Differential Diagnosis Of Tremors

Dr Ahmad Shahir MawardiNeurology Department,Hospital Kuala Lumpur

20 March 2017

Outlines

• Introduction• Types of tremor• Common tremors• Approach• Summary

Tremor

• The most common involuntary movement disorders seen in clinical practice

• A rhythmic, involuntary, oscillating movement of a body part occurring in isolation or as part of a clinical syndrome.

Types of tremor Descriptions

Resting tremor occurs when the affected extremity is at complete rest and diminishes with movement

Postural tremor occurs when the affected limb is held in sustention against gravity.

Action or kinetic tremor occurs during voluntary movement.

Intention tremor marked increase in tremor amplitude during the terminal portion of a targeted movement

Task-specific tremor.emerges during a specific activity. e.g primary writing tremor

PATHOPHYSIOLOGY

• not fully understood

• combinations of these mechanisms produce tremor in different disease states

PATHOPHYSIOLOGY• Mechanical oscillations of the limb can occur at a particular joint; this

mechanism applies in cases of physiologic tremor.

• Reflex oscillation is elicited by afferent muscle spindle pathways and is responsible for stronger tremors by synchronization. This mechanism is a possible cause of tremor in hyperthyroidism or other toxic states.

• Central oscillators are groups of cells in the central nervous system in the thalamus, basal ganglia, and inferior olives. These cells have the capacity to fire repetitively and produce tremor. Parkinsonian tremors may originate in the basal ganglia, and essential tremors may originate within the inferior olives and thalamus.

• Abnormal functioning of the cerebellum can produce tremor. PET studies have shown cerebellar activation in almost all forms of tremor.

Tremor Characteristics by Condition

Tremor Characteristics by Condition

Physiologic Tremor

• Very-low-amplitude, fine tremor (6–12 Hz) that is barely visible to the eye.

• Present in every normal person while a posture or movement is being maintained.

• Does not interfere with ADL.

Enhanced Physiologic Tremor

• A high-frequency, low-amplitude, visible when a specific posture is maintained.

• Induced by : – drugs and toxins– hyperthyroidism, liver disease,anxiety, and

hypoglycemia.

Enhanced physiologic tremor

• Tx: – Metabolic etiology (eg, thyroid, glucose)--> treated

accordingly. – Anxious patient--> treatment of the anxiety – Drug-induced--> decrease the dose or stop the drug

Essential Tremor

• The most common • Prevalence: 0.4% to 6.7% in persons over 40 years

• Postural and action tremors• Frequency: 4 and 8 Hz.• Symmetriccal onset (most patients)

• Most commonly : – hands (95%) head (34%) and voice (12%)

• Less common: – legs (20%) trunk and face (5%)

• with increasing time, frequency decreases and the amplitude may increase

Essential Tremor

• In familial ET(AD) with incomplete penetrance.– A positive family history (50% to 70%)

• Worsens during eating, drinking, and writing.

• Long-standing ET Mild resting tremor

• A ‘‘no-no’’ or ‘‘yes-yes’’ head tremor is characteristic of ET

*Elble RJ. Diagnostic criteria for essential tremor and differential diagnosis.Neurology 2000;54:S2–6.

Essential Tremor

• Improves with relaxation and alcohol (50%)

• Associated symptoms: gait difficulty (manifested as tandem walking), decreased hearing.

• Variants of essential tremor:– Task-specific tremor (eg, primary writing tremor)– Isolated voice tremor– Isolated chin tremor

Essential Tremor

• Tx not recommended for mild cases

• Decision to treat is based on:– age, coexistent conditions, prior exposure to drug therapy,

concurrent drug therapies, contraindications, physician and patient bias, benefits and potential adverse effects

• Different body parts a may also have different pharmacological responsiveness

Essential tremor

• First-line treatments: Propranolol and primidone– better response for hand tremors than for voice and head tremors

Essential tremor

Other therapies:

1. Botulinum toxin injections - limb, head, vocal, palatal, and other tremors.

2. Anxiolytics (Tremors associated with anxiety)

3. Refractory thalamic lesioning or DBS

Parkinson’s Disease

• Tremor:– a low-frequency resting tremor :70% (pill-rolling tremor)– asymmetric– 4 to 6 Hz, (distal --> proximal --> contralat) – Some patients: postural and action tremors. – Re-emergent tremor (occurs a few seconds after the hands have

been held in sustention and action).

