HARSH GUPTA, MDPGY4 RESIDENT

DEPARTMENT OF NEUROLOGYUNIVERSITY OF ARKANSAS FOR MEDICAL

SCIENCES

Movement Disorders forthe Internist

“To study the phenomenon of disease without books is to sail an uncharted sea, while to study books without patients is not to go to sea at all” - William Osler

Brigadier General in World War IGeneral of the Army in World War IISupreme Commander in KoreaOn 11 April 1951, U.S. President Harry S.

Truman relieved him of his commands for making public statements that contradicted the administration’s policies.

Suggested that MacArthur had Parkinson’s Disease (PD), and that his military judgment might have been influenced by this condition.

A combination of dynamic personality and iron will, MacArthur avoided medical examination for most of his military career.

The literature of the system of health care practices in India from 5000 to 3000 BC, the term kampa was used to mean a tremor, and kampavata, an imbalance due to tremor.

In the Western religious tradition, the Book of Ecclesiastes (200 BC) includes the passage

Rest tremor – aggravated by stress or when movements of other body parts are performed.

Postural tremor Kinetic tremorIntention tremor

Parkinson’s Disease

Bradykinesia TremorRigidityPostural disturbances

If etiology is known then it is called as Secondary or Symptomatic Parkinsonism.

Parkinson’s-plus syndrome or Atypical Parkinson’s.

Parkinson’s Disease

Majority patients lack family history. Usually unilateral onset and becomes symmetrical

as the age advances. Tremor – typically rest. Stress or walking makes

it worse. Disappears during sleep. Rigidity. Bradykinesia. Posture – Falls, loss of stability, flexion attitude,

difficulty initiating or stopping. Tremor accentuated while walking. Running???

TRAP

Masked face or hypomimia.Decreased blinking.Hypophonia (soft voice). Positive glabellar tap.

Non-Motor manifestations.

Sialorrhea.Weight loss.Swallowing difficulty.No objective deficit but patients complain of

numbness and tingling. Bradyphrenia.Dementia.Insomnia, nightmares, and daytime sleepiness. Personality change, hallucination, and depression. Dysautonomia.

Levodopa – combined with CarbidopaDopamine agonistAdverse effects: Peripheral v/s Central. DyskinesiaFluctuationsPsychosis – esp. hallucinationsSleep attacksOrthostatic hypotensionObsessionsImpulsivity

COMT inhibitorsMAO-B inhibitorsAmantadineAnticholinergicsSurgical treatment

ESSENTIAL TREMOR

The term ‘essential’ was applied to a number of different disease entities in the 19th century.

The term ‘essential’ was applied to tremor.Some texts use the term ‘essential tremor’

while others continue to refer this as ‘familial tremor’.

“Benign” is a word used to indicate an illness that is either of a mild type, does not threaten life, has no significant effect, not recurrent, or progressive.

A positive family history is very common in patients with ET.

A considerable number of families with presumably autosomal dominant ET exist.

ET patients with a positive family history have a younger age at onset.

Kinetic tremor. Frequency of tremor between 4 to 12 Hz –

inversely related to age. Postural tremor.Neck tremor, jaw tremor, and voice tremor. Rest tremor (severe ET). Somatotopic spread of tremor over time.

- The large number of ET cases had no, minimal, or mild postural tremor.

- In 95% of cases, kinetic tremor was more severe than the postural tremor

- Patients were asked to close their eyes.- Asked to hold their fingers for 15 secs.- Positive response was considered as displacement of at least 5 cms.

- On arm extension, a higher proportion of ET than PD patients had isolated proximal tremor whereas a higher proportion of PD patients had distal tremor.

- Tremor in ET involved the wrist more than distal hand joints. - ET patients had more wrist flexion-extension tremor than wrist

pronation-supination tremor. - Isolated thumb tremor was not a useful test to differentiate between the

two.

TREATMENT

There is no neuroprotective therapy.In milder cases of ET, psychostimulants such

as coffee, tea, and soda should be avoided. Ethanol is a potent suppressor of ET. In mild cases, alcohol can be suggested about

30 mins prior to a tremor discomfort activity. Rebound is a side effect.

Primidone – mechanism of action unknown. Start with 25mg at bedtime and increase

gradually to a target dose of 250mg at bedtime. If there is a partial response then dose can be increased to 500mg at bedtime.

Primidone – level A.

Propranolol 60-320 mg/day; long acting form preferred by some patients.

Atenolol and Sotalol – level B.Nadolol – level C.Metoprolol – uncertain benefit in ET. Benefit is maintained for 1 year and usually

increase in dose is required after this time period.

Other drugs and Surgical treatment.

Botulinum toxin type A (BoNT-A) has modest benefit for limb tremor associated with ET.

Dose dependent hand weakness.BoNT-A for voice tremor is associated with

breathiness, hoarseness, and swallowing difficulty.

No clinical studies using botulinum toxin type B for ET.

Botulinum toxin type A – level C

Differential Diagnosis

Enhanced physiological tremor- Caffeine- Lithium- Sodium valproate- Beta agonist- Hyperthyroidism

- Sudden onset- Short duration- Spontaneous remission- Distractibility – best test- Suggestibility- Entrainment – poor sensitivity but high specificity

DYSTONIA

What is a dystonia? “a sign, a symptom, or a diagnosis”

A syndrome dominated by sustained muscle contractions, frequently causing twisting and repetitive movements or abnormal postures.

Fast, visible, and irregular. Age of onset (early- and late-onset dystonia) Etiology (primary and secondary)Body distributionTask specificSensory tricks (geste antagoniste)

RESTLESS LEG SYNDROME

Urge to move associated with paresthesias. Worse at night and relieved with movement. Primary v/s SecondaryIron deficiency – always check Ferritin!!ESRDDM MSPDPregnancy

LevodopaDopamine agonistBenzodiazepinesOpioidsGabapentin Pregabalin

Take home message

ET – bilateral but asymmetric.PD typically starts unilaterally.Pronation-supination v/s Flexion-extension. Finger displacement test. Check gait. Tremor can be absent in patients with PD. The diagnosis of psychogenic movement

disorder should be made cautiously.

Questions?Please page @ 405-5033!