RELATION
OF
OPTIC
NEURITIS
TO
MULTIPLE
SCLEROSIS
IN CHILDREN
Charles Kennedy, M.D., and Sidney Carter, M.D.
Neurological Institute, Presbyterian Hospital, New York, and Department of Neurology (Division of Child Neurology), College of Physicians and Surgeons, Columbia University
PRESENT ADDRESS: (C.K.) Children’s Hospital of Philadelphia, 1740 Bainbridge Street, Philadelphia
46, Pennsylvania.
PEDIATRICS, September 1961 377
O
PTIC NEURITIS has been defined as theresult of involvement of any part of the optic nerve by a disease process, whether inflammatory, vascular or degen-erative, which impairs nerve conductivity as indicated by visual field changes.1 When this process is proximal to the point of emergence of nerve fibers into the orbit, such that the optic disk is either normal or pale in appearance it is called retrobulbar neuritis, whereas if there are vascular changes or swelling around the papilla the term papillitis may be applied. Under these circumstances the appearance of the ocular fundus resembles that of papilledema such that the two conditions cannot be differ-entiated by ophthalmoscopic examination, and the need arises to rule out the various causes of increased intracranial pressure by other criteria.2
Optic neuritis occurs relatively infre-quently in childhood and is seen most com-monly as a complication of an acute in-fectious disease such as measles,3 mumps,4 chickenpox or pertussis,” or following im-munization procedures.1’7 Hereditary forms may result in severe visual impairment dat-ing from birth or may cause visual failure at almost any age,81#{176} and in some such cases there may later evolve more wide-spread degenerative changes in the nervous system.’ Malnutrition of a severe degree has been found to be associated with visual failure and partial optic atrophy in children in the British West Indies,12 but to date no cases have been seen in the United States. Toxic agents and a number of drugs may also result in selective injury to the optic nerve at any age.’ Finally, optic neuritis
may occur as an isolated episode unassoci-ated with any clinical circumstance to sug-gest its pathogenesis, although the observa-tions by Fleischerll and later by Lillie’4 in-dicated that in young adults it is often the first manifestation of multiple sclerosis.
More recent follow-up studies have
shown that one-third or more of those who
suffer an attack of unexplained optic neuri-tic have evidence of dissemination within a 10-to-15-year period.1517 That multiple sclerosis may also develop in children with optic neuritis is shown in the cases cited by Ford18 and in the one mentioned by Taub and Rucker.6 In a recent report of 30 chil-dren with optic neuritis, 8 were found to have developed clinical symptoms of dis-serninated involvement in a variable period after, and in one case before, the visual symptoms.18 In view of the dearth of clini-cal reports in children, dealing with the re-lationship between these two entities, it was considered of value to present the find-ings in these eight cases in some retail. One case (L.F.) had been included in the report of Low and Carter,19 but the remainder are additions to their group of cases of multiple sclerosis having its onset before the age of 15 years. In each instance the
diagnosis was made on the basis of the usual clinical criteria.20
CLINICAL MATERIAL
The age of the children at the time of the
charac-Case Sex
Optic Neuritis
Firs! Attack
Visual
Field Defect
At-tacks (no.)
Interval to First
Symp-torn of
.
Multiple
.
Sclerosis
Last Follow-up
-.
Age Neurologw
(yr) Status
Age
(yr)
Lalerality of
.
Vrsual
Symptoms
I F S Simultaneously
bilateral
Peripheral
constriction
S If I 1 (Remission)
F 13 Unilateral Central
scotoma
24 (Died)
3 M 10 Unilateral Central 4
scotoma
13 Partial remission from
para-plegia
4 F 1 Unilateral Central
scotoma
S 1 24 Partial remission from
para-paresis
5
6
F 14 Unilateral Central
scotoma
15 Partial remission from
quad-riparesis and ataxia
F i5 Unilateral Central
scotoma
I (S wk) 4 (Complete remission)
7 F 8 Simultaneously
bilateral
Uncertain I 8f (Hemiparesis and ataxia)
8 M 5 Simultaneously
bilateral
Uncertain 1 16 Transverse myelopathy
TABLE I
SUMMARY OF CLINICAL FINDINGS-CHILDREN WITH OPTIC NEURITIS ASSOCIATED WITH MULTIPLE SCLEROSIS
* Neurologic symptoms preceded optic neuritis by 34 years.