• Most commonly affected: hands, legs, chin, and jaw.• a/w, bradykinesia, rigidity, micrographia

Parkinsonian Versus Essential Tremor

Micrographia - PD hand writing

ET handwriting

*DA are useful in advanced PD pts with tremor that is refractory to levodopa & anticholinergics

Parkinson’s Diasease

• Medication-refractory tremors functional neurosurgery– Lesioning procedures (eg, thalamotomy) – DBS (the thalamus, globus pallidus, or subthalamic

nucleus

Cerebellar Tremor

• slow-frequency tremor (3 and 5 Hz)

• Occurs during the execution of a goal-directed (intentional) movement

• can be associated with a postural component.

• S&Sx of cerebellar dysfunction may be present (ataxia, dysmetria, dysdiadochokinesia, and dysarthria)

• best elicited during the finger-nosefinger or heel-shin-heel tests

Cerebellar Tremor

• Titubation tremor: – described as a slow-frequency “bobbing”

motion of the head or trunk.– usually seen in MS, hereditary ataxia

syndromes, cerebellar infarction, and traumatic brain injury.

Cerebellar tremor

• No medication has been consistently successful

• Medications that can be tried:– clonazepam, propranolol, trihexyphenidyl,

levodopa, physostigmine, and topiramate.

• Thalamic stimulation for disabling tremor may be an option

Psychogenic Tremors

• onset a/w a stressful life event• irregular frequency & amplitude• sudden onsets and remissions.• increase of their tremor amplitudes during loading• selective disability• Most often: right hand (84%), followed by legs (28%),

generalized (20%), left arm (8%) and head (8%)• Uncommon: voice, face, tongue, and fingers

*Manyam BV. Uncommon forms of tremor. In: Watts RL, Koller WC, eds. Movement disorders: neurologic principles and practice. 2nd ed. New York: McGraw-Hill, 2004:459–80.

Psychogenic tremor

1. Suggestibility– Suggestion and placebo can to exacerbate or relieve tremor

2. Distractibility

3. Entrainability – tremor automatically changes to the frequency that is being enforced

on the uninvolved hand or foot

4. Coactivation sign– presence of voluntary coactivation of agonist and antagonist muscles

of the respective joint – fluctuation of the tone with reduction or increase in the tremor– produce bizarre positioning of the hands when they are outstretched.

Other tremors

• A• B• C• D• E

Holmes Tremor

• a combination of rest, postural, and action tremors due to midbrain lesions in the vicinity of the red nucleus.

• Irregular and of low frequency tremor(2–4 Hz)• predominantly proximal limbs• Signs of ataxia and weakness may be present.

• Common causes : CVA, MS with a possible delay of 2 weeks to 2 years in onset and sx.

• The tremor is disabling and resistant to treatment.

Dystonic Tremor

• tremor that occurs in a body region affected by dystonia.

• Postural/action tremor with irregular amplitudes and frequencies.

• E.g: no-no or yes-yes head tremor associated with spasmodic torticollis.

• Irregular or arrhythmic tremor and may improve with a “sensory trick” (geste antagoniste)

Dystonic tremor.

• botulinum toxin injections.

• Other medications:– anticholinergic agents, levodopa, propranolol,

and clonazepam

Neuropathic tremor

• Neuropathic tremors are mostly postural or action tremors that occur in the setting of a peripheral neuropathy.

• 3 and 6 Hz in the hand and arm muscles.

• They are more commonly a/w demyelinating neuropathies of the dysgammaglobulinemic type.

• The exact etiology of this tremor is unknown.

