Neurologic Disorders


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Neurologic Disorders

  1. 1. TAB W – NEUROLOGIC DISORDERS PEDIATRIC QUESTIONS AND ANSWERS 1. A previously healthy 8-month-old boy who lives on a farm is brought to you because of progressive lethargy, loss of developmental milestones, constipation, and recent feeding problems. The child is hypotonic with flaccid extremities; head control and sucking are both poor. This child has likely been fed: A) canned meat preparation for infants. B) homemade sausage. C) unpasteurized cow’s milk. D) goat’s milk. E) honey. ANSWER: E Infants under 1 year of age have been found to have severe infections with type B botulism from ingestion of honey. The neurologic deficits (progressive lethargy, loss of developmental milestones, constipation, and hypotonia with flaccidity and feeding problems) found in this patient are classical for infant botulism. Appropriate supportive management and IV hydration therapy will usually allow the child to recover. Home-prepared sausage, unpasteurized cow’s milk, canned meat preparation for infants, and goat’s milk are not associated with this neurologic picture. Honey should not be given to children less than one year of age. Botulism has also been associated with home canned jalapeños. Ref: Infant botulism-Massachusetts. MMWR 1984; 33:165-166.2) Rudolph AM (ed): Pediatrics, ed 18. Appleton & Lange, 1987, pp 1668-1669. 2. A 5-year-old black male was witnessed to fall off his bicycle and hit the back of his head on a hard surface. There was no loss of consciousness. No other injury was noted. He was obviously agitated and restless, and his only complaint was a loss of vision. When you see him, his examination is unremarkable except for moderate swelling over the occipitoparietal area of the scalp. His skin is intact, and no gross neurologic deficit is noted except for the visual loss. A CT scan is negative. An EEG shows only slight slowing of activity. Which one of the following statements is true regarding this patient? A) The loss of vision is probably caused by damage to the optic nerve. B) It will be months before the child’s vision returns. C) The child should be hospitalized for 72 hours for further observation. D) The child’s vision will probably return within 24 hours. E) The child should have a referral to Child Protective Services Division of the Department of Protective Regulatory Services. ANSWER: D Transient cortical blindness following mild head trauma is usually associated with a benign outcome. The special features are mild head trauma, no loss of consciousness, onset of blindness occurring within hours of the trauma, duration of blindness less than 24 hours, absence of skull fracture or visible injury on CT scan, and no other neurologic deficits. The EEG shows initial slowing with normalization on follow-up. This case describes an accident, and is a witnessed event with the history compatible with the physical findings. In child abuse [(non- accidental trauma (NAT)], legislation in all fifty states mandates expedient reporting to authorities of all suspected cases of abuse and/or neglect. Ref: Yamamoto LG, Bart RD, Jr: Transient blindness following mild head trauma. Clin Pediatr 1988; 27(10): 479-483. Neurologic Disorders - page 1 of 10
  2. 2. 3. A 5-year-old child has just been hospitalized with meningococcemia. Family members and close contacts should be given which one of the following? A) Penicillin orally B) Rifabutin (Mycobutin) orally C) Erythromycin orally D) Rifampin (Rifadin) orally E) No chemoprophylaxis ANSWER: D Exposed household, school, or day-care contacts of children with meningococcemia should receive chemoprophylaxis as soon as possible. The drug of choice is rifampin (10 mg/kg, up to 600 mg) every 12 hours for 48 hours. Rifampin concentrates in the nasal secretions and thus is effective in the nasopharyngeal eradication. Rifampin recipients often have orange stained urine and tears, and contacts may be permanently stained. Sulfisoxazole may be used for the unusual meningococcus susceptible to sulfonamides. Ceftriazone and ciprofloxacin are alternative choices for adolescents and adults. Ref: Behrman RE, Kliegman RM (eds): Nelson Textbook of Pediatrics, ed 14. WB Saunders Co, 1992, p 716. 