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Neurology

Question 1

A 4-year-old child with cerebral palsy comes to your clinic for the first time for a routine visit. He walks with the help of leg braces and a walker, and his speech is slow and slurred. His mother tells you that he has never been hospitalized, and he has never had any problems with swallowing. He began walking at age 2.5 years, his speech is limited 10 short phrases, and he is unable to take of his clothes or use the toilet without assistance. On examination, you find that the boy has only minimally in¬creased tone in the upper extremities and that he has good fine motor coordination, but that he has significantly increased tone and deep tendon reflexes in the lower extremities. How would you categorize this child’s cerebral palsy?
A. Mild, diplegic
B. Mild, hemiplegic
C. Moderate, diplegic
D. Moderate, quadriplegic
E. Severe, diplegic

Answer

C. Diplegia means all four extremities are affected, more in the lower extremities.

Question 2

A 17-year-old woman is brought to the hospital by ambulance after a motor vehicle accident. She and her boyfriend had been drinking beer at a party and were on their way home when she lost control of the car and hit the wall of the local police station. She reportedly had a brief loss of consciousness but is currently oriented to name, place, and time. She responds appropriately to your questions. While waiting for her cervical spine series, how¬ever, she vomits and lapses into unconsciousness. She becomes bradycardic and develops irregular respirations. Several days after emergent management of her injury, the ado¬lescent in the above case is transferred to you from the intensive care unit. The brain in¬jury most likely in this case is:
A. Subdural hemorrhage
B. Epidural hemorrhage
C. Intraventricular hemorrhage
D. Posttraumatic epilepsy
E. Concussion

Answer

B. This teen displays the typical adult course in epidural hemorrhage, with an initial period of altered mental status (concussion), followed by a period of lucidity, and then redevelopment of altered mental status and symptoms of increased intracranial pressure (hematoma effect). Young children typically do not display this pattern. Immediate neurosurgical evaluation is appropriate for her.

Question 3

She is concerned about her prognosis. You tell her:
A. She will need extensive neuropsychiatric evaluation before she can return to school.
B. She will likely have headaches, fatigue, nausea, and sleep disturbances.
C. She will likely develop seizures and will need to take pro-phylactic anticonvulsants for 2 years.
D. She can no longer be legally permitted to drive because she has had brain surgery.
E. She should have few long-term problems.

Answer

E. Although the mortality for patients with acute epidural hemorrhage is higher than that for patients with acute subdural hemorrhage, long-term morbidity is minimal. The complaints in an¬swer B are common after a subdural hemorrhage. While a seizure disorder may preclude driving, simply having had cra¬nial surgery does not.

Question 4

A 7-month-old child presents to the emergency room after re¬portedly falling from his high chair. The parents report no loss of consciousness, and no other trauma or medical problems. Your physical examination reveals a few old bruises but no evidence of acute trauma or fracture. The child is irritable, however, and you request a CT scan of the brain without contrast. The pediatric ra¬diologist reports bilateral frontal subdural hematomas, and notes two healing skull fractures that she estimates to be about 2 weeks old. The best next step in this child’s management is to:
A. Observe him for 6 hours in the emergency center.
B. Assess bleeding time and prothrombin time.
C. Order magnetic resonance imaging of the head.
D. Discharge him from the emergency center with head injury precautions.
E. Order an electroencephalography and a neurology consultation

Answer

C. This child has evidence of old skull fractures with subdural hematomas. Magnetic resonance imaging of the head would help to determine the age of the hematomas. Should the age of the blood in the hematomas corretate with the estimated age of the skull fractures, child abuse should be considered. Neurology may be helpful later in the child’s evaluation, but a consultation would be of limited benefit before additional data were gath¬ered. Discharge with the’ information presented in the case would be dangerous; the child likely requires admission to the hospital. Bleeding studies are unlikely to be helpful; the child has no history consistent with a bleeding disorder, nor would a bleeding disorder explain the old fractures.

Question 5

A 7-year-old girl presents with poor school performance. She is repeating the first grade because she did poorly in reading. Her mother reports that she cannot finish her homework without repeated encouragement and occasional threats. She notes that girl does not like to go to school, and every morning she complains of a headache or stomachache to avoid attending school. Her teachers report that she does not pay attention in class, that she gets up frequently and walks around the room, and that she is always misplacing her school supplies, frequently leaves her backpack at school. Her physical exami¬nation, including a funduscopic examination, is normal. The best way to evaluate this child is:
A. Electroencephalograph (EEG).
B. Magnetic resonance imaging of her brain .
C. Electrolytes and a complete blood count.
D. Parental reassurance.
E. Parent and teacher input into an ADHD evaluation tool .

Answer

E. The history suggests attention deficit hyperactivity disorder. Rating scales that include parent and teacher questionnaire can be helpful in this evaluation. Laboratory evaluation would not be helpful, and a central nervous system neoplasm is unlikely in this child with a normal physical examination. Reassurance in this situation without more information would be a disservice to the child.

Question 6

The emergency center physician admits a 17-year-old boy for new onset seizures. A first semester freshman at the local college, he awoke to go to class but then had a generalized tonic-clonic seizure, witnessed by his roommate. He reports that he spent the night before studying and denies illicit drug use. With prompting he reports that for several years he has had iso¬lated muscle twitches in the mornings, but because they always resolved after an hour or so he did not seek medical attention. He has no family history of seizures. He is currently afebrile, and his physical examination is completely normal. He ques¬tions why he had the seizure and what is wrong with him. Which of the following is the best diagnosis and treatment plan
A. Absence seizures; 1 to 2 years of anti epileptic medications.
B. Febrile seizures; no need for medications.
C. Lennox-Gastaut syndrome; lifelong antiepileptic medication.
D. Juvenile myoclonic epilepsy; lifelong antiepileptic medication.
E. Benign myoclonus; no need for medications.

Answer

D. This is a typical presentation of Janz syndrome, or juvenile myoclonic epilepsy. Prognosis is generally excellent with med¬ication, but cessation of medication frequently results in relapse

Question 7

A 7-year-old girl, born at 26 weeks gestation, has global developmental delay and functions at the level of a 4-year-old child She has a history of staring spells that last several minutes and include unilateral upper extremity jerking. Treatment has been difficult, requiring two anti epileptic medications to achieve adequate control. The parents have read information suggesting that most children with absence seizures improve with age and are developmentally normal. They ask if they can expect the same from their child. You respond that they can expect:
A. Complete resolution of the absence seizures and normal development.
B. Complete resolution of the absence seizures and continued developmental delay.
C. Persistent seizures but eventual normal development.
D. Persistent seizures and continued developmental delay.
E. Partial resolution of the seizures and normal development.

Answer

D. The child described has atypical or complex absence seizures. These children usually do not have resolution of seizure activity as they get older; rather, they commonly develop other types of seizures.

Question 8

A 2-year-old boy who had a simple brief febrile seizure comes to your office one day after his emergency center visit. He is currently afebrile, is happily pulling the sphygmomanometer off the wall, and from the mother you learn that he is taking antibi¬otics for an ear infection diagnosed the previous day. She wants to know what to expect in the future regarding his neurologic status. You tell her that:
A. He has no risk of further seizures because he was age 2 years at the time of his first febrile seizure.
B. He will need to take anticonvulsant medications for 6 to 12 months to prevent further seizure activity.
C. You want to schedule an EEG and a magnetic resonance scan of his head.
D. While he does have a risk of future febrile convulsions, seizures of his type are generally benign and he is likely to outgrow them.
E. This is an isolated disorder and his children will not have seizures.

