Overview of movement disorders - short note

1. JMJ Movement Disorders JMJ
IDIOPATHIC PARKINSON’S DISEASE (PD)
SYMPTOMS AND SIGNS
Prodromal premotor symptoms Motor symptoms
 Anosmia (90%)- olfactory bulb is one of the
first structure to be affected
 Depression / anxiety (50%)
 Aches and pains
 REM sleep behavior disorder
 Autonomic features- urinary urgency,
hypotension
 Constipation
 Restless leg syndrome
 Tremor
 Rigidity
 Akinasia
 Postural and gait disturbance
RISK FACTORS
 Age – more than 70 years
 Gender – male
 Environmental factors –
Rural living
Drinking well water
Pesticide exposure
High oxidative stress
Non-smoker
 Genetic factors- usually non-familial
PATHOLOGY
1. Presence of neuronal inclusions (Lewy
bodies)
2. Loss of dopaminergic neurons – pars
compacta of substantia nigra in the midbrain
The extent of nigrostriatal dopaminergic cell loss
correlates with the degree of akinasia
AKINASIA
 Cardinal clinical feature of PD
 Main cause of disability
 Difficulty initiating movement
 Upper limb usually affect first
 Almost always unilateral for the first
years
 Rapid dexterous movements are impaired
causing difficulty in writing (micrographia)
 Facial immobility- mask like semblance of
depression
 Spontaneous blinking diminished –
serpentine stare
TREMOR
 Present in 70% of patients
 Almost always starts in fingers and hands
 Initially unilateral
 Spreading later to the legs on the same side
 Then the opposite arm
 Present at rest
 Reduces or stops completely when the hand
is in motion
 “pill-rolling”
 Tremor made worse by emotion or stress

2. JMJ Movement Disorders JMJ
RIGIDITY
 Sign rather than a symptom
 Stiffness on passive limb movement,
 Present throughout the range of movement
- “lead-pipe”
 Is not dependent on speed of movement
 When stiffness occurs with tremor-
ratchet-like jerkiness is felt – Cog-Wheel
rigidity
POSURAL AND GAIT CHANGES
 Stooping posture is characteristic
 Gait gradually becomes
o Shuffling
o Small stride length
o Slow turns
o Freezing
o Reduced arm swelling
 Postural stability deteriorated – leading to
fall
SPEECH AND SWALLOWING
 Speech become quite, indistinct and flat
 Drooling – present
 Swallowing difficulty is late
 Can lead to aspiration pneumonia
COGNITIVE AND PSYCHIATRIC CHANGES
 Cognitive impairment
 Dementia
 Visual hallucinations on treatment
 Psychosis – not uncommon
 Depression is common
DIAGNOSIS
 No laboratory test
 Diagnosis is made by physical signs
 MRI – normal
 Dopamine transporter (DaT) imaging –
assess the extent of nigrostriatal cell loss
TREATMENT
 Dopamine replacement with levodopa or a
dopamine agonist – improves motor
symptoms
 Non motor symptoms – depression,
constipation, pain and sleep disorders
(Quality of life)

3. JMJ Movement Disorders JMJ
TREATMENT
LEVODOPA DOPAMINE AGONIST
 Most effective treatment
 Combine with a dopa decarboxylase inhibitor
 Can combine with levodopa
 As initial monotherapy in younger patients with
mild to moderate impairment
 Less well tolerated
 Effective than levodopa
OTHER DRUGS USED IN PD
SELEGILINE
 5-10 mg daily
 Mono amine oxidase B inhibitor
 Reduced catabolism of dopamine in
brain
 RASAGILINE
AMANTIDINE
 Modest anti-parkinsonism drug
 Mainly used to improve dyskinesia
in advanced disease
ANTICHOLINERGICS
 May help tremor
 Rarely used in PD except in
younger patients
 High doses cause confusion in
older patients
APOMORPHINE
 Potent, short acting
 Administrated SC
 Used in advanced PD
MANAGEMENT OF PD
1. Dose fractionation of levodopa –
a. increasing dose frequency
2. Addition of COMT inhibitor entacapone to prolong duration of action.
3. Slow release levodopa –
a. mostly used in overnight symptoms as absorption is erratic and difficult to predict, so limiting
effectiveness in control of daytime symptoms
4. Avoiding protein-rich meals (which impair levodopa absorption) and taking doses at least 40 minutes prior
to meals
5. Apomorphine continuous SC infusion
6. Deep brain stimulation and L-dopa intestinal gel
DEEP BRAIN STIMULATION (DBS)
 Usually under 70 years
 Stereotactic insertion of electrodes
into brain
 Selected patients with dyskinesia and
motor fluctuations not adequately
controlled with medical therapy
L-DOPA INTESTINAL GEL INFUSION
 Infusion into small intestine via a jejunostomy
 To selected patients with severe motor
complications
 At present – used where apomorphine or DBS
contraindicated

4. JMJ Movement Disorders JMJ
TISSUE TRANSPLANTATION
 Transplantation of embryonic
mesencephalic dopaminergic cells
directly into the putamen
PHYSIOTHERAPY, OT AND PHYSICAL AIDS
 To reduced disability, speech and
swallowing problems and falls