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Questions and Answers 

Questions and Answers
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Questions and Answers
Author(s):

Kelly D. Flemming

DOI:
10.1093/med/9780190244927.003.0031
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Questions

Multiple Choice (choose the best answer)

IV.1. A 64-year-old woman presents for evaluation of increasingly frequent episodes of syncope and, more recently, some dream reenactment behavior. On examination, her blood pressure decreases from 170/85 mm Hg supine to 90/60 mm Hg standing without a corresponding increase in heart rate. Her cognitive function is normal. She has moderate diffuse bradykinesia and rigidity, postural instability, and mild appendicular ataxia. Which of the following is the most appropriate next step in her management?

  1. a. Refer her for polysomnography to assess for nocturnal laryngeal stridor

  2. b. Initiate high-dose parenteral corticosteroids

  3. c. Initiate donepezil

  4. d. Recommend that she avoid sleeping with the head of the bed elevated

  5. e. Initiate a low-dose β‎-blocker for orthostatic intolerance

IV.2. A 69-year-old man is referred for increasingly frequent falls. On examination, he has very slow saccadic velocities, occasional ocular square wave jerks, and severe axial rigidity with impaired postural reflexes. Which of the following imaging findings would you most likely encounter in the evaluation of this patient?

  1. a. Cruciform pontine T2-signal hyperintensity on magnetic resonance imaging (MRI) (“hot cross bun” sign)

  2. b. Asymmetric frontoparietal cortical atrophy on MRI

  3. c. Normal fluorodopa F 18 positron emission tomographic imaging

  4. d. Midbrain atrophy on MRI, resulting in the “hummingbird” sign

  5. e. Symmetric, extensive calcification of the putamen, pallidum, and dentate nuclei

IV.3. A 17-year-old adolescent boy presents for evaluation of social withdrawal and new upper limb tremor. On examination, he has a high-amplitude, irregular, postural tremor of the upper limbs most prominent in proximal muscles. On ocular examination, a brownish limbic corneal discoloration is noted. Aspartate aminotransferase (AST) and alanine aminotransferase (ALT) levels are significantly elevated. Which of the following genes is most likely involved in the diagnostic abnormality in this patient?

  1. a. PANK2

  2. b. THAP1

  3. c. NKX2.1

  4. d. TOR1A

  5. e. ATP7B

IV.4. A 16-year-old adolescent girl presents for evaluation of abnormal movements. For several years, she has had a fluctuating problem with briefly suppressible urges to shake her head. These are worse when she is “stressed out,” and they are socially embarrassing. During the interview she makes subtle, intermittent grunting noises. Which of the following is the most appropriate next step?

  1. a. Urgent referral to a psychiatrist for treatment of a psychogenic movement disorder

  2. b. Lumbar puncture for measurement of cerebrospinal fluid catecholamines

  3. c. Initiation of low-dose oral clonidine

  4. d. Referral to a medical geneticist for assessment of trinucleotide repeats in the huntingtin gene

  5. e. Urgent electroencephalography to exclude nonconvulsive status epilepticus

IV.5. A 23-year-old man is evaluated for imbalance. On examination, he has severe axial and appendicular ataxia. He also has nearly complete loss of proprioception and vibration sensation, absent muscle stretch reflexes throughout, and extensor plantar responses. Which of the following is most likely to apply?

  1. a. Significant GAA expansion in both FRDA alleles

  2. b. Elevated serum alpha-fetoprotein level

  3. c. Acanthocytes on blood smear

  4. d. Extensive subcortical T2-signal hyperintensity on MRI

  5. e. Elevated serum lactate level

IV.6. A 59-year-old man is referred by his primary care physician for evaluation of falls. On examination he has an ataxic, wide-based gait and mild appendicular ataxia. Imaging of his brain demonstrates significant atrophy of the cerebellum vermis. Use of which of the following medications would be important for you to assess in your history?

  1. a. Fluoxetine

  2. b. Lovastatin

  3. c. Azithromycin

  4. d. Hydroxychloroquine

  5. e. Phenytoin

IV.7. A 72-year-old woman comes to your clinic for evaluation of right hand tremor. In addition to the tremor, she has had several years of intermittent dream reenactment behavior and almost complete anosmia. On examination, you note diffuse bradykinesia and hypokinesia. She has a slow resting tremor of the right hand and moderate cogwheeling rigidity in all limbs, which is also worse on the right. Which of the following statements regarding her treatment is most accurate?

  1. a. She should be referred for consideration of early subthalamic nucleus deep brain stimulation

  2. b. She should be started on high-dose pramipexole

  3. c. Midodrine should be prescribed to prevent inevitable orthostatic hypotension

  4. d. Carbidopa-levodopa should be taken only well before or after meals

  5. e. Regular exercise should be avoided to reduce the risk of falls

IV.8. You have diagnosed slowly progressive idiopathic Parkinson disease in a 78-year-old man. His bradykinesia and rigidity are well controlled on a regimen of carbidopa-levodopa. Which of the following potential adverse effects should you make the patient aware of?