Answer

D. Part of the anticipatory guidance for parents of children with febrile seizures is to impress upon them that the child may have another seizures; it is similarly important to emphasize the usual benign nature of this condition. In a simple febrile seizure, imaging and EG generally are not recommended, nor are prophylactic anticonvulsants. Because febrile seizures seem to have a genetic basis; it is possible that your patient’s children will also have febrile seizures.

Question 9

A 10-month-old boy presents to the ED with a I-day history of fever to 104°F (40°C), increased irritability, decreased breast¬feeding, and refusal of solid foods. The parent brought him in after two 30-second episodes of generalized jerking that oc¬curred over a 20-minute span. Your examination reveals an awake but lethargic infant. The anterior fontanelle is flat, the tympanic membranes and oropharynx are moist and not erythe¬matous, the lungs are clear, and the heart and abdominal exam¬inations are normal. He has no focal neurologic findings. The next step in management should be:
A. Intravenous ceftriaxone
B. Admission overnight for observation
C. Computerized tomography of the head
D. Discharge from ED to follow-up with his primary care pro¬vider in 24 hours
E. Lumbar puncture

Answer

E. While this child may ultimately be diagnosed as having had a simple febrile seizure, the patient’s age (younger than 1 year) is such that a neck examination will not be reliable. A lumbar puncture is required to evaluate the child for meningitis. Administering antibiotics before the lumbar puncture (or other cul-tures are obtained) is inadvisable unless the patient’s condition is such that he would not tolerate the procedure.

Question 10

The father of a 4-year-old girl calls your office to report her sec¬ond febrile seizure. He states that this seizure was identical to the first one that happened 4 months ago: she developed an el¬evated temperature and within a short time had a generalized convulsion lasting 90 seconds. She was sleepy for about 2 min¬utes after the seizure. When she woke up, the family gave her ibuprofen suspension. She is now running around the house chasing her younger brother. The parents wonder if she needs to be on anticonvulsants now that she has had another seizure. You tell the father that:
A. Febrile seizures are frequently recurrent, but they usually have no significant long-term effect.
B. You will prescribe an anticonvulsant because they reduce the risk of epilepsy in the future.
C. You will order an EEG and CT scan of her head to be done on an outpatient basis
D. He needs to take his daughter to the hospital for in patient admission.
E. He should stop the ibuprofen and observe the fever curve.

Answer

A. Some children will develop recurrent febrile seizures. Data suggest that anticonvulsant medications will decrease the risk of further febrile seizures; however, no evidence suggests the no¬tion that anticonvulsant therapy with decrease the risk of devel¬oping epilepsy. The possible adverse reactions with antiepilep¬tic medications are numerous, including severe allergic reactions and interference with school performance, and in most cases, the benefit is not worth the risk. Fever reduction with medications is generally encouraged in children with a history of febrile seizure.

Question 11

year-old child with cerebral palsy comes to your clinic for the first time for a routine visit. He walks with the help of leg braces and a walker, and his speech is slow and slurred. His mother tells you that he has never been hospitalized, and he has never had any problems with swallowing. He began walking at age 2.5 years, his speech is limited 10 short phrases, and he is unable to take of his clothes or use the toilet without assistance. On examination, you find that the boy has only minimally in-creased tone in the upper extremities and that he has good fine motor coordination, but that he has significantly increased tone and deep tendon reflexes in the lower extremities. How would you categorize this child’s cerebral palsy?
A. Mild, diplegic
B. Mild, hemiplegic
C. Moderate, diplegic
D. Moderate, quadriplegic
E. Severe, diplegic

Answer

C. Diplegia means all four extremities are affected, more in the lower extremities.

Question 12

A 17-year-old woman is brought to the hospital by ambulance after a motor vehicle accident. She and her boyfriend had been drinking beer at a party and were on their way home when she lost control of the car and hit the wall of the local police station. She reportedly had a brief loss of consciousness but is currently oriented to name, place, and time. She responds appropriately to your questions. While waiting for her cervical spine series, how¬ever, she vomits and lapses into unconsciousness. She becomes bradycardic and develops irregular respirations. Several days after emergent management of her injury, the ado¬lescent in the above case is transferred to you from the intensive care unit. The brain in¬jury most likely in this case is:
A. Subdural hemorrhage
B. Epidural hemorrhage
C. Intraventricular hemorrhage
D. Posttraumatic epilepsy
E. Concussion

Answer

B. This teen displays the typical adult course in epidural hemorrhage, with an initial period of altered mental status (concussion), followed by a period of lucidity, and then redevelopment of altered mental status and symptoms of increased intracranial pressure (hematoma effect). Young children typically do not display this pattern. Immediate neurosurgical evaluation is appropriate for her.

Question 13

She is concerned about her prognosis. You tell her:
A. She will need extensive neuropsychiatric evaluation before she can return to school.
B. She will likely have headaches, fatigue, nausea, and sleep disturbances.
C. She will likely develop seizures and will need to take pro-phylactic anticonvulsants for 2 years.
D. She can no longer be legally permitted to drive because she has had brain surgery.
E. She should have few long-term problems.

Answer

E. Although the mortality for patients with acute epidural hemorrhage is higher than that for patients with acute subdural hemorrhage, long-term morbidity is minimal. The complaints in an¬swer B are common after a subdural hemorrhage. While a seizure disorder may preclude driving, simply having had cra¬nial surgery does not.

Question 14

A 7-month-old child presents to the emergency room after re¬portedly falling from his high chair. The parents report no loss of consciousness, and no other trauma or medical problems. Your physical examination reveals a few old bruises but no evidence of acute trauma or fracture. The child is irritable, however, and you request a CT scan of the brain without contrast. The pediatric ra¬diologist reports bilateral frontal subdural hematomas, and notes two healing skull fractures that she estimates to be about 2 weeks old. The best next step in this child’s management is to:
A. Observe him for 6 hours in the emergency center.
B. Assess bleeding time and prothrombin time.
C. Order magnetic resonance imaging of the head.
D. Discharge him from the emergency center with head injury precautions.
E. Order an electroencephalography and a neurology consultation

Answer

C. This child has evidence of old skull fractures with subdural hematomas. Magnetic resonance imaging of the head would help to determine the age of the hematomas. Should the age of the blood in the hematomas corretate with the estimated age of the skull fractures, child abuse should be considered. Neurology may be helpful later in the child’s evaluation, but a consultation would be of limited benefit before additional data were gath¬ered. Discharge with the’ information presented in the case would be dangerous; the child likely requires admission to the hospital. Bleeding studies are unlikely to be helpful; the child has no history consistent with a bleeding disorder, nor would a bleeding disorder explain the old fractures.

