iSMART quiz on Nervous System & Rehab disease

A 7-year old boy was seen in your clinic due to double vision that worsens during the day. On PE he had unilateral ptosis but the pupillary reaction to light was normal. He had difficulty sustaining an upward gaze. What diagnostic procedure will you do to confirm your diagnosis?

• repetitive nerve stimulation

• serum creatinine kinase

• serum potassium

• muscle biopsy

Which is TRUE of Duchenne muscular dystrophy?

• histologic evidence of myopathy at birth

• normal creatine kinase at birth

• short stature seen in symptomatic phase

• honeymoon period is due to improvement in motor milestone

Which of the following cases qualifies a diagnosis of epilepsy?

• An 8-year-old male who had recurrent tonic clonic seizures during his sleep 2-3 months apart.

• A 3-year-old girl who had 4 seizure events since 6 months old. In all events, she had fever.

• A 10-year-old having recurrent seizures after falling off his bike.

• An 11-year-old girl had one afebrile seizure prior to consult. She had two febrile seizures when she was 3yr old.

A 5-year old boy complains that objects are moving towards him. He is frightened every time he experiences it. This was also associated with diaphoresis, nausea, vomiting and on and off clicking sound on his right ear. What is your consideration?

• Focal aware seizure

• Benign paroxysmal vertigo of childhood

• Focal to bilateral tonic-clonic seizure

• Focal aware seizure and Benign paroxysmal vertigo of childhood

A 4-year old girl was brought to the clinic due to recurrent seizures. You noted several skin lesions. What is the most likely diagnosis?

• Tuberous sclerosis complex

• Aicardi Syndrome

• Neurofibromatosis Type 1

• Sturge Weber Syndrome

A 3-day old baby girl was admitted due to jaundice. she was delivered uneventful to a 20-year old G1P1 (1001) via NSD at home assisted by a midwife. On the 2^nd day of life, she started to have jaundice in the face and eventually progressed all over her body. She was immediately brought to the hospital. At the ER she developed generalized tonic-clonic seizure for about 5 minutes. Your consultant asked for TBB1B2 and revealed extremely high B1. Your plan is to do double exchange transfusion. What will be the possible outcome of this neonate?

• Cerebral palsy hemiplegia type

• Cerebral palsy athetoid type

• Cerebral palsy diplegia type

• Cerebral palsy paraplegia type

A 5-year old previously healthy boy was having difficulty doing monkey bars and climbing up the stairs. On examination, he had proximal weakness, big calves and normal reflexes. He was seen by a general physician who suspected a neuromuscular condition. Which neuro-diagnostic procedure will you request initially?

• serum creatinine kinase

• multiplex ligation probe amplification for dystrophin gene

• muscle biopsy

• electromyography with rapid nerve stimulation (RNS)

A 12-year- old girl was brought to the clinic for evaluation due to seizures. You noted her unique facial findings. What is your diagnosis?

• Aicardi Syndrome

• Tuberous sclerosis

• Von Recklinghousen disease

• Sturge-Weber Syndrome

Clinical picture not consistent with a peripheral cause of hypotonia in a child

• Lethargy

• Feeding impairment

• Respiratory distress

• Profound weakness

A 15-year-old girl was brought to ER for visual loss. This was preceded by fever, headache, and eye pain. She had EBV infection 10 days ago. What is the most likely diagnosis?

• central retinal artery occlusion

• retinal migraine

• optic neuritis

• non-organic visual loss