4. A 2-year-old brought to you because of a high fever is having convulsion. Attempts at establishing an intravenous line are unsuccessful. Which one of the following agents has the most established efficacy when given rectally? A) Diazepam (Valium) B) Lorazepam (Ativan) C) Phenytoin (Dilantin) D) Phenobarbital E) Valproic acid (Depakene) ANSWER: A Rectal solutions of diazepam provide prompt and reliable absorption. Peak levels are reached in 10 minutes. The rectal dose is 0.5 mg/ kg, with 20 mg the maximum recommended dose. It is common practice in England to allow parents to administer rectal diazepam to children with recurrent febrile seizures either as a prophylactic with acute fever or to stop a recurrent complex febrile seizure. The drug should not be given if the seizure has stopped. Lorazepam is useful when given intravenously during acute convulsions. Some consider it the drug of choice. It does not currently have an established record of use by the rectal route. Phenytoin and phenobarbital are useful when given parentally or orally for seizure control, but are not administered rectally. Valproic acid has been administered rectally, but its absorption is slow and therefore not acceptable for acute seizures. Ref: Treatment of convulsive status epilepticus: Recommendations of the Epilepsy Foundation of America’s Working Group on Status Epilepticus. JAMA 1993; 270(7):854-859. 2) Valman HB: ABC of one to seven: Febrile convulsions. BMJ 1993;306(6894):1743-1748. 5. Which one of the following anti-seizure medications is used exclusively for absence (petit mal) seizures? A) Ethosuximide (Zarontin) B) Gabapentin (Neurontin) C) Primidone (Mysoline) D) Pheriobarbital E) Phenytoin (Dilantin) ANSWER: A Ethosuximide has proved to be a safe and efficacious agent that is used exclusively for absence (petit mal) seizures. The other drugs listed are used for other types of seizures. Ref: Rakel RE (ed): Conn's Current Therapy 2000. WB Saunders Co, 2000, pp 860-861. (ABFP, 2000) 6. A 20-month-old male is seen by you approximately 1 hour after having a generalized seizure that lasted “2 or 3 minutes” according to his mother. His past medical history is unremarkable except for two episodes of otitis media. On examination you note that he has a Neurologic Disorders - page 2 of 10
  3. 3. fever of 38.9C (102.0F), and is awake, interactive, and consolable, with obvious otitis media of the left ear. Neurologic examination is unremarkable and there are no meningeal signs. Which one of the following would now be most appropriate? A) Lumbar puncture B) Electroencephalography C) Neuroimaging studies D) Serum levels of electrolytes, calcium, phosphate, and magnesium, plus a blood glucose level and a CBC E) No diagnostic studies at this time ANSWER: E This patient has experienced a classic simple febrile seizure and no additional diagnostic studies are recommended. A lumbar puncture following a seizure is not routinely recommended in a child over 18 months old, since by that age a patient with meningitis would be expected to demonstrate meningeal signs and symptoms or clinical findings suggesting an intracranial infection. There is no evidence to suggest that routine blood tests or neuroimaging studies are useful in a patient following a first simple febrile seizure, and it has not been shown that electroencephalography performed at the time of presentation or within the following month will predict the occurrence of future afebrile seizures. Ref: Provisional Committee on Quality Improvement, Subcommittee on Febrile Seizures: Practice parameter: The neurodiagnostic evaluation of the child with a first simple febrile seizure. Pediatrics 1996;97(5): 769-772. 