Gangguan Motorik- appetizer ^^v

Ersifa fatimahPPDS Neurologi

RSUD Dr Soetomo – Univ. Airlangga 2013

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Referensi

Disorder of Motility• Motor paralysis• Abnormal of movement &

posture due to disease of Basal Ganglia

• Incoordination & other disorders of Cerebellar function

• Tremor, Myoclonus, Focal dystonias, and Tics

• Disorder of Stance & Gait(Adams & Victor’s, 2005)

Motor Disturbances• Muscle• LMN• UMN• Basal Ganglia• Cerebellum(Waxman, 2010)

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The Motor System (DeJong, 2010)

Muscle Tone

Muscle Volume & Contour

Level: Motor unit, Spinal cord, Pyramidal, Extrapyramidal

Motor strength & Power

Abnormality of Movement

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Knowing the structure and function of the different levels of motor control,

the relationships between the motor systems,

and the changes in motor activity that occur in disease

helps in understanding disorders of the motor system.

DeJong, 2010

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Sensory and motor functions are interdependent in the performance of volitional movement,

and it is not possible to consider the motor system apart

from the sensory system. Impairment of sensation may affect

all aspects of motion—volitional, reflex, postural, tonic, and phasic.

DeJong, 2010

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The axons arising from the large pyramidal cells in layer V (Betz's cells) of area 4 contribute only about 5% of the fibers of the corticospinal tract and its pyramidal portion.

Waxman, 2010

55%35%

10%

Corticospinal & Corticobulbar Tracts, Composition

area 4 & 6area 3, 1, 2Others: frontal & parietal

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Disorder of Motility

Large motor neurons in the anterior horns of the spinal cord & the motor nuclei of the brainstem the axons comprise the anterior spinal roots , the spinal nerves & cranial nerves innervate the skeletal muscles.The LMNs are the final common path by which all neural impulses are transmitted to muscle.

Paralysis due to an interruption of LMNs.Complete lesions of LMN result in a loss of all movement—voluntary, automatic, postural, and reflex.

Motor neurons in the frontal cortex adjacent to the rolandic fissure connect with the spinal motor neurons by a system of fibers known as the pyramidal tract.Since the motor fibers that extend from the cerebral cortex to the spinal cord are not confined to the pyramidal tract , they are more accurately designated as the corticospinal tract, or UMNs,to distinguish them from the LMNs.

Paralysis due to dysfunction of upper motor (corticospinal) neurons.

The following parts of the nervous system are engaged primarily in the control of movement and, in the course of disease, yield a number of characteristic derangements(Adams & Victor, 2005)

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Several brain stem nuclei that project to the spinal cord, notably the pontine and medullary reticular nuclei, vestibular nuclei, and red nuclei. + their descending fibers posture & movement (particularly when

movement is highly automatic and repetitive)Certain of these brain stem nuclei are influenced by the motor or premotor regions of the cortex, e.g., via corticoreticulospinal relays.Two subcortical systems: the basal ganglia (striatum, pallidum, and related structures, including the substantia nigra and subthalamic nucleus) & the cerebellum Play important role in the control of muscle tone, posture, and coordination of movement by virtue of its connections with the corticospinal system (via thalamocortical fibers) and other descending cortical pathways

Involuntary movements & abnormalities of posture due to disease of the basal gangliaAbnormalities of coordination (ataxia) due to lesions in the cerebellum.

Disorder of Motility (Adams & Victor, 2005)

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Several other parts of the cerebral cortex, particularly the premotor & supplementary motor cortices. These structures are involved in the programming (i.e., the sequencing and modulation) of voluntary movement.