Question 15

A 7-year-old girl presents with poor school performance. She is repeating the first grade because she did poorly in reading. Her mother reports that she cannot finish her homework without repeated encouragement and occasional threats. She notes that girl does not like to go to school, and every morning she complains of a headache or stomachache to avoid attending school. Her teachers report that she does not pay attention in class, that she gets up frequently and walks around the room, and that she is always misplacing her school supplies, frequently leaves her backpack at school. Her physical exami¬nation, including a funduscopic examination, is normal. The best way to evaluate this child is:
A. Electroencephalograph (EEG).
B. Magnetic resonance imaging of her brain .
C. Electrolytes and a complete blood count.
D. Parental reassurance.
E. Parent and teacher input into an ADHD evaluation tool .

Answer

E. The history suggests attention deficit hyperactivity disorder. Rating scales that include parent and teacher questionnaire can be helpful in this evaluation. Laboratory evaluation would not be helpful, and a central nervous system neoplasm is unlikely in this child with a normal physical examination. Reassurance in this situation without more information would be a disservice to the child.

Question 16

The emergency center physician admits a 17-year-old boy for new onset seizures. A first semester freshman at the local college, he awoke to go to class but then had a generalized tonic-clonic seizure, witnessed by his roommate. He reports that he spent the night before studying and denies illicit drug use. With prompting he reports that for several years he has had iso¬lated muscle twitches in the mornings, but because they always resolved after an hour or so he did not seek medical attention. He has no family history of seizures. He is currently afebrile, and his physical examination is completely normal. He ques¬tions why he had the seizure and what is wrong with him. Which of the following is the best diagnosis and treatment plan
A. Absence seizures; 1 to 2 years of anti epileptic medications.
B. Febrile seizures; no need for medications.
C. Lennox-Gastaut syndrome; lifelong antiepileptic medication.
D. Juvenile myoclonic epilepsy; lifelong antiepileptic medication.
E. Benign myoclonus; no need for medications.

Answer

D. This is a typical presentation of Janz syndrome, or juvenile myoclonic epilepsy. Prognosis is generally excellent with med¬ication, but cessation of medication frequently results in relapse

Question 17

A 7-year-old girl, born at 26 weeks gestation, has global developmental delay and functions at the level of a 4-year-old child She has a history of staring spells that last several minutes and include unilateral upper extremity jerking. Treatment has been difficult, requiring two anti epileptic medications to achieve adequate control. The parents have read information suggesting that most children with absence seizures improve with age and are developmentally normal. They ask if they can expect the same from their child. You respond that they can expect:
A. Complete resolution of the absence seizures and normal development.
B. Complete resolution of the absence seizures and continued developmental delay.
C. Persistent seizures but eventual normal development.
D. Persistent seizures and continued developmental delay.
E. Partial resolution of the seizures and normal development.

Answer

D. The child described has atypical or complex absence seizures. These children usually do not have resolution of seizure activity as they get older; rather, they commonly develop other types of seizures.

Question 18

A 2-year-old boy who had a simple brief febrile seizure comes to your office one day after his emergency center visit. He is currently afebrile, is happily pulling the sphygmomanometer off the wall, and from the mother you learn that he is taking antibi¬otics for an ear infection diagnosed the previous day. She wants to know what to expect in the future regarding his neurologic status. You tell her that:
A. He has no risk of further seizures because he was age 2 years at the time of his first febrile seizure.
B. He will need to take anticonvulsant medications for 6 to 12 months to prevent further seizure activity.
C. You want to schedule an EEG and a magnetic resonance scan of his head.
D. While he does have a risk of future febrile convulsions, seizures of his type are generally benign and he is likely to outgrow them.
E. This is an isolated disorder and his children will not have seizures.

Answer

D. Part of the anticipatory guidance for parents of children with febrile seizures is to impress upon them that the child may have another seizures; it is similarly important to emphasize the usual benign nature of this condition. In a simple febrile seizure, imaging and EG generally are not recommended, nor are prophylactic anticonvulsants. Because febrile seizures seem to have a genetic basis; it is possible that your patient’s children will also have febrile seizures.

Question 19

A 10-month-old boy presents to the ED with a I-day history of fever to 104°F (40°C), increased irritability, decreased breast¬feeding, and refusal of solid foods. The parent brought him in after two 30-second episodes of generalized jerking that oc¬curred over a 20-minute span. Your examination reveals an awake but lethargic infant. The anterior fontanelle is flat, the tympanic membranes and oropharynx are moist and not erythe¬matous, the lungs are clear, and the heart and abdominal exam¬inations are normal. He has no focal neurologic findings. The next step in management should be:
A. Intravenous ceftriaxone
B. Admission overnight for observation
C. Computerized tomography of the head
D. Discharge from ED to follow-up with his primary care pro¬vider in 24 hours
E. Lumbar puncture

Answer

E. While this child may ultimately be diagnosed as having had a simple febrile seizure, the patient’s age (younger than 1 year) is such that a neck examination will not be reliable. A lumbar puncture is required to evaluate the child for meningitis. Administering antibiotics before the lumbar puncture (or other cul-tures are obtained) is inadvisable unless the patient’s condition is such that he would not tolerate the procedure.

Question 20

The father of a 4-year-old girl calls your office to report her sec¬ond febrile seizure. He states that this seizure was identical to the first one that happened 4 months ago: she developed an el¬evated temperature and within a short time had a generalized convulsion lasting 90 seconds. She was sleepy for about 2 min¬utes after the seizure. When she woke up, the family gave her ibuprofen suspension. She is now running around the house chasing her younger brother. The parents wonder if she needs to be on anticonvulsants now that she has had another seizure. You tell the father that:
A. Febrile seizures are frequently recurrent, but they usually have no significant long-term effect.
B. You will prescribe an anticonvulsant because they reduce the risk of epilepsy in the future.
C. You will order an EEG and CT scan of her head to be done on an outpatient basis
D. He needs to take his daughter to the hospital for in patient admission.
E. He should stop the ibuprofen and observe the fever curve.

Answer

A. Some children will develop recurrent febrile seizures. Data suggest that anticonvulsant medications will decrease the risk of further febrile seizures; however, no evidence suggests the no¬tion that anticonvulsant therapy with decrease the risk of devel¬oping epilepsy. The possible adverse reactions with antiepilep¬tic medications are numerous, including severe allergic reactions and interference with school performance, and in most cases, the benefit is not worth the risk. Fever reduction with medications is generally encouraged in children with a history of febrile seizure.

Question 21

The parents of a 3-year-old child are worried about the child’s apparent clumsiness with frequent falls and a waddling gait. Which of the following is consistent with DMD?
A. Female sex
B. Hypertrophy of the quadriceps
C. 22-year old sister with DMD
D. Gower sign
E. Positive antinuclear antibodies in the blood

Answer

D. Duchenne muscular dystrophy, an X-linked recessive dis¬ease, is clinically evident only in males. Affected boys may show hypertrophy of the calves These boys will generally develop a Gower sign-a stereotypical set of movements used to rise from a sitting position on the floor.

Question 22

The best screening test for the child mentioned above is:
A. A muscle biopsy
B. Measurement of serum creatinine
C. An electromyogram
D. Blood analysis for antinuclear antibodies
E. Measurement of serum creatine kinase

Answer

E While a definitive diagnosis can be made by using immuno¬chemical testing of muscle biopsy tissue, the use of serum cre¬atine kinase measurement is preferred because it is less invasive and results can be obtained rapidly. Electromyography will re¬veal nonspecifIc myopathy.