7. A 9-year-old white female is brought to your office shortly after a fainting spell at school. The child reported a sense of warmth, nausea, and feeling “funny in the head” before falling down from a standing position. The episode occurred during assembly in the school auditorium where the child had been scheduled to sing a solo. There is no family or personal history of cardiac disease, exercise-induced problems, or palpitations. The most likely cause of this episode is: A) vasovagal syncope. B) a seizure disorder. C) panic disorder. D) hypertrophic cardiomyopathy. E) long QT syndrome. ANSWER: A Vasovagal syncope is a common cause of syncope in children. It is often accompanied by a sense of warmth, nausea, lightheadedness, and visual “grayout.” Vasovagal syncope often occurs in hot, humid, or crowded settings, and can be triggered by stressful situations, mild physical trauma, or the sight of blood. Ref: Gutgesell HP, Barst RJ, Humes RA, et al: Common cardiovascular problems in the young: Part I. Murmurs, chest pain, syncope and irregular rhythms. Am Fam Physician 1997:56(7): 1825-1830. Neurologic Disorders - page 3 of 10
  4. 4. 8. In a majority of patients, which one of the following types of tremor can be treated effectively with beta-adrenergic blockers? A) Essential tremor B) Tremor with peripheral neuropathy C) cerebellar postural tremor D) Orthostatic tremor E) Tremor at rest ANSWER: A Proper therapy of tremor depends on an accurate diagnosis. The tremor at rest of Parkinson’s disease or other Parkinsonian states improves with the use of dopaminergic agents or anticholinergic agents. Essential tremor responds in 70% of cases to beta-adrenergic blockers. Clonazepam appears useful in orthostatic tremor. In some cases, severe cerebellar postural tremor can be treated with isoniazid in combination with pyrodoxine hydrochloride. Carbamazepine has been useful in a small number of cases. Patients with the tremor of peripheral neuropathy have slowing of motor conduction velocity and are not helped by beta blockers. Ref: Hallett M: Classification and treament of tremor. JAMA 1991;266(8): 1115-1117. 2) Fauci AS, Braunwald E, Isselbacher KJ, et al (eds): Harrison’s Principles of Internal Medicine, ed 14. McGraw-Hill, 1998, p 2359. 9. The parents of a 2-year-old come in to discuss their child’s febrile seizures. The child has experienced four seizures, each associated with fever (usually from an ear infection). Each seizure lasted less than 2 minutes and was generalized tonic-clonic. The child was usually postictal for about 60 minutes but then returned to his normal level of mental function. The parents are concerned about the long-term significance for these seizures, specifically about any permanent brain damage and retardation. They ask if their child should be on medication to prevent the seizures. Which one of the following should you tell them? A) Children with a history of febrile seizures usually go on to a more complicated seizure pattern as they age. B) Children with a history of febrile seizures typically perform less well on standardized school tests. C) Children with febrile seizures typically are growth retarded. D) Children with febrile seizures are at greater risk for premature death than the general public. E) Most children who experience febrile seizures develop normally. ANSWER: E Febrile seizures are frightening to parents and frustrating to health care providers. They are nearly impossible to predict and to control. In the past, phenobarbital and other anti-seizure medications were used in an attempt to control or totally prevent febrile seizures. Unfortunately, these medications had multiple side effects, not the least of which was sedation. Parents and physicians have been concerned about the long-term consequences of these seizures. A 10-year follow-up study of 398 school-age children in England showed that children who experienced simple febrile seizures did equally well with their classmates and had no increased incidence of subpar intellectual development. Ref: Verity CM, Greenwood R, Golding J: Long-term intellectual and behavioral outcomes of children with febrile convulsions. N Engl J Med 1998;338(24): 1723-1728. Neurologic Disorders - page 4 of 10
