Essential tremor - Clinicals, Diagnosis, and Management

Neurology

Clinicals - History

Fact Explanation
Tremulous upper/lower extremities. Shaky head movement ET is generally recognized as due to cerebellar system dysfunction and the presence of several types of structural-anatomical changes in the cerebellum.
Shaky legs/feet is less common.
Other affected body parts may include eyelids and trunk.
Head movement in an up-and-down or side-to-side motion.
Tremors worsen with voluntary movement, stress, anxiety, excitement, emotional upset, fatigue and cold temperatures.
Improvement in tremor is witnessed on alcohol consumption.
Tremulous upper/lower extremities. Shaky head movement
ET is generally recognized as due to cerebellar system dysfunction and the presence of several types of structural-anatomical changes in the cerebellum.
Shaky legs/feet is less common.
Other affected body parts may include eyelids and trunk.
Head movement in an up-and-down or side-to-side motion.
Tremors worsen with voluntary movement, stress, anxiety, excitement, emotional upset, fatigue and cold temperatures.
Improvement in tremor is witnessed on alcohol consumption.
Difficulty performing tasks Performing tasks such as writing,brushing, dressing up, sewing, eating or drinking is difficult due to the kinetic tremor of the hands. Difficulty performing tasks
Performing tasks such as writing,brushing, dressing up, sewing, eating or drinking is difficult due to the kinetic tremor of the hands.
Trembling voice when speaking Larynx (voice box) is affected. Trembling voice when speaking
Larynx (voice box) is affected.

Clinicals - Examination

Fact Explanation
Positive family history 50-70% of the ET patients have been found to have a positive family history - Autosomal dominant inheritance. Positive family history
50-70% of the ET patients have been found to have a positive family history - Autosomal dominant inheritance.
Regular tremor Regular tremor on out-stretched hands and also on finger-to-nose maneuver is observed. Bilateral persistent symmetrical postural or kinetic tremors.
Generally recognized as due to cerebellar system dysfunction and the presence of several types of structural-anatomical changes in the cerebellum.
Regular tremor
Regular tremor on out-stretched hands and also on finger-to-nose maneuver is observed. Bilateral persistent symmetrical postural or kinetic tremors.
Generally recognized as due to cerebellar system dysfunction and the presence of several types of structural-anatomical changes in the cerebellum.
Mild regular “waviness” seen when writing or drawing spirals Caused because of the hand tremor due to cerebellar system dysfunction Mild regular “waviness” seen when writing or drawing spirals
Caused because of the hand tremor due to cerebellar system dysfunction
"Ratchety” quality "Ratchety" quality felt in the tone of the controlateral arm when performing voluntary movement with one hand.
Generally recognized as due to cerebellar system dysfunction and presence of several types of structural-anatomical changes in the cerebellum.
"Ratchety” quality
"Ratchety" quality felt in the tone of the controlateral arm when performing voluntary movement with one hand.
Generally recognized as due to cerebellar system dysfunction and presence of several types of structural-anatomical changes in the cerebellum.

Investigations - Diagnosis

Fact Explanation
Tremor focused neurological examination Clinical criteria for ET (MDS consensus statement Deuschl et al., 1998):

INCLUSION CRITERIA:
*A bilateral, largely symmetric, postural or kinetic tremor which involves both hands and forearms, and is visible and persistent.
*Additional/isolated tremor in the head, but absence of abnormal posturing.

EXCLUSION CRITERIA: 
*Other abnormal neurological signs (especially dystonia).
*Presence of known causes of enhanced physiologic tremor.  
*Historical or clinical evidence of psychogenic tremor.  
*Convincing evidence of sudden onset or step-wise deterioration.  
*Isolated voice, tongue, chin, leg tremor.
*Isolated position- or task-specific tremor.
Tremor focused neurological examination
Clinical criteria for ET (MDS consensus statement Deuschl et al., 1998):

INCLUSION CRITERIA:
*A bilateral, largely symmetric, postural or kinetic tremor which involves both hands and forearms, and is visible and persistent.
*Additional/isolated tremor in the head, but absence of abnormal posturing.