Question 23

A 12-year-old previously healthy boy presents to your office be¬cause his parents have noticed, that he appears to have some muscle weakness. He has had increasing difficulty with lifting his backpack (الشنطة) and walking long distances. He has not had any trouble with his schoolwork, and continues to play the piano and video games without tiring. His 38-year-old maternal uncle re¬cently became wheelchair-bound for unclear reasons. Of the fol¬lowing, the most likely diagnosis is:
A. Cerebral palsy
B. Duchenne muscular dystrophy
C. Myasthenia gravis
D. Becker muscular dystrophy
E. Guillain-Barre syndrome

Answer

D. This patient has a history of muscle weakness that does not preclude extended use of distal muscles (hands). With the child’s presentation at the age of 12 years and a 38-year-old maternal uncle with muscle weak¬ness requiring a wheelchair, it is most likely that he has Becker muscular dystrophy.

Question 24

Which of the following statements best describes the prognosis for the 12-year-old boy discussed in the question above?
A. His prognosis for full recovery is excellent.
B. He will need to take daily medication to alleviate his symptoms.
C. It is likely that he will continue to be capable of ambulation for several years.
D. He will progressively lose fine motor skills such as writing.
E. Plasmapheresis is likely to ameliorate his symptoms.

Answer

C. Patients with Becker muscular dystrophy have a genetic de¬fect at or near the Xp21.2 locus resulting in abnormal dys¬trophin. The clinical course is more benign than that of Duchenne muscular dystrophy. Males with Becker muscular dystrophy have elevated creatine kinase and marked hypertro¬phy of the calves. The average age for loss of ambulation is early in the fourth decade of life.

Question 25

A 5-month-old female infant presents to the emergency depart¬ment with sudden onset of torticollis and with some facial gri¬macing. The infant has been alert and interactive during this time. The infant has been doing well and gaining weight for the last month after having been prescribed ranitidine and metoclo¬pramide for gastroesophageal reflux disease diagnosed by her primary care physician. Family history is negative for epilepsy. Which of the following statements is true?
A. The infant most likely is having a partial-complex seizure and needs an immediate electroencephalograph.
B. A lumbar puncture for cell count, glucose, and protein is warranted.
C. Measurement of serum electrolytes and glucose is unnecessary.
D. The infant is most likely having a dystonic reaction to one of her medications.
E. A magnetic resonance image (MRI) of the cervical spine is likely to show a congenital abnormality

Answer

D. This infant has sudden onset of the dystonic features of torti¬collis and facial grimacing. Although it is most likely a dystonic reaction to metoclopramide, initial evaluation for seizures inc1uding measurement of serum electrolytes, glucose, and calcium is indicated. Administration of diphenhydramine may rapidly reverse this drug-induced dystonia. An MRI is unlikely to demon¬strate a cervical abnormality since the onset of the condition was abrupt. Analysis of the cerebrospinal fluid as a first step is un¬likely to result in obtaining an etiology to this type of torticollis.

Question 26

A l-week-old female infant presents with her new adoptive par¬ents. The family complains that the child seems to have a twisted neck. The limited information they can provide includes an unremarkable prenatal history but delivery was almost a C-section because the baby was lying sideways. The infant has been feeding well and has had appropriate urine and stool out¬put for the last 24 hours that the family has had the child. Physi¬cal examination is significant for torticollis. Which of the fol-lowing statements is true?
A. This infant is at significant risk for aspiration pneumonia.
B. The parents should immediately begin a regimen of gentle stretching of the neck.
C. Radiographs of the cervical spine should be obtained.
D. Immediate orthopedic consultation should be arranged.
E. Immediate neurologic consultation should be arranged.

Answer

C. This child appears to have had a difficult delivery, making muscular torticollis likely. However, cervical spine abnormali¬ties must be excluded. If radiography of the cervical spine is normal, the parents can begin gentle stretching to move the head in a neutral position. If the condition persists, orthopedic refer¬ral is necessary.

Question 27

You are the team physician for a high school varsity football team .On a Friday night during the first quarter of the game, you watch as your star quarterback is sacked with a helmet-to-helmet tackle .He does not get up from the initial impact, and the other are waving frantically. You sprint on the field and assess the injured player. He is breathing and has a steady pulse, but unconscious. As you begin your evaluation, he wakes up remembers his name but cannot remember the day, his position in the team, the coach’s name or how he got to the game. He has no sensory or motor deficit that would suggest a cervical spine injury, and eventually you assist him off the field. After 10 minutes he is fully oriented (although he still cannot remember what he had for breakfast) and wants to go back in. The coach tells him he is sitting out for the rest of the game. The player appeal to you which of the following is the most appropriate management:
A. Affirm the coach’s decision and tell the player that he will need sequential evaluations before he can come back to practice.
B. Affirm the coach’s decision and tell the player he can come back and practice tomorrow.
C. Refute the coach’s decision and tell the player he can resume playing now.
D. Refute the coach’s decision and tell the player he can resume playing after half-time.
E. Strap the player to a backboard and take him to the hospital.

Answer

A. Although there is debate as to the proper length of time a patient needs to refrain from sports activities after a concussion, most organizations recommend that a player who sustains a concussion resulting in loss of consciousness refrain from play the remainder of the day. The American Academy of Pediatrics currently endorses findings of the 1997 Concussion Workshop sponsored by the American Orthopedic Society for Sports Medicine. Despite paucity of data, this report suggests that individualized and frequent reassessment of the player over time is more useful than a predetermined length of time to refrain from additional activity.

Question 28

The parents of a 1-year-old boy come to the office for a second opinion. The boy has a history of infantile spasms beginning at 6 months of age. He was porn via emergency caesarean section at 30 weeks gestation after his mother was in a motor vehicle accident and developed placental abruption. His previous doctor tried many different anti epileptic medications with little success for seizure control. The child’s most recent EEG demonstrated hypsarrhythmia. The parents are interested in the possibility of new medications and ask about their child’s long-term progno¬sis. You tell them:
A. Infantile spasms are difficult to treat. While the child will likely outgrow them, he will have significant mental retar¬dation and may have other types of seizures.
B. Infantile spasms are best treated with phenobarbital, which will decrease the child’s seizures and improve his prognosis.
C. Most children grow out of infantile spasms and develop nor-mally; medication for seizure control is unnecessary.
D. The EEG finding indicates the child does not have infantile spasms; the movements that the parents are seeing are likely caused by gastroesophageal reflux and he will be com¬pletely cured with reflux medication.
E. Infantile spasms are best prevented by antipyretic medica¬tion, and usually resolve by 5 years of age.

Answer

A. An EEG demonstrating hypsarrhythmia is typical of a patient with infantile spasms. Secondary infantile spasms are notoriously difficult to treat. Only a small percentage of children with infantile spasms will have normal development mostly the primary type and can be treated by ACTH; this patient, with proba¬bly underlying central nervous system injury secondary to peri¬natal asphyxia, will probably have persistent developmental de¬lay. Phenobarbital is not usually effective in this disorder, and there is no evidence suggesting that medications will affect long-term prognosis.

