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UNIT 13

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Unit 13 - Clinical Syndromes

Unit 13 Learning Objectives

A. Stroke
B. Cerebral Palsy
C. Parkinson’s Disease (Paralysis Agitans)
D. Cerebellar Disorders

A. Stroke

1. Definition

Refers to the cutting off, or severe decrease of blood supply to a part of the brain tissue (or its afferent and efferent fibers in the internal capsule as well as various brainstem structures) that leads to an impairment of function.

2.  Causes

Associated with age, arterial insufficiency, hypertension, cardiac disease, diabetes, and elevated blood cholesterol.

Fig. 1 –

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Click for Printable PDF Figure 1.

3.  Types of Strokes

a.   Thrombosis

An occlusion of an intracranial vessel by blood clot or other substance which occurs in the area of occlusion.   Approximately 50%-75% of cases of stroke are of this type.  

b. Hemorrhage

Bleeding within the substance of the brain due to a rupture of a vessel.   The most common type of hemorrhagic stroke is due to an aneuryism, which is a rupture of an abnormal sac within a vessel.   Approximately 10%-25% of cases of stroke are hemorrhagic (30% of these being fatal).

c.   Embolus

Obstruction of an intracranial vessel by a substance that has arisen in another location.    Approximately 1%-5% of cases of stroke are of this type.

4.  Signs and Symptoms

Clinical signs and symptoms are dependent on the area of the brain which has been affected.   They will depend on the artery that is involved, the point at which the artery is involved (main trunk or side branch), and the etiology of the stroke.

5.  Clinical Syndromes (according to artery involvement)

a.   Middle Cerebral Artery (Dominant)

1. Hemiplegia - arm worse than leg
2. Hemianesthesia - arm worse than leg, including a tactile and proprioceptive agnosia
3. Homonymous hemianopsia
4. Aphasia - motor and/or sensory depending on site of lesion
5. Impairment of analytical skills
6. Problems distinguishing right from left; finger agnosia
7. Chorea, Athetosis, and Dystonia
8. Hearing loss or agnosia
9. Occular control problems
10. Limbic and memory deficits
11.  Loss of taste

b.   Middle Cerebral Artery (Non-dominant)

1. Hemiplegia - arm worse than leg
2. Hemianesthesia - arm worse than leg, including a tactile and proprioceptive agnosia
3. Homonymous hemianops
4. Parietal Lobe Syndrome - this causes impairment of perceptual skills and  visual, temporal, and spatial skills which can result in ideomotor ot ideatonial apraxia
5. Loss of creative and artistic talents, including gesturing to express oneself
6. Loss of reflexive speech
7. Chorea, Athetosis, and Dystonia
8. Hearing Loss
9. Occular control problems
10. Limbic and memory deficits
11. Loss of taste

c.   Anterior Cerebral Artery

1. Hemiplegia - leg involved
2. Hemianesthesia - seen over involved leg, including a tactile and proprioceptive agnosia
3. Some degree of aphasia
4. Partial incontinence
5. Mental changes - Dominant hemisphere: loss of inhibition
Non-dominant hemisphere: Patient becomes lethargic and unmotivated.

 d.   Anterior Communicating Artery (most common site of aneurysm)

1. Paraplegic resolving to paraparesis
2. Impairment of intellect
3. Incontinence
4. Loss of inhibition
5. Loss of sensation in lower extremities (including a tactile and proprioceptive agnosia)

e.   Posterior Cerebral Artery

1. Fleeting hemiparesis (vertebral or basilar artery involvement)
2. Fleeting hemianesthesia (vertebral or basilar artery involvement)
3. Ataxia (vertebral or basilar artery involvement)
4. Intolerable pains and sensations - Thalamic Pain Syndrome
5. Visual deficits
6. Occular control deficits
7. Hemibalismus
8. Olfactory deficits
9. Memory and limbic deficits
10. Constructional apraxia
11. Olfactory deficits

Fig. 2 –

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Click for Printable PDF Figure 2.

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B. Cerebral Palsy

1.  Definition

Literally, cerebral palsy means “damage to C.N.S. which results in motor impairment”.   Clinically, it is referred to as any dysfunction (sensory, motor, perceptual, cognitive) that occurs secondary to brain injury either prenatally, perinatally, or within the first two postnatal years of life.   The lesion which occurs interferes with the normal motor development of the child.

