Video Monitoring Recording

Date of Procedure: Date started: 06/28/07
Date completed: 06/29/07

Procedure: 24-hour Video Monitor Recording

Clinical History: This is a 22 month old male with seizure like episodes

Medications: Vigabatrin and Zonegran

Recording Date: The scalp electrodes were applied according to the International 10/20 system of electrode placement. Zygomatic electrodes were also used. The recording was made on the Nihon Kohden digital EEG system.

Digital EEG Analysis: The automatic seizure-spike detection programs were applied.

Findings: Background activity: The background activity during the awake state consisted of posterior dominant theta activity at 5-6 Hz. No consistent background asymmetry was noted. Frequent independent spike and wave activity was noted in the left and right temporal region independently. The frequency of interictal spike and wave activity were higher on the left side. The frequency of interictal epileptiform activity was increased during sleep. Some of the spike and wave activity in the left temporal region showed secondary bilaterally synchrony.

During sleep, vertex waves, sleep spindles were noted bilaterally.

Interictal Findings: No electrographic seizures were captured on this recording.
Clinical Events: A total of six clinical events were documented by the family. The events consisted of either startling (brief body stiffening) or rapid eye twitching. None of the events showed significant evolution of epileptiform activity on EEG. No subclinical seizures were captured.

Impression: This is an abnormal video EEG monitoring. The background activity was within lower normal limits. Independent spike and wave activity in the left and right temporal regions may suggest increase risk of epileptic seizures. Otherwise, no subclinical seizures were captured. No electrographic seizures were captured. None of the clinical events showed EEG correlations. Therefore, these episodes are probably nonepileptic events.

PET Scan

D, a 1 year 7 month old boy, was evaluated in the PET center as a participant in a research study. As part of this evaluation D's mother, completed the Vineland which is a questionnaire that asks about his functioning in numerous domains including communication, daily living, socialization and motor skills. The scores of this questionnaire are reported below, and indicate how mothers perception of D's functioning compares to the functioning of other children his age. It is important to note that this assessment was performed as part of a standardized research protocol and is not intended to be used in lieu of a full clinical evaluation. In addition, clinical history is required for appropriate interpretation of the results. A full clinical evaluation, or referrals, is available at the office. However, these results may be useful to school, medical and other professionals who are following D.

Vineland Adaptive Behavior Scales, Second Edition

COMPOSITE Subscale V-Scaled Score (M=15: SD=3) Standard Score (m=100; SD=15)

Communication Composite Stand. Score 77 Descriptive Range: Borderline
Receptive Communication Scaled Score 12
Expressive Communication Scaled Score 11
Daily Living Skills Composite Stand. Score 80 Descriptive Range: Low Average
Personal Scaled Score 11
Domestic Scaled Score 12
Community Scaled Score 13
Socialization Composite Stand. Score 90 Descriptive Range: Average
Interpersonal Relationship Scaled Score 12
Play and Leisure Time Scaled Score 12
Coping Skills Scaled Score 16
Motor Skills Composite Stand. Score 57 Descriptive Range: Low
Gross Motor Scaled Score 6
Fine Motor Scaled Score 8
Adaptive Behavior Composite Stand. Score 72 Descriptive Range: Borderline

Clinic Letter

*Final Report*
Age: Patient is an 18-month boy.
CHIEF COMPLAINT: Perisylvian syndrome/polymicrogyria

HISTORY OF PRESENT ILLNESS: Parents report that "D" has had no recent seizures or spasms. His last seizure occurred over a year ago. He will occasionally startle with loud noises. Parents report that he will extend his neck and flex his arms forward when this occurs. It appears that D has lost 1 pound since his last visit. The dietitian was consulted and met with the parents to discuss methods for increasing D's caloric intake.

CURRENT DEVELOPMENT: D is in EI program where he receives PT and OT. He also receives PT/OT and speech at Beaumont Hospital twice a week each. D has been making small gains in his development. He has started using his upper extremities more frequently. He is reaching out for objects. He is able to roll from side to side. He is using a gait trainer, taking a few steps in physical therapy. He is babbling a few consonants, but has no speech yet.

