BASAL GANGLIA AND DEGENERATIVE DISEASE

DJADJANG SUHANA

Department of NeurologyMedical Faculty Padjadjaran UniversityBandung

MOTOR SYSTEM.PIRAMYDAL SYSTEMEXTRAPYRAMIDAL SYSTEM

EXTRAPYRAMIDAL SYSTEM : NUCLEI CIRCUITS DESCENDENCE PATHWAYS

ANATOMI1. 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

SIRKUITSIRKUIT 1.KORTEK SEREBRI STRIATUM GLOBUS PALIDUS TALAMUS KORTEK SEREBRI

SIRKUIT 2.A. KORTEK SEREBRI STRIATUM SUBSTANSIA NIGRAB.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 4KORTEK 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. PIRAMIDALISTR. KORTIKORUBROSPINALISTR. KORTIKORETIKULARISTR.KORTIKORETIKULOSPINALIS

FUNGSI SISTEM EKSTRAPIRAMIDALISMENGATUR :GERAKAN ASOSIATIFPOSTURALINTEGRASI OTONOMI

LESI EKSTRAPIRAMIDALISMENYEBABKAN: GERAKAN INVOLUNTER GANGGUAN TONISITAS GANGGUAN POSTURAL

PATOFISIOLOGI# HUBUNGAN SINAPTIK SIRKUIT : FASILITASI ATAU INHIBISI# GANGGUAN FUNGSI INHIBISI FUNGSI FASILITASI : RELEASE PHENOMENONCONTOH: TREMOR PARKINSON

GANGGUAN MOTORIK EKSTRAPIRAMIDALIS1. AKINESIA (BRADIKINESIA, HIPOKINESIA) GANGGUAN KECEPATAN DAN SPONTANITAS GERAKANTAK ADA KELUMPUHANCONTOH :GERAKAN HABITUAL TERGANGGUMUKA TOPENGMARS A PTIT PASMIKROGRAFIGERAKAN REAKTIF MELAMBAN (PROPULSI, RETROPULSI DAN LATEROPULSI)

GANGGUAN MOTORIK EKSTRAPIRAMIDALIS2. TREMOR

GERAKAN OSILASI RITMIK DAN REGULERRESTING TREMORPARKINSON ROLLING PILL

GANGGUAN MOTORIK EKSTRAPIRAMIDALIS3. GANGGUAN POSTURAL (SIKAP)

FLEKSI BATANG TUBUH, TUNGAKI DAN KEPALA.DISEBABKAN GANGGUAN KONTROL PSOTURAL NORMALDIAGNOSA BANDING : GANGGUAN PROPIOSEPTIF LABIRIN VISUAL

GANGGUAN MOTORIK EKSTRAPIRAMIDALIS3. PERUBAHAN TONUS OTOT (RIGIDITAS)

MENINGKAT KONTINU ATAU INTERMITENINTERMITEN : COGWHEEL PHENOMENONDIAGNOSA BANDING : SPASTISITAS (CLASP KNIFE PHENOMENON)PADA OTOT FLEKSOR DAN EKSTENSORREFLEKS TENDON TAK MENINGGI

GANGGUAN MOTORIK EKSTRAPIRAMIDALIS4. KHOREA

GERAKAN GESIT DAN CEPAT, BERUBAH ARAHPADA SELURUH PERSENDIANGERAKAN OTOT WAJAH TIDAK JELAS ATAU MINIMALLESI : KORPUS STRIATUM

GANGGUAN MOTORIK EKSTRAPIRAMIDALIS5. ATHETOSIS

MENYERUPAI GERAKAN MENARIGERAKAN LAMBAT, JANGKAUAN LEBIH PANJANGGERAKAN 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

DEGENERATIVE DISEASEINTRODUCTION.

PROGRESSIVE SELECTIVE GENETIC AND FAMILIAL PATHOMECHANISM IS UNKNOWN

PATOLOGY.

NEURONAL LOSS, WITH GLIOSIS LONG TRACTS INVOLVEMENT

CHARACTERISTIC. INCIDIOUS OF ONSET PRECIPITATED BY STRESS FAMILIAL PROGRESSIVE SYMETRIC BILATERAL LESION SELECTIVE NEURONAL INVOLVEMENT DYSINTEGRATION OF CELL BODIES, AXONAL, DENDRITICAL WITHOUT CELLULAR AND TISSUE RESPONS.