Apraxic or nonparalytic disturbances of purposive movement due to involvement of association pathways in the cerebrum

The prefrontal cortex planning & initiation of willed movement. Fibers from the prefrontal cortex project to the supplementary & premotor cortex and provide the input to the some restrictly motor areas. Similarly, certain parietal cortical areas (superior parietal lobule) supply the somatic sensory information that activates the premotor and supplementary motor cortices and leads to directed movement. In addition, other parts of the nervous system concerned with tactile, visual, and auditory sensation are connected by fiber tracts with the motor cortex. These association pathways provide their own sensory regulation of motor function

Disorder of Motility (Adams & Victor, 2005)

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• Motor Unit (LMN)• Segmental/spinal cord• Suprasegmental

– Various descending systems modulate activity in segmental level

– Brainstem, cerebellar, extrapyramidal, pyramidal

– Psychomotor/ cortical associative

Levels:

Fig: The most important descending pathways that act upon the anterior horn cell of the spinal cord (deJong 2010)

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• Lat ReticuloSpinal: – influence the musculature of the

trunk.• Med TectoSpinal:

– reflex movement– Trunk, neck, eyes ~ visual stimuli

• Lat CorticoSpinal:– Hand, finger movement

• RubroSpinal:– Hand, finger movement– Flexor muscle tone– Postural mechanism,

standing/righting reflex• Lat VestibuloSpinal:

– Postural mechanism, standing/righting reflex

(DeJong, 2010)

Mainly control trunk & proximal extremity

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DeJong, 2010

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Waxman, 2010

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Motor ParalysisTerminology

Paralysis Loss of involuntary movement due to interruption of motor pathway (cerebrum – muscle fiber)

Paresis A lesser degree of paralysis / partial loss

Plegia Severe / complete loss of motor function

Focal ~ asymmetry Ex: hemi-

Generalized ~ symmetry (although the weakness not truly general, ex.: scapuloperoneal syndrome)Truly generalized bulbar is impaired

Multifocal Ex.: bilateral CTS

Non-focal Generalized, predominantly proximal, predominanly distal

These various patterns have differential diagnostic and localizing significance. Identification of the process causing weakness is further aided by accompanying signs, such as reflex alterations and sensory loss. (DeJong, 2010)

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Pattern of Paralysis & Their Diagnosis

Adams & Victor, 2005

Apraxia: a state in which a clear-minded patient with no weakness, ataxia, or other extrapyramidal derangement, and no defect of the primary modes of sensation, loses the ability to execute highly complex and previously learned skills and gestures.

NMJ

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Bradley, 2010

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Bradley, 2010

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Mumenthaler, 2004

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Bradley, 2010

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Bradley, 2010

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Bradley, 2010

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Mumenthaler, 2004

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Bradley, 2010

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Bradley, 2010

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Bradley, 2010Proximal, Distal & General Weakness

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Adams & Victor’s, 2005

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Features of UMN vs LMN Weakness(DeJong, 2005)

+ UMN LMN

Weakness distribution

Corticospinal distribution, hemi-, para-, quadri-, mono-, faciobrachial

Generalized, focal, predominantly distal, predominantly proximal, No preferential involvement of corticopsinal innervated muscle

Sensory loss distribution

Central pattern None, stocking glove, peropheral / root distribution

Sphincter function Sometimes impaired Normal (except for cauda equina lesion)

Pain No Sometimes

Other CNS sign Possibly No

Corticospinal distribution:• Distal muscle• Upper ext: esp hand; wrist, finger & elbow extnsor, supinator, external rotator,

abductor shoulder• Lower ext: foot & toe dorsiflexor, knee flexor, hip flexor & internal rotator,

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Muscle Tone (DeJong, 2010)

Hypotonia Hypertonia

Myopathy Cerebellar disease

Spasticity Rigidity Dystonia:transient or sustained hypertonic conditions that do not fit into the other categories

most marked near the middle of the range of motion, more apparent with fast than with slow passive movement

the increased tone occurs to more or less the same degree throughout the range of passive motion of a limb, and is independent of the speed of the movement

Lead pipe Cogwheel Paratonia:increase in tone in a limb more or less proportional to the examiner's attempt to move it.(Gegenhalten)

smooth resistance throughout the range independent of the rate of move.

ratchety, jerky, tremulous variation in the hypertonia, due primarily to superimposed tremor

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Muscle Volume & Contour (DeJong, 2010)

Atrophy Hypertrophy

Neurogenic Myogenic Disuse True Pseudo

Weakness // wastingMay be severe

Anterior horn cell peripheral nerve

UMN:Usually not followed by atrophy

Weakness >> wastingMild-moderate

Immobilization:Wasting >> weaknessMild-moderateRecovers quickly with resumption of uses

Physiologic apparent muscle enlargement due to replacement of diseased muscle by fat and fibrous tissueEx: Duchenne

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Abnormal of movement & posture due to disease of Basal Ganglia

• Akinesia: absence of movement

• Bradykinesia: abnormally slow movement

• Hypokinesia: a decreased amount or amplitude of movement.

