Movement DisordersStrange and abnormal movements are the

hallmarks of a number of neurological diseases, which are collectively termed

movement disorders. The movements are clearly visible and allow the observer to

suggest proper diagnosis from the movements. All these movements (and lack of movements) are involuntary .

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DYSKINESIA - disturbance of movement

1) Hypokinesia a. bradykinesia b. rigidity 2) Hyperkinesla a. tremor b. chorea c. athetosis d. hemiballism e. dystonia

PARKINSONISM:

Tremor - rhythmic and regular to 4 to 6 Hertzpresent at rest and stops when limb Is put into action

usually most obvious In fingers, hands, wrist and mouth disappears in sleep.

Rigidity - constant resistance to muscle stretching cogwheel rigidity Bradykinesia - poverty of movements little apparent arm swing gait consists of small, shuffling steps mask-like fades few eye blinks

ESSENTIAL TREMOR

:Benign monosymptomatic disorderTremor most evident In upper extremities, head and neck.Definitely exacerbated when the hands are used in maneuvers that require precision.Neither life-threatening nor life-shorteningFamilial

CEREBELLAR TREMOR:

Intention or action tremor ONLY occurs during voluntary action and the

amplitude of the tremor increases as the limb is extended or as the limb approaches the target.

Course tremor usually consisting of side-to-side movement of arms and legs.

Handwriting very much Impaired

WING-BEATING TREMOR:

Marked flapping tremor One or both arms jerk violently resembling a

bird beating its wings. Present at rest and may be heightened by

extension of the arms.

CHOREA:

Rapid, jerky, uncoordinated and purposeless movements which Involve the extremities, tongue,and trunk and face.

Disappear during sleep Patient may appear to be restless or fidgety. Often exacerbated by nervousness or

emotional upset.

ATHETOSIS:

Slow, wormlike muscular contractions, somewhat grotesque, writhing movements, not only

extremities but also of the trunk, neck and even the face.

Most frequently a result of brain damage or anoxia at or before birth.

May not develop for several years. Choreo-athetosis - combination of chorea and

athetosis.

DYSTONIA:

Slow, Involuntary movements which result in a wide variety of strange contortions of the body that may last seconds to hours or may be sustained.

Early manifestations Include a peculiar way of holding the head or extremity.

Gradually involves trunk muscles and produces contorted posture

SPASMODIC TORTICOLLIS

Partial dystonia Neck involuntarily deviates to one

side or backwards Patient can usually bring the neck

back to midline with concentration, but there will be an involuntary slow return of the abnormal neck posture.

HEMIBALLISMUS

Violent, flaying movement (wild, forceful swinging) of one arm or leg

Ballismus - bilateral movements

TARDIVE DYSKINESIA:

Associated with chronic use of neuroleptic drugs Onset usually after 1 -2 years of treatment with the neuroleptic

drug. Withdrawal of the drug does not always mean the tardive

dyskinesla will stop. If withdrawal Is done early, there is a higher chance that the

tardive dyskinesia will stop. in large percentage of patients, It becomes an irreversible problem.

Consists clinically of lingual-faclal-buccal chewing type movements as well as lipemaking and sucking movements.

The patient’s fingers, hands, arms and feet may show choreo-athetoid type and ballistic type movements. The patient may appear agitated.

Can produce a technically poor EEG. Be sure to note if the patient has this problem.

HUNTINGTON'S CHOREA (CHRONIC PROGRESSIVE CHOREA)

Inherited in ancruto:smnal C!9minant patter!1.

Onset of symptoms may be at any time but occurs usually between ages 30 and 50 years.

Characterized by chorea (abnormal spontaneous move.ments that are short, irregular, rapid, nonrhythmic and uncoordinated), intellectual decline and emotional disturbances.

HUNTINGTON'S CHOREA (CHRONIC PROGRESSIVE CHOREA)

Mental symptoms are diffuse and include changes in personality, impulsive behavior and a progressive impairment of memory and intellectual powers.

Self-care skills are eventually lost.

HUNTINGTON'S CHOREA (CHRONIC PROGRESSIVE CHOREA)

The major pathologic changes are found in the basal ganglia.

The caudate nucleus and putamen show severe atrophy.

Huntington's chorea is ultimately fatal within 15 – 20 years with suicide a frequent cause of death.

