Case: Parkinson’s

CASE STUDY: 61-year-old woman with “the shakes”

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“My hand tremors are getting worse and are getting in the way of my normal activities. Even my handwriting is getting difficult to read. Do I have Parkinson’s? What’s wrong with me?”

Case: Ms. Johnson is a 61-year-old woman presenting today with a chief complaint of a hand tremor that has worsened over the past three years. The tremor occurs when she uses her hands for activities such as slicing vegetables, sewing, putting on her lipstick, or typing at her computer. Her handwriting has become messy and sprawling and very difficult to read. She has worked for 23 years as an executive assistant; as a result of her tremor and the associated difficulties, she is considering retirement. She notes that on the rare occasion that she consumes alcohol, the tremor is somewhat improved.

Family history is positive for two grandparents who also “had the shakes” later in life. She assumes that they both had Parkinson’s disease and is afraid “that’s what’s wrong with me, too.”

Medications include an antihypertensive, a statin, and an estrogen patch. Today her BP is 120/62. Height is 5’6”, weight 139 lbs. On physical examination, mental status, cranial nerves, sensation, muscle strength, tone, and deep tendon reflexes are all normal. There is a mild tremor bilaterally in both hands as she writes her name. No tremor at rest. There is no bradykinesia or rigidity noted. Voice is somewhat tremulous. There is a slight involuntary back-and-forth horizontal rotation of the head.

Lab data, including lipids, TSH and FBS are all within normal limits.

 

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Ms. Johnson clearly has a tremor disorder. However, as there are several types of tremor associated with various clinical presentations, it’s important to differentiate between different tremor types. The two basic tremor types are resting and action. Resting tremors typically occur when the affected body part is fully supported and at rest. Action tremors are typically worse when the affected part of the body is in motion, such as writing or slicing vegetables. They are most commonly caused by enhanced physiologic tremor or essential tremor and tend to be disabling.[1,2]

Resting tremor is typically a sign of Parkinson’s disease, along with rigidity and bradykinesia. The tremor associated with Parkinson’s disease generally decreases or disappears when the affected body part is moved. And although patients with Parkinson’s disease may also exhibit action tremors, resting tremors—not action tremors—are the hallmark of Parkinson’s disease.[3,4]

Action tremors are almost always worse when the affected part of the body is in motion than when it is at rest. The most common tremor is “essential” tremor, a slowly progressive neurological disorder characterized by a tremor of the arms or hands that is apparent during voluntary movements such as eating or writing. Essential tremor affects about 10 million people in the United States and is more common than Parkinson’s disease.[5] The tremor usually affects both upper extremities, although mild asymmetry is not uncommon. The tremor may also affect the head, jaw, and voice. Although essential tremors are often mild, many patients have difficulty performing typical activities of daily living. Other conditions associated with action tremors include adult-onset idiopathic dystonia and Wilson’s disease, although these occur in less than 1% of patients.

Although some evidence suggests that this disorder is heterogeneous,[6] essential tremor generally is considered to be monosymptomatic (tremor only)[7]. Some patients, however, will present with abnormalities in gait and balance. If patients do present with such abnormalities, the diagnosis should be carefully considered, because most patients with essential tremor do not have gait abnormalities. Other findings associated with essential tremor include changes in cognition, personality, mood, and hearing.[8,9] Based on aggregated epidemiologic data from three independent studies, it is likely that people with essential tremor are 3 to 13 times more likely to develop Parkinson’s disease.[10]

While there is no gold standard for diagnosis, the following clinical criteria tend to accurately differentiate essential tremor from other tremor conditions, including Parkinson’s disease.[1]

  • Postural tremor of moderate amplitude is present in at least one arm during at least four tasks: pouring water, using a spoon to drink water, drinking water, finger-to-nose maneuver, and drawing a spiral
  • Tremor must interfere with at least one activity of daily living
  • The tremor is not caused by hyperthyroidism, alcohol, medications, or other neurologic conditions

Ms. Johnson: Parkinson’s or essential tremor?

