MOVEMENT DISORDER

BASJIRUDDIN ABAGIAN/SMF ILMU PENYAKIT SARAF FK UNAND/

RS DR M DJAMIL PADANG

MOVEMENT DISORDER GENERAL OVERVIEW

A. HYPERKINESIA TREMORChoreaDystonia Athetosis Tics

B. HYPOKINESIAParkinsonismSpasticityDrop attack

A. HYPERKINESIA

• TREMOR : rhytmical involuntary oscilations around a fixed point occur at rest outstretching (postural), during anxiety,

caffeine drugs On action (intention) :

Cerebral dysfunctionDrugs (phenitoin)StrokeTrauma

TREMOR

• Essential tremor : retlatively benign, embarrasing disorder, familial, sporadic forms, aggravated by stress,excitement

• Dystonic tremor : involuntary torsion movement, affected muscle group, movement ussually slow

• Resting tremor (Parkinsonism)

• Exaggerated physiologic tremor : Small amplitude, high frequency

ESSENTIAL TREMOR

• Upper extremity tremor with posture and/or action

• Bilateral, usually roughly symmetric

• Tremor may produce disability

• No clear association with other diseases or disorders

ESSENTIAL TREMOR contd….

TREATMENT

• Primodone

• Propanolol 10-20 mg/day and other Beta blockers

• Tremors of some patients are quite responsive to alcohol, and patients may self-medicate

CHOREA • Excessive spontaneous movements , rapid, arrhytmic

movements of muscle group• The movement are often incorporated into deliberate

movements by the patient to camouflage their disorder • Irreguler, brief and aburpt non stereotype (non

repetitive)• Distal predominance • Facial grimacing

A. HYPERKINESIA Tremor CHOREA Dystonia Athetosis Tics

CHOREA contd….

CAUSES :

• Medications – Haldol, other antipsychotics – Reglan is an important cause of tardive dyskinesia

• Huntington’s disease

• Hemibalism

• Post-infection

CHOREA

1. Chorea sydenham 2. Huntington disease

1. Chorea sydenham• Acute movement• Paroxismal• Uncoordinated movement• Involuntary• Emotional disturbances• Diminish while sleeping and increase by stress

CHOREA contd….

2. Huntington disease : is a neurodegenerative disease charactized by progresive choreoathetosis, psychological changes

• Clinical appearanceChoreaticCognitive dysfunctionGait disorderClumsiness Speech disorderBladder and bowel incontinence Sexual dysfunction

CHOREA contd….

TREATMENT

• Valproic acid : 15-20 mg/kg/days

• Carbamazepine : 10-15 mg/kg/days

• Corticosteroid

• Dopaminergic blocker : • Halloperidol : 3 -40 mg/days• Primazole

DYSTONIA • Dystonia is a slow, purpose, involuntary movements

affecting muscle groups of face, limb, trunk• Agonist and antagonist • Clinical findings : – Repetitive twisting and squeezing movements – Fixed posture

Caused :• Idiopathic (most cases)• Drug related : – Antipsychotics and Reglan

A. HYPERKINESIA Tremor ChoreaDYSTONIA Athetosis Tics

ATHETOSIS

• A movement charactherized by slow, writhing of groups of muscle

• More pronounce in the distal extremities

• Associated with weakness and rigidity• Aggravated by stress• Disappears during sleep• Athetosis is slower than chorea and may

occur together

A. HYPERKINESIA Tremor Chorea Dystonia ATHETOSIS Tics

TICS

• Definition : brief, sudden, irresistible, inapposite, reccurent movement

• These movements are either isolated or represent an act for a particular purpose

• For a time tics can be suppresed or inhibited• Patients often feel actively in performing a tic• Tics can be tiggered by environmental

stimuli, exciting events or life event

A. HYPERKINESIA Tremor Chorea Dystonia Athetosis TICS

PARKINSON’S DISEASE

• Parkinson’s disease is a chronic neurodegenerative disease associated with substantial morbidity, increased mortality, and high economic burden

• Parkinson’s results from the degeneration of dopamine-producing nerve cells in the brain, specifically in the substantia nigra.

B. HYPOKINESIAPARKINSON Spasticity Drop attack

PARKINSON DISEASE contd….

EPIDEMIOLOGY

• The most common movement disorder affecting 1 – 2 % of the general population over the age of 65 years.

• Prevalence rate in men are slightly higher than in women

• Age onset usually between 50-70 years

• Rarely in people less than 30 years old

• Incidence is 20 every 100.000 population

PARKINSON DISEASE contd....

RISK FACTORS Age - the most important risk factor Positive family history Male gender Environmental exposure: Herbicide and pesticide

exposure, metals (manganese, iron), well water, farming, rural residence, wood pulp mills; and steel alloy industries

Race Life experiences (trauma, emotional stress,

personality traits such as shyness and depressiveness)?

An inverse correlation between cigarette smoking and caffeine intake in case-control studies

PATHOPHYSIOLOGY

• The etiology of parkinson disease is not yet clear

• It’s widely believed that genetic and enviromental factor induce neuronal death

• The most common pathological feature is degeneration of dopaminergic neurons in pars compacta of substansia nigra

• The lost of dopaminergic neuron decreased activity of thalamus,thus reducing excitatory input to motor cortex and initiate ivoluntary movement

• The presence of lewy bodies is another classic

pathological finding in parkinson disease

CLINICAL FEATURES

• Four cardinal symptoms:

® Resting tremor

® Bradykinesia (generalized slowness of movements)

® Muscle rigidity

® Postural instability

CLINICAL FEATURES contd….

