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BASAL GANGLIA AND DEGENERARIVE DISEASE

Djadjang Suhana

Department of Neurology
Medical Faculty Padjadjaran University
Bandung
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 DISEASE
INTRODUCTION.
• 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
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 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
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.