[HF4088 Multiple sclerosis 1967, Length: 00:26:25, Color, Sound.
This Beta SP was duplicated from a 16mm answer print by Bono Film & Video, Inc
for the National Library of Medicine, February, 2010.]
[Dark, then film leader and countdown]
[This film has been aquired for distribution by the National Medical Audiovisual Center]
[Narrator:] The patient with multiple sclerosis staggers down a long road,
weaving between despair on the one side and hope on the other.
The doctor who must guide this patient is faced with a disorder in which the diagnosis
is a gamble of odds and probabilities.
The etiology is hidden in a jungle of clues and the therapy stirs a broth of heated dispute.
It is hoped that this film will enable the practitioner and the student to better understand
this elusive disease.
[The National Multiple Sclerosis Society presents]
[Multiple Sclerosis]
[supervised by The Division of Neurology of The The School of Medicine,
University of California at Los Angeles]
[Narrator:] This is a...at 19, she had a two-week episode of burning and hypersensitivity
of the right arm.
A second episode occurred nine months later, beginning with sharp pains in the right forehead
and scalp, then numbness and tingling in the right fingers and toes, ascending in three days
to include the entire right side below the neck.
After one month, these symptoms had almost cleared, but she then developed loss of vision
in the left eye over a three-day period, followed in two weeks by blurring of vision in the right eye
with pain on movement of the eyes.
At this time, she also noted a tingling down the back, on bending the head forward.
On examination at the peak of this attack, she could only count fingers with the left eye,
and the visual field showed a huge scotoma involving the central region and the area around the blind spot.
On the right, visual acuity was 20 over 40 and field-testing showed a scotoma involving the area
around the blind spot and the entire inferior nasal quadrant, but sparing central vision.
The optic discs appeared normal.
These findings of optic neuritis gradually disappeared over the next two months.
In the four years since then, she has remained well, except for one persistent symptom.
[Patient:] When I bend my neck down, I get an electric shock sensation that extends
from my neck down my back, through my legs.
[Narrator:] This electric shock-like sensation on flexion of the neck is called Lhermitte's sign
and is considered a manifestation of posterior column damage in the cervical spinal cord.
This is a 43-year-old sales manager for a dairy company.
At 33, he had an episode of left-sided numbness and weakness, unsteady walk, and double vision,
with complete recovery in six months.
At 36, he developed slurred speech, difficulty focusing his eyes, clumsiness of the left arm and leg,
hypersensitivity of the right side of the body, and Lhermitte's sign.
He improved after one month but was left with permanent residual.
This stayed the same for the next four years, then slowly worsened.
Ten years after onset, he demonstrates the following:
His eye movements are impaired in all directions of gaze.
When he looks to the sides, the in-turning eye is paralyzed.
The out-turning eye shows horizontal nystagmus.
There is weakness of upward gaze, left more than right, and vertical nystagmus
on upward and downward gaze.
This disconjugate gaze difficulty is called internuclear ophthalmoplegia.
It represents damage to the median longitudinal fasciculus, a tract that runs through
the dorsal portion of the brain stem on each side of the midline.
Bilateral internuclear ophthalmoplegia is considered by many authorities
to be pathognomonic of multiple sclerosis.
His speech shows dysarthria characterized by jerky rhythm and slurring.
[Patient 2:] On July 24th, 1858, after consulting friends about the proposal, Abraham Lincoln,
the Republican candidate for senator, challenged his Democratic opponent, the incumbent,
Stephen A. Douglas, to divide time and address the same audience during the present canva....
[Narrator:] Testing of his hands shows good coordination on the right.
But on the left, there is a tremor of the outstretched hand accentuated
by performing any accuracy test.
There is also inability to perform rapid alternating movements with this hand.
These signs represent the ataxia of cerebellar disease.
Testing of his legs reveals the weakness on the left.
[The doctor examines the way each leg reacts to stretches.]
There is ankle clonus on the left.
There are bilateral upgoing toes on plantar stimulation.
These signs are indicative of damage to the pyramidal tract somewhere between
the cerebral cortex and the lumbar cord.
This is a 23-year-old insurance company clerk.
Beginning at age 18, she has had one episode each year.