terized by 1) abrupthess of onset with a severe degree of visual impairment, 2) im-pairment of the central portion of the vis-sual field in most instances, and 3) a tend-ency to remission and relapse. Although papillitis was present in four cases, in only one of these was a pneumoencephalogram
necessary to rule out the possibility of an
intracranial mass lesion. In one case the
spinal cord and brain stem symptoms pre-ceded the optic neuritis; in seven the visual episode occurred first, followed by the de-velopment of other neurologic signs after a variable interval from 3 weeks to 16 years. In all but two, dissemination had occurred within 2% years after the first attack of op-tic neuritis. The cerebrospinal fluid was normal in all of the children at the time of
their visual involvement, and in only one instance was there even a borderline in-crease in the percentage of total protein due to gamma globulin (13% in Case 1).
CASE REPORTS
Case 1 (PH., 26-41-48)
HISTORY: An 8%-year-old girl was first seen in February, 1956, because of pain in the eyes and bilateral impairment of vision. Two weeks previously she had had a res-piratory infection followed by pain in the right eye, and a day or two later pain de-veloped in the left eye also. This pain was
aggravated on movement of the eyes.
clearly. This child had been in a poor state of general health for the previous year, during which she had had measles, chick-enpox, pertussis, and a recurrent sinusitis. She had been aware of severe episodes of diplopia, which was not present in the 2
weeks prior to her being seen.
PHYSICAL EXAMINATION: The tempera-tare was 103#{176}F(39.4#{176}C), but there were no positive physical findings. The neurologic examination was also negative. Her vision was 20/50 on the right and was reduced to hand movements at 1 ft on the left. There was a marked decrease in the direct pupil-lary reaction to light on the left and
blur-ring and elevation of both optic disks, this being more marked also on the left. The visual field showed a constriction to 20 de-grees with an enlarged blind spot on the
right, but it could not be plotted on the left.
LABORATORY FirnNcs: The leukocyte count was 11,000/mm3, with a normal dif-ferential count. Urinalysis revealed nega-tive findings. Cerebrospinal fluid studies revealed normal pressure; protein, 33 mgI
100 ml, 13% of which was gamma-globulin; and a negative result of Kolmer test. Roent-genograms of the skull revealed negative findings, except for evidence of maxillary sinusitis, presumably due to a hemolytic Staphylococcus aureus which was isolated from the nose.
Cou1sE: The child was hospitalized and given aureomycin, erythromycin and corti-sone. During a 2-week period there was im-provement in the vision in the left eye, the field defect in which was found to be an irregular constriction with greatest involve-ment of the temporal field. There was con-tinued, gradual improvement, and the pa-tient was discharged after 2 months with no medication, but she was readmitted 6 days later with fever and visual impairment in the left eye as severe as on her original admission. She also complained of severe pain in her heels on the second admission, the cause for which was not determined. Again she was given steroids, and during a 10-day period her vision in the left eye
improved to 12/200. The heel pain also subsided.
Two months later vision in the right eye was reduced to the ability to count fingers at 6 in., simultaneously with a relapse in the vision on the left to 1/200. At this time the tendon reflexes were increased and were more active on the right side than on the left, and vibration sense was diminished in both legs. Treatment with steroids (pred-nisone) was again associated with a remis-sion on visual difficulties. Pallor was later noted in both disks.
A third episode of sudden visual failure occurred (3 months later, this time reducing the vision in the left eye to light perception only. A repeat lumbar puncture disclosed no increase in pressure, leukocytes or pro-tein, the gamma-globulin component of which 7% of the total protein. On steroid therapy the vision returned to 20/70 on the left. She continued to receive small doses of steroids (prednisone 5 mg/day) for the next 5 months, but discontinuance was
again followed shortly by a relapse, and reinstatement by a remission in visual acu-ity. During this time she had had periods of urinary urgency and a recurrence of pains in her heels.