  5. 5. 10. A 7-year-old white female is brought to your office because of a 3 day history of an increasingly clumsy gait. The mother noted that she first observed the problem 2 days earlier when her daughter climbed out of the bathtub and had difficulty standing up while being dried off. At the time she thought the child was just playing. The next day the child seemed better and went to school, but was sent home because she was having difficulty on the playground and said that “it was hard” going up the stairs. The child had a minor upper respiratory illness, as did other family members, but otherwise was not particularly sick. However, on the morning of this office visit, the child was unable to stand up when she got out of bed and had difficulty using her spoon to eat her cereal. She had no difficulty swallowing. The family reports she never had any similar symptoms. The family lives in a rural area near woods; she has two older brothers. Family pets include two dogs, a cat, and a parakeet. No family member or animals are sick. Blood counts, chemistries, urinalysis, cerebrospinal fluid, CT scan of the head, and a chest roentogenogram are all normal. Examination is within normal limits, except for a slightly runny nose which drains clear, watery mucus. There is no evidence of ear infection or sinusitis. She has profound weakness, but not total paralysis, of the lower extremities, and to a lesser extent, the upper extremities. There is no sensory deficit and no difficulty with urination. A tick is found in her scalp. Which one of the following statements is true concerning this patient’s condition? A) The most likely diagnosis is tick paralysis; the tick should be removed and the patient followed. B) Since the patient had progressive symptoms for 3 to 4 days, removing the tick at this point will probably not lead to total recovery. C) Tick paralysis is fatal to the majority of patients. D) Treatment includes physical therapy and broad-spectrum antibiotics. E) The paralysis is caused by a toxin produced by Babesia microti. ANSWER: A With all laboratory tests being normal, finding a tick on this patient strongly suggests that the diagnosis is indeed tick paralysis. Untreated, tick paralysis can have 10% to 12% mortality rate. The paralysis is thought to be caused by a neurotoxin produced by the tick’s salivary glands, and not an infection. Thus, recovery is usually prompt when the tick is removed, and neither physical therapy nor broad-spectrum antibiotics would be necessary to hasten recovery. Babesia microti causes babesiosis, and paralysis is not part of its symptom complex. Ref: 1) Spach DH, Liles WC, Campbell GL, et al: Tick-borne diseases in the United States. N Engl J Med 1993; 3296 (13): 936-947. 2) Fauci AS, Braunwald E, Isselbacher KJ, et al (eds): Harrison’s Principles of Internal Medicine, ed 14. McGraw-Hill Inc, 1998, pp 1188-1189, 2549. 11. A 4-month-old infant is brought to your office for well baby care. She was born elsewhere, 6 weeks prematurely, and has just been adopted. An examination results in normal findings, with the exception of a head circumference of 45 cm, which is well over the 95th percentile. Developmental screening is normal. You order a CT scan which shows dilated ventricles. You can inform the parents A) that the diagnosis was evident at birth but was missed by the physician at that time. B) that there is an increased risk for a variety of developmental disabilities. C) that the defect was most likely caused by a maternal infection such as toxoplasmosis. D) that with modern neurosurgical shunting procedures their child will most likely develop normally. E) that this is likely to recur in subsequent siblings born at term. ANSWER: B The clinical presentation of hydrocephalus is variable and depends on many factors. In an infant, an accelerated rate of enlargement of the head is the most prominent sign. A CT scan and/or an MRI along with ultrasound are the most important studies to identify the specific cause of hydrocephalus. Neurologic Disorders - page 5 of 10
