Pick Your Brain !

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Section Editor: Dr. Gurpreet Singh Kochar

Dear friends, welcome to the Third Edition of ‘Pick your brain’, the Child Neurology Quiz to engage, enlighten and empower you. Here, we come with five more clinical questions to stimulate your brain! The correct answers for the quiz can be found at the bottom of this page

Case 1

20 month old girl with Global developmental delay, Bilateral ptosis and hypotonia with normal DTRs

  1. What are the MRI findings & possible diagnosis?
  2. What are additional 2 clinical features often identified with this disease?
  3. What is the predominant inheritance pattern?

Case 2

A 15-year-old girl presents to the emergency with difficulty breathing, diaphoresis, muscle rigidity, confusion, and agitation. She is on some medications for her psychosomatic symptoms. Her pulse rate is 130/min, respiratory rate 28/min, and temperature 104 F. She is agitated and has altered sensorium.

  1. What is the possible diagnosis?
  2. What group of medications possibly the patent might be taking?
  3. Name 2 medications that may be used for treatment of the condition

Case 3

A preterm newborn baby if found outside a orphanage. He is brought to hospital for neurological evaluation. His EEG is done for evaluating background activity. The EEG was reported normal by pediatric neurologist.

 

  1. What are the findings in EEG?
  2. Peak incidence of this finding is seen at what gestational age?
  3. By what age these finding (arrow) usually disappear?

Case 4

A 13-year-old girl presents with acute onset ascending paralysis. A day before, she had gone for a visit to religious place where she walked for 8-9 Km. She also has history of 2 other episodes of weakness in last 3 years which resolved on their own. There are no cranial nerve deficits. The motor examination shows bilateral proximal lower limb > upper limb weakness. There are no sensory deficits, plantar is flexor bilaterally. Laboratory work reveals sodium 136 mEq/L, potassium 1.9 mEq/L, BUN 30 mEq/L, creatinine 0.7 mg/dL, chloride 106 mEq/L, bicarbonate 22mEq/L. ECG shows depressed ST-segment and tall T waves.

  1. The most common familial form of this disorder is caused by a gene mutation for which ion channel?
  2. What is expected in Nerve conduction studies?
  3. What is the drug to treat acute attack and drug used for prevention?

Case 5

This is a graph showing relationship of PaCO2, PaO2 and Mean arterial pressure (MAP) with Cerebral Blood flow (CBF).

  1. Label the respective graphs (a,b,c)
  2. What value of cerebral perfusion pressure should be targeted in older children with traumatic brain injury?
  3. Threshold for treatment of intracranial hypertension <………….mmHg

Answers

Q1

  1. Molar tooth sign. Joubert syndrome
  2. Abnormal breathing pattern (alternating apnea/ tachypnea); Abnormal eye movements
  3. AR

Q2

  1. Neuroleptic malignant syndrome
  2. Dopamine-receptor antagonist- antipsychotics (haloperidol, fluphenazine)
  3. Dantrolene, dopamine agonists (bromocriptine)

Q3

  1. Delta brushes, Discontinuity
  2. 31-35 weeks
  3. 38-42 weeks (40 weeks)

Q4

A- Hypokalemic periodic paralysis

  1. Calcium channel-CACNA1S
  2. Reduced CMAP amplitudes (normal velocities)
  3. Acetoazolamide, Dichlorphenamide

Q5

  1. a- PaO2; b- PCO2; c- MAP
  2. 40-50 mmHg
  3. < 20 mmHg