t speak but makes
sounds in anger. No cranial nerve paresis. Nutrition of the muscles poor but equal in all 4 limbs.
Moves all 4 limbs, but left hand paucity of movement present, minimum weakness present in the l
eft sided limbs. Walks normally. No neurocutaneous markers, no congenital anomalies.
Blood investigations were within normal limits.

MRI revealed enlarged right temporal lobe, with increased grey matter going in to and replacing the white matter,enlarged right lateral ventricle (temporal horn) polymicrogyria and macrogyria.
Frontal lobe of the right side and all the lobes on the left side were normal



On 28 –05 –2004 right fronto parieto occipito temporal craniotomy and excision of the pathological temporo-parieto-occipital region
was done. Per operatively the brain right side was found to be abnormal as the gyri were prominent in the temporal lobe and poly
microgyri in the parieto occipital lobe was found. Other parts of the brain were looking normal.
Total excision of the abnormal looking brain was done.
Post operatively she recovered well.
She was seizure free immediately after the surgery and remains so till last review(2 mths post-op)  
There was no new neurological deficit.