  6. 6. The prognosis depends on the cause of the dilated ventricles and not on the size of the cortical mantle at the time of operative intervention. Hydrocephalic children are at increased risk for a variety of developmental disabilities. The mean IQ is reduced compared with the general population, particularly for performance tasks as contrasted to verbal abilities. Most children have abnormalities in memory function, and visual problems are common. Ref: Behrman RE, Kliegman RM, Arvin AM (eds): Nelson Textbook of Pediatrics, ed 15. WB Saunders Co, 1996, pp 1683-1685. DIRECTIONS: Below is a multiple true-false problem which consists of a stem and four or five lettered options. For each of these lettered options, indicate “T” if the option is true or “F” if the option is false. 12. An 8-year-old white male is seen because he complains of frequent headaches. For the past 9 months, he has experienced bifrontal headaches, sometimes with associated photophobia, phonophobia, and nausea, averaging twice weekly. His symptoms are refractory to both acetaminophen and Motrin and usually dissipate with sleep. His father had “migraine headaches” as a college student. The patient has a lifelong history of motion sickness. His physical examination, including measurement of blood pressure and careful neurologic assessment, is normal. Which of the following would be considered suitable initial therapy for this patient? A) Propranolol (Inderal) B) Amitriptyline (Elavil) C) Cyproheptadine (Periactin) D) Methysergide (Sansert) E) Zoloft ANSWERS: A) True, B) True, C) True, D) False, E) True This patient likely has common migraine headaches, judging from the characteristics of the headaches, personal and family history, and normal examination. In children, common migraine headaches are the most commonly experienced migraines and are typically bifrontal; classic migraines are experienced less frequently and the two are typically unrelated. Given the frequency of the headaches, prophylactic therapy for migraines is very reasonable. Suitable drugs include propranolol, amitriptyline, cyproheptadine, and zoloft. The use of methysergide in children is controversial, and would therefore not be appropriate as initial therapy. Ref: Oski FA (ed): Principles and Practice of Pediatrics. JB Lippincott Co, 1990, p 1948. 13. A 17-year-old white male has been in rehabilitation for C6 complete quadriplegia. His health had been good prior to a diving accident 2 months ago which caused his paralysis. The patient has been catheterized since admission and his recovery has been steady. His vital signs have been normal and stable. The nurse calls you and tells you that for the past hour the patient has experienced seating, rhinorrhea, and a pounding headache. His heart rate is 55/min and his blood pressure is 220/115 mm Hg. His temperature and respirations are reported as normal. There has been no vomiting and his neurologic examination is unchanged. The most likely diagnosis is: A) cluster headache. B) autonomic hyperreflexia. C) sepsis. D) intracranial hemorrhage. E) progression of the spinal cord lesion. ANSWER: B Neurologic Disorders - page 6 of 10
  7. 7. Autonomic hyperreflexia is characterized by the sudden onset of headache and hypertension in a patient with a lesion above the T6 level. There may be associated bradycardia, sweating, dilated pupils, blurred vision, nasal stuffiness, flushing, or piloerection. It usually occurs several months after the injury and has an incidence as high as 85% in quadriplegic patients. Frequently, it subsides within 3 years of injury, but it can recur at any time. Bowel and bladder distention are common causes. Hypertension is the major concern because of its potential sequelae of cerebral hemorrhage and associated seizures or neuronal injury. Cluster headaches have a constant unilateral orbital localization. The pain is steady (non-throbbing) and lacrimation and rhinorrhea amy be part of the syndrome. Sepsis is usually manifested by chills, fever, nausea, and vomiting. Common signs include tachycardia and hypotension rather than bradycardia and hypertension thus decreasing the likelihood of this diagnosis. There are no neurologic findings or history which suggest progression of the patient’s lesion at C6. Ref: Braunwald E, Fauci AS, Kasper DL, et al (eds): Harrison’s Principles of Internal Medicine, ed 15. McGraw-Hill, 2001, pp 70, 78, 799, 2385, 2419. (ABFM 2002) 14. When evaluating a pediatric patient on chronic phenytoin (Dilantin) therapy for a seizure disorder, which one of the following is a sign of toxicity? A) Peripheral neuropathy B) Ataxia C) Clonus D) Ballistic movements E) Photophobia ANSWER: B Pediatricians and family physicians will follow patients with seizure disorders well controlled by phenytoin (Dilantin) monotherapy. However, due to its many side