History

Benign essential tremor:

Follow-up Questions/Answers

This lady’s main concern is clearly that of a tremor. Can you recount the salient points
of the history relevant to the tremor, and then give me a differential diagnosis to the
cause?
o This lady presents with a right-sided tremor; she does not have any on her left
side.
o The tremor is worse at rest or when holding her hand in a fixed position.
o On the initiation of movement, the tremor improves.
o Two specific scenarios are most troublesome to her:
• she is a cellist – when holding her cello bow in a fixed position prior to the
initiation of bowing
• when she is holding her pen prior to writing.
o The tremor improves with alcohol.
o She has also noticed some abnormality in her voice, particularly when singing.
o She is an asthmatic who infrequently uses a salbutamol inhaler.
o Her father has recently been found to have a bilateral tremor.
o The General Practitioner (GP) is concerned about Parkinson’s disease, and has
referred him to the neurology clinic.
What do you think the differential diagnosis is here?
o The most likely diagnosis is a benign essential tremor.
o It is unilateral, improves with movement and has been relieved previously by
alcohol.
o There is also the potential for an iatrogenic cause of the tremor, particularly with
the salbutamol inhaler.
o This could also be Parkinsonian given her father’s potential diagnosis, but it is
highly unlikely, as she doesn’t have any other Parkinsonian features, and the
tremor is unilateral.
How would you investigate this lady?
o A blood test for thyroid function screening to ensure that she does not have any
thyroid abnormality.
o If the thyroid screen were negative, I would treat with medications and a review in
six months’ time.
o If the symptoms had improved, then I would be happy that this was a diagnosis of
benign essential tremor.
o If symptoms had not improved with treatment or she had developed any other
neurological symptoms, then I would consider further investigation, which I would
discuss with my consultant.
History

What treatments do you know for benign essential tremor?

o The most common treatment is a beta-blocker such as propranolol. In this case, it
is not appropriate because the lady has asthma.
o Therefore, I would consider primidone treatment.
o In some cases, benzodiazepines can be used. There is a risk of addiction, so I
would not use it as a first-line choice.
o In very severe, treatment-resistant cases, deep brain stimulation can be used. I
would refer her to the neurologist for further opinion if my initial treatments were
not getting control of her symptoms.
Her father has a presumed diagnosis of Parkinson’s disease. Let’s say that, on review, he
actually has a very similar tremor. Is there any known familiar tendency or genetic
component to benign essential tremor?
o Yes, benign essential tremor is a genetic condition which tends to be inherited in
an autosomal-dominant fashion.
Is it always an inherited condition?
o Not always, no. But in the majority of cases, it is autosomal dominantly inherited.
History

Benign essential tremor:

Key Words and Phrases

This lady gives a classical history of benign essential tremor – uncontrollable tremor
of her hand, particularly when holding a fixed position, such as holding her bow or
when carrying out movement with her hand, including writing and typing.

Purposeful movements usually improve the tremor – explaining why this lady’s tremor is
often more obvious between writing words rather than when she is writing the words.

Essential tremor usually begins in one arm or hand and is not present at rest. It is likely
to progress to the other arm or other hand within a year or two. The tremor can also
affect the legs, head, voice, jaw or face.

The tremor is often made worse by stress, tiredness, caffeine, hunger, anger or even
extremes of temperature.

Improvement in symptoms following alcohol is classical of benign essential tremor.

In order to be satisfied that the tremor is not due to another underlying disorder, the
candidate should be screening the patient for other neurological symptoms, such as
headache, visual disturbance, cognitive impairment, weakness, paraesthesiae,
anesthesiae and impaired coordination.

The candidate should also ask about symptoms of hyperthyroidism, which is another
common cause of hand tremor.

About half of all benign essential tremors are familial and are inherited in an autosomal-
dominant fashion, so a detailed family history is important.
Treatment may be used for significant problems with tremor, and beta-blockers such as
propranolol are commonly tried and are effective in around 50% of patients.

Anti-epileptic medication may be tried in patients who do not respond to beta-blockers,

as may benzodiazepines.

Head and voice tremors may be considered for treatment with Botox®.

Deep brain stimulation would only be considered in the most severe, treatment-resistant
cases.