EXCLUSION CRITERIA: 
*Other abnormal neurological signs (especially dystonia).
*Presence of known causes of enhanced physiologic tremor.  
*Historical or clinical evidence of psychogenic tremor.  
*Convincing evidence of sudden onset or step-wise deterioration.  
*Isolated voice, tongue, chin, leg tremor.
*Isolated position- or task-specific tremor.
Electromyography or accelerometry Assess tremor frequency, rhythmicity, and amplitude.
*Differentiate between ET and PK: a tremor frequency below 5.5 Hz suggests PD; a tremor frequency above 6 Hz suggests ET.
*When orthostatic tremor is suspected EMG is needed to confirm the diagnosis - typical high-frequency (13–18 Hz) EMG pattern appearing after a short period of standing.
Electromyography or accelerometry
Assess tremor frequency, rhythmicity, and amplitude.
*Differentiate between ET and PK: a tremor frequency below 5.5 Hz suggests PD; a tremor frequency above 6 Hz suggests ET.
*When orthostatic tremor is suspected EMG is needed to confirm the diagnosis - typical high-frequency (13–18 Hz) EMG pattern appearing after a short period of standing.
Lab test: Standard electrolyte panel, R/o metabolic disturbances (most common: hypoglycemia) Lab test: Standard electrolyte panel,
R/o metabolic disturbances (most common: hypoglycemia)
Lab test: Thyroid function tests Recommended in patients with an action tremor.
R/o thyroid diseases (most common: hyperthyroidism)
Lab test: Thyroid function tests
Recommended in patients with an action tremor.
R/o thyroid diseases (most common: hyperthyroidism)
Lab test: Serum ceruloplasmin Considered in patients with an unexplained tremor and under 55 years of age. R/o Wilson disease Lab test: Serum ceruloplasmin
Considered in patients with an unexplained tremor and under 55 years of age. R/o Wilson disease
MRI of the head Indicated if the tremor has an acute onset or a stepwise progression.
Also for exclusion of inflammatory (including multiple sclerosis) and structural lesions and Wilson disease.
MRI of the head
Indicated if the tremor has an acute onset or a stepwise progression.
Also for exclusion of inflammatory (including multiple sclerosis) and structural lesions and Wilson disease.
Single-photon emission CT (SPECT) scanning using ioflupain 123 I (DaTSCAN) to rule out parkinsonism. Single-photon emission CT (SPECT) scanning using ioflupain 123 I (DaTSCAN)
to rule out parkinsonism.
Screen for drugs of abuse and alcohol consuption Alcohol overuse and withdrawal can cause tremor. Conversely, small amounts of alcohol can temporarily relieve essential tremor and hence can be a clue to the diagnosis. Screen for drugs of abuse and alcohol consuption
Alcohol overuse and withdrawal can cause tremor. Conversely, small amounts of alcohol can temporarily relieve essential tremor and hence can be a clue to the diagnosis.

Investigations - Management

Fact Explanation
Adjusting of medication doses may be needed over time. Because essential tremor is a slowly progressive disorder there may be lose of benefit and adverse reactions on long term use. Adjusting of medication doses may be needed over time.
Because essential tremor is a slowly progressive disorder there may be lose of benefit and adverse reactions on long term use.
Follow-up with family members. During follow-ups of the patient, since essential tremor is familial it is appropriate to make family members aware of it and examine them as well when suspected of an ET. Follow-up with family members.
During follow-ups of the patient, since essential tremor is familial it is appropriate to make family members aware of it and examine them as well when suspected of an ET.

Management - Supportive

Fact Explanation
Avoid caffeine and smoking To prevent induction of tremors. Avoid caffeine and smoking
To prevent induction of tremors.
Get enough sleep Tremors disappear during sleep. Get enough sleep
Tremors disappear during sleep.
Alcohol (30ml) 1-2 drinks. Drinking should not be recommended as a treatment for non-alcoholics but this can be used an alternative for intermittent tremor control in those who consume alcohol. Alcohol (30ml)
1-2 drinks. Drinking should not be recommended as a treatment for non-alcoholics but this can be used an alternative for intermittent tremor control in those who consume alcohol.
Use of larger utensil handles or wrist weights Helps in coping with kinetic tremor of the hands Use of larger utensil handles or wrist weights
Helps in coping with kinetic tremor of the hands

