What is Causing Tremors in this Elderly Man’s Hand?

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What is Causing Tremors in this Elderly Man’s Hand?

Question 1

Questions answered incorrectly will be highlighted.

A 61-year-old right-handed man presents with involuntary twitches of his left hand. He first noticed between 6 months and 1 year ago that when he is at rest, his left hand shakes. He can stop the shaking by looking at his hand and concentrating. The shaking does not impair his activities in any way. He has no trouble holding a glass of water. There is no tremor in his right hand, and his lower extremities are not affected. He has had no trouble walking, and there have been no falls. There have been no behavioral or language changes. On examination, a tremor of the left hand is evident when the man is distracted. His handwriting is mildly tremulous. He has bilateral cogwheel rigidity with contralateral activation, which is worse on the left. His rapid alternating movements are bradykinetic on the left. Which of the following is the most likely diagnosis in this case?

Epilepsy

Guillain-Barré syndrome

Multiple sclerosis

Parkinson’s disease

Stroke

The answer is d, Parkinson’s Disease [1]

The tremor is of a Parkinsonian type. The patient also has the classic findings of Parkinson’s disease: asymmetric tremor, rigidity, and bradykinesia. Epilepsy is characterized by repeated unprovoked seizures. Hand shaking can be the result of a focal motor seizure, but the presentation overall makes epilepsy an unlikely diagnosis. Guillain-Barré syndrome is a peripheral demyelinating disease that usually presents as an ascending motor deficit. Multiple sclerosis is a central nervous system (CNS) demyelinating disease. It presents with individual episodes of CNS deficits, which usually recover to some extent. Stroke is characterized by the acute onset of a neurological deficit due to nerve infarction. Tremor would be an exceedingly rare presentation for stroke, and it would not evolve over 6 to 12 months.

Question 2

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Which of the following brain structures are currently targets for deep brain stimulation in patients with Parkinson’s disease?

Globus pallidus, medulla, and parietal lobe

Globus pallidus, subthalamic nucleus, and thalamus

Hippocampus, medulla, and thalamus

Medulla, occipital lobe, and subthalamic nucleus

Parietal lobe, temporal lobe, and thalamus

The answer is b, Globus pallidus, subthalamic nucleus, and thalamus [2] (Bradley, pp 2138–2139.)

Current theory of Parkinson’s disease pathology is based on the premise that the substantia nigra pars compacta has decreased dopamine production, which eventually leads to overinhibition of thalamocortical pathways. The thalamus may be directly intervened on to decrease this overinhibition. Alternatively, the globus pallidus interna may be lesioned or stimulated, because it directly inhibits the thalamus. A third approach is to lesion or stimulate the subthalamic nucleus, which has an excitatory connection on the globus pallidus interna and substantia nigra pars reticulata. The medulla, hippocampus, temporal lobe, and occipital lobe are not involved in this pathway.

Question 3

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A 25-year-old man has had motor tics since age 13. They seem to be getting worse, and now he also has involuntary obscene vocalizations. He may have largely normal behavior while being treated with which of the following?

L-dopa

Trihexyphenidyl (Artane)

Phenytoin (Dilantin)

Carbamazepine (Tegretol)

Haloperidol (Haldol)

The answer is e, Haloperidol (Haldol) [3]

The scenario described is that associated with Tourette syndrome. The affected person is usually over 21 years of age and cannot control the obscene and scatological remarks. With Tourette syndrome there appears to be an autosomal dominant pattern of inheritance with variable penetrance. Most affected persons are men. A variety of drugs may help suppress the tics that are characteristic of this syndrome. These include haloperidol, pimozide, trifluoperazine, and fluphenazine. Antiepileptics, such as carbamazepine and phenytoin, are not useful. Trihexyphenidyl and benztropine are useful in suppressing the parkinsonism that may develop with haloperidol administration, but are not useful in the management of Tourette syndrome.

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