• Dyskinesia: involuntary, abnormal movement

• Tremor• Athetosis: slow,

writhing movements of the extremities & neck musculature

• Chorea: quick, repeated, involuntary movements of distal extremity muscle, face, tongue, ~ corpus striatum

Waxman, 2010

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Extrapyramidal SystemA: Basal ganglia: major structures. MD, medial dorsal; VA, ventral anterior; VL, ventral lateral nuclei of thalamus

Extrapyramidal motor system:

– Corpus striatum– Subthalamic nucleus– Substansia nigra– Red nucleus– Brain stem reticular

formation– Descending spinal cord

tract other than corticospinal tract

(Waxman, 2010)

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Waxman, 2010

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Adams & Victor, 2005Waxman, 2010

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Adams & Victor, 2005

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Adams & Victor’s, 2005

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Incoordination & other disorders of Cerebellar function

Truncal ataxia, drunken gait

Loss of equilibrium, nystagmus

Ataxia, asynergy, dysmetria,

dysdiadocho-kinesia, intention tremor, rebound

phen.

Adams & Victor’s, 2005 | Waxman, 2010

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There is a somatotopic organization of body parts within the cerebellar cortex. In addition, the cerebellum receives collateral input from the sensory and special sensory systems.

Waxman, 2010

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Waxman, 2010

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Via this pathway, activity in the dentate nucleus & other deep cerebellar nuclei modulates activity in the contralateral motor cortex. This crossed connection, to the contralateral motor cortex, helps to explain why each cerebellar hemisphere regulates coordination and muscle tone on the ipsilateral side of the body.

Waxman, 2010

Efferents from Cerebellum: the dentatorubrothalamocortical pathway

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Waxman, 2010

TerminologyDysmetria Past-pointing phenomenon, unable to estimate

the distance involved in muscular acts their attempt to touch the object will overshoot the target

Dysdiadochokinesia Inability to perform rapidly alternating movementsRebound phenomenon Loss of interaction between agonist & antagonist

smooth muscle

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Signs of Various Lesions of Human Motor system (Waxman, 2010)

* Fasciculations are spontaneous, grossly visible contractions (twitches) of entire motor units

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Abnormality of movement (DeJong, 2005)

Hypokinetic• Parkinson's disease• Parkinsonian syndromes

– Progressive supranuclear palsy– Multisystem atrophy– Olivopontocerebellar degeneration (sporadic

form)– Striatonigral degeneration– Shy-Drager syndrome– Diffuse Lewy body disease– Corticobasal degeneration– Drug-induced parkinsonism– Dopa responsive dystonia

• Other non-Parkinson's akinetic-rigid syndromes

• Huntington's disease (rigid or juvenile form)

• Wilson's disease• Essential tremor• Depression• Arthritis, polymyalgia, fibromyalgia

Hyperkinetic• Regular/predictable

– Tremor– Hemiballism– Palatal myoclonus

• Intermediate– Dystonia– Myokymia– Athethosis– Tic– Stereotypy– Myorythmia

• Fleeting/unpredictable– Fasciculations– Myoclonus– Chorea– Dyskinesias

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Bradley, 2010

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Bradley, 2010

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Adams & Victor’s, 2005

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Bradle

y, 201

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Disorder of Stance & Gait

Adams & Victor’s, 2005

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Disorder of Stance & Gait

Adams & Victor’s, 2005

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Bradley, 2010

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Apraxia

Bradley, 2010

http://thebrain.mcgill.ca

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Bradley, 2010

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Bradley, 2010

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Finally, it should be remarked once again that the complexity of motor activity is

almost beyond imagination.Adam & Victor, 2005