Abnormal Movements (Case 55)

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Chapter 63
Abnormal Movements (Case 55)

Joseph Rudolph MD and Michele Tagliati MD

Case: A 68-year-old man has been noticing a tremor in his right hand for the past year. The tremor began in his thumb, but it has spread to the entire hand and now his arm. It generally appears while he is watching television, walking, or is otherwise occupied, and lately it has been occurring more frequently. The shaking has been accompanied by a loss of dexterity of the same hand. He is finding it hard to button his left sleeve cuff and to tie his shoes. In addition, his wife has noticed a change in his gait. He seems to drag the right foot slightly when he takes a step, and he appears not to be able to walk as fast he used to. During the interview the patient’s wife also comments that her sleep has been interrupted because the patient has had several outbursts of yelling and flailing of his arms and legs during his sleep. He adds that he has been having some wild dreams. Upon direct questioning, he also admits that he has not been able to smell his wife’s cooking as well for the past 10 years.

Differential Diagnosis

Parkinson disease (PD)

Multiple system atrophy (MSA)

Progressive supranuclear palsy (PSP)

Essential tremor (ET)

Drug-related parkinsonism

Vascular parkinsonism

 

Speaking Intelligently

PD is the most common movement disorder. Despite its being a progressive, degenerative disease, a variety of treatments can help patients with PD to live a normal life span, with a greatly improved quality of life. In evaluating someone with possible PD, establish that he or she presents with the key clinical criteria. Specifically, look for tremor at rest, stiffness (rigidity), slowness of movement (bradykinesia), abnormalities of gait or balance (postural instability), and an overall development of these symptoms in a unilateral or at least asymmetric pattern. The patients may not be aware of some of the issues in which you are interested, but a spouse or family member may have noticed other problems. In addition, there are nonmotor symptoms of which lay people are unaware that are connected with PD but that become relevant and more disabling with disease progression. These include depression, anxiety, and cognitive dysfunction with slowed processing, but most functions are still generally intact, except for planning and judgment (frontal lobe symptoms), autonomic dysfunction including neurogenic bladder, erectile dysfunction, orthostatic hypotension, rapid eye movement (REM) behavior disorder (the seeming acting out of dreams), and loss of sense of smell. In more advanced cases a discussion with the caregiver assumes greater significance as specific issues that make it difficult to care for the patient but do not seem to be specifically part of the disease may come to light.

PATIENT CARE

Clinical Thinking

• PD appears in 12 to 20 of every 100,000 people per year, approaching a prevalence of 1 in 2000 people.

• Be aware of subtle symptoms or findings that may have been ignored by the patient or family but may be relevant to the diagnosis. For example, the patient may hold one arm slightly stiff while walking or may seem not to blink quite as frequently as one would expect in a healthy person.

• Early diagnosis and initiation of management with medications, dietary changes, and exercise may slow disease progression (although there is no true neuroprotective agent available).

• Stretching exercises may lower the risk of contractures, and exercising will keep the muscles active, minimizing the development of rigidity. Patient mobility is, of course, a significant issue for the patient as well as for the caregivers.

History

• Look for a collection of symptoms and signs that demonstrate loss of dexterity and flexibility.

• Does the patient recall loss of fine motor control, or perhaps muscle cramping in one limb?

• Is there difficulty swallowing with typically associated excess saliva pooling in the mouth?

• When the patient is walking, is one leg being dragged, or are there episodes of freezing (no obvious weakness, but seeming inability to move the legs, often occurring in doorways and approaching a chair or a crowd)?

• Does the patient cut his or her own food?

• Is there dizziness upon standing?

• Ask about the shrinking of handwriting size, the loss of voice volume, and the lack of facial expression (so-called mask facies).

• Utilize the Unified Parkinson’s Disease Rating Scale (UPDRS), which is routinely performed on all new PD patients. This scale includes cognitive concerns, ADLs, motor complaints, medication complications, a detailed movement-disorders exam, as well as several other details. Applying this scale at each visit enables the physician to compare each symptom, finding, and complaint across visits. Following the total UPDRS score over time is one way to monitor the degree and rapidity of disease progression.

• Be sure to ask about use of neuroleptic medications, as antipsychotics are dopamine antagonists and may induce a parkinsonian condition. Typically, drug-induced parkinsonism has a symmetrical onset, while idiopathic PD begins unilaterally, although this is not always the case.

• To help distinguish between the tremor of PD and that of essential tremor, the tremor of ET is generally more symmetrical and manifests during action, while that of PD is an asymmetric resting tremor. Also, ET may improve after the ingestion of alcohol, while PD will be unaffected or worsen.

Physical Examination

• A typical neurologic exam may hide as much as it reveals in a patient with a movement disorder.

• The examiner must check the tone of the patient’s muscles to evaluate for rigidity, both with and without reinforcement or distracting contralateral movements.

• Rapid alternating movements (finger tapping, opening and closing a fist, tapping the foot) may highlight slight asymmetric bradykinesia.

• Testing for postural instability is performed by pulling on the patient in an attempt to put him or her off balance (being ready to catch, if needed).

• The UPDRS, as described above, includes many of the relevant physical exam maneuvers that can be used at each visit to evaluate the severity of the disease.

Tests for Consideration

• There are currently no diagnostic tests for PD or other movement disorders.

• There are several genetic tests for cerebellar degenerative conditions, as well as for Huntington disease (HD), should these entities appear on the differential diagnosis.

• A number of genetic abnormalities associated with PD have been recently identified, but genetic testing is currently not recommended for the diagnosis of PD.

IMAGING CONSIDERATIONS

→ A brain MRI may help rule in or out other parkinsonian syndromes, particularly when the presentation is equivocal.

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→ A PET scan can be done, and this may show metabolic changes in the basal ganglia. However, a PET scan is not a necessary test and is more frequently used in the research setting, or to distinguish PD from an atypical parkinsonian syndrome.

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Parkinson Disease

Pφ

The central component of PD is the loss of the dopamine-secreting cells originating in the substantia nigra pars compacta. This causes a chain reaction throughout the basal ganglia that results in higher levels of inhibitory signals sent to the thalamus and motor cortex, thus slowing voluntary movement. There are also many nonmotor symptoms of PD, which are probably due to widespread neurodegeneration, suggesting involvement of neurotransmitters other than dopamine. They may include mood disorders, cognitive decline, orthostatic hypotension, speech difficulty, and dysphagia. Pathologically, the cells of the substantia nigra demonstrate Lewy bodies, which are intracellular collections of abnormal proteins, including α-synuclein (a membrane protein) and ubiquitin.

TP

PD usually presents unilaterally, with abnormal movements in one arm or hand or occasionally one leg. Frequently, tremor is the initial symptom, but not always. In non–tremor-predominant variants (i.e., akinetic-rigid syndromes), patients may notice rigidity and loss of dexterity in their hands before any tremor. Patients may recall a permanent loss of sense of smell, often beginning several years before the onset of motor symptoms. There may also be a history that sounds similar to REM behavior disorder (which involves acting out of dreams or even just talking in one’s sleep) and constipation.

Dx

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