effects, careful monitoring of patients receiving phenytoin is required. Screening for ataxia, which is often subtle, must be performed at each visit, even when following blood levels at regular intervals. Ref: Victor M, Ropper AH: Adams and Victor’s Principles of Neurology, ed 7. McGraw-Hill, 2001, pp 356-361. (ABFM 2002) 15. Which of the following therapeutic agents is most appropriate for daily use in the prevention of migraine headaches of adolescents? A) Dihydroergotamine (DHE 45) B) Amitriptyline (Elavil) C) Sumatriptan (Imitrex) D) Aspirin/caffeine/butalbital (Fiorinal) E) Acetaminophin/ hydrocodone bitartrate (Vicodin) ANSWER: B Antidepressants, β-adrenergic blockers, anticonvulsants, calcium channel blockers, NSAIDS, and serotonin antagonists are the major classes of drugs used for preventive migraine therapy. All of these medications result in an approximately 50% reduction in the frequency of headaches. The other drugs listed are useful for the treatment of acute migraine, but not for prevention. Ref: 1) Noble SL, Moore KL: Drug treatment of migraine: Part II, Preventive therapy. Am Fam Physician 1994:50(6):1325-1332. 2) Goadsby PJ, Lipton RB, Ferrari MD: Migraine – Current understanding and treatment. N Engl J Med 2002:346(4):257-270. (ABFM 2002) Neurologic Disorders - page 7 of 10
  8. 8. 16. A 17-year-old white male with cerebral palsy who also has a poorly controlled major seizure disorder and a 6-week history of recurrent fever, anorexia, and persistent coughing visits your office. On physical examination he is noted to have a temperature of 38.3 C (101.0 F), a respiratory rate of 16/min, gingival hyperplasia, and a fetid odor to his breath. Auscultation of the lungs reveals rales in the mid-portion of the right lung posteriorly. Which one of the following is most likely to be found on a chest radiograph? A) Sarcoidosis B) Miliary calcifications C) Lung abscess D) Right hilar mass E) Right pleural effusion ANSWER: C Anaerobic lung abscesses are most often found in a person predisposed to aspiration who complains of a productive cough associated with fever, anorexia, and weakness. Physical examination usually reveals poor dental hygiene, a fetid odor to the breath and sputum, rales, and pulmonary findings consistent with consolidation. Patients who have sarcoidosis usually do not have a productive cough and have bilateral physical findings. A persistent productive cough is not a striking finding in disseminated tuberculosis, which would be suggested by miliary calcifications on a chest film. The clinical presentation and physical findings are not consistent with a simple mass in the right hilum nor with a right pleural effusion. Ref: 1) Goldman L, Bennett JC (eds): Cecil Textbook of Medicine, ed 21. WB Saunders Co, 2000, pp 439-441. 2) Braunwald E, Fauci AS, Kasper DL, et al (eds): Harrison’s Principles of Internal Medicine, ed 15. McGraw-Hill, 2001, pp 1013-1014, 1478. (ABFM 2002) 17. You are called to the hospital to examine a male infant born 12 hours earlier. The mother had noticed that the baby was not moving one of his arms, and asked that you be called immediately. The mother had a difficult vaginal delivery after a labor of 18 hours. This is her first child. Apgar scores were 7 and 9. The infant weighs 4800 g (10 lb 9 oz). When you examine the baby, you elicit a Moro response and note the infant’s left arm stays motionless by his side, remarkably different from the normal movement of the right arm. The remainder of the examination is within normal limits. Your tentative diagnosis is Erb-Duchenne palsy. Other diagnoses associated with this neurological finding include: 1. fracture of the left clavicle. 2. intracranial hemorrhage. 3. hypoglycemia. 4. pyridoxine deficiency. Findings in this infant which are associated with an increased risk for Erb-Duchenne palsy include: 5. birth weight. 6. length of labor. 7. mother’s parity. 8. Apgar scores. Appropriate advice to the parents would include which of the following? 9. Physical therapy with passive range of motion of the shoulder is indicated. 10. The majority of infants with this condition have permanent residual arm weakness. ANSWERS: 1) T F F F 2) F 5) T 8) F 3) F 6) T 9) T 4) 7) 10) Neurologic Disorders - page 8 of 10