Question 29

Paramedics bring a 7 -month-old infant to the emergency room with seizure activity. The father reports the infant was in a nor¬mal state of health until about 3 days ago when she developed a febrile illness, diagnosed by her physician as a viral upper res¬piratory tract infection. Approximately 30 minutes ago the child began having left arm jerking, which progressed to whole-body jerking. The infant stopped having the seizure activity in the am¬bulance on the way to the hospital. Vital signs include a heart rate of 90 beats per minute, a respiratory rate of 25 breaths per minute, and a temperature of 100.4°P (38°C). Your examination reveals a sleeping infant in no respiratory distress with an intra¬venous catheter in the left arm. The child’s anterior fontanelle is full. The oropharynx is clear, and crusted mucous is found in the nares. The tympanic membranes are dark. The lungs are clear, and the heart and abdominal examinations are normal. The skin examination is significant for a bruise over the occiput and sev¬eral parallel bruises along the spine. The next step in manage¬ment is:
A. Observation
B. Lumbar puncture
C. Computerized tomography of the head
D. Phenobarbital
E. Electroencephalogram

Answer

C. This child’s history is worrisome for trauma. The fontanelle is full, bruises are found along the spine and on the occiput, and he has evidence of hemotympanum. A CT scan is of paramount importance, as this child likely had a seizure as a result of acute intracranial hemorrhage associated with physical abuse. While this child is febrile and is within the proper age range for febrile seizure, the history and physical examination findings are more consistent with a diagnosis other than febrile seizure.

Question 30

A 6-month-old child was noted to be normal at birth, but over the ensuing months you have been somewhat concerned about his slow weight gain and his mild delay in achieving developmental milestones. The family calls you urgently at 7:00 a.m. noting that their child seems unable to move the right side of his body. Which of the following conditions might explain this child’s condition?
a. Phenylketonuria
b. Homocystinuria
c. Cystathionuria
d. Maple syrup urine disease
e. Histidinemia

Answer

The answer is b. Homocystinuria can cause thromboembolic phenom¬ena in the pulmonary and systemic arteries and particularly in the cerebral vasculature; vascular occlusive disease is, in turn, one of the many causes of acute infantile hemiplegia. None of the other disorders listed in the ques¬tion is associated with acute hemiplegia. Phenylketonuria causes retarda¬tion and, on occasion, seizures; maple syrup urine disease, an abnormality of the metabolism of leucine, leads to seizures and rapid deterioration of the central nervous system in newborn infants; and histidinemia and cystathionuria are most likely a benign amino-aciduria with no effect on the central nervous system.

Question 31

An otherwise healthy 6-month-old girl presents to your office with an unusually shaped head. You note that the child has a long and narrow skull. She has a prominent occiput, broad forehead, no anterior fontanel. and a prominent bony ridge over the sagittal suture. Delivery of this child was by cesarean section because of failure to progress. The rest of the exam¬ination is normal. Which of the following is the most appropriate step in management of this patient?
a. Obtain a head ultrasound
b. Skeletal survey
c. Obtain chromosomes on patient and parents
d. Perform lumbar puncture to obtain opening pressures
e. Referral to neurosurgery for possible surgical procedure

Answer

The answer is e. The infant in the question, who is completely healthy otherwise, appears to have simple, primary craniosynostosis (simple = only one suture; primary = not because of failure of brain growth). This condi¬tion is usually sporadic (occurring in births) and more commonly affects the sagittal suture resulting in scaphocephaly (a long and narrow skull). The therapy for this condition is controversial, but might include surgery; consultation with a neurosurgeon would be indicated.
A child whose examination or history is not normal (developmental delay, polydactyly or syndactly unusually shaped digits or eyes, etc.) is more likely to have a genetic defect as the cause of the craniosynostosis. In these latter groups of patients, chromosome evaluation for conditions such as Chotzen, Pfeiffer, Crouzon, Carpenter, or Apert syndromes is appropri-ate. An ultrasound of the head in the child in this question would be impossible; the anterior fontanelle is closed. Skeletal surveys are appropri¬ate for the child with suspected child abuse, a condition not likely in this child. Lumbar puncture for opening pressures is unnecessary as increased intracranial pressure is unlikely in this patient; if increased intracranial pressure were being considered, imaging (CT or MRI) prior to an LP would be considered.

Question 32

A 4-year-old child is observed to hold his eyelids open with his fingers and to close one eye periodically, especially in the evening. He has some trouble swallowing his food. He usually appears sad. although he laughs often enough. He can throw a ball, and he runs well. Which of the following is most likely to aid in the diagnosis?
a. Muscle biopsy
b. Creatine phosphokinase (CPK)
c. Effect of a test dose of edrophonium
d. Chest x-ray
e. Antinuclear antibodies (ANAs)

Answer

The answer is c. Myasthenia gravis is an autoimmune disorder in which circulating acetylcholine receptor-binding antibodies result in neuromuscular blockade. The earliest signs of myasthenia gravis are ptosis and weakness of the extraocular muscles, followed by dysphagia and facial muscle weakness. The distinguishing hallmark of this disease is rapid fatiguing of the involved muscles. The conduction velocity of the motor nerve is normal in this condition. When the involved muscle is repetitively stimulated for diagnostic purposes, the EMG shows a decremental response, which can be reversed by the administration of cholinesterase inhibitors. In older children this test is accomplished with edrophonium chloride, but it should be avoided in young infants, as cardiac arrhythmias may result (neostigmine would be used instead). Cholinesterase inhibitors are the primary therapeutic agents. Other therapeutic modalities for myas¬thenia gravis include immunosuppression, plasmapheresis. thymectomy (an enlarged thy i a us is frequently seen on chest x-ray), and treatment of hypothyroidism. CPK should be normal.

Question 33

A 3-year-old child is unconscious, with symmetrically small pupils. These findings occur in which of the following?
a. Pontine hemorrhage
b. Phenylephrine overdose
c. Trauma with resultant third nerve palsy
d. Atropine-induced coma
e. Cyclopentolate exposure

Answer

The answer is a. Pin¬t pupils are found in coma, narcotics use, pilocarpine treatment, Horner syndrome, pontine hemorrhage, posterior synechia formation, pes¬ticide or nerve gas exposure, and tertiary syphilis. Dilated pupils are seen with drugs such as epinephrine, phenylephrine, topical antihistamines/ vasoconstrictor combinations and atropine-like agents, and closed-angle glaucoma

Question 34

A 5-month-old child was normal at birth, but the family reports that the child does not seem to look at them any longer. They also report the child seems to “startle” more easily than he had before. Testing of his white blood cells identifies the absence of hexosaminidase A activity, confirming the diagnosis of which of the following?
a. Niemann-Pick disease, type A.
b. Infantile Gaucher disease
c. Tay-Sachs disease
d. Krabbe disease
e. Fabry disease

Answer

The answer is c. Children who have Tay-Sachs disease are characterized by progressive developmental deterioration; physical signs include macular cherry-red spots and exquisite and characteristic sensitivity to noise. Diagnosis of this disorder can be confirmed biochemi¬cally by the absence of hexosaminidase A activity in white blood cells. Tay-Sachs disease is inherited as an autosomal recessive trait; frequently, affected children are of Eastern European Jewish ancestry.

