Essential Tremor, diagnosis and treatment: Implications for the elderly

Fenna Phibbs M.D. Assistant Professor of Neurology

Also known as..

Familial tremor Benign essential tremor Intention tremor Hereditary tremor

What is Essential Tremor?

A neurological disorder characterized by rhythmic, involuntary shaking of a part or parts of the body

Most commonly seen in the arms/hands Can also be seen in the head, voice, legs and trunk

What is Essential Tremor?

Action/Kinetic Tremor
Occurs during voluntary action such as eating, drinking, writing

Postural Tremor
Occurs in positions against gravity such as holding your arms out in front of your body

Internal sense of tremor

What is affected

Body parts affected: Hands 69% Voice 40% Head 17% (no-no 79%) Leg 13% Jaw 7% Others-face trunk and tongue Can start at any age, which does not have bearing on the rate of progression Slowly progressive, defined as increased tremor amplitude and/or extension to previously unaffected body parts Not associated increased mortality

Genetics of ET

50% of patients have a family history Autosomal dominant pattern, most common

Prevalence Rate

4 to 5.6 percent of people aged 40 to 60 have ET

Age 60 and older is estimated at 6.3 to 9%

(Benign) Essential tremor

Disability

Worse with anxiety, fatigue and illness Socially withdraw Physical disability related to age, tremor amplitude, ability to execute fine motor tasks such as writing and eating. Anxiety and depression Gait changes, possible dementia Medication side effects

Normal

Is it ET or PD?
Essential Tremor Action tremor More rapid frequency Not associated with slow movements, muscle rigidity and postural changes Often affects both sides Often familial Parkinsons Disease Resting tremor Slower frequency Associated with slowing, shuffling gait, rigidity, stooped posture, imbalance Usually worse on one side Rarely familial

Other Causes of Tremor

Enhanced physiologic tremor

Occurs during stressful or fear-provoking times

Medication induced tremor

Over the counter, herbal and prescription medications

Steroids, Depakote, Lithium, TCAs, Amiodarone, dopamine antagonists, Cyclosporin, stimulants, drug withdrawal

Post traumatic tremor resulting from head injury Thyroid disease

Other Causes of Tremor

Blood sugar fluctuations

Excessive use of caffeine or other stimulants (supplements) Presence of another neurological disorder, most commonly Parkinsons Disease

Treatment

Treatment Goal

Reduce the severity, not complete resolution To improve daily functioning Improve quality of life Medications can help mild to moderate tremor the best Aim for patient satisfaction

Treatment

Beta-blockers

Propranolol (Inderal) Metoprolol (Lopressor) Atenolol (Tenormin) Sotalol (Betapace)

Concern for side effects, sedation, fatigue, exercise intolerance and coexisting depression. Often already on due to coexisting heart disease, coordinate care!

Treatment
Anticonvulsants Primidone (Mysoline) Gabapentin (Neurontin) Topiramate (Topamax)

Generally well tolerated Primidone is generally first line, give at bedtime to reduce problems with sedation Topiramate can cause cognitive side effects, less appealing for elderly

Treatment

Benzodiazepines

Alprazolam (Xanax) Clonazepam (Klonopin) Diazepam (Valium)

Can be used prn for anxiety provoking situations Generally not good to use long term due to sedation, worsened balance

Treatment

Botulinum toxin

Blocks synaptic transmission to the muscle

Generally 3-6 month duration of response

Most useful for head or jaw tremor but sometimes used for hand and voice tremor Postural tremor responds better Smaller effect on intention tremor

Dependent on physicians expertise

Treatment

Alcohol

Alcohol reduces tremor in some patients Rebound tremor may occur after excessive alcohol intake - tremor can be temporarily more severe the next day Often pre-treating prior to event helpful Avoid excessive use of alcohol Do not drink and drive

Alternative Therapies

Benefit is unconfirmed when using alternative therapies such as acupuncture, hypnosis, massage therapy For people whose tremor worsens with stress, biofeedback or behavioral therapy may be helpful Occupational therapy has been found helpful assistance with adaptive devices such as weighted utensils, plate guards, etc.

Alternative therapies

Deep Brain Stimulation

Consider when:

Medications have failed to provide adequate relief Dose limiting side effects Moderate to severe tremor

Most efficacious tremor treatment

Tremor amplitude and frequency

80-95% reduction in extremity tremor 50-85% reduction in midline tremor (head, voice), often requires bilateral stimulation

Deep Brain Stimulation

Target the ventral intermediate nucleus of the thalamus (VIM) Does not help

Balance Paraesthesias Contractions Dysarthria Gait?

Side effects

DBS background

1946 first stereotatic surgery 1952, improvement in symptoms following ligation of the anterior choroidal artery. 1960s Thalamotomies for PD 1990s Pallidotomies 1993 first report of chronic high frequency stimulation of the thalamus in essential tremor 1998 STN published as a target for Parkinsons disease

DBS background

1997 Essential tremor 2002 Parkinsons disease 2003 Dystonia 2009 Obsessive compulsive disorder Future uses

Epilepsy Depression Tourettes syndrome Obesity Migraine Bipolar disorder

DBS Mechanism of action

Activation of inhibitory inputs Exhaustion of neuronal signal Jamming the abnormal signaling pathways

Deep Brain Stimulation process

Neurologic evaluation Pre-op screening

WHIGET tremor rating scale Neuropsychiatric testing

DBS conference Neurosurgical evaluation Insurance approval

Deep Brain Stimulation process

Surgery steps

Step 1: Bone markers placed with CT and MRI Step 2: electrode placement

Done week after step 1 Done with patient awake Overnight stay in the hospital Done 1-3 weeks after step 2 Done 4 weeks after step 2 Done every 1-6 months, will require fine tuning as the disease progresses

Step 3: Battery placement

Step 4: Initial programming

Programming maintenance

DBS for Essential tremor

No age cut off at Vanderbilt

Over 70 electrode placements are staged with at least one week between sides

Can consider unilateral if higher surgical risk We have consider lesional surgery if there is concern from infection risk, significant cognitive changes

Questions???