The first attack consisted of buzzing and decreased hearing in the left ear, double vision,
and impaired balance, with complete recovery in six weeks.
The second attack included left-sided tinnitus and hearing loss, staggering walk, double vision,
slurred speech, tingling in the right hand, and numbness in both legs below the knees.
After six months, these symptoms improved, but she was left with permanent nystagmus
and ataxia.
Her third attack began with pain and foggy vision in the right eye, reaching a peak in one month,
at which time the visual acuity was 20 over 30, and the visual fields showed a scotoma adjacent
to the macular region.
This paracentral scotoma of optic neuritis remitted in another month.
A right oculomotor nerve palsy lasting five weeks constituted the fourth attack.
She is now in her fifth attack with symptoms of vertigo, slurred speech, double vision,
staggering gait, and numbness in the right hand.
Examination of eye movements reveals coarse horizontal nystagmus on lateral gaze,
vertical nystagmus on gaze up and down.
A rhythmic head tremor may also be seen.
This represents damage to vestibular pathways.
When she holds out the left hand, there is a flapping tremor at the wrist,
intensified by performance of the finger-to-nose test.
This is independent of whether the eyes are open or closed.
It represents the intention tremor of cerebellar disease.
The right hand exhibits searching movements of the fingers when the eyes are closed,
and hesitancy on the finger-to-nose test, which is not present when the eyes are open,
indicating position sense loss due to posterior column damage in the spinal cord.
Her gait is weaving and lurching because of ataxia in the legs.
She has bilateral ankle clonus.
And Babinski signs as a result of pyramidal tract damage.
This is a 41-year-old housewife and mother whose first symptoms occurred at age 30,
when she developed weakness of the left leg.
At 33, she had nystagmus and an ataxic gait.
Since 35, both legs have become progressively weaker and stiffer.
There have been no remissions.
After 11 years, she shows the following.
Testing of eye movements demonstrates bilateral internuclear ophthalmoplegia,
with weakness of the adducting eye and jerking of the abducting eye on gaze to each side.
There is a mild intention tremor of both hands, representing damage to the cerebellar system.
Babinski signs are present bilaterally.
Both legs show spasticity and weakness, the left more than the right.
The patellar reflexes are hyperactive bilaterally.
This spastic paraparesis with hyperactive and pathological reflexes denotes pyramidal tract
damage in the spinal cord.
This is a 41-year-old schoolteacher whose symptoms began at age 31 when he developed
difficulty focusing his eyes with apparent movement of objects upon which he would fix.
This represented nystagmus.
It occurred intermittently for one year, then disappeared.
At 33, he noted intermittent balance difficulty and weakness of the legs.
At 34, incoordination of the hands and feet.
At 36, his left leg and arm became weak and have remained so ever since.
After ten years, he shows the following.
There is mild ataxia of all four limbs on coordination testing.
This is more prominent on the left.
A slight head tremor occurs intermittently with effort.
[The doctor watches the way the patient's wrists and ankles tremble as he attempts to stretch.]
Spasticity in the left arm and leg is demonstrated when he walks.
The left arm does not swing, and the left leg is used stiffly.
[The patient walks around to demostrate the difference in the way each leg works.]
The reflexes are hyperactive everywhere.
[The patient's reflexes are tested in various parts of his body.]
Hoffmann signs are present bilaterally.
There are bilateral Babinski signs.
This patient's findings indicate damage to the pyramidal tract and the cerebellar system
bilaterally, most likely in the brain stem.
Multiple sclerosis can be diagnosed with certainty only at autopsy
when a picture of patchy demyelination in the central nervous system is seen.
The glial scars or plaques may be seen and felt on the surface of the brain or spinal cord,
as grayish-brown, firm, translucent spots.
All of these pictures are from one patient, a 24-year-old lady with a four-year history
of multiple sclerosis.
Here on the pons is a large, brown, sclerotic plaque with two smaller lesions above it.
These lesions represent a late stage in the pathological process, the demyelinated patch
having been filled in by a scar of glial cell overgrowth.
When cut sections of brain or cord are viewed, foci of demyelination appear as brown patches
in the white matter.
Cerebral foci have a predilection for the white matter immediately adjacent to the ventricles,
especially posterior and inferior horns,
and the white matter about the cortical and basal nuclear gray matter.