Neuropathic tremors.

• No successful pharmacologic treatment has been reported.

• Pharmacotheraphy: – clonazepam, primidone, and propranolol

(inconsistent benefit).

• Disabling neuropathic tremor thalamic DBS

Palatal Tremor

• brief, rhythmic, involuntary, low-frequency movements of the soft palate.

• Classified in two forms:

1. Symptomatic palatal tremor – arise from a lesion of the brainstem or cerebellum (within the Guillain-

Mollaret triangle), resulting in a rhythmic contraction of the levator veli palatini.

– Movement of the edge of the palate is appreciated.

2. Essential palatal tremor(often a/w an ear click)– not associated with CNS lesions – a result of the rhythmic contractions of the tensor veli palatini– Movement of the roof of the palate is also seen.

Palatal tremors

• usually not disabling.

• Patients who are bothered by the ear click may benefit from trihexyphenidyl, valproate, or flunarizine.

• Injection of botulinum toxin in the tensor veli

Drug-Induced Tremors

• Types of tremors include enhanced physiologic tremor, rest tremor, and action tremor.

• The signs and symptoms depend on the drug used and on a patient’s predisposition to its side effects.

• Some drugs cause extrapyramidal side effects manifesting as bradykinesia, rigidity, and tremor.

• Tx: discontinuation/reduction of the dose of the offending agent

List of Potential Toxins and Drugs Inducing Tremor

Orthostatic Tremor

• Middle aged/elderly people

• characterised by unsteadiness on standing– tremors remit on walking, but disappear when sitting or lying down

• Confirmation : EMG showing a 16 Hz pattern

• The treatment of choice : – low-dose clonazepam.– Other options: Phenobarbital, primidone, propranolol, levodopa,

pramipexole, and gabapentin

Tremor in Wilson’s disease

• All tremor types can be seen. – Most common: resting and postural tremors.– “wing-beating tremor” late stage,refractory to medication

• Other neurological manifestations: Ataxia, parkinsonism, dysarthria, dystonia, and risus sardonicus.

• Often accompanied by liver disease and psychiatric manifestations (eg, depression, anxiety, psychosis).

• This diagnosis should be considered for any patient with a movement disorder presenting before the age of 50 years.

Risus sardonicus

Wilson’s disease.

• Low copper diet.

• Copper chelation agent: – penicillamine (1–2 g/d) with pyridoxine (50 mg/d), – trientine (500 mg 2 times daily)– tetrathiomolybdate (80–120 mg daily in 3 to 4 divided doses)– zinc (50 mg/d without food).

• Thalamotomy.

EVALUATION OF THE PATIENT WITH TREMOR

• History– history of the neuropathy, drug use, and toxic

exposure and the family history• Examination• Workout

– not necessary for most patients

Examination

W O R K O U T S

*Deuschl G, Bain P, Brin M; Ad Hoc Scientific Committee. Consensus statement of the Movement Disorder Society on Tremor. Mov Disord. 1998;13(suppl 3):2-23.

Summary

• Make sure it is Tremor• Predominenat tremor• Evidence of functional tremor• Additional sign

– Parkisonism– Cerebellar– Dystonia– Peripheral neuropathy– Hyperthyrodism

References

1. A Practical Approach to Movement Disorders, 2nd Edition, Diagnosis and Management-Hubert Fernandez, Andre Machado, Mayur Pandya Demos Medical (2014)

2. R Bhidayasiri, Differential diagnosis of common tremor syndromes, Postgrad Med J 2005;81:756–762.

3. PAUL CRAWFORD, MD ETHAN E. ZIMMERMAN, MD, Differentiation and Diagnosis of Tremor, American Family Physician

4. Elble RJ. Diagnostic criteria for essential tremor and differential diagnosis.Neurology 2000;54:S2–6.

5. Deuschl G, Bain P, Brin M; Ad Hoc Scientific Committee. Consensus statement of the Movement Disorder Society on Tremor. Mov Disord. 1998;13(suppl 3):2-23.

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