  1. a. Livedo reticularis

  2. b. Impulsive behaviors such as inappropriate gambling

  3. c. Hypertension

  4. d. Dry mouth

  5. e. Hepatotoxicity

IV.9. A 48-year-old surgeon is referred to you for evaluation of several years of upper limb tremor (right worse than left). On examination, she has a low-amplitude 8-Hz postural tremor that abolishes at rest. She has normal muscle tone and postural reflexes and no evidence of bradykinesia. Her father had a similar tremor. Which of the following statements regarding this diagnosis is most accurate?

  1. a. Excellent response to low-dose domaminergic agents is diagnostic of this disorder

  2. b. Failure to improve with ethanol excludes this diagnosis

  3. c. The diagnosis can be confirmed only at autopsy

  4. d. The tremor may eventually involve the voice

  5. e. Selegiline has been shown to slow the progression of the tremor

IV.10. A 59-year-old man is referred to your clinic for evaluation of lower limb discomfort. On questioning, he describes an unpleasant sensation in both lower limbs that improves with walking or after sitting. He has had no falls. Neurologic examination findings are normal, although when asked to stand after surface electromyography electrodes are applied over his lower limb muscles, he remarks that the transduced waveforms on the speaker “sound like a helicopter.” Which of the following is the most likely diagnosis?

  1. a. Orthostatic tremor

  2. b. Holmes tremor

  3. c. Idiopathic Parkinson disease

  4. d. Dentatorubral-pallidoluysian atrophy

  5. e. Psychogenic movement disorder

IV.11. A 52-year-old man comes to your clinic for evaluation of uncontrollable movements. He has a history of severe depression. On examination, diffuse choreic movements most prominently affect the upper limbs. On further questioning, he reports that his father had a similar movement disorder before committing suicide at the age of 61. Which of the following statements regarding his diagnosis is correct?

  1. a. Genetic counseling should not be performed until the mutation is confirmed

  2. b. Structural brain imaging with MRI is normal even in advanced disease

  3. c. Reserpine would have to be used with caution given his history of depression

  4. d. The presence of psychosis would effectively exclude the most likely diagnosis in this case

  5. e. Striatal stem cell implantation provides significant benefit in slowing disease progression

IV.12. A 32-year-old woman reports a 2-year history of painful, uncontrollable head movements. Initially, she described intermittent forced head turning to the left. More recently, she has had difficulty with involuntary simultaneous bilateral eye closure. On examination, she has intermittent leftward torticollic head turns and marked hypertrophy of the right sternocleidomastoid muscle. Which of the following statements regarding her treatment is most accurate?

  1. a. Treatment with carbamazepine or its derivatives eliminates symptoms in a majority of patients

  2. b. Surgical denervation of affected cervical muscles is the most appropriate first-line therapy

  3. c. Given the high suspicion for a psychogenic cause for the movements, paroxetine should be administered

  4. d. Chemodenervation with botulinum toxin injection is an appropriate first-line therapy but may result in dysphagia

  5. e. Close observation is appropriate since most cases will spontaneously remit

Answers

IV.1. Answer a.

Christine CW, Aminoff MJ. Clinical differentiation of parkinsonian syndromes: prognostic and therapeutic relevance. Am J Med. 2004 Sep 15;117(6):412–9.

IV.2. Answer d.

Christine CW, Aminoff MJ. Clinical differentiation of parkinsonian syndromes: prognostic and therapeutic relevance. Am J Med. 2004 Sep 15;117(6):412–9.

IV.3. Answer e.

Singer HS, Mink JW, Gilbert DL, Jankovic J. Movement disorders in childhood. Philadelphia (PA): Saunders/Elsevier; c2010. 279 p.

IV.4. Answer c.

Fernandez-Alvarez E, Aicardi J. Movement disorders in children. London (UK): Mac Keith for the International Child Neurology Association; c2001. 263 p. (International Review of Child Neurology Series).

IV.5. Answer a.

Manto M, Marmolino D. Cerebellar ataxias. Curr Opin Neurol. 2009 Aug;22(4):419–29.

IV.6. Answer e.

Subramony SH. Ataxic and cerebellar disorders. In: Daroff RB, Fenichel GM, Jankovic J, Mazziotta JC, editors. Bradley’s neurology in clinical practice. 6th ed. Philadelphia (PA): Elsevier/Saunders; c2012. p. 224–229.

IV.7. Answer d.

Adler CH, Ahlskog JE, editors. Parkinson’s disease and movement disorders: diagnosis and treatment guidelines for the practicing physician. Totowa (NJ): Humana Press; c2000. 474 p.

IV.8. Answer b.