Management - Specific

Fact Explanation
First line drugs Beta-adrenergic blockers-
Propranolol: 40-320mg/d ) - a better choice for younger patients than older patients because of serious adverse effects (dizziness, confusion, memory loss) in older adults. Contraindicated for use in patients with asthma, diabetes, cardiac conduction block or heart failure. (Primidone is preferable for those patients.)
Anti-convulsants-
Primidone: (50-1000mg twice a day) - drug of choice for older adults. Contraindicated in patients with hypersensitivity to phenobarbital and porphyria.
First line drugs
Beta-adrenergic blockers-
Propranolol: 40-320mg/d ) - a better choice for younger patients than older patients because of serious adverse effects (dizziness, confusion, memory loss) in older adults. Contraindicated for use in patients with asthma, diabetes, cardiac conduction block or heart failure. (Primidone is preferable for those patients.)
Anti-convulsants-
Primidone: (50-1000mg twice a day) - drug of choice for older adults. Contraindicated in patients with hypersensitivity to phenobarbital and porphyria.
Second line drugs Benzodiazepines-
Alprazolam(0.125–0.5 mg/d): Effective in patients whose tremor is frequently aggravated by anxiety or other stressors.
Clonazepam(0.5-4mg twice a day): Helpful in patients who failed other first and second lines of therapy.
GABA analogs-
Gabapentin (100-3600mg three times a day): Low effectiveness in patients who have failed primidone or propranol.
Pregabalin (25-75mg twice a day): Low dosage is recommended because doses past 300mg have increased incidence of side effects.
Anti-convulsants-
Topiramate (25-400mg twice a day): Most effective second-line agent and can be used in patients not responding to propranolol and primidone.
Botulinum toxin - useful in the treatment of head and voice tremor. Given once every 3 months :
for head tremor - 50 units to 400 units
for voice tremor - 0.6 to 14 units
Second line drugs
Benzodiazepines-
Alprazolam(0.125–0.5 mg/d): Effective in patients whose tremor is frequently aggravated by anxiety or other stressors.
Clonazepam(0.5-4mg twice a day): Helpful in patients who failed other first and second lines of therapy.
GABA analogs-
Gabapentin (100-3600mg three times a day): Low effectiveness in patients who have failed primidone or propranol.
Pregabalin (25-75mg twice a day): Low dosage is recommended because doses past 300mg have increased incidence of side effects.
Anti-convulsants-
Topiramate (25-400mg twice a day): Most effective second-line agent and can be used in patients not responding to propranolol and primidone.
Botulinum toxin - useful in the treatment of head and voice tremor. Given once every 3 months :
for head tremor - 50 units to 400 units
for voice tremor - 0.6 to 14 units
Third line drugs (mostly suitable for patients who are not candidates for surgical therapy.) Calcium channel blockers-
Nimodipine (30mg four times a day): May be considered in patients who have failed other commonly used medications.
Atypical neuroleptic agents-
Clozapine(25-75mg/d): May be considered in medically refractory ET if other nonpharmacological options are either contraindicated or not desired by the patients.
Third line drugs (mostly suitable for patients who are not candidates for surgical therapy.)
Calcium channel blockers-
Nimodipine (30mg four times a day): May be considered in patients who have failed other commonly used medications.
Atypical neuroleptic agents-
Clozapine(25-75mg/d): May be considered in medically refractory ET if other nonpharmacological options are either contraindicated or not desired by the patients.
Surgical management Indicated in rare cases where patients have disabling and medically refractory tremor; and also useful in reducing head and voice tremor.
Deep brain stimulation (DBS) : Can be performed bilaterally with fewer side effects than thalamotomy.
Thalamotomy (Rest tremor gets completely suppressed)
Surgical management
Indicated in rare cases where patients have disabling and medically refractory tremor; and also useful in reducing head and voice tremor.
Deep brain stimulation (DBS) : Can be performed bilaterally with fewer side effects than thalamotomy.
Thalamotomy (Rest tremor gets completely suppressed)

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