• Resting tremor: most common first symptom, usually asymmetric and most evident in one hand with the arm at rest.

Shaking or trembling in the hand, arm, leg, face, and it spreads, sometimes affecting only one side of the body.– Worsen when the muscles are relaxed or individual is stressed– Dissapears during sleep or during intentionally moved

• Bradykinesia: spontaneus and automatic movement are lost and all movement becomes extremely slow. Diffiulty with daily activities such as writing, shaving, using a knife and fork and opening buttons

Decreased blinking, masked facies, slowed chewing and swallowing.

CLINICAL FEATURES contd....

• Rigidity: muscle tone increased in both flexor and extensor muscles providing a constant resistance to passive movements of the joints

Stooped posture, anteroflexed head, and flexed knees and elbows.

• Postural instability: due to loss of postural reflexes. balance and coordination become impaired.

Patients tend to lean forward or backward, and to develop a stooped posture. Walking with quick and small steps.

ADDITIONAL CLINICAL FEATURES

• Dysfunction of the autonomic nervous system: impaired gastrointestinal motility, bladder dysfunction, excessive head and neck sweating, and orthostatic hypotension.

• Depression: mild to moderate depression in 50% of patients.

• Cognitive impairment: mild cognitive decline including impaired visual-spatial perception and attention

Slowness in execution of motor tasks At least 1/3 become demented during the course of the

disease.

OTHER SYMPTOMS

• Difficulty swallowing or chewing• Urinary problems• Constipation• Irregular sleep• Short breathing

NON-MOTOR FEATURES OF PD :

• Include :– mental health problems

• depression• psychotic symptoms• dementia

– sleep disturbance– falls– autonomic disturbance

PARKINSON DISEASE contd….

DIAGNOSIS

• No specific test or marker for PD

• Diagnosis is made on clinical ground

• Depends on the presence of at least two of the three major signs : tremor at rest, rigidity, and bradykinesia.

• Bradykinesia is tested by determining how quickly the person can tap the finger and thumb together.

Clinical criteria for diagnosis (by Hughes)

Possible• Alt least one of TRAP symptoms (tremor, rigiditas,

akinesia, postur tak stabil)

Probable• Combining 2 major symptoms (including postural

instability) or 1 of 3 asymetrical cardinal signs

Definite• Combining 3 of 4 major symptoms or 2 symptoms with

another asymetrical symptom (3 cardinal signs)

TREATMENT

• The goal of therapy is to reverse functional disability, abolition of all symtoms and signs is not currently possible even with high dose of medication

• Treatment highly individualized

• no universal first choice drug therapy

• choice of adjuvant drug should take into account• clinical and lifestyle characteristics• patient preference

TREATMENT contd….

1. Supporting treatment– Explanation to the patient, giving support, and and

occupational counseling

– education for the patient, in order obtain general picture of the disease

– Emotional support and professsional counseling

– Training in accordance with their physical condicions

TREATMENT contd….

2. Medication

• Anticholinergic : benztropine mesylate 1-8 mg/day

thyhexyphenidil 3 -6 mg/ day

• NMDA antagonist : amantadine (symetrel) 100-300mg/day

• Dopaminergic : carbidopa+levodopa 10/100mg, 25/100mg, 25/250 mg

• Dopamine agonist : bromocryptine 5-40mg/day

pramipexole 1,5-4,5 mg/day

ropinirole 0,75-2,4mg/day

• COMT inhibitors : entacapone 200 mg/day

• MAO-B inhibitors : selegiline 5 mg/day

TREATMENT contd….

2. Operative treatment

Deep brain Stimulation

2. Rehabilitation treatment: physic, occupation, speech, psychotherapy

SUGGESTED ACTIONS

• make sure there are enough– physiotherapists– occupational therapists– speech and language therapists

• PD patients should have regular access to– monitoring and alteration of medication– a continuing point of contact– a reliable source of information

COMPLICATION OF PARKINSON DISEASE

Complication of long-term levodopa therapy• Dyskinesia• Freezing• Falls• Response fluctuations

Behavioral / psychiatric disorder• Dementia• Depression• Psycoses

REFERENCE

1. Waters CH. Diagnosis and maanagement of Parkinson’s disease, second edition. Caddo, Professional Communications nc; 1999: 31-71.

2. Basjiruddin A. Management of late’s Parkinson’s disease. In: Sjahrir H, dkk (eds). Parkinson’s disease and other movement disordres. Medan; 2007: 124-43.

3. Wolter’s EC, Bosboom JLW. Parkinson’s disease. In: Wolters et al (eds). Parkinsonism and related disorders. Amsterdam, VU University Press; 2007:143-155.

4. Parkinsons: Clinical features and differential diagnosis. Fahn S, Jankovic J. Principles and practice of movement disorders. Philadelphia, Churchill Livingstone Elsevier; 2007: 79-96.

5. Benazzouz A. Parkinson’s disease and implication of basal ganglia in its pathoophysiology. Egypt, June 2009.

6. NHS National Institu for Health and Clinical Excellence. Parkinson’s disease.June, 2006

7. Jakala P. Parkinson’s disease, finland, 2008

THANK YOU