EXTRAPYRAMIDAL SYSTEM and BASAL GANGLIA Burcu Ormeci,MD Department of Neurology

Extrapyramidal System 2 main system manage motor functions Pyramidal system Corticospinal tract Extrapyramidal system Basal ganglia caudate putamen globus pallidus EPS is created by the basal ganglia Consist of: -den oluşmaktadır

Neurotransmitters/Neuromodulators at Basal Ganglia GABA  inhibitory Glutamate  excitatory Dopamine  D1 receptors  excitatory D2 receptors  inhibitory Acetylcholine modulator Not excitatory or inhibitory It changes excitability

Motor Functions of the 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 Ball is played, ball was played This plan is made by the basal ganglia

Classification of The Movement Disorders Movement disorders are classified according to dominant abnormal or involuntary movements hypokinetic (reduced movement) hyperkinetic (increased movement)

HYPOKINETIC MOVEMENT DISORDERS

Hypokinetic Movement Disorders It is named «Akinetic-rigid syndromes» Parkinson's syndrome is prototype of Akinetic-rigid syndromes Cogwhile rigidity

Parkinson’s Syndrom Symptoms Bradykinesia-akinesia Rigidity Postural instability Resting tremor may be accompanied, but it is not necessary Fall down

Parkinson’s Syndrom DEGENERATİVE 2 - Parkinson's plus syndromes 1 - Idiopathic Parkinson's disease (IPD) 2 - Parkinson's plus syndromes multisystem atrophy, Progressive supranuclear palsy corticobasal degeneration, Lewy Body Dementia 3 - Parkinsonism associated other degenerative diseases Spinocerebellar ataxia type 2,3,17 Huntington's Disease Striapallidodentat bilateral calcinosis (Fahr's Disease) Frontotemporal dementia-parkinsonism complex Dentatorubropallidolusian atrophy Nöroakantositoz ect Underlying pathology of these diseases are degenerative cell loss and not be detected in another reason

Parkinson’s Syndrom Symptomatic 1 - Vascular (small vessel disease-related subcortical encephalopathy, multiple lacunar infarcts, basal ganglia and Brainstem hemorrhages and infarcts) 2 - Normal pressure hydrocephalus 3 - Tumors 4 - 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 Diseases 10 - Psychogenic Parkinsonism

IDIOPATIC PARKINSON’S DISEASE 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

TREATMENT of 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)

PARKINSON’S DISEASE TREATMENT 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 PLUS SYNDROMS Common features Classic signs of Parkinson's syndrome (bradykinesia, rigidity, tremor, postural reflex abnormalities) + Additional signs and symptoms

HYPERKINETIC MOVEMENT DISORDERS

DYSTONIA 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

Type of Dystonias Hemifasyal Spasm Blepharospasm 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) Laterocollis Anterocollis Retrocollis "Task Specific” dystonia Occurs only during selective motor functions The most common is "writer's cramp"

CHOREA and BALLISM 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

TREMOR Tremor, is involuntary, rhythmic oscillations occur as a result of synchronous contraction of the reciprocal muscles 1- Kinetic tremor - Intensiyenel tremor (cerebellum) 2- Postural Tremor 3-Resting tremor Aksiyon Tremoru (kinetik tremor) Bir ekstremiteyi aktif olarak kullanırken, hareket esnasında ortaya çıkar. Genelde yüksek frekanslıdır ancak genliği az ya da çok olabilir, genlik hareket esnasında hep aynı kalır. Postüral tremor gibi aksiyon tremoru da en sık esansiyel tremor hastalığında görülür. İntansiyon Tremoru Bu tip tremorun en büyük özelliği istemli hareket esnasında ortaya çıkması ve hedefe yaklaşırken tremorun genliğinin çok artmasıdır. Örneğin parmak-burun testinde hasta parmağını burnuna yaklaştırdıkça tremorun genliği artar ve hasta hedefi bulamaz. İntansiyon tremoru düşük frekanslı, kaba bir tremordur ve kural olarak serebellum ve bağlantılarının tutulması sonucu ortaya çıkar. Dizartri ve nistagmus tremora eşlik edebilir.

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

MYOCLONUS 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 Essential 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

Sleep-related Movement Disorders 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

Drug-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

PSYCHOGENIC 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

Treatment of Hyperkinetic Movement Disorders Treatment is symptomatic Anticholinergics Antidopaminergics (neuroleptics) Baclofen Benzodiazepines Botulinum toxin