PENYAKIT DEGENERATIF DAN

MOVEMENT DISORDER
FITRIYANI

Department of Neurology
Medical Faculty Padjadjaran University
Bandung

Lesion of Extrapyramidal System

The main sign :
Disorders of muscle tone ( dystonia )
Involuntary movement disorders ( hyper/hypo/a-kinesia )
absent during sleep

Syndromes of extrapyramidal lesions:

1. Hypokinesia hypertonia
2. Hyperkinesia - hypotonia

1. Hypokinesia hypertonia syndrome

a. Parkinsons disease = paralysis agitans= shaking palsy
b. Parkinson syndrome = parkinsonism
Parkinsons Disease
Degeneratif process hereditary
Loss of melanin containing neurons of substantia
nigra & of dopaminergic neurons that connect with the
striatum
Bilateral
Unilateral

3 cardinal sign of Parkinsons Disease

a. Brady / akinesia
Starting a movement becomes very difficult,
the mobility decreases.
The entire body anteflexed position,
no swing during walking
Hypo / amimia a mask face
Speech monotonous, dysarthic
Postural reflex decreases pro / latero / retropultion

3 cardinal sign of Parkinsons Disease

b. Rigidity
Cogwheel phenomenon
c. Tremor
Passive tremor : 4 8 movements / sec
Pill rolling = money counting movement
Resting tremor

Parkinson syndrome = parkinsonism

Neuronal loss in the substantia nigra caused by :
Postencephalitic parkinsonism
Cerebral arteriosclerosis
Trauma
Tumors
Intoxication by Co, Mg, etc.
phenotiazines, reserpin

2. Hyperkinetic hypotonic syndrome

Lesion in neostriatum
Occasionally glob. pallidus, thalamus,cerebral cortex

dan

Chorea
penyakit yg ditandai dg kontraksi otot2 badan
wajah secara tdak dikehendaki, tdak teratur,
tersentak2 dg cepat
short, fast, jerky movements
distal proximal portion of the extremities
Chorea minor

= Sydenhams chorea
= St Vitus dance
- in children
- acute, selflimiting
- linked to rheumatic fever ( = chorea infectiosa)

Chorea gravidarum
- acute chorea
- early pregnancy
Huntingtons chorea = chorea major
dominant, hereditary, degenerative disease
middle age

Hemiballism = ballistic syndrome

Gerakan involunter yg bersifat kasar dr bagian proksimal dan
distal anggota tubuh,grkn brsfat sbntar dan berulang2,krna
cepatny tbuh seakan2 berputar2 dan terlempar.
lesion of the subthalamic nucleus ( Luysi ) and its
the muscle of shoulder & pelvis out reaching &

connection

hurling movements
Spasmodic torticollis, torsion dystonia
biochemical abnormallity of the putamen, thalamus
(centro median nucleus & in other extrapyramidal nuclei)

Athetosis
Keadaan yg ditandai dg gerakan2 anggota
badan at bagian tbuh lain scra meliuk2,
lambat dan tdk disadari
Continuous, arrhytmic, slow, wormlike
movements
Distal portion of the extremities
Posture & movement are bizarre
Caused by perinatal damage to the striatum

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

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