The lesions in the cerebral hemisphere vary in size from that of a pinhead
to several centimeters in diameter.
Similarly, numerous areas of discoloration are found in the brain stem and cerebellum.
Early in the plaque formation, there is perivascular inflammation.
Then tissue is destroyed, myelin preponderantly.
Myelin sheaths fragment into globules, fat is liberated, and macrophages ingest the fat.
As the lesion ages, glial cells proliferate and produce fibrils, which give the older lesions
their sclerotic appearance and make them visible to the naked eye.
Here on a freshly cut section of the spinal cord, only a faint area of discoloration is seen
in the posterior columns.
When stained for myelin, the lesions appear as pale foci within the darkly staining white matter.
Many more plaques are now apparent.
The lesions are often far in excess of what one would've expected
from the patient's symptoms and signs during life.
Thus, many plaques may be clinically silent, such as this demyelinated patch
in the optic radiation of the cerebrum.
In fact, there is evidence that a patient may go through life with pathological demyelination
and yet have no clinical manifestations of multiple sclerosis.
[Louis J. Rosner:] The only relatively consistent laboratory abnormalities in multiple sclerosis
are spinal fluid changes.
The spinal fluid may be entirely normal.
This occurred in 40 percent of patients in the UCLA Multiple Sclerosis Clinic.
An elevated gamma globulin content, over 13 percent of the total protein, was found in 58 percent.
The colloidal gold curve was abnormal, the numbers adding up to ten or more in 36 percent.
This test is an indirect measure of relative gamma globulin elevation.
There was an elevated protein in 35 percent, an increased white cell count in 20 percent.
With no pathognomonic laboratory test by which to confirm the diagnosis, the doctor must rely
on his evaluation of the history and the physical examination.
The clinical criteria for the diagnosis of multiple sclerosis are two.
Number one, episodic course.
Number two, multiple symptoms and signs pointing to optic nerve, brain stem, or spinal cord.
Optic nerve manifestations are: decreased visual acuity, central scotoma,
or paracentral, cecocentral, or peripheral on visual field examination
and optic atrophy on ophthalmoscopic examination.
Brain stem manifestations include nystagmus, the disconjugate gaze difficulty known as
internuclear ophthalmoplegia, trigeminal neuralgia, facial sensory deficit, facial weakness,
vertigo, dysarthria, dysphagia, emotional lability, and ataxia of the head, trunk, or limbs.
Spinal cord manifestations consist of the electric sensations on flexing the neck
known as Lhermitte's sign, tight band feelings around the trunk called girdle sensations,
superficial or proprioceptor sensory deficit, spastic weakness with hyperactive and pathological reflexes,
and neurogenic bladder disorder.
Variations in the course and in the signs define four varieties of multiple sclerosis,
the incidence of which is as follows.
Number one, episodic disseminated, the classical type, 78 percent.
Number two, progressive disseminated, 15 percent.
Number three, episodic non-disseminated, five percent.
Number four, progressive non-disseminated, two percent.
Returning to our five patients, it will be seen that the first three represent
the classical type of multiple sclerosis.
The other two, atypical types.
The college student in the first year of her illness had intermittent sensory symptoms
with no objective signs.
She was considered neurotic at that time.
Many patients in the early stages of multiple sclerosis are given the diagnosis of psychoneurosis.
The differential can be a difficult one.
It is recommended that multiple sclerosis be diagnosed only if there are
documented, objective abnormalities on examination.
When this young lady developed the scotomas of optic neuritis and the Lhermitte's sign
of spinal cord involvement, there was objective evidence of neurological disease
and definite evidence of dissemination.
The spinal fluid was normal, except for an elevated gamma globulin of 20 percent.
Then the diagnosis of multiple sclerosis could be made.
The excellent remission further confirmed this impression.
The sales manager began with an attack suggesting both brain stem
and spinal cord pathology.
With complete remission of these symptoms, multiple sclerosis was suspected,
but other remitting conditions occurring at the cervicomedullary junction region
were still possible.
After a two-year interval, he developed the disconjugate gaze difficulty of brain stem disease
and the spinal cord sign of Lhermitte.