DEMENTIA. PROGRESSIVE DISORDER OF INTELECTUAL CAPACITY CAUSED BY THE DISEASE OF THE BRAIN. 80% DUE TO: ALZHEIMER MULTIPLE INFARCTION OTHERS DUE TO: HUTINGTON DISEASE PICK DISEASE NPH

ALZHEIMER DISEASE ETIOLOGY IS UNKOWN NEURONAL LOSS PATHOPHYSIOLOGY: DECREASE OF CHOLINE ACETHYLTRANSAMINASE

ALZHEIMER DISEASE MACROSCOPIC APPEARANCE: DIFFUSE BRAIN ATROPHY WIDTH OF SULCI SHALLOWNESS OF GYRI ENLARGE OF VENTRICLES MICROSCOPIC APPEARANCE: NEURONAL LOSS IN GRAY AND WHITE AREAS NEUROFIBRILLARY TANGLES SENILE PLAQUES.

CLINICAL MANIFESTATION. BOTH OF SEX OFTEN > 65 YEAR CARDINAL SIGNS: DISORDER OF MEMORY (RECENT MEMORY) DISORDER OF CALCULATION AND ABSTRACTION DISORDER OF JUDGMENT

CLINICAL MANIFESTATION (CONT.)OTHERS :

LOSS OF INSIGHT APATHY, AGITATION, AGGRESSION IRRITABILITY, EUPHORIA, DEPRESSION COMBINATION.

FOCAL SIGNS : DYSPHASIA DYSCALCULI DYSLEXIA DYSGRAPHIA DYSPRAXIA GAIT APRAXIA (PARKINSONIM)

PHYSICAL EXAMINATION MENTAL EXAMINATION : MMSE (MINI-MENTAL SCORE EXAMINATION) MOTORIC EXAMINATION : NORMAL DEGENERATIVE REFLEX : SNOUT PALMOMENTAL GRASP INCREASE OF PHYSIOLOGIC REFLEXES PATHOLOGIC REFLEXES ARE POSITIVE

SPARING CRANIAL AND PERIPHERAL NERVES CEREBELLUM LOCOMOTION

SENSIBILITY NORMAL

DIAGNOSTIC PROCEDURES. EEG CT-SCAN

AMYOTROPHIC LATERAL SCLEROSIS(MOTOR NEURON DISEASE) CHRONIC DISEASE PROGRESSIVE DEGENERATION OF MOTOR NEURONS OF: THE ANTERIOR HORN OF THE SPINAL CORD MOTOR NUCLEI IN BRAIN STEM MOTOR NEURONS IN CEREBRAL CORTEX THE ETIOLOGY IS UNKNOWN, MAY BE CAUSE BY METAL INTOXICATION OR VIRAL INFECTION

TYPES OF ALS.1. PROGRESSIVE MUSCULAR ATROPHY

NEURONAL LOSS IN THE ANTERIOR HORN OF SPINAL CORD FIRSTLY IN CERVICAL REGION CORTICOSPINAL AND SENSORY TRACT ARE INTACK. CLINICAL FINDING: PARESIS FASCICULATION MOVEMENTS.

TYPES OF ALS (CONT.)

2. PROGRESSIVE BULBAR PALSY. NEURONAL LOSS OF BRAINSTEM NUCLEI CLINICAL MANIFESTATION : # DYSARTHRIA # DYSPHAGIA # FASCICULATION OF TONGUE MUSCLES # EXTERNAL EYE MOVEMENT IS NORMAL

TYPES OF ALS

3. PRIMARY LATERAL SCLEROSIS NEURONAL LOSS OF CEREBRAL CORTEX AND ASSOSCIATIVE CORTEX INVOLVE OF CORTICOSPINALIS TRACT CLINICAL FINDING: PARESIS TENDON REFLEXES ARE ABNORMAL ATROPHY AND FASCICULATION ARE NEGATIVE

4. COMBINATION PARESIS OF TRUNK AND FACIAL MUSCLES ATROPHY OF MUSCLES FASCICULATION ABNORMALITY OF REFLEXES SENSIBILITY IS NORMAL

DIAGNOSIS EMG BIOPSY MUCLE ENZYME LUMBAL PUNCTION

PARKINSON CARDINAL SIGNS :

RIGIDITY TGREMOR BRADYKINESIA

PARKINSONM (CONT.)CLINICAL CATEGORIES :1. PARALYSIS AGITANT (IDIOPATHIC PRAKINSONM)2. POSTENCEPHALYTIC PARKINSONISM3. ARTERIOSCLEROSIS PARKINSONISM4. DRUG-INDUCED PARKINSONISM5. PARKINSONM DUE TO INFECTION6. TOXIC PARKINSONISM7. ANOXIC ENCEPHALOPATHY PARKINSONISM8. PARKINSONISM IN ALZHEIMER DISEASE

THE ETIOLOGY IS DECREASE OF DOPAMIN ACTIVITY. RELEASE ACTIVITY FAILURE DECREASE OF OUTPUT RESEPTOR DOPAMINE BINDING FAILUREINCREASE 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 MANIFESTATIONTREMOR : 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 TRUNCALI 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)

HUNTINGTONS 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.