  • Head tremor is uncommon in early Parkinson’s disease
  • Action tremors are associated with essential tremor; resting tremors are associated with Parkinson’s disease
  • Small amounts of alcohol do not usually mitigate the tremor associated with Parkinson’s disease
  • People with essential tremor typically exhibit macrographia compared with the micrographia associated with Parkinson’s disease
  • Essential tremor is more apt to exhibit a genetic component than Parkinson’s disease. According to the Parkinson Research Foundation, 60% of patients with essential tremor have some genetic connection to the disease, while only 10% of patients with PD report a strong family history.[1,11,12,13]Ms. Johnson asks you about levodopa for treating her tremor. While you are likely to refer her to a neurologist, you can talk with her about the more probable diagnosis of essential tremor and possible treatments. Unlike the tremor of Parkinson’s disease, essential tremor does not improve in response to levodopa. According to the American Academy of Neurology most recent evidence-based guideline for the treatment of essential tremor,[3] primidone and propranolol are the cornerstones of maintenance medical therapy for essential tremor, although primidone is not FDA-approved for this indication.[11] In many patients, these medications provide good benefit, reducing tremor amplitude in approximately 50% to 75% of patients. If monotherapy with primidone or propranolol is not beneficial, the two agents may be used in combination. Moderate evidence suggests that botulinum toxin type A injections may be effective, particularly in the management of head and voice tremor.[12] Benzodiazepines such as diazepam, alprazolam, and clonazepam improve the symptoms in some patients with essential tremor, perhaps due in part to their anxiolytic effects. However the adverse events and the abuse potential are likely to limit their clinical use.[12]Some patients may require only intermittent tremor reduction, such as when attending a meeting or engaging in a social activity. For these patients, an alcoholic beverage prior to the activity may be sufficient. Obviously, alcohol consumption is not an appropriate maintenance therapy for patients who seek tremor reduction throughout the day; therefore, an alternative is propranolol (10-40 mg) approximately one-half hour prior to the event.Data suggest, however, that at least 50% of patients may not improve on these medications.[13] If these patients become sufficiently disabled, surgery may be an option, with unilateral thalamotomy and deep brain stimulation the procedures of choice.[14] Both procedures offer some success in reducing tremors in the contralateral arm; research also suggests that these procedures are also useful in reducing head and voice tremor.

     

    Jill Shuman, MS, ELS
    Published on August 7, 2012
    Updated on August 14, 2012

     

    References

    1. Louis ED. Essential tremor. N Engl J Med. 2001;345:887-891.
    2. Wyne KT. A comprehensive review of tremor. JAAPA. 2005 Dec;18(12):43-50.
    3. Lees AJ, Hardy J, Revesz T. Parkinson’s disease. Lancet. 2009;373:2053-2066.
    4. Louis ED, Klatka LA, Liu Y, Fahn S.  Comparison of extrapyramical features in 31 pathologically confirmed cases of diffuse Lewy body disease and 34 pathologically confirmed cases of Parkinson’s disease.  Neurology 1997;48:376-380
    5. American Academy of Neurology. AAN summary of evidence-based guidelines for patients and their families. AAN; 2011. http://www.aan.com/practice/guideline/uploads/494.pdf
    6. Deng H, et al. Genetics of essential tremor. Brain. 2007 Jun;130(Pt 6):1456-64.
    7. Elble RJ. Essential tremor is a monosymptomatic disorder.  Mov Dis 2002;17:4;633-637
    8. Chatterjee A, Jurewicz EC, Applegate LM, Louis ED. Personality in essential tremor: further evidence of non-motor manifestations of the disease. J Neurol Neurosurg Psychiatry. 2004;75(7):958-961.
    9. Benito-Leon J, Louis ED, Bermejo-Pareja F. Reported hearing impairment in essential tremor: a population-based case-control study. Neuroepidemiology. 2007;29(3-4):213-217.
    10. LaRoia H,  Louis ED. Association between Essential Tremor and Other Neurodegenerative Diseases: What Is the Epidemiological Evidence? Neuroepidemiology. 2011;37(1):1-10.
    11. Zesiewicz TA, Elble RJ, Louis ED, et al. Evidence-based guideline update: treatment of essential tremor. Neurology. 2011;77:1752-1755.
    12. Laskawi R. The use of botulinum toxin in head and face medicine: an interdisciplinary field. Head Face Med. 2008 Mar 10;4:5.
    13. Diaz NL, Louis ED. Survey of medication usage patterns among essential tremor patients: movement disorder specialists vs. general neurologists. Parkinsonism Relat Disord. 2010;16:604-607.
    14. Fytagoridid A, Sandvik U, Astrom M, Bergenheim T, Blomstedt P. Long term follow-up of deep brain stimulation of the caudal zona incerta for essential tremor. J Neuro Neurosurg Psychiatry. 2012;83:258-262.