2.  Causes

a.   Prenatal

1. Hereditary

2. Other

a. Virus or bacteria
b. Drug use or abuse
c. Age of the mother
d. Prematurity
e. Rh- incompatibility

b.   Perinatal

1. Trauma during delivery

       a. Anoxia
       b. Forceps

c.   Postnatal

1. Infectious disease
2. Trauma - abuse, accidental
3. Neonatal malnutrition

3.  Normal Motor Development vs. Abnormal Motor Development

In normal development, head and trunk control are established first.   This will allow the child to control their head and trunk against gravity and establish rotation within the body axis.   These skills become functionally established between the fourth and sixth months of life.   During this period, flexion is favored in the extremities and little functional use has been achieved.   It is not until flexor and extensor control are equal (which occurs between the fourth and sixth month) that the extremities can begin to be used for postural purposes and disassociation of movement can occur.   At this point, the child will begin to rapidly progress through the developmental sequence which will progress a child from a prone/supine posture to a bipedal posture, culminating in independent walking.   The levels of this sequence are:

1. Head Control
2. Trunk Control
3. Quadruped
4. Sitting
5. Standing

This sequence is completed somewhere between the twelfth and fifteenth month of life.   This process also includes the development of our postural control mechanism which allows us to maintain or regain our balance and equilibrium.   This is accomplished through what are referred to as compensatory responses.   At first these responses will support the establishment of our developmental skills, then they will monitor them.   Disassociation of movement, which is also occurring at this time, will occur from proximal to distal.   Following the functional establishment of these developmental skills, the child will then concentrate their efforts on developing functional movement patterns to deal with their everyday needs. Refinement of these functional skills continues up until the eighth year of life.

In Cerebral Palsy, the lesion that has occurred interferes with this orderly process of development limiting the child to nonfunctional patterns of movement in the extremities and lack of postural control.   Also, hypertonus will develop in the form of spasticity or rigidity, or as intermittant increases of postural tone in response to stimulation.
The extent and types of abnormalities will vary according to the severity and location of the lesion.

4.  Classification of Cerebral Palsy

a.   Topographical  (body parts involved)

1. Quadriplegia - all four extremities involved, with the upper extremities more involved than the lower extremities

2. Hemiplegia - one side involved

3.  Diplegia - all four extremities involved with lower extremities worse than the upper extremities

NOTE: May also see Paraplegia or Monoplegia which are usually mild cases of Diplegia and Hemiplegia accordingly.

b.   Based on Motor Symptomatology

1. Spastic Paralysis

Caused by damage to Areas 4-6.   The child will have a lack of head and trunk control, as well as an increase of tone in antigravity musculature to the extent that they may be locked into a fixed posture (flexion of the upper extremities and extension of the lower extremities) referred to as stiffness of posture.   The level of these symptoms will be proportional to the amount of brain damage.

2. Choreo-athetotic Condition

Caused by damage to the corpus striatum. A child may have one or both of these conditions depending on the type of damage to the corpus striatum.  Athetotic children will demonstrate constant slow writhing, purposeless movements of the extremities, neck, and trunk.  The child’s face and tongue may be involved resulting in problems with swallowing and speech.  Children with choreoform movements will exhibit rapid irregular purposeless movements of the proximal musculature and fingers.  In both cases, postural control or stability will be impaired.  Both types, like spastics, will have gradations in severity depending on the amount of brain damage.  Many of the children with damage to the corpus striatum will demonstrate a combination of both disorders.

3. Flaccid Paralysis

This type of hypotonia is an indication of massive brain damage.   It results in NO tone at all; completely floppy limbs.   Many orthopedic deformities are related to this type of CP.

4. Hypotonia - Low Tone

This type of hypotonia is seen in slow motor developers.   These children have low tone and soft muscles to touch.   No abnormal movement patterns are seen, but it will take the child longer to progress through the developmental levels of motor control.

5. Rigidity

De-Cortical Rigidity
Results from damage of the cortex, usually Areas 4-6.   Symptoms are seen as a spastic paralysis, with the upper extremities in flexion and the lower extremities in extension.

De-Cerebrate Rigidity
Results from massive brain damage to all parts of the C.N.S. from the mid-brain through the cerebral cortex.   The child will exhibit a tonic contraction of the extensor musculature of the body.