  9. 9. Of the upper brachial plexus root injuries, Erb-Duchenne palsy is the most common. The differential diagnosis includes fractures of the clavicle or humerus. Intercranial hemorrhage, hypoglycemia, and pyridoxine deficiency cause generalized neuromuscular problems rather than isolated limb findings. Obstetric and fetal factors that may lead to brachial plexus injuries include multiparty, prolonged or augmented labor, abnormal presentations, shoulder dystocia, increased birth weight, and signs of fetal distress with low Apgar scores. In this case, the only factors present were prolonged labor and increased birth weight. . Therapy should begin after the first week (to allow for resolution of initial inflammation), using regular passive range-of-motion exercises plus hand and wrist splinting when appropriate. Generally, the prognosis depends on the severity of the injury. However, complete recovery of function is seen in over 90% of infants with brachial plexus injuries, usually by 3 months of age Ref: Taeusch HW, Ballard RA: Avery’s Diseases of the Newborn, ed. 7. WB Saunders Co, 1998, pp 865-866. 2) McMillan JA, DeAngelis CD, Feigin RD, et al (eds): Oski’s Pediatrics: Principles and Practice, ed 3. Lippincott Williams & Wilkins, 1999, p 2122. (ABFM 2002) 18. A 10-year-old white male with a family history of migraine has experienced three migraine attacks. The first attack was at age 7, and the second was 2 years later. In each of these attacks the child exhibited the symptoms of migraine with aura in the form of fortification spectra for 15-20 minutes. These visual symptoms were followed by a severe throbbing headache distributed globally over the entire head. He had another identical episode 2 weeks ago. His physical examination is normal. At this time, the primary consideration in your selection of an appropriate treatment regimen should be which one of the following? A) The presence or absence of an aura B) The duration of symptoms with each attack C) The frequency of attacks D) The patient's body mass index (BMI) E) Any onset of new headaches in siblings ANSWER: C Migraine in children is diagnosed and managed much the same way as in adults. Laboratory or imaging studies are usually not needed unless there are questionable findings in the history or physical examination. The main treatment consideration is whether to treat each acute attack or to try to prevent future attacks. The choice depends on the frequency of the headaches and their impact on the child's daily activities. The use of prophylactic drugs should be strongly considered if a child experiences more than 2-4 severe episodes monthly or is unable to attend school regularly. While multiple drug regimens are available for both abortive and prophylactic treatment, it is always advisable to initiate management with a thorough attempt to eliminate certain migraine-activating stimuli, such as stress, fatigue, anxiety, triggering foods, flashing lights, excessive physical exertion, mild head trauma, loud noises, and motion sickness. Ref: McMillan JA, DeAngelis CD, Feigin RD, et al (eds): Oski's Pediatrics: Principles and Practice, ed 3. Lippincott Williams & Wilkins, 1999, pp 2027-2029. 2) Eehffi1an RE, Kliegman RM, Jenson HE (eds): Nelson Textbook of Pediatrics, ed 16. WE Saunders Co, 2000, pp 1832-1834. (ABFP, 2001, 126). 19. A 2-year-old white male is seen for a well care visit. His mother is concerned because he is not yet able to walk. The routine physical examination, including an orthopedic evaluation, is unremarkable. Speech and other developmental landmarks seem normal for his age. Which one of the following tests would be most appropriate? A) A TSH level B) Random urine for aminoaciduria C) Phenylketonuria screening D) A serum creatine kinase level E) Chromosome analysis ANSWER: D The diagnosis of Duchenne's muscular dystrophy, the most common neuromuscular disorder of childhood, is usually not made until the affected individual presents with an established gait abnormality at age 4-5. By then, parents unaware of the X-linked inheritance may have had additional children who would also be at risk. The disease can be diagnosed earlier by testing for elevated