Bill Lyons, M.D. UNMC Geriatrics Asst. Professor wlyons@unmc.edu SELECTED GERIATRIC NEUROLOGIC DISEASE Module 2 Essential Tremor, PSP & Other Movement disorders Bill Lyons, M.D. UNMC Geriatrics Asst. Professor wlyons@unmc.edu Welcome to the modules on selected neurologic diseases in the geriatric population. Over the next many slides we will attempt to review the most important aspects of this important subject. We hope that you will have as much fun learning from this material as we had making it.

Objectives Upon completion the learner regarding Essential Tremor, Progressive Super nuclear Palsey ( PSP) and drug-induced movement disorders,will be able to: Describe diagnostic features of each disease.. Differentiate Parkinson’s disease from PSP

BENIGN ESSENTIAL TREMOR Action tremor of hands, head, voice, legs Shaking while bringing cup to lips Ethanol may improve Many, but less than half, familial Treat: propranolol or primidone Less effective with head and voice tremor This is the start of the second module on the subject of common neurologic problems in the geriatric population. You should already have completed the module on parkinson’s disease. The current module briefly covers some other common movement disorders seen in the older population. The first disorder we will discuss is benign essential tremor. This is characteristically an action tremor – seen, for example, when a person brings a cup or glass of liquid to the lips to drink. The tremor of parkinson’s disease, in contrast, is a rest tremor. Although patients with benign essential tremor most commonly experience the tremor in their hands, it is also seen in the head or the legs, or heard in the voice. Patients often remark that the tremor improves if they have an alcoholic beverage, and this can be helpful diagnostically. Although the tremor is often called benign familial tremor, familial cases are still in the minority, so a negative family history should not cause you to discard this as a possible diagnosis. Treatment of benign essential tremor is with either propranolol or primidone; since both of these agents can cause significant side effects, it is generally best not to initiate treatment until a patient finds that the tremor impairs her function. The tremor unfortunately tends to be progressive. Medications tend to be more effective in controlling hand tremor than tremor of the voice or head.

PROGRESSIVE SUPRANUCLEAR PALSY (PSP) M>F, starts when in 50s or 60s About 1/10th as common as idiopathic PD Compare PSP and PD: Early PSP greater tendency to falls PSP patients often fall backwards PSP patients may complain of vision PSP patients less likely with tremor PSP rigidity axial, PD in limbs Progressive supranuclear palsy, or PSP, is an uncommon movement disorder seen in the elderly population. Estimates are that it is about a tenth as common as idiopathic parkinson’s disease. Progressive supranuclear palsy generally begins when a patient is in his 50s or 60s, and it is a bit more common in men than in women. It can be instructive to compare progressive supranuclear palsy to parkinson’s disease. Generally speaking, patients with early PSP tend to have a greater tendency to fall than do parkinson’s patients. Also, PSP patients characteristically tend to fall backwards, probably because they show a truncal rigidity in extension. As we’ll discuss in a minute, patients with PSP characteristically have problems with voluntary gaze, and this may cause them to complain of visual problems to a greater extent than parkinson’s patients do. PSP is not characterized by tremor, in contrast to most cases of parkinson’s disease. Finally, although both PSP patients and parkinson’s patients experience rigidity, there is a difference. The rigidity of PSP is characteristically axial, that is, it affects the trunk more than the limbs. The rigidity of parkinson’s disease, in contrast is more apparent in the limbs.

PSP, cont’d Vertical gaze paresis – trouble looking down Trouble eating (soiled shirt, tie, blouse) Men have difficulty at urinal Danger walking down stairs Patients with PSP typically have a vertical gaze paresis, which means they have difficulty looking down. This can create characteristic practical problems for these patients. First, these patients can have problems eating neatly, and frequently soil their shirt, tie, or blouse at mealtime. This finding has been coined the “dirty shirt sign.” Men may have difficulty using a stand-up urinal because of difficulty looking down. Lastly and most importantly, these patients may find walking downstairs to be difficult and dangerous.

PSP, cont’d Speech troubles Trouble chewing and swallowing Hypernasal, strained, monotone Trouble initiating speech Speech arrest Trouble chewing and swallowing Emotional incontinence Patients with PSP develop peculiar problems with their speech. Often their voice can sound especially nasal, or strained, or monotonous. These patients can have difficulty initiating speech, or may find that they experience speech arrest or freezing while speaking. Another apparent bulbar deficit shown by patients with PSP is difficulty with chewing and swallowing. Some patients also demonstrate what is termed emotional incontinence, in which they may laugh or cry without experiencing internally the emotions you’d expect from these behaviors.

PSP, cont’d Cognitive deficits Prognosis typically worse than PD Memory (usually mild) Executive dysfunction Prognosis typically worse than PD Little or no response to PD medications Treat: PT, OT Patients with PSP over time develop cognitive decline. Memory deficits are present but are typically mild relative to patients with Alzheimer’s disease. More characteristic are problems of executive dysfunction, with difficulty making conceptual shifts, for example. The overall prognosis for PSP is generally worse than that of parkinson’s disease, as patients tend to show a more rapid decline in their motor and cognitive abilities. The response to medicines used for parkinson’s disease is usually disappointing. As a result, management involves referral to physical and occupational therapy, as well as assistance with advance care planning.

DRUG-INDUCED MOVEMENT DISORDERS Many from antipsychotics: Parkinsonism Akathisia Acute dystonic reactions Tardive dyskinesia Chorea Metoclopramide can cause parkinsonism, too Health professionals with much experience caring for older patients know that many movement abnormalities are the result of medication adverse effects. Antipsychotic agents probably lead the list in this context. Use of these drugs has been associated with parkinsonian symptoms resembling idiopathic parkinson’s disease, akathisia or an internal feeling of motor restlessness, acute dystonic reactions, tardive dyskinesia, and choreiform movements. Metoclopramide, a drug very commonly employed to treat nausea, can also cause parkinsonism.

DRUG-INDUCED, cont’d Chorea – levodopa, bromocriptine, phenytoin, carbamazepine, lithium Dystonia – levodopa, bromocriptine, lithium, metoclopramide, carbamazepine Tremor – theophylline, lithium, valproic acid Besides antipsychotic drugs, other medications have been associated with drug-induced chorea. These include levodopa or bromocriptine, phenytoin, carbamazepine, and lithium. This same set of drugs can also induce dystonias. Finally, tremors are often drug-induced, and theophylline, lithium, and valproate are drugs whose use should raise your suspicion.

Post –test 1 Which one of the following statements regarding progressive supranuclear palsy (or PSP) and Parkinson's disease is correct? Patients with early PD are more likely to fall than patients with early PSP Patients with PSP more often fall backwards than patients with PD Patients with PD are more likely to have visual complaints than patients with PSP. Patients with PD are more likely to demonstrate emotional incontinence than are patients with PSP.

Correct Answer:   B. Patients with PSP more often fall backwards than patients with PD Feedback:The correct answer is (b), patients with PSP more often fall backwards than patients with Parkinson's disease. This occurs because PSP patients tend to have axial rigidity in extension, whereas Parkinson's patients have more truncal flexion. Answer (a) is wrong because patients with early PSP tend to fall more than patients with parkinson?s disease. Answer (c) is wrong because patients with PSP have more visual complaints, on average, than patients with Parkinson's disease, by virtue of the associated gaze palsy. Item (d) is wrong because it's PSP patients, not Parkinson's patients, who are more likely to have emotional incontinence. And answer (e) is incorrect not because of differences in upper and lower extremity rigidity between the two disease, but rather because the rigidity in PSP is more axial, while that of Parkinson's disease is more in the extremities. End