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BASAL GANGLIA AND

DEGENERATIVE DISEASE
MOTOR SYSTEM.

 PIRAMYDAL SYSTEM
 EXTRAPYRAMIDAL SYSTEM

EXTRAPYRAMIDAL SYSTEM :
 NUCLEI
 CIRCUITS
 DESCENDENCE PATHWAYS
ANATOMI

1. KORTEKS EXTRAPIRAMIDALIS
(AREA BRODMANN 4, 6 DAN 8)
2. GANGLIA BASALIS :
 KORPUS STRATUM
 NUKLEUS KAUDATUS
 NUKLEUS LENTIFORMIS
(GLOBUS PALIDUS DAN PUTAMEN)
 NUKLEUS SUBTALAMIKUS
(KORPUS LUYSI)
 TALAMUS
(NUKL. VENTROLATERALIS TALAMI)
ANATOMI (LANJ.)

3. NUKLEUS RUBER
4. SUBSTANSIA RETIKULARIS BATANG OTAK
5. SEREBELUM
SIRKUIT

SIRKUIT 1.
KORTEK SEREBRI  STRIATUM  GLOBUS
PALIDUS  TALAMUS  KORTEK SEREBRI

SIRKUIT 2.
A. KORTEK SEREBRI  STRIATUM 
SUBSTANSIA NIGRA
B.1. SUBSTANSIA NIGRA  STRIATUM
2. SUBSTANSIA NIGRA  TALAMUS 
KORTEK SEREBRI (BRODMANN 4, 6 DAN 8)
SIRKUIT (LANJ.)

SIRKUIT 3.
GLOBUS PALIDUS  NUKLEUS SUBTALAMIKUS
(NUKLEUS LUYSI)  GLOBUS PALIDUS

SIRKUIT 4
KORTEK SEREBRI  NUKLEUS PONTIS
IPSILATERAL (TR. KORTIKOPONTIS) 
SEREBELUM (TR. PONTOSEREBELARIS) 
TALAMUS  KORTEK SEREBRI.
SIRKUIT (LANJ.)

SIRKUIT 5.

KORTEK EKSTRAPIRAMIDALIS  NUKLEUS


BATANG OTAK DAN MEDULA SPINALIS:
MELALUI TARKTUS-TRAKTUS:
 TR. PIRAMIDALIS
 TR. KORTIKORUBROSPINALIS
 TR. KORTIKORETIKULARIS
 TR.KORTIKORETIKULOSPINALIS
FUNGSI SISTEM EKSTRAPIRAMIDALIS

MENGATUR :
GERAKAN ASOSIATIF
POSTURAL
INTEGRASI OTONOMI

LESI EKSTRAPIRAMIDALIS
MENYEBABKAN:
 GERAKAN INVOLUNTER
 GANGGUAN TONISITAS
 GANGGUAN POSTURAL
PATOFISIOLOGI

# HUBUNGAN SINAPTIK SIRKUIT :


FASILITASI ATAU INHIBISI
# GANGGUAN FUNGSI INHIBISI  FUNGSI
FASILITASI :
RELEASE PHENOMENON
CONTOH:
TREMOR PARKINSON
GANGGUAN MOTORIK
EKSTRAPIRAMIDALIS

1. AKINESIA (BRADIKINESIA, HIPOKINESIA)


 GANGGUAN KECEPATAN DAN SPONTANITAS
GERAKAN
 TAK ADA KELUMPUHAN
 CONTOH :
 GERAKAN HABITUAL TERGANGGU
 MUKA TOPENG
 MARS A PTIT PAS
 MIKROGRAFI
 GERAKAN REAKTIF MELAMBAN (PROPULSI,
RETROPULSI DAN LATEROPULSI)
GANGGUAN MOTORIK
EKSTRAPIRAMIDALIS

2. TREMOR

 GERAKAN OSILASI RITMIK DAN REGULER


 RESTING TREMOR
 PARKINSON ROLLING PILL
GANGGUAN MOTORIK
EKSTRAPIRAMIDALIS

3. GANGGUAN POSTURAL (SIKAP)

 FLEKSI BATANG TUBUH, TUNGAKI DAN


KEPALA.
 DISEBABKAN GANGGUAN KONTROL
PSOTURAL NORMAL
 DIAGNOSA BANDING :
 GANGGUAN PROPIOSEPTIF
 LABIRIN
VISUAL
GANGGUAN MOTORIK
EKSTRAPIRAMIDALIS

3. PERUBAHAN TONUS OTOT (RIGIDITAS)

 MENINGKAT KONTINU ATAU INTERMITEN


 INTERMITEN : COGWHEEL PHENOMENON
 DIAGNOSA BANDING : SPASTISITAS (CLASP
KNIFE PHENOMENON)
 PADA OTOT FLEKSOR DAN EKSTENSOR
 REFLEKS TENDON TAK MENINGGI
GANGGUAN MOTORIK
EKSTRAPIRAMIDALIS