creatine kinase in boys who are slow to walk. The mean age for walking in affected boys is 17.2 months, whereas over 75% of normal children in the United States walk by 13.5 months. Massive elevation of creatine kinase (CK) from 20 to 100 times normal occurs in every young infant with the disease. Early detection allows appropriate genetic counseling regarding future pregnancies. Hypothyroidism and phenylketonuria could present as delayed walking. However, these diseases cause significant mental retardation and would be associated with global developmental delay. Furthermore, these disorders are now diagnosed in the neonatal period by routine screening. Disorders of amino acid metabolism present in the newborn period with failure to thrive, poor feeding, and lethargy. Neurologic Disorders - page 9 of 10
  10. 10. Gross chromosomal abnormalities would usually be incompatible with a normal physical examination at 18 months. Ref: McMillan JA, DeAngelis CD, Feigin RD, et al (eds): Oski's Pediatrics: Principles and Practice, ed 3. Lippincott Williams & Wilkins, 1999, pp 1972-1973. 2) Hoekelman RA (ed): Primary Pediatric Care, ed 4. Mosby, 2001, pp 457-466. 20. You diagnose attention-deficit/hyperactivity disorder in a 10-year-old male, and the parents ask about medication treatment. Which one of the following would you tell the parents with regard to potential side effects of medication treatment? A) Methylphenidate (Ritalin) may precipitate seizures. B) Although anorexia may occur, it will not lead to substantial short term weight loss. C) Methylphenidate has significantly fewer side effects than dextroamphetamine (Dexedrine). D) The effect on eventual adult height is minimal in patients treated with methylphenidate or dextroamphetamine. E) Children treated with stimulants have an increased risk of developing addiction to illegal drugs in adolescence. ANSWER: D Stimulant medications do not precipitate seizures and can be given safely to patients with a history of seizures. Substantial weight loss may occur in up to 15% of patients, but eventual adult height and weight are not affected. The number of side effects is similar for methylphenidate and dextroamphetamine. No significant changes in pulse or blood pressure occur either at rest or with exercise. Studies have shown that children treated with stimulants do not have an increased risk of developing addiction to illegal drugs in adolescence, and they may actually have a decreased incidence of developing addiction to illegal drugs when compared with attention- deficit/hyperactivity disorder patients who are not treated with stimulants. Ref: Elia J, Ambrosini PJ, Rapoport JL: Treatment of attention-deficit-hyperactivity disorder. N Engl J Med 1999; 340(10): 780-788. 21. Positron emission tomography (PET) scans used to detect cancer most commonly use a radioactive tracer tagged to a molecule that is an analogue of: A) oxygen. B) glucose. C) hemoglobin. D) nitrate. E) phosphate. ANSWER: B The fluorine-18-labeled tracer fluorodeoxyglucose (FDG) is a glucose analogue taken up by metabolically active cells using glucose as a substrate for their metabolism. This enables the PET scanner to detect metabolically active tissues such as cancer metastases. Ref: Schwartz SI (ed): Principles of Surgery, ed 7. McGraw-Hill, 1999, pp 339-340. 2) Positron Emission Tomography, An Introduction. GE Medical Systems, 2000, pp 1-14. 22. Which one of the following is useful in migraine prophylaxis? A) Thiamine (vitamin B1) B) Riboflavin (vitamin B2) C) Ascorbic acid (vitamin C) D) Cholecalciferol (vitamin D) E) Tocopherol (vitamin E) ANSWER: B A daily oral dose of 400 mg of riboflavin (vitamin B 2) has been shown to be superior to placebo for migraine prophylaxis. The effect of riboflavin begins after 1 month of treatment and is maximal after 3 months of treatment. Its effect is most pronounced on attack frequency and the number of days patients have a headache. For comparison, one Centrum chewable multivitamin contains 1.7 mg of Riboflavin. Ref: Schoenen J, Jacquy J, Lenaerts M: Effectiveness of high-dose riboflavin in migraine prophylaxis: A randomized controlled trial. Neurology 1998; 50(2):466-470. Neurologic Disorders - page 10 of 10