The final schedule of invited lectures and the CPC case for your

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39th ANNUAL B.C. NEUROSCIENCE ACADEMIC DAY
Friday March 11, 2016
Paetzold Centre, Vancouver General Hospital
“This event is an Accredited Group Learning Activity eligible
for up to 5.5 Section 1 credits as defined by the Maintenance of
Certification program of the Royal College of Physicians and
Surgeons of Canada. This program has been reviewed and
approved by UBC Division of CPD. Each physician should
claim only those credits he/she actually spent in the activity.”
Registration:
8:30 – 9:00 (coffee)
Morning Session: Experts in their Field
9:00–9:30
Professor Blair Leavitt “Gene silencing therapy for Huntington’s disease”
9:30-10:00
Dr. Navraj S. Heran “The modern era of aneurysm care”
10:00–10:30 Dr. Julie Robillard “Online brain health resources: ethics needed”
10:30-11:00
Professor Haakon Nygaard “Drug repurposing to accelerate new Alzheimer’s
disease therapeutics”
11:00-12:00
2016 Dr. P.D. MOYES LECTURE
“Surgical management of thalamic and chiasmatic – hypothalamic tumours of
childhood”
Dr. Jefffrey Wisoff
Professor and Director
Division of Pediatric Neurosurgery
New York University Langone Medical Center
12:00-13:00
BUFFET LUNCHEON
at Medical Student Alumnae Centre with
B.C. Neurological and Neurosurgical Nurses Association
13:30-14:30
10th annual Ken Berry Clinical Pathological Conference
Discussant: Dr. Kathy Selby
Moderator:
Dr. Chris Dunham
(case history attached)
Afternoon Session: Resident, Fellow and Nursing Research Presentations
14:30 –16:00 “TBA
16:00 ADJOURNMENT
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Course Objectives:
Following these sessions, participants will be better able to understand: gene therapy for
Huntington’s, the clinical management of cerebral aneurysms, the ethical dilemmas associated
with internet health information, drug therapy for Alzheimer’s, thalamic and chiasmatichypothalamic tumors, and the latest clinical research in the neurosciences occurring in BC.
Each lecture will include 25% time for questions.
Comments (if required):
SUGGESTED TOPICS FOR 2017 Meeting:
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BC Neuroscience Day 2016: 11th annual Ken Berry CPC
Clinical summary: PJ currently is a 4 y/o girl that originally presented to Dr. E. Roland
(Pediatric Neurology) at 2.5 y/o with features suspicious for a myopathy.
PJ was the product of a normal pregnancy and weighed 7.5 lbs. at birth. Her mother noted leg
weakness from 4 months onward. PJ started walking at 17 months. She was being seen by a
physiotherapist, who eventually noted signs of developmental regression. Toilet training,
previously achieved, became non-existent. PJ started to fall more frequently and exhibited
poor protective reflexes. PJ’s mother noted that walking seemed to become more difficult at
3 y/o. Her ability to climb stairs/furniture was lost and she was not running or jumping. On
exertion, PJ complained about leg aches; the latter would often keep her up at night. With
minimal physical activity, she fatigued quickly and was breathless post activity, but there was
no increased work of breathing per se. In sum, there was evidence of delayed gross motor
and developmental milestones.
Further history suggested that PJ had no cognitive, speech or fine motor issues. However, she
did suffer from occasional headaches, constipation and sensitivity to loud noises. Family
history revealed that PJ’s mother suffered from Graves’ disease, while her father had
ulcerative colitis. A maternal sister had migraines. The maternal grandmother had diabetes
and suffered a stroke at 58 y/o. Both the maternal grandmother and great grandmother were
diagnosed with multiple sclerosis.
On examination, PJ had normal vital signs. She was alert and cooperative. She had thin muscle
bulk. PJ walked with a waddling gait and had hyperlordosis of the lumbar spine. There was no
facial dysmorphism. She exhibited a positive Gowers’ sign. PJ was hypotonic and displayed
proximal muscle weakness. The remainder of the neurologic and physical exam was normal.
An extensive workup was performed. Radiologic investigations included a normal MRI of the
head and spine, with the exception of “fatty infiltration” noted in the paraspinal muscles (T12
and inferior) and in the glutei. Pelvic x-ray was normal. Electrophysiology revealed a normal
EEG, EMG and NCS. Genetic testing revealed a normal chromosomal microarray and SMN
analysis was normal. Blood work included: CBC with differential, electrolytes, phosphorus,
magnesium, calcium, transaminases, CPK, LDH, lactate, acylcarnitine profile, TSH/T4, urine
purine/pyrimidines, plasma amino acids, and urine organic acids; no abnormalities were
detected.
Both Cardiology (Dr. M. Hosking) and Biochemical Diseases (Dr. S. Stockler-Ipsiroglu) were
consulted. Cardiac exam, ECG and echocardiogram were all normal, suggesting the absence of
a cardiomyopathy. Metabolic assessment did not reveal evidence of a metabolic disorder.
A muscle biopsy of the left vastus lateralis was subsequently performed.
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