Question 35

Examination of the cerebrospinal fluid of an 8-year-old, mildly febrile child with nuchal rigidity and intermittent stupor shows the fol¬lowing: white blood cells 85/uL (all lymphocytes), negative Gram stain, protein 150 mg/dL, and glucose 15 mg/dL. A CT scan with contrast shows enhancement of the basal cisterns by the contrast material. Which of the following is the most likely diagnosis?
a. Tuberous sclerosis
b. Tuberculous meningitis
c. Stroke
d. Acute bacterial meningitis
e. Pseudotumor cerebri

Answer

answer is b: viral meningitis, tuberculous meningitis, meningeal leukemia, and medulloblastoma, all of which can cause pleocytosis as well as elevated protein and lowered glucose concentrations in cerebrospinal fluid (CSF). Of the four diseases (and the likely finding of this patient), tuberculous meningitis is associated with the lowest glucose levels in CSF. The CT scan with contrast can be an excellent clue for diag¬nosing tuberculous meningitis. Exudate in the basal cisterns that shows enhancement by contrast material is typical; tuberculomas, ringed lucencies, edema, and infarction can be apparent; and hydrocephalus can develop. Confirmation with culture is mandatory. The x-ray of the chest will be likely to show signs of pulmonary tuberculosis. A high index of sus¬picion is necessary to diagnose tuberculous meningitis early.
The cellular response to viral meningitis eventually will be predomi¬nantly lymphocytic. Cells found in the CSF of a child who has meningeal leukemia most commonly are lymphocytes or lymphoblasts. Children who have a medulloblastoma generally present with the signs and symptoms caused by a mass in the posterior cranial fossa; their pleocytotic cere-brospinal fluid contains unusual-appearing cells of the monocytic variety. In pseudotumor cerebri, the constituents of CSF are generally normal except for low protein content in some instances and high opening pressure. Acute bacterial disease must be considered for this patient, but typically causes polymorphonuclear cells and positive Gram stains. Neither tuberous sclero¬sis nor stroke typically cause these findings on CFS examination.

Question 36

An irritable 6-year-old child has a somewhat unsteady but nonspe­cific gait. Physical examination reveals a very mild left facial weakness, brisk stretch reflexes in all four extremities, bilateral extensor plantar responses (Babinski reflex), and mild hypertonicity of the left upper and lower extremities; there is no muscular weakness. Which of the following is the most likely diagnosis?

  1. Pontine glioma
  2. Cerebellar astrocytoma
  3. Tumor of the right cerebral hemisphere
  4. Subacute sclerosing panencephalitis
  5. Progressive multifocal leukoencephalopathy

Answer

The answer is a. A child who has a sub-acute disorder of the central nervous system that produces cranial nerve abnormalities (especially of cranial nerve VII and the lower bulbar nerves), long-tract signs, unsteady gait secondary to spasticity, and some behavioral changes is most likely to have a pontine glioma. Tumors of the cerebellar hemispheres can, in later stages, produce upper motor neuron signs, but the gait disturbance would be ataxia. Dysmetria and nystagmus also would be present. Supratentorial tumors are quite common in 6-year-old chil­dren; headache and vomiting likely would be presenting symptoms and papilledema a finding on physical examination. Subacute sclerosing panencephalitis is a rare disorder seen in children having experienced an episode of measles; extremely rare cases of SSPE can be associated with the vaccine. The patients have insidious behavior changes, deterioration in schoolwork, and finally dementia. No other findings are usually seen. Pro­gressive multifocal leukoencephalopathy, caused by a Human polyomavirus 2 infection, is usually found in patients with immune deficiencies; although still rare, it has become more common as the incidence of AIDS increased.

Question 37

A 2-year-old boy has been doing well despite his diagnosis of tetral¬ogy of Fallot. He presented to an outside emergency room a few days ago with a complaint of an acute febrile illness for which he was started on a “pink antibiotic.” His mother reports that for the past 12 hours or so he has had a headache and is more lethargic than normal. On your examination he seems to have a severe headache, nystagmus, and ipsilateral ataxia. Which of the following would be the most appropriate first test to order?
a. Urine drug screen
b. Blood culture
c. Lumbar puncture
d. CT or MRI of the brain
e. Stat echocardiogram

Answer

The answer is d. The patient in the question has a brain abscess, a condition more commonly seen in patients with cardiac defects that have right-to-left shunts associated with them. The antibiotics that he received from the emergency room kept the condition somewhat under control, but was unlikely to cure the condition, which ultimately progressed. The diag¬nostic tool of choice is imaging, and either CT or MRI is indicated. Lumbar puncture would be contraindicated in this patient until after imaging (the patient is at risk for brain herniation) and the CSF (and blood) cultures are usually negative. The patient is not experiencing new cardiac problems, so an echocardiogram is not indicated. If the patient has negative imaging of the brain, then a urine drug screen might be indicated, but not as a test of first choice.

Question 38

A 6-year-old child is hospitalized for observation because of a short period of unconsciousness after a fall from a playground swing. He has developed unilateral pupillary dilatation, focal seizures, recurrence of depressed consciousness, and hemiplegia. Which of the following is the most appropriate management at this time?
a. Spinal tap
b. CT scan
c. Rapid fluid hydration
d. Naloxone
e. Gastric decontamination with charcoal

Answer

The answer is b. Compression of cranial nerve III and distortion of the brainstem, resulting in unilateral pupillary dilatation, hemiplegia, focal seizures, and depressed consciousness, suggest a progressively enlarging mass, most likely an epidural hematoma. Such a hematoma displaces the temporal lobe into the tentorial notch and presses on the ipsilateral cranial nerve III. Brainstem compression by this additional tissue mass leads to progressive deterioration in consciousness. Rising blood pressure and falling pulse rate (“Cushing’s triad”) are characteristic of increasing intracranial pressure. The most urgent test to diagnose this condition is a CT scan. Spinal taps in a patient with evidence of an expanding intracranial mass are contraindicated and may result in herniation and death; this patient has no evidence of intravascular fluid depletion, and rapid fluid hydration may exacerbate the increased intracranial process. Naloxone is indicated for an opiate ingestion, but such an ingestion would be expected to produce bilateral pinpoint pupils. Gastric decontamination with char¬coal rarely is used, but would be indicated for some suspected ingestions.

Question 39

A 6-year-old boy is brought to the clinic with the complaint of poor growth. His growth curves demonstrate his linear development to be well below the fifth percentile. Upon close questioning, his family reports that he has had headaches in the past 2 or 3 weeks and occasional vomiting. Your physical examination reveals mild papilledema. Which of the follow-ing might also be expected to be seen in this patient?
a. Sixth nerve palsy
b. Unilateral cerebellar ataxia
c. Unilateral pupillary dilatation
d. Unilateral anosmia
e. Bitemporal hemianopsia

Answer

The answer is e. The findings of poor growth and papilledema could be ex-plained by a craniopharyngioma. This tumor is one of the most common supratentorial tumors in children, often causing growth failure through disruption of pituitary excretions such as growth hormone. Upward growth of a craniopharyngioma results in compression of the optic chiasm. Par¬ticularly affected are the fibers derived from the nasal portions of both retinas (i.e. from those parts of the eyes receiving stimulation from the temporal visual field). Early in the growth of a craniopharyngioma, a uni¬lateral superior quadrantanopic detect can develop, and an irregularly growing tumor can impinge upon the optic chiasm and cause homonymous hemianopia.