Adler CH, Ahlskog JE, editors. Parkinson’s disease and movement disorders: diagnosis and treatment guidelines for the practicing physician. Totowa (NJ): Humana Press; c2000. 474 p.

IV.9. Answer d.

Bain PG. The management of tremor. J Neurol Neurosurg Psychiatry. 2002 Mar;72 Suppl 1:I3-I9.

IV.10. Answer a.

Bain PG. The management of tremor. J Neurol Neurosurg Psychiatry. 2002 Mar;72 Suppl 1:I3-I9.

IV.11. Answer c.

Bhidayasiri R, Truong DD. Chorea and related disorders. Postgrad Med J. 2004 Sep;80(947):527–34. Erratum in: Postgrad Med J. 2004 Nov;80(949):649.

IV.12. Answer d.

Gonzalez-Alegre P. The inherited dystonias. Semin Neurol. 2007 Apr;27(2):151–8.

Suggested Reading

Adler CH, Ahlskog JE, editors. Parkinson’s disease and movement disorders: diagnosis and treatment guidelines for the practicing physician. Totowa (NJ): Humana Press; c2000. 474 p.Find this resource:

    Bain PG. The management of tremor. J Neurol Neurosurg Psychiatry. 2002 Mar;72 Suppl 1:I3-I9.Find this resource:

      Bhidayasiri R, Truong DD. Chorea and related disorders. Postgrad Med J. 2004 Sep;80(947):527–34. Erratum in: Postgrad Med J. 2004 Nov;80(949):649.Find this resource:

        Christine CW, Aminoff MJ. Clinical differentiation of parkinsonian syndromes: prognostic and therapeutic relevance. Am J Med. 2004 Sep 15;117(6):412–9.Find this resource:

          Deuschl G, Wilms H. Clinical spectrum and physiology of palatal tremor. Mov Disord. 2002;17 Suppl 2:S63–6.Find this resource:

            Dooley JM. Tic disorders in childhood. Semin Pediatr Neurol. 2006 Dec;13(4):231–42.Find this resource:

              Fernandez-Alvarez E, Aicardi J. Movement disorders in children. London (UK): Mac Keith for the International Child Neurology Association; c2001. 263 p. (International Review of Child Neurology Series).Find this resource:

                Gonzalez-Alegre P. The inherited dystonias. Semin Neurol. 2007 Apr;27(2):151–8.Find this resource:

                  Jung HH, Danek A, Walker RH. Neuroacanthocytosis syndromes. Orphanet J Rare Dis. 2011 Oct 25;6:68.Find this resource:

                    Kojovic M, Cordivari C, Bhatia K. Myoclonic disorders: a practical approach for diagnosis and treatment. Ther Adv Neurol Disord. 2011 Jan;4(1):47–62.Find this resource:

                      Manto M, Marmolino D. Cerebellar ataxias. Curr Opin Neurol. 2009 Aug;22(4):419–29.Find this resource:

                        Morgante F, Edwards MJ, Espay AJ. Psychogenic movement disorders. Continuum (Minneap Minn). 2013 Oct;19(5 Movement Disorders):1383–96.Find this resource:

                          Schneider SA, Walker RH, Bhatia KP. The Huntington’s disease-like syndromes: what to consider in patients with a negative Huntington’s disease gene test. Nat Clin Pract Neurol. 2007 Sep;3(9):517–25.Find this resource:

                            Shahwan A, Farrell M, Delanty N. Progressive myoclonic epilepsies: a review of genetic and therapeutic aspects. Lancet Neurol. 2005 Apr;4(4):239–48.Find this resource:

                              Singer HS, Mink JW, Gilbert DL, Jankovic J. Movement disorders in childhood. Philadelphia (PA): Saunders/Elsevier; c2010. 279 p.Find this resource:

                                Subramony SH. Ataxic and cerebellar disorders. In: Daroff RB, Fenichel GM, Jankovic J, Mazziotta JC, editors. Bradley’s neurology in clinical practice. 6th ed. Philadelphia (PA): Elsevier/Saunders; c2012. p. 224–229.Find this resource:

                                  Subramony SH, Xia G. Disorders of the cerebellum, including the degenerative ataxias. In: Daroff RB, Fenichel GM, Jankovic J, Mazziotta JC, editors. Bradley’s neurology in clinical practice. 6th ed. Philadelphia (PA): Elsevier/Saunders; c2012. p. 1802–23.Find this resource:

                                    van Gaalen J, van de Warrenburg BP. A practical approach to late-onset cerebellar ataxia: putting the disorder with lack of order into order. Pract Neurol. 2012 Feb;12(1):14–24.Find this resource:

                                      Wenning GK, Litvan I, Tolosa E. Milestones in atypical and secondary Parkinsonisms. Mov Disord. 2011 May;26(6):1083–95.Find this resource: