PENYAKIT DEGENERATIF DAN MOVEMENT DISORDER

FITRIYANI

Department of NeurologyMedical 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. Parkinson’s disease = paralysis agitans= shaking palsy

b. Parkinson syndrome = parkinsonism

Parkinson’s 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 Parkinson’s 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

b. Rigidity

Cogwheel phenomenon

c. Tremor

Passive tremor : 4 – 8 movements / sec

Pill – rolling = money counting movement

Resting tremor

3 cardinal sign of Parkinson’s Disease

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

Choreashort, fast, jerky movementsdistal proximal portion of the extremities

Chorea minor = Sydenham’s chorea = St Vitus dance

- in children- acute, selflimiting- linked to rheumatic fever ( = chorea

infectiosa)

Chorea gravidarum

- acute chorea

- early pregnancy

Huntington’s chorea = chorea major

dominant, hereditary, degenerative disease

middle age

cortical neuron degenerate dementia

Hemiballism = ballistic syndrome

lesion of the subthalamic nucleus ( Luysi ) and its connection

the muscle of shoulder & pelvis – out reaching &

hurling movements

Spasmodic torticollis, torsion dystonia biochemical abnormallity of the putamen, thalamus

(centro median nucleus & in other extrapyramidal nuclei)

Athetosis

Continuous, arrhytmic, slow, wormlike movementsDistal portion of the extremitiesPosture & movement are bizarreCaused 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

•