Question 40

A 6-year-old boy presents with the sudden onset of ataxia. Which of me following is the most likely cause?
a. Drug intoxication
b. Agenesis of the corpus callosum
c. Ataxia telangiectasia
d. Muscular dystrophy
e. Friedreich ataxia

Answer

The answer is a. Cerebellar ataxia in childhood can occur in association with infection, metabolic abnormalities, ingestion of toxins, hydrocephalus, cerebellar lesions, multiple sclerosis, labyrinthitis, polyradiculopathy, and neuroblastoma. Although the other listed diagnoses (except agenesis of the corpus callosum) can cause ataxia, symptoms would be more chrome in nature than the acute episode described. Ingestion (intentional or accidental) of barbiturates, phenytoin, alcohol, and other drugs also must be considered. Agenesis of the corpus callosum is usually diagnosed by imaging studies; however, it does not cause acute ataxia.

Question 41

A 9-year-old child has developed headaches that are more frequent in the morning and are followed by vomiting. Over the previous few months, his family has noted a change in his behavior (generally more irri¬table than usual) and his school performance has begun to drop. Imaging of this child is most likely to reveal a lesion in which of the following regions?
a. Subtentorial
b. Supratentorial
c. Intraventricular
d. In the spinal canal
e. In the peripheral nervous system

Answer

The answer is a. Between 50 and 60% of tumors of the nervous system in children 4 to 11 years old are infratentorial (posterior fossa) and include cerebellar and brainstem tumors, often either medulloblastoma or cerebellar astrocytoma. In adults and infants, most intracranial tumors originate above the tentorium; only 25 to 30% of brain tumors in adults are subtentorial.

Question 42

At birth, an infant is noted to have an abnormal neurologic examina¬tion. Over the next few weeks he develops severe progressive central ner¬vous system degeneration, an enlarged liver and spleen, macroglossia, coarse facial features, and a cherry-red spot in the eye. Which of the fol¬lowing laboratory findings most likely explains this child’s problem?
a. Reduced serum hexosaminidase A activity
b. Deficient activity of acid beta-galactosidase
c. A defective gene on the X chromosome
d. Complete lack of acid beta-galactosidase activity
e. Deficient activity of galactosyl-3-sulfate-ceramide sulfatase
(cerebroside sulfa-tase)

Answer

The answer is b. The cherry-red spot represents the center of a normal retinal macula that is sur¬rounded by ganglion cells in which an abnormal accumulation of lipid has occurred, thus altering the surrounding retinal color so that it is yellowish or grayish white; it is seen more often in such disorders as GM1 generalized gangliosidosis type 1, Sandhoff disease, and Niemann-Pick disease type A, in which there is lipid material deposited in the ganglion cells. Generalized gangliosidosis type 1 (type 1 GM1 gangliosidosis) presents as noted in the question, with symptoms often present at birth; these infants have a com¬plete lack of acid beta-galactosidase activity. Other findings with general¬ized gangliosidosis type 1 (and not listed in the question) include gingival hyperplasia, hernias, joint stiffness, dorsal kyphosis, and edema of the ex¬tremities. Hexosaminidase A deficiency (GM2 gangliosidosis. type 1, or Tay-Sachs disease) presents as psychomotor retardation and hypotonia beginning at about 6 to 12 months of age; the children are usually normal at birth. A pronounced startle reflex and severe hyperacusis, seizures, loss of vision (with cherry-red macular spots), and macrocephaly are seen.
Reduced activity of alpha-galactosidase (Fabry’s disease) presents in older children as acroparesthesia (numbness or tingling in one or more extremities), intermittent painful crises of the extremities or the abdomen, frequently low-grade fevers, and sometimes cataracts. Patients with Rett syndrome (the etiology of which has been traced to a defective gene on the X chromosome) present as normal children at birth, but then have a rapid decline in motor and cognitive functions beginning between 6 and 18 months of age. Affected girls demonstrate loss in the use of their hands and loss in their ability to communicate and socialize.
Metachromatic leukodystrophy (deficient activity of galactosyl-3-sulfateceramide sulfatase) has its onset between 1 and 2 years of age and is notable for progressive ataxia, weakness, and peripheral neuropathy. In this disorder, gray macular lesions can be seen that look somewhat similar to cherry-red spots.

Question 43

An 18-month-old child is brought to the emergency center after the family reports she had a “seizure.” They note that the child was in her nor¬mal good state of health at bedtime, but when they went to investigate noises they heard coming from her room they found her having a “seizure.” They picked her up to put her on the floor and noticed that she was very warm. Her “seizure” resolved, and by the time they have reached the emergency room she appears to be back to her normal state of good health. Which of the following statements portends the most positive outcome?
a. Mild pleocytosis is found in the cerebrospinal fluid (CSF)
b. The child has an otitis media on examination, but is otherwise healthy
c. The seizure lasted 30 minutes
d. The child was born prematurely with an intraventricular hemorrhage
e The family reports the child to have had right-sided tonic-clonic activity only

Answer

The answer is b. The child in the question likely had a febrile seizure. Febrile seizures usually occur in children between the ages of 9 months and 5 years, generally in association with upper respiratory illness, roseola, shigellosis, or gastroenteritis. The generalized seizures are mostly brief (2 to 5 min), and the cerebrospinal fluid is normal. Infants who have seizures that are prolonged (longer than 15 min), focal, or lateralized, or who had neurologic problems before the febrile seizure, are at a higher risk of developing an afebrile seizure disorder during the subsequent 5 to 7 years.

Question 44

A 4-year-old patient in coma with severe brain injury has developed very dilute urine with an output of 100 mL/h. This condition can be managed by which of the following?
a. Replacing excessive urine output with a solution of 5% glucose and normal saline
b. Fluid restriction
c. Oral (or nasogastric) hypoglycemic agents
d. Giving insulin
e. Giving DDAVP (synthetic vasopressin) intranasally

Answer

The answer is e. The child in the question likely has devel¬oped diabetes insipidus, a common complication of severe head trauma, due to a deficiency in secretion of antidiuretic hormone. It must be distin¬guished from the polyuria that can occur several days after head injury, as the high antidiuretic hormone levels associated with the head injury resolve. Diabetes insipidus associated with head trauma can be treated by replacement of urinary losses intravenously or enterally with solutions low in sodium as long as it is possible to keep up with the losses. Alternatively,: vasopressin given as an intranasal preparation makes care for these patients easier and safer as long as water balance is carefully monitored.