EEG CHANGES WITH HUNTINGTON'S CHOREA

Low to extremely low voltage alpha activity and a lack of rhythmicity. \

The EEG has shown no predictive value in determining who will develop Huntington's Chorea in an affected family.

PARKINSON'S DISEASE

Common degenerative disease affecting people in their 50's to 60's and beyond.

It is estimated that l out of 40 people will develop Parkinson's.

Parkinson's is due to the degeneration of the substantia nigra and the lack of the neurotransmitter dopamine.

PARKINSON'S DISEASE

Characterized clinically by resting tremor, rigidity, mask-like facies, bradykinesia (slowness and poverty of movements) and postural instability.

Intellectual faculties are usually unimpaired. Many patients become depressed due to the slowness of their body.

EEG CHANGES WITH PARKINSON'S DISEASE

Usually normal.

If the patient does have an alteration in mental status, the EEG may show excessive slow activity either posteriorly or diffusely.

WILSON'S DISEASE

Signs and symptoms of Wilson's disease are characterized by degeneration of the basal ganglia accompanied by cirrhosis of the liver.

Inherited through a recessive gene.

Due to inborn defect in the metabolism of copper.

WILSON'S DISEASE

A greenish brown pigmentation of the cornea near the sclera junction (Kayser-Fleischer ring) is present in the majority of cases.

The ring is formed by copper deposits within the eye.

Onset of symptoms between 11 and 25 years.

WILSON'S DISEASE

No one clinical picture is characteristic because the neurological manifestations are so varied.

Course is progressive unless treated.

Treated with a copper poor diet' and with potassium sulfide to minimize copper absorption.

EEG CHANGES WITH WILSON'S DISEASE

Usually normal. May show some slowing of the alpha.

There is tendency for the patient with abnormal EEGs to be those with the most severe clinical symptoms.

TOURETTE'S SYNDROME

Tourette's syndrome is defined clinically as a chronic tic disorder beginning in childhood with both involuntary motor and vocal tics.

Onset between age 5 - 10 years with a male to female ratio of 4: 1.

Repetitive eyelid blinking, facial tics, involuntary obscenities and constant repetition of other people's words are common clinical characteristics.

TOURETTE'S SYNDROME

Normal intelligence.

Haldol is used for treatment.

Recent theory that it is a maturation disorder involving the neurotransmitters and their receptors.

EEG CHANGES WITH TOURETTE'S SYNDROME

The EEG is severely marred by muscle and movement artifacts but is usually normal.

DISEASES THAT AFFECT THE SPINAL CORD

ALS (AMYOTROPHIC LATERAL SCLEROSIS)

ALS is a chronic, progressive disease of unkown etiology.

It is characterized by atrophy and fibrillations of the muscles.

Degeneration occurs in the motor cells in the spinal cord and in the medulla.

Patients retain their normal intelligence.

EEG CHANGES WITH ALS

EEG remains normal.

DEMYELINATING DISEASES

MULTIPLE SCLEROSIS

MS is the most common cause of neurological disability of men and women in their productive years - ages 15-55 years.

MS is a chronic illness that varies in severity. Pain and loss of mental faculties are rare.

Demyelination occurs in the CNS only and can involve most motor and sensory functions.

MULTIPLE SCLEROSIS

The cause of the demyelination is unknown although there are numerous theories.

There is no specific treatment and nothing alters its unpredictable course.

Weakness involving one or both legs is the most common initial symptom.

MULTIPLE SCLEROSIS

The symptoms have a tendency to have cycles of remission and exacerbation.

The cycles come without warning.

The majority of cases tend to level out and not exhibit a progressive decline.

EEG CHANGES WITH MULTIPLE SCLEROSIS

The EEG is usually normal.

The abnormalities seen depend on the location of the CNS involvement and the stage of the disease.

The abnormalities may be focal or diffuse and are more likely to be seen during periods of exacerbation.

EVOKED POTENTIALS IN MULTIPLE SCLEROSIS

Evoked potentials have been found to be extremely useful in diagnosis of MS, particularly VEP’s.

Many patients with or without history or signs of visual disturbance will have abnormally slow VEP's.

BAER's can evaluate the brainstem.

SEP's can evaluate the peripheral nervous system as well as the CNS.