Now, the diagnosis of multiple sclerosis was definite on the basis of episodic course
and disseminated signs.
A normal spinal fluid reinforced this conclusion.
The pretty young clerk had only brain stem manifestations with her first attack.
Multiple sclerosis could not be diagnosed at that time, as several other diseases
can give a similar picture with remission.
For example, cholesteatoma in the posterior fossa.
In the second attack, there were sensory symptoms suggestive of spinal cord involvement
in addition to the previous brain stem signs.
There was still the possibility of a compressive lesion at the foramen magnum.
Only with her third attack, when signs of optic neuritis occurred,
was dissemination unequivocal.
The diagnosis could not be made with assurance then, until this, the third year of her illness.
The housewife has had a progressive, non-remitting course.
This occurs in ten to 40 percent of MS patients in different large series.
It is more common with an older age of onset and with a spinal cord type of onset.
With time, it became apparent that this lady had damage in both the brain stem and spinal cord,
as evidenced by the nystagmus and ataxia on the one hand and the spastic paraparesis on the other.
The further development of bilateral internuclear ophthalmoplegia confirms the diagnosis
of multiple sclerosis.
The schoolteacher initially had only nystagmus, which remitted.
After a one-year interval, intermittent ataxia appeared.
Subsequently, the ataxia became constant, and he developed a spastic left hemiparesis
with bilateral hyperreflexia and pathological reflexes.
All signs could be localized to the brain stem.
The early remissions suggested multiple sclerosis, but the absence of
signs of dissemination necessitated the performance of many special tests.
The entirely normal spinal fluid was further evidence of multiple sclerosis.
However, in this type of case, the doctor should be especially cautious in his diagnosis.
After the diagnosis, then what?
Although multiple sclerosis has been known for almost 100 years, its etiology and its treatment
are still unsettled.
One important clue is the epidemiology of the disease.
The geographical distribution is that of a low prevalence in tropical zones
and a high prevalence in temperate and northern zones where studies have been made.
Certain factors commonly found to precipitate exacerbations of the disease include
physical exhaustion, infection, surgical procedures, and emotional crises.
This patient's most recent exacerbation was in association with an emotional crisis.
When an attack occurs, bed rest at the beginning may decrease the duration
and the severity of the relapse.
Symptomatic treatment includes the use of anti-vertigo drugs, muscle relaxants,
antispasmodics for the bladder, and physical therapy.
Most important of all is the psychological support that the doctor can render the patient
by giving him attention, encouragement, and hope.
Specific treatment for multiple sclerosis must await the discovery of the cause.
Many theories have been proposed, but none has been proved.
Research programs are now studying the problems of precipitants in the exacerbations of MS:
geographical factors in its incidence, the nature and function of myelin, the significance of
anti-myelin antibodies, and the experimental demyelinating disease of animals.
The solution to multiple sclerosis will open the door to an understanding
of many other neurological problems.
Speaking for the National Multiple Sclerosis Society is Dr. Augustus S. Rose,
Professor of Neurology at the University of California, Los Angeles.
[Dr. Augustus S. Rose:] It is generally believed that patients derive encouragement and hope
through the effort and programs of the National Multiple Sclerosis Society.
Not many years ago, there was comparatively little scientific and public interest in the problem
of multiple sclerosis.
Today, it is recognized as the leading neurological disorder of our time and a major health problem.
A large and diversified research program is being conducted in many laboratories throughout
the world and with promising results.
Through research and research fellowship training grants, the National Multiple Sclerosis Society
supports fundamental, applied, and clinical studies considered to have relationship
to the problem of multiple sclerosis and demyelination.
Positions and research persons at a postdoctoral level interested in these programs
should communicate with the medical and research director of the National Multiple Sclerosis Society
in New York City.
[written and narrated by Louis J. Rosnerm M.D., assistant Professor of Neurology, U.C.L.A.
in association with Augustus S. Rose, M.D., Professor of Neurology, U.C.L.A.]
[Produced and donated to The Multiple Sclerosis Society by]
[Rex Fleming Productions Santa Barbara, Calif.]
[presented by the]
[National Multiple Sclerosis Society 257 Park Avenue South New York, N.Y. 10010]