Burcu Ormeci,MDBurcu Ormeci,MD

Department of NeurologyDepartment of Neurology

2 main system manage motor functions

Pyramidal system Corticospinal tract

Extrapyramidal system Basal ganglia

caudate putamen globus pallidus

Extrapyramidal System

GABA inhibitory Glutamate excitatory Dopamine

D1 receptors excitatory D2 receptors inhibitory

Acetylcholine modulator Not excitatory or inhibitory It changes excitability

Neurotransmitters/Neuromodulators at Basal

Ganglia

The initiation and planning of movements Adjusting speed and magnitude of movement Automatically implementation of learned

motor programs (walking, cycling, etc.) Implementation of consecutive or

simultaneous movements Adjustment of muscle tone Truncal stability

Motor Functions of the Basal Ganglia

Movement disorders are classified according to

dominant abnormal or involuntary movements

hypokinetic (reduced movement)

hyperkinetic (increased movement)

Classification of The Movement Disorders

HYPOKINETIC MOVEMENT DISORDERS

It is named «Akinetic-rigid syndromes»

Parkinson's syndrome is prototype of Akinetic-rigid syndromes

Hypokinetic Movement Disorders

Symptoms

Bradykinesia-akinesia Rigidity Postural instability Resting tremor

may be accompanied, but it is not necessary

Parkinson’s Syndrom

DEGENERATİVE

1 - Idiopathic Parkinson's disease (IPD)2 - Parkinson's plus syndromes

multisystem atrophy, Progressive supranuclear palsycorticobasal degeneration, Lewy Body Dementia

3 - Parkinsonism associated other degenerative diseasesSpinocerebellar ataxia type 2,3,17Huntington's DiseaseStriapallidodentat bilateral calcinosis (Fahr's Disease)Frontotemporal dementia-parkinsonism complexDentatorubropallidolusian atrophyNöroakantositoz ect

Underlying pathology of these diseases are degenerative cell loss and not be detected in another reason

Parkinson’s Syndrom

Symptomatic1 - Vascular (small vessel disease-related subcortical encephalopathy,

multiple lacunar infarcts, basal ganglia and Brainstem hemorrhages and infarcts)

2 - Normal pressure hydrocephalus3 - Tumors4 - Drug-induced (neuroleptics, dopamine exhausting drugs, calcium

channel blockers, antiemetics, valproic acid, lithium)5 - Intoxications (carbon monoxide, manganese, potassium

permanganate, ephedrine abuse, mercury and other heavy metals, organic solvents, paint thinner, carbon disulfide, MPTP, cyanide)

6 - Infections (encephalitis, prion disease, neurosyphilis, toxoplasmosis)7 - Metabolic causes (hypoxia, hypoparathyroidism, extra-pontine

miyelinozis, chronic liver disease, Wilson's disease)8 - Head injury, dementia pugilistica (boxer)9 - Demyelinating Diseases10 - Psychogenic Parkinsonism

Parkinson’s Syndrom

Constitutes 80-85% of parkinsonism

The average age of onset is 50-60 years Early-onset <40 years Juvenile <20 years

Loss of dopaminergic cells in the substantia nigra pars compacta

Lewy bodies

When dopaminergic cell loss reach to 60-70%, clinical symptoms start to occur

IDIOPATIC PARKINSON’S DISEASE

The cardinal symptoms of Parkinson's

disease Bradykinesia

+ Rigidity Resting tremor Postural instability

*Not need to be all-in-one*The disease often begins from half of the body

asymmetrically

IDIOPATIC PARKINSON’S DISEASE

Other clinical symptoms of Parkinson's

disease Anteflexion posture Freezing phenomenon Gait disorders Speech disorders Micrography, dysphagia Autonomic disorders Mental-emotional symptoms Sleep disorders Odor and visual perception disorders

IDIOPATIC PARKINSON’S DISEASE

Resting Tremor

Often, the first clinical finding is motor symptoms, 15% never seen

Frequency is 4-6 Hz Usually emerges on the hands, sometimes

on the feet, tongue, jaw and lip Audio and head tremor never emerges Increases with stress, mental activity and

walking

IDIOPATIC PARKINSON’S DISEASE

Bradykinesia / Akinesia

The most characteristic symptom of basal ganglia disorders

Presence is necessary to diagnosis Bradimimi, micrography, decrease of

associate movements and sialorrhoea develop because of this symptom

The most basic symptom of disability

IDIOPATIC PARKINSON’S DISEASE

Rigidity

Due to agonist and antagonist muscles have increased tone simultaneously

Called cogwheel phenomenon Emerges by passive motion Increases with other extremity motion

(Froment's sign) Patients describe as stiffness or hardness

IDIOPATIC PARKINSON’S DISEASE

Postural instability

The most common cause of disability Occurs in later stages of the disease Due to loss of postural reflex The most common cause of falls Pull test is used for diagnosis Patients fall down while sitting as a block

