Movement Disorders & Motor Delays in Pediatrics Flashcards
Which of the following in not a RF for CP? A. Prematurity B. <2500g birth weight C. Teratogens D. Birth asphyxia E. C. section delivery
E. C. section delivery. The remainder of the answers ARE RFs for CP!
Mom presents to clinic with her 7 month old boy, Stanley, due to ongoing feeding difficulties. Stanley is an ex-prem. She says that he tends to have more spills than most babies but otherwise she hasn’t noticed any other issues since discharge from hospital. On exam, Stanley requires additional support to hold up his head, his legs tend to scissor and he has a positive Moro reflex. What is the likely diagnosis?
This child is premature and this is a RF for CP. Additionally, the exam has multiple red flags for CP. Therefore, the likely diagnosis is CP.
Kyle is a 5 year old male with known CP. He presents to clinic today for abdominal pain and constipation. On exam, you note that his left arm is stiff and pronated, with milder stiffness occurring in hits L leg. He also has strabismus and a history of seizures. How would you classify his CP? A. Spastic Hemiplegia B. Dyskinetic Hemiplegia C. Spastic Diplegia D. Dyskinetic Diplegia E. Spastic Quadriplegia F. Dyskinetic Quadriplegia
A. Spastic Hemiplegia
Spastic CP accounts for 75-85% of CP. Hemiplegia is a unilateral UMN syndrome with arms being affected greater than legs, visual field defects (ex. homonymous hemianopsia, and strabismus), specific LD and seizures.
Shane is a 7 year old male with known CP. He has a scissoring gait pattern and walks on his toes. He muscle stiffness and pains tend to be worse in his legs than his arms. How would you classify his CP? A. Spastic Hemiplegia B. Dyskinetic Hemiplegia C. Spastic Diplegia D. Dyskinetic Diplegia E. Spastic Quadriplegia F. Dyskinetic Quadriplegia
C. Spastic Diplegia
Diplegia is a bilateral UMN syndrome. This results in legs being more severely effected than the arms, a scissoring gait pattern, hip flexion/adduction, knees flexed/valgus, equinus ankles, and less commonly, seizures.
Dad presents to your clinic with 3 month old Hayden with complaints that he is a poor feeder and hates tummy time. On exam you note significant head lag but appropriate grasp strength. Dad states that Hayden has not yet started to socially smile. Which of the following would not be on the DDx? A. Hypoxic ischemic encephalopathy B. Trisomy 21 C. Congenital brain anomaly D. Muscular dystrophy
D. Muscular dystrophy. A, B and C are all causes of central hypotonia. Muscular dystrophy causes peripheral hypotonia. The DDx of central hypotonia includes: hypoxic ischemic encephalopathy (HIE is MOST common cause), intracranial hemorrhage, congenital brain anomaly/cerebral malformations, TORCH infections, sepsis, drug effects (ex. benzos), encephalopathy (seizures), chromosomal abnormalities (T21, Prader Willi), paroxysmal disorders (ex. Zellweger).
Tim is a 4 month old "floppy baby". On exam he is alert and exams normally with the exception of profound weakness in his extremities. Dad says that he takes interests in toys but does not swat at them and is unable to bring his hand to his mouth. Which of the following is not on your DDx? A. Hypoxic ischemic encephalopathy B. Spinal muscular atrophy C. Myasthenia gravis D. Botulism E. Muscular dystrophy
A. Hypoxic ischemic encephalopathy should not be on the DDx as it is a cause of central hypotonia and this baby has peripheral hypotonia. The remaining options are causes of peripheral hypotonia.
A 2 year old male presents with progressive proximal muscle weakness. He uses his arms to stand, walks on his toes and waddles when he walks. What is the inheritance pattern of this disease?
This child likely has Duchenne Muscular Dystrophy. It is an X-Linked recessive mutation in the dystrophin gene (1/3 mutations are sporadic). The missing dystrophin results in muscle fibre fragility and breakdown. The clinical presentation includes progressive proximal muscle weakness, cardiac muscle degeneration, Gower’s sign (uses arms to stand), toe walking, waddling gait, calf pseudo hypertrophy (muscles are replaced by fat), thing muscle atrophy, decreased reflexes and cognitive impairment.