4. KHOREA

 GERAKAN GESIT DAN CEPAT, BERUBAH ARAH


 PADA SELURUH PERSENDIAN
 GERAKAN OTOT WAJAH TIDAK JELAS ATAU
MINIMAL
 LESI : KORPUS STRIATUM
GANGGUAN MOTORIK
EKSTRAPIRAMIDALIS

5. ATHETOSIS

 MENYERUPAI GERAKAN MENARI


 GERAKAN LAMBAT, JANGKAUAN LEBIH
PANJANG
 GERAKAN PADA SELURUH OTOT
(EKSTRIMITAS, WAJAH, LIDAH)
BALLISM
 ABRUPT ONSET OF VIOLENT
FLINGING
MOVEMENTS.
 AFFECTING THE LIMBS, NECK, TRUNK
 OFTEN ON ONE SIDE OF THE BODY 
HEMIBALLISM
 A SINGLE LIMB  MONOBALLISM
 DUE TO A LESION INVOLVING THE
SUBTHALAMIC NUCLEUS
DYSTONIA
 THE MOVEMENT  ATHETHOSIS
MOVEMENT
 USUALLY CONTORTION MOVEMENT OF
THE TRUNK, LIMBS, HEAD AND NECK.
 THE SITE OF LESION: CORPUS
STRATUM AND GLOBUS PALLIDUS
PARKINSON
 CARDINAL SIGNS :

• RIGIDITY
• TGREMOR
• BRADYKINESIA
PARKINSONM (CONT.)
CLINICAL CATEGORIES :
1. PARALYSIS AGITANT (IDIOPATHIC
PRAKINSONM)
2. POSTENCEPHALYTIC PARKINSONISM
3. ARTERIOSCLEROSIS PARKINSONISM
4. DRUG-INDUCED PARKINSONISM
5. PARKINSONM DUE TO INFECTION
6. TOXIC PARKINSONISM
7. ANOXIC ENCEPHALOPATHY
PARKINSONISM
8. PARKINSONISM IN ALZHEIMER DISEASE
THE ETIOLOGY IS DECREASE OF
DOPAMIN ACTIVITY.

 RELEASE ACTIVITY FAILURE


 DECREASE OF OUTPUT
 RESEPTOR DOPAMINE BINDING FAILURE

INCREASE OF ACTIVITY OF
OTHER NEUROTRANSMITTER
IDIOPATHIC PARKINSONISM
 THE ETIOLOGY IS UNKOWN, MAY BE
VIRUS.
 MACROSCOPIC APPEARANCE:
• MELANINE LOSS IN NIGRAL
SUBSTANTIA
 MICROSCOPIC APPEARANCE:
• NEURONAL LOSS
• LEWY BODIES
CLINICAL MANIFESTATION
TREMOR :
• USUALLY UNILATERAL, UPPER
EXTRIMITY
• PIL-ROLLING TREMOR
• ADVANCE: BILATERAL, HEAD, NECK
(TITUBATION), FACE, TONGUE AND
MANDIBULAR
• INCREASE IN TENSION, AND NEGATIVE
IN SLEEPING
CLINICAL MANIFESTATION (CONT.)
RIGIDITY
• HYPERTONUS, AGONIST AND ANTAGONIST
• COGWHEEL PHENOMENON
• ALL EXTRIMITIES
• ADVANCE: EXTRIMITIES, NECK AND
TRUNCAL
I• INITIAL SYMPTOMS:
•LOST OF ASSOCIATIVE MOVEMENT.
CLINICAL MANIFESTATION (CONT.)
BRADYKINESIA.
• MASK-LIKE FACE
• SPEECH IS SLOWLY, SIALORRHEA.
• MICROGRAPHY
• MARS A PTIT PAS
• FLEXED POSTURE
• PROPULTION, LATEROPULTION,
RETROPULTION
CLINICAL MANIFESTATION (CONT.)
OTHERS:

• POSTURAL HYPOTENSION
• DEPRESSION
• DEMENTIA
MANAGEMENT

 L-DOPA / LEVODOPA
 TRIHEXYLPHENIDYL (ANTICHOLINERGIC)
 BROMOCRIPTINE (ADJUNCT THERAPY)
 AMANTADINE (INCREASE DOPAMINE
RELEASE)
HUNTINGTON’S DISEASE
(HUNTINGTON CHOREA)
• HEREDITARY DEGENERATIVE
• CHOREOATHETOSIS AND DEMENTIA
• CHROMOSOME-4 DISORDER
• CORTICAL ATROPHY, ESPECIALLY IN
FRONTAL LOBE
• VENTRICEL DILATATION
• NEURONAL LOSS, REACTIVITY OF GLIAL
CELL (ASTROCYTES)
• DEFCIENCY OF GABA, ACETHYLCHOLINE,
SUBSTANCE-P, DYNOPHINE.

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