IDIOPATIC PARKINSON’S DISEASE

Dopaminergic drugs

Levodopa (dopamine precursor) Dopamine agonists These drugs mimic the effect of dopamine as

binding to dopamine receptors Anticholinergic agents

Re-provide deteriorated balance between dopamine and acetylcholine in the basal ganglia

MAO-B inhibitors Main enzyme which destroyed the dopamine Dopamine stays in the synaptic gap for longer time

COMT inhibitors Amantadine (PK-Mertz)

TREATMENT ofPARKINSON’S DISEASE

SURGICAL

Destruction of problematic areas Stereotactic surgery Gamma-knife

Deep brain stimulation High-frequency electrical impulses via deep

electrodes reduce the activity of the cells in problematic areas

PARKINSON’S DISEASETREATMENT

Common features Classic signs of Parkinson's syndrome (bradykinesia,

rigidity, tremor, postural reflex abnormalities)+

Additional signs and symptoms

PARKINSON PLUS SYNDROMS

HYPERKINETIC

MOVEMENT DISORDERS

Characterized by involuntary, continuous,

twisting, rotating muscle contraction Recurrent Results with temporary or permanent

abnormal postures Disappears during sleep Dystonic contractions temporarily loss with

touch Sensory trick

DYSTONIA

Hemifasyal Spasm Only on half of the face May be continuous or intermittent Semi-rhythmic contractions

Blepharospasm Increasing of eye blink frequency functional

blindness Oromandibulolingual Dystonia

Dystonia around the mouth-jaw-tongue Laryngeal Dystonia (pasmodic Dysphonia)

Difficulty of voicing and speech

Type of Dystonias

Cervical Dystonia (spasmodic torticollis)

Torticollis Laterocollis Anterocollis Retrocollis

"Task Specific” dystonia Occurs only during selective motor functions The most common is "writer's cramp"

Type of Dystonias

Arrhythmic, rapid, saltatory or smooth, simple

or complex movements Chorea

Amplitude is smaller Usually seen in distal of extremity

Ballism Amplitude is greater Usually seen in proximal of extremity

CHOREA and BALLISM

Tremor,

is involuntary, rhythmic oscillations occur as a result of synchronous contraction of

the reciprocal muscles

1- Kinetic tremor- Intensiyenel tremor (cerebellum)

2- Postural Tremor3-Resting tremor

TREMOR

Physiological tremor 10-12 hz, postural tremor with low-amplitude Etiology

Mental reasons (anxiety, stress, fatigue), Metabolic causes ( fever, thyrotoxicosis,

hypoglycaemia, ect.), Drugs (neuroleptics, beta-adrenergic agonists,

valproic acid, lithium, steroids, dopamine agonists, antidepressants),

Toxins (mercury, lead, arsenic) Some food ingredients (caffeine)May lead to increased physiological tremor

Tremor

Sudden, short-term (<100ms), as fast

as lightning Form of throwing, jumping

Positive myoclonus The sudden contraction of a particular muscle

group Negative myoclonus (asterixis)

Sudden relaxation of the muscles while maintain a posture

MYOCLONUS

Physiological myoclonus

While falling asleep, after excessive exercise , in anxiety

Essential myoclonus There is no pathological signs and symptoms related

to another disorders Reduce the symptoms with Alcohol is typically

Symptomatic myoclonus During the course of diseases such as Liver failure,

uremia, electrolyte imbalance, poisoning, head trauma, hypoxic-ischemic ensafalopati (Lance-Adams syndrome), neurodegenerative diseases, central nervous system infections, prion diseases… etc

MYOCLONUS

Restless Legs Syndrome

It is identified leg discomfort at rest or before sleep

Periodic limb movements of sleep Semirhythmic involuntary movements during

sleep especially in the feet

Sleep-related Movement Disorders

Acute Dystonic Reactions

Within a few hours after drug intake Especially around the head, neck, face, tongue

and mouth Form of dyskinetic and dystonic involuntary

movements Subacute Parkinsonism or Akathisia

Neuroleptic useing for last 1-3 months Usually reversible

Tardive Syndromes As a result of long-term use of neuroleptic Sometime irreversible

Drug-related Movement Disorders

Can mimic all kinds of movement disorders such as

dystonia, myoclonus, tremor, tic disorder Symptoms onset suddenly Emerge after psychic trauma There is serious handicaps It is bizaare, doesn’t like normal forms If the patient's attention is disrupted, symptoms

ameliorate There are some periods of self-recovery Positive response to placebo treatment The presence of secondary gain Neurological examination is completely normal The presence of symptoms that suggestive

psychopathology

PSYCHOGENIC MOVEMENT DISORDERS

Treatment is symptomatic

Anticholinergics Antidopaminergics (neuroleptics) Baclofen Benzodiazepines Botulinum toxin

Treatment of Hyperkinetic Movement

Disorders