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Tremor, rigidity, bradykinesia, and postural instability are the cardinal features of parkinsonism and may be present in any combination There may also be a mild decline in intellectual function The tremor of about four to six cycles per second is

clear palsy, bradykinesia and rigidity are accompanied by a supranuclear disorder of eye movements, pseudobulbar palsy, and axial dystonia Creutzfeldt-Jakob disease may be accompanied by features of parkinsonism, but dementia is usual, myoclonic jerking is common, ataxia and pyramidal signs may be conspicuous, and the electroencephalographic findings are usually characteristic In cortical-basal ganglionic degeneration, parkinsonism is accompanied by conspicuous signs of cortical dysfunction (eg, apraxia, sensory inattention, dementia, aphasia)

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most conspicuous at rest, is enhanced by emotional stress, and is often less severe during voluntary activity Although it may ultimately be present in all limbs, the tremor is commonly confined to one limb or to the limbs on one side for months or years before it becomes more generalized In some patients, tremor is absent Rigidity (an increase in resistance to passive movement) is responsible for the characteristically flexed posture seen in many patients, but the most disabling symptoms of parkinsonism are due to bradykinesia, manifested as a slowness of voluntary movement and a reduction in automatic movements such as swinging of the arms while walking Curiously, however, effective voluntary activity may briefly be regained during an emergency (eg, the patient is able to leap aside to avoid an oncoming motor vehicle) Clinical diagnosis of the well-developed syndrome is usually simple The patient has a relatively immobile face with widened palpebral fissures, infrequent blinking, and a certain fixity of facial expression Seborrhea of the scalp and face is common There is often mild blepharoclonus, and a tremor may be present about the mouth and lips Repetitive tapping (about twice per second) over the bridge of the nose produces a sustained blink response (Myerson s sign) Other findings may include saliva drooling from the mouth, perhaps due to impairment of swallowing; soft and poorly modulated voice; a variable rest tremor and rigidity in some or all of the limbs; slowness of voluntary movements; impairment of fine or rapidly alternating movements; and micrographia There is typically no muscle weakness (provided that sufficient time is allowed for power to be developed) and no alteration in the tendon reflexes or plantar responses It is difficult for the patient to arise from a sitting position and begin walking The gait itself is characterized by small shuffling steps and a loss of the normal automatic arm swing; there may be unsteadiness on turning, difficulty in stopping, and a tendency to fall.

Treatment is symptomatic No therapy has been shown to slow disease progression, but trials of several putative neuroprotective agents are in progress, as are various trials of gene therapy

Drug treatment is not required early in the course of parkinsonism, but the nature of the disorder and the availability of medical treatment for use when necessary should be discussed with the patient 1 Amantadine Patients with mild symptoms but no disability may be helped by amantadine This drug improves all of the clinical features of parkinsonism, but its mode of action is unclear Side effects include restlessness, confusion, depression, skin rashes, edema, nausea, constipation, anorexia, postural hypotension, and disturbances of cardiac rhythm However, these are relatively uncommon with the usual dose (100 mg twice daily) 2 Anticholinergic drugs Anticholinergics are more helpful in alleviating tremor and rigidity than bradykinesia Treatment is started with a small dose (Table 24 6) and gradually increased until benefit occurs or side effects limit further increments If treatment is ineffective, the drug is gradually withdrawn and another preparation then tried Common side effects include dryness of the mouth, nausea, constipation, palpitations, cardiac arrhythmias, urinary retention, confusion, agitation, restlessness, drowsiness, mydriasis, increased intraocular pressure, and defective accommodation Anticholinergic drugs are contraindicated in patients with prostatic hyperplasia, narrow-angle glaucoma, or obstructive

Example A-59. One solution to Exercise 20-1 (continued)

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