Video Monitoring Recording

Date of Procedure: Date started: 01/12/2009
Date completed: 01/13/2009

Procedure: 23-hour Video Monitor Recording

Clinical History: This is a 3 yr old child with a history of bilateral perisylvian syndrome, infantile spasms, now having startle episodes again.

Medications: None

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 which was bilaterally symmetrical.

During sleep, sleep spindles were seen bilaterally.

Interictal Findings: Interictally there was presence of right temporal-central as well as right temporal-central spike and wave activity seen.

Clinical Events: There were spasms captured. These spasms showed minimal EEG correlation or sometimes the EEG correlation was obscured by EMG activity. Some spasms showed diffuse spike wave activity followed by attenuation. We would not localize the seizure onset..

Impression: This is an abnormal video EEG monitoring. The background activity is slow for age which suggests diffuse neuronal dysfunction. There is present of multi-focal spike and wave activity as well as multiple spasms captured during the recording.

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

Video EEG

Date of Procedure: November 18, 2006

CLINICAL HISTORY: D is a 15 month male born at full-term with history of infantile spasms and developmental delay

RECORDING DATA: 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 system.

DIGITAL EEG ANAYSIS: Automatic spike and seizure detection programs were applied.
Background activity: The background during the awake state consisted of a mixture of felta, theta, and alpha activities ranging from 4-7 Hz, which was bilaterally symmetrical. During sleep, the background consisted of a mixture of delta and theta activity ranging from 2-5 Hz. Sleep spindles, vertex waves, and K complexes were seen bilaterally.

INTERICTAL FINDINGS: Occasional spike and wave activity was seen in the right temporal-partietal-occipital region and independently occasionally spike and wave activity was seen at left temporal-parietal-occipital region. No ongoing seizure was noted.

CLINICAL EVENTS: A total of 10 events were recorded by the parents. These events were characterized by rapid eye blink. None of these episodes showed evolution of epileptiform activity.

IMPRESSION: This was an abnormal video monitoring recording.
1) The background was slow. This finding may suggest diffuse neuronal dysfunction.
2) Occasional spike and wave activity was seen in the tight temporal-parietal occipital region and independently occasional spike and wave activity was seen in the left temporal parietal occipital region. These findings may suggest increased risk of epileptic seizures.
3) A total of 10 events were recorded by the parents. These events were characterized by rapid eye blink and staring. None of these episodes showed evolution of epileptiform activity so therefore; these events were probably nonepileptic in nature.
Clinical correlation was suggested.

Speech and Language Pathology

Evaluation date: 10/12/06

Initial Evaluation

Age: 14 months
Initial Communicative Diagnosis: Severe oral motor delay, Severe expressive language delay, Moderate receptive language delay
Mother accompanied her son, D, to our Center for Childhood Speech and Language Disorders on 10/12/2006 for a speech and language evaluation.

Background Information: information was obtained through a case history and parent interview. Developmental milestones for early speech and language development were reported to be delayed. D did vocalize and babble as an infant. Babbling and some jargon persist. First words have not yet emerged. Mom reported that she first became concerned regarding D's speech and language development following his diagnosis of CBPS, IS, PMG. In February of 2006 D began therapy with Carol, physical therapist. Mom indicated that D has been receiving both PT and OT from February 2006 to the present.

Dr. Cukrowski, Ophthalmolgist, and Dr. Dobyns, Genetics, Neurology, Pediatrics both indicated that D's vision is good with no apparent issues.

Mom reported that audiological screening was conducted during D's hospital stay following his birth. No apparent difficulty with hearing acuity was revealed at that time.

Dr. Alpiner, Pediatric and Adult Physical Medicine and Rehabilitation indicated that D is behind on his motor skills.

Mom reported that D will be seen by the early intervention program through the public school system in June of 2007 for PT, OT and speech and language treatment.

Behavioral Observation:
Upon meeting D int eh waiting room, he was seating in a reclining position within his stroller. He was noted to be smiling and quite interactive with his mother. Separation was not attempted and mom and D accompanied this clinician to the diagnostic suite where they remained for the entire 45 minute session. D was cooperative throughout the evaluation and demonstrated good eye contact and facial expression. Play skills were clearly delayed due to D's reduced upper extremity control.

Receptive Language Functioning:
Mom was invited to remain in the diagnostic suite and act as an informant as the Rosetti Infant Toddler Language Scale (RITLS) was administered. The RITLS is a developmental checklist, which assesses different areas of development in different age ranges. This test is based on development in different age ranges. This test is based on developmental norms.

Receptive Language Functioning:
Results of the RITLS placed D receptive language skills within the six to nine month range. He demonstrated most of the skills in the upper age range such as recognizing family members names, attending to music or singing, responding to "no" most of the time, stopping when name is called, attending to pictures and occasionally waving "bye-bye" During the evaluation D demonstrated difficulty gesturing in response to verbal requests, following simple commands that required movement, and verbalizing or vocalizing in response to verbal requests. It should be noted that vocalizations and verbalization were present but were delayed in nature. It appeared that D had difficulty initiating oral/vocal motor movement. Based on observation and developmental checklist of the RITLS D is demonstrating a moderately severe receptive language delay. It should be noted that it is probably that demonstration of auditory comprehension is greatly effected by D's difficulty with initiation of motor movements as well as difficulty with gross and fine motor planning.
Play Skills:
The RITLS also assesses play skills. D is demonstrating a severe delay in play skills at this time as his scores are falling the three to six month age range. D's delay in development of play skills is clearly directly related to his decreased motor planning. For example, D smiles at himself in the mirror and enjoys frolic play, however, he had great difficulty initiating reaching for objects or participating in games with adults due to his decreased upper extremity motor planning.
Oral Motor Development:
D presented with symmetrical facial features. He appeared to have proper lip closure. D demonstrated production of numerous vowel sounds and when babbling he produced age appropriate phonemes such as /p, b, m, g,d/. An oral peripheral examination was not completed on this date due to D's young age and inability to imitate oral motor movements at this time. Motor planning for oral motor movement was reduced, however, spontaneous smiling was frequent.

Mom reported that D is receiving feeding treatment by an OT. She reported that D sometimes chokes on food, does not yet chew, and is taking stage 3 baby foods.

Expressive Language Functioning:
The RITLS was used to assess D's expressive language skills. At the time of evaluation D demonstrated the ability to vocalize four different syllables and vocalization of two syllable combinations such as "mama" is emerging. Mom reported that D is beginning to repeat syllables but only for the word "mama' at this time. D is demonstrating a severe expressive language delay.

Summary:
D is a 14 month old child who participated in a speech and language evaluation at WBH. Based on parent reported, informal observation and development checklist, D is presenting with a moderate receptive language delay, a severe oral motor delay and a severe expressive language delay.

Recommendations:
A conference was held immediately following the diagnostic session with mom. At that time the above mentioned diagnostic impressions as well as treatment recommendations were discussed. Recommendations are as follows:
1) That D participate in an individual speech and language treatment stimulation program within our center one time weekly for one half hour session.
2) That the family continue with the early intervention program at home and within their public school system

Long Term Goals:
Goal for the first interim may include, but are not limited to the following:
1)Provide the family with an intensive language stimulation home program.
2) Provide a speech and language stimulation treatment program within our center with Anna, SLP. Anna is a speech and language Pathologist who has certification in neurodevelopmental training.