PHYSICAL EXAMINATION: D weighs 23 lbs; heart rate is 101 and blood pressure 103/66. D is well-nourished, well-developed, in no acute distress. He is alert, interactive and making good eye contact. Of note, he is drooling quite a bit. Lungs are clear to auscultation bilaterally. Heart has a regular rate and rhythm. Abdomen is soft and not tender. On musculoskeletal exam, strength is 4/5 in upper and lower extremities. D has good head and neck control, but very poor trunk control. He has increased tone in his bilateral upper extremities, bilateral lower extremities. NO parachute reflex was noted.

NEUROLOGICAL EXAMINATION: D is alert, interactive. Pupils are equal, round and reactive. Extraocular movements are intact. There is a positive red reflex. Fundi are not well visualized. NO facial asymmetry. Palate elevates with phonation. Deep tendon reflexes are +2 throughout.

PREVIOUS DIAGNOSTIC STUDIES: D has a VMR in November 2006 which was abnormal with a slow background, occasional spike and wave activity was seen in the right temporo-parieto-occipial region. There were ten events by the parents that were not associated with epileptic activity. D had a CT scan in August 2006 which showed PMG along the perisylvian areas of the brain with a moderate degree of ventriculomegaly. There was no evidence of craniosynostosis on this exam. D also has a barium swallow recently which showed no aspiration, but did show some oral motor dysfunction

IMPRESSION: Perisylvian syndrome, polymicrogyria and epilepsy

TREATMENT PLAN: Continue vigabatrin 250 mg in the morning and 500 mg at bedtime. D will be scheduled for a PET scan. Parents are to call with any questions or concerns. D should return to clinic in four months.

Modified Barium Swallow

Procedure done: 01/08/2007

Procedure: Modified Barium Swallow

Notes: D is a 17 month old infant male who has a diagnosis of congential bilateral perisylvian syndrome, polymicrogyria, and infantile spasms. He is presently recieving out patient OT/PT and speech. D was referred by OT for a modified barium swallow (MBS) to rule out aspiration and assess swallowing safety. Mom reported that Daniel presently eats baby food (stage 2 and some 3's), thick liquids and mashed potatoes. He will eat crackers that will melt in his mouth but nothing that requires mastication. Mom reports that D does not demonstrate any side to side movements with his tongue. D was evaluated this pm before radiology for a MBS. His mother was present. D was positioned upright in the red tumble form feeding chair for the assessment. He was offered the following foods mixed with barium.
Milk via a bottle: D demonstrated severe oral motor dysfunction with the bottle. He demonstrated lingual cupping and stripping of the tongue. He is able to compression and express milk from the bottle. However noted increased spillage from his oral commissions along with increased drooling. Noted consistent protrusion beyond the labial border with a wide jaw excursion. Quality of his suck is poor, but functional for him. No penetration or aspiration noted.
Sweet Potatoes mixed with cereal via a spoon: oral motor central remaining the same as above with no penetration or aspiration noted.
Cheese puff and meat stick: D was given a small (cheerio-sized) piece of meat stick and cheese puff on the lateral sides of his oral cavity. Noted slightly better mobility of the food when placed on the Right vs. the Left. Ability to manipulate the bolus with his tongue and gums is poor. However, he is sensorily aware of the bolus. Oral transit time is significantly delayed. NO penetration or aspiration noted.
Impression: D is a delightful 17 month old little boy with significant oral motor dysfunction. His mother has done an excellent job working with him at home. He receives outpatient OT/PT a& speech and has made gains (per his mother) Mom has a home program that she with with D on.
Recommendations: Offer D thick liquids and pureed foods, soft mechanical diet, soft semi-solid foods to work on mobility lip function and facial tone.
Increase OT oral motor therapy to 2-3x per week