6. Ataxic

Caused by damage to the cerebellum.   Results in ataxia and other coordination problems.

7.  Dystonia

Caused by damage to the corpus striatum.  Children with this disorder will demonstrate initially slow, writhing purposeless movements as seen in athetotic children.  However, as time goes on these movements become sustained, locking the child into abnormal postures for sustained periods causing multiple skeletal deformities.

8.  Hemiballismus

Caused by damage to the subthalamus.  Children with this disorder will exhibit sudden wild flinging of the arms and legs alternating between total flexion and extension.  Usually seen on one side of the body but can be both.

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C. Parkinson’s Disease (Paralysis Agitans)

Parkinson’s Disease is a disease of the substantia nigra which prevents the necessary production of the neurotransmitter dopamine.   Dopamine acts to control the activity of the corpus striatum and other cortical structures.   If the corpus striatum is deprived of this neurotransmitter it results in a progressive set of symptoms and eventually the deterioration of our motor control mechanism, as well as cognitive and limbic function.   To understand the various types of symptoms exhibited by a patient with Parkinsonism it is necessary to understand the function of the corpus striatum.   The function of the corpus striatum is to plan movement strategies (their force, rate, direction, and range), to initiate the movement, as well as to stop or change the movement.   In addition, the corpus striatum helps regulate the activity of the alpha motor neurons through its inhibitory effect on Area 4, as well as exerting a facilitory effect on the gamma motor neuron via the cerebellum.   Thus the symptoms the patient will exhibit will relate in some way to these functions.   These symptoms are first seen unilaterally in the upper extremities then become bilateral progressing to the lower extremities.

1.  Causes

a. Epidemic Encephalitis
b. Cerebral Arteriosclerosis
c. Carbon Monoxide Poisoning
d. Manganese Poisoning
e. Neurosyphilis
f. CVA

2.  Clinical Findings

a. Tremors

Rhythmic or alternating movements from contraction of opposing muscle groups.   Three to six per second with rest.   Seen greatest in the fingers, hands, and wrists (example - pill rolling), and disappears with sleep or voluntary movement.

b.  Bradykinesia

c.  Diminution of associated movements

d.  Immobile mask-like facial expressions

e.   Stooped Posture

Tendency to develop thoracic kyphosis and an anterior pelvic tilt in the late manifestation of the disease.   This is characterized by a forward leaning posture.

f.   Propulsive or Festinating Gait

Tendency to break into a trot when standing or walking.   Shuffling steps will be seen, beginning slowly and becoming more rapid.   Rate of progression (step length) will decrease as patient continues to walk.

g.   Rigidity

Persistent simultaneous activity occurring in agonist and antagonist muscles, despite attempted relaxation (example: lead pipe or cogwheel movements).

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D. Cerebellar Disorders

Damage to the cerebellum will result in ataxia, which is incoordination of muscular movement resulting in intermittant and jerky movements of body musculature.   This occurs due to the irregular contraction of muscles regarding force, rate, direction, and range.
Damage to the archicerebellum will result in an unsteady, wide based gait where the patient sways side to side with poor balance which is referred to as cerebellar ataxia.   A nystagmus may be present, which is the inability to control occular musculature, and hypotonia in the body musculature may also be seen. Damage to the neocerebellum and paleocerebellum results in a variety of disorders referred to as asynergy.

1.  Dysmetria - Past Pointing

Dysmetria is the inability to gauge or predict how far movements will go.   Attempts at movements will either overshoot or undershoot the target.

2.  Dysdiadochokinesia

The loss of the ability to complete rapid alternate movements is called dysdiadochokinesia.

3.  Intention Tremors

Cerebellar tremors, or intention tremors, are jerk-like motions which most frequently occur at the end of a particular movement.

4.  Decomposition of Movement

Decomposition of Movement is the separation of voluntary movements (that normally flow smoothly) into mechanical puppet-like movements which occur one joint at a time.

5.  Dysarthria

Dysarthria is a term that refers to slurred speech.   It is caused by damage to the cerebellum which results in a decreased control over the muscles of the face and throat that are used for speech.

6.  Hypotonia

This refers to a low tone in the muscles which leads to quick tiring and decreased endurance.   

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