Clinic Letter

D was follwed up in the clinic today. he is an 11-month old boy being seen in the clinic for infantile spasms. He also has bilateral perisylvian syndrome. He is accompanied by both of his parents. He was last seen in teh clinc on 05/17/06.

He started having seizures at 4 1/2 months of age. His seizures at the time were happening at a frequency of about 10-12 clusters per day and during each cluster, we would have approximately 15 spasms. He was tried at first on Topamax and Klonopin without much affect.

He was seen at Children's and was started on vigabatrin during Feb. of 2006 His parents reported that within a few days of starting Vigabatrin, he had significant improvements, and his last spasm was on 02/14/06, which is two days after starting the vigabatrin. At the time of the last visit, he also used to have daily episodes of blank stares with pupillary dilatation. THe family did not report any episodes of blank stares since he was started on Vigabatrin.

He is attaining his developmental milestones fasher, as per the parents. As per the parents, he has attained good head support; he can sit up unsupported. He stands up with support. He started doing that a month back. He tracks, he recognizes peop.e. He mostly babbles. He has started sayng "Ma". He laughs. He is learning how to drink out of a suppy cup. He is eating mostly mashed food at presen, but he has started eating some solid food.

He is getting OT/PT and is on the Early Intercention program. He also had a recent ophthamology ev aluation. There have been no interval medical problems since the last visit.

He sleeps well. There has been no behavioral abnormalities. He was born full-tern. He weighed 5 pounds 8 ounces. He is on vigabatrin 250 mg in the a.m. and 500 mg at night.

Review of System: Review of systems is negative for 10 systems unless otherwise mentioned.

Physical Examination: We couldn't obtain his blood pressure or heart rate. His height is 76.7, weight 9.6 kg. He was well nourished. His head circumference was 42, which is below the two standard deviations for his age. He had persistent drooling. He was awake, alert. He ws trackign well. He was smiling. He was interactive. He had good eye contact.

His pupils were 3mm reacting well to 2 mm. He had full range of conjugate eye movements. Facee appeared symmetric on smiling. He had mildly increased tone in both upper extremities, but the tone in the legs seemed normal. He had good strength in the limbs. His deep tendon reflexes were 2+ symmetrically. His planars were equivocal.

A review of the previous evaluations showed a VEP and WERG done during 07/11/06. which was normal. He had a VMR during April of 2006 which showed interictally frequent independant spikes in teh right temporal, central and parietal and occassional independant spikes int eh bilateral occipial area. There was one cluster of habitual epileptic spasns, which was manifested as a subtle body jerk. The EEG at this time of this spasm showed diffuse seizure onset.

He had an MRI during February of 2006 whcih showed Polymicrogyria with evidence of cortical dysplasia, predominantly in perisylvian distribution.

Impression: D has infantile spasms. He has been seizure free since vigabatrin was started. We would like to repeat his VMR after the next visit. We have advised the parents to continue the vigabatrin at the same dose.

He has a brain malformation with bilateral perisylvian synfrome and polymicrogyria. Considering that he has microcephaly, we would like to frule out cranial synostosis. We have requested a CT of the head with bony reconstructions to look for cranial synostosis. If cranial synostosis is present, we may have to consider surgery.

We have advised the parents on the importance of PT/OP and continuing with the early intervention program. We also discussed in detail with the family the future prognosis rearding perisylvian syndrome.

Visual Evoked Potential and ERG

Date of Procedure: 07/11/2006

Procedure: Visual Evoked Potential and Electroretinogram

Clinical History: D is an 11 month old male w/history of Infantile Spasms
Medications: Current medication include Vigabatrin
Recording Data: The visual evoked potentials were recorded by stroboscopic flash to each individual eye using a rate of 1.7 Hz, a duration of 100 microseconds, and filter setting of 5-100 HZ. A total of 100 sweeps were averaged each time and the study was repeated at least once for confirmation. The recordings were made MO.

Findings: The visual evoked potential by flash P100 peak on the left side was 82 and P100 peak on the right side was 83.5.

Electroretinogram. Wave A on the right side was 24.5 and wave B on the right side was 35.8. Wave A on the left side was 23.0 and wave B on the left side was 37.0.

Impression: There was a normal visual evoked potential and electroretinogram