Question 45

A 3-year-old boy’s parents complain that their child has difficulty walking. The child rolled, sat, and first stood at essentially normal ages and first walked at 13 months of age. Over the past several months, however, the family has noticed an increased inward curvature of the lower spine as he walks and that his gait has become more “waddling” in nature. On examination, you confirm these findings and also notice that he has enlargement of his calves. Which of the following is the most likely diagnosis? :
a. Occult spina bifida
b. Muscular dystrophy
c. Brain tumor
d. Guillain-Barre syndrome
e. Botulism

Answer

The answer is b. The most common form of muscular dystrophy is Duchenne muscular dystrophy. It is inherited as an X-linked recessive trait. Male infants are rarely diagnosed at birth or early infancy since they often reach gross milestones at the expected age. Soon after beginning to walk, however, the features of this disease become more evident. While these children walk at the appropriate age, the hip girdle weakness is seen by age 2. Increased lordosis when standing is evidence of gluteal weakness. Gower sign (use of the hands to “climb up” the legs in order to assume the upright position) is seen by 3 to 5 years of age. as is the hip waddle gait. Ambulation ability remains through about 7 to 12 years of age. after which use of a wheelchair is common. Associated features include mental impairment and cardiomyopathy. Death due to respiratory failure, heart failure, pneu¬monia, or aspiration is common by early adulthood.

Question 46

About 12 days after a mild upper respiratory infection, a 12-year-old boy complains of weakness in his lower extremities. Over several days, the weakness progresses to include his trunk. On physical examination, he has the weakness described and no lower extremity deep tendon reflexes, mus¬cle atrophy, or pain. Spinal fluid studies are notable for elevated protein only. Which of the following is the most likely diagnosis in this patient?
a. Bell palsy
b. Muscular dystrophy
c. Guillain-Barre syndrome
d. Charcot-Marie-Tooth disease
e. Werdnig-Hoffmann disease

Answer

The answer is c. The paralysis of Guillan-Barre often occurs about 10 days after a nonspecific viral illness. Weakness is gradual over days or weeks, beginning in the lower extremities and progressing toward the trunk. Later, the upper limbs and the bulbar muscles can be¬come involved. Involvement of the respiratory muscles is life-threatening. The syndrome seems to be caused by a demyelination in the motor nerves and, occasionally, the sensory nerves. Measurement of spinal fluid protein is helpful in the diagnosis; protein levels are increased to more than twice normal, while glucose and cell counts are normal. Hospitalization for observation is indicated. Treatment can consist of observation alone, intra¬venous immunoglobulin, steroids, or plasmapheresis. Recovery is not always complete. Bell palsy usually follows a mild upper respiratory infec¬tion, resulting in the rapid development of weakness of the entire side of the face. Muscular dystrophy encompasses a number of entities that in-clude weakness over months. Charcot-Marie-Tooth disease has a clinical onset including peroneal and intrinsic foot muscle atrophy, later extending to the intrinsic hand muscles and proximal legs. Werdnig-Hoffmann dis-ease is an anterior horn disorder that presents either in utero (in about one-third of cases) or by the first 6 months of life with hypotonia, weakness, and delayed developmental motor milestones.

Question 47

Your 6-year-old son awakens at 1:00 a.m. screaming. You note that he is hyperventilating, is tachycardic, and has dilated pupils. He cannot be consoled, does not respond, and is unaware of his environment. After a few minutes, he returns to normal sleep. He recalls nothing the following morning. Which of the following is the most likely diagnosis?
a. Seizure disorder
b. Night terrors
c. Drug ingestion
d. Psychiatric disorder
e. Migraine headache

Answer

The answer is b. Night terrors are most common in boys. The child awakens suddenly appears frightened and unaware of his surroundings, and has the clinical signs outlined in the question. He cannot be consoled by the parents. After a few minutes, sleep returns, and the patient cannot recall the event in the morning. Sleepwalk¬ing is common in these children. Exploring the family dynamics for emotional disorders may be helpful; usually pharmacologic therapy is not required, and family reassurance is indicated.

Question 48

A 8 month old infant is noted to have developed constipation over the past week, and then facial diplegia and difficulty sucking and swallowing. The child has been colicky, and the maternal grandmother has been treating the child with a mixture of weak tea, rice water, and honey. Which of the fol¬lowing disorders is the most likely cause?
a. Infantile spinal muscular atrophy
b. Myasthenia gravis
c. Congenital myotonic dystrophy
d. Duchenne muscular dystrophy
e. Botulism

Answer

The answer is e. Although the other possibilities listed in the question may be the etiology, it seems most likely that botulism is the most likely. Botulism clearly has been associated with the inges¬tion of raw honey, and in young children often presents with constipation before the development of the other symptoms listed; constipation is an unusual presenting feature in the other disorders.
Constipation in a young infant is a common complaint of parents, and potentially is unrelated to this child’s problem. Some of the other diagnos-tic considerations listed in the question are possibilities if the constipation is a red herring. Spinal muscular atrophy in a neonate is associated with hypotonia and feeding difficulties; a muscle biopsy can confirm this diag-nosis. Neonatal myasthenia gravis, although uncommon, must be consid-ered in a newborn infant who has the symptoms described in the question. The symptoms presented (excepting prominent constipation) also could represent myotonic dystrophy; this diagnosis is confirmed by examination of both parents for percussion and grip myotonia, and by electromyographic depiction of myotonic discharges. Duchenne (pseudohypertrophic) muscular dystrophy clinically appears in children who are about 2 or 3 years of age.

Question 49

An 18-year-old female presented with bilateral ptosis and tiredness towards afternoons. She had a short tensilon (edrophonium test which was positive). A diagnosis of myasthenia Gravis was made and she was stated on pyridostigmine. She now relapses and is given edrophonium intravenously. However, her condition deteriorates and her forced expiratory volume falls to 1.0. She is transferred to the high dependency units. An initial CT scan and chest x-ray were normal.
What should be the next management step?

a) Azathioprine
b) Emergency thymectomy
c) Intravenous methylprednisolone
d) Neostigmine
e) Plasmapheresis

Answer

The answer is e
The diagnosis is Myasthenia crisis. The treatment of choice is either iv immunoglobulins or plasma exchange. Transfer to ICU is essential because patients may deteriorate rapidly needing intubation and ventilation.

Question 50

A 17-year-old man has been diagnosed with schizophrenia 4 weeks ago. He was started on haloperidol.
Two weeks later he was found confused and drowsy. On examination he was pyrexial (40.7 C), rigid with
blood pressure of 200/100. Which of the following treatment will you initiate?

a) phenytoin
b) diazepam
c) cefuroxime
d) acyclovir
e) dantrolene

Answer

the answer is e
Neuroleptic malignant syndrome is the most likely diagnosis. Its major features are rigidity, altered
mental state, autonomic dysfunction, fever, and high creatinine kinase. It is usually caused by potent
neuroleptics. The treatment of choice is dantrolene and bromocriptine. Withdrawal of neuroleptic
treatment is mandatory. Rhabdomyolysis and acute renal failure are potential complications.

Question 51

Which visual field defect is most likely to occur with multiple sclerosis?

a) bitemporal hemianopia
b) central scotoma
c) homonymous hemianopia
d) increased blind spot
e) tunnel vision

Answer

the answer is b
Central scotoma likely with retrobulbar neuritis and optic atrophy. Tunnel vision occurs in glaucoma,
retinitis pigmentosa and retinal panphotocoagulation. Increased blind spot occurs with papilloedema,
which may lead to optic atrophy. Optic chiasma compression causes bitemporal heminopia.

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