In October, 1957, the neurologic examina-tion disclosed nystagmus on lateral gaze, more marked on the right. Tendon reflexes were again hyperactive, with right-sided preponderance. The plantar response was bilaterally upward. When last seen in April, 1958, she had had two periods of relapse and remission of visual acuity, both cor-related with cessation and reinstitution of steroid therapy.
COMMENT: In addition to recurrent epi-sodes of visual impairment, this child had had diplopia (which had preceded the op-tic neuritis), nystagmus, pyramidal tract signs and diminution of vibratory sensa-tion. Therefore, the diagnosis of multiple sclerosis seems virtually certain.
groups of patients there seems to be no conclusive evidence that steroids affect the natural course of optic neuritis.5
Case 2 (R.S., 47-00-97)
HISTORY: A 13-year-old girl was first seen at the Columbia Presbyterian Medical
Cen-ter on November 15, 1935, because of dif-ficulty in reading. Her vision was found to be 20/200 on the right and 20/30 on the left; on refraction the deficit could not be corrected, and no lenses were advised. Visual fields were not plotted at that time. Twenty-two months later she returned com-plaining of pain in the left eye.
PHYSICAL EXAMINATION: The vision was
20/70 on the right and 20/50 on the left,
and definite pallor of both disks was noted. Bilateral central scotomas were found. Re-sults of a neurologic examination were en-tirely negative, as were roentgenographic findings of the skull and optic foramina.
COURSE: In October, 1937, occasional horizontal nystagmus on gaze to the left was noted, and the patient recalled that 3 months previously she had had a period of numbness in the fingertips of one hand, lasting about a week. Some weeks later this occurred in the opposite hand also. In November, 1937, nystagmus was marked on extreme lateral gaze, and patient com-plained of numbness in the left leg below the knee, which disappeared in a few days. In the subsequent year she became ataxic in all four extremities and had particular dif-ficulty in handwriting. Examination of cere-brospinal fluid in May, 1938, revealed two
leukocytes, no elevation of protein content, and a colloidal gold curve of 1111100000. Results of serologic studies of the blood were negative. The vision continued to fail gradually, and the scotomas enlarged in size.
The patient was hospitalized in March, 1939, and the previously noted ataxia
seemed more pronounced and was most
marked in the right upper extremity. Her speech was scanning and explosive in char-acter. On sensory examination there was impairment of two point discrimination in
both legs as well as loss of vibratory sensa-tion. The tendon reflexes were increased in the right arm, and the plantar response
was upward on the left. In the subsequent
year the course of her illness progressed rapidly, her vision being reduced to
5/200
on the right and 3/200 on the left, and her locomotion impaired by both ataxia and spasticity such that she became confined to a wheel chair. She then developed bowel and bladder impairment and became bed-ridden. She died at the age of 24 years, 10% years after the onset of her vision fail-ure.COMMENT: This patient developed evi-dence of disseminated involvement at the age of 15 years, 2 years after her first com-plaint of visual failure. The course of her illness is somewhat atypical in that it was steadily progressive from the onset until her death. However detailed review of the rec-ord indicates that there were short remis-sions in the sensory complaints, i.e., numb-ness and paresthesia, and also in visual acuity as judged subjectively by the patient herself from day to day.
Case 3 (W.S., 98-13-27)
HISTORY: A 10-year-old boy was seen
J
anuary 4, 1949, because of loss of vision in the right eye, which had first been no-ticed 9 days previously. This had been dis-covered accidentally when the boy had placed his hand over the left eye and found he was unable to see. There was no head-ache, no eye pain or other complaint, and the boy had had no recent respiratory or other acute infection. However this child had been subject to almost continuous chronic respiratory infections since infancy, and during the previous year he had had a number of episodes, even during thesum-mer months. He lost weight and had a
cough with productive sputum that was particularly severe 3 months prior to the onset of visual failure.
and rhonchi were heard throughout the chest. Results of neurologic examination were entirely negative. Vision was reduced to light perception in the temporal field on the right but was 20/15 on the left. The central and peripheral fields were normal
in the left eye. On ophthalmologic
examina-tion there was blurring of the right disk, which was elevated 2 to 3 diopters.
LABORATORY FiDINcs: Roentgenograms
of the chest showed evidence of broncho-pneumonia at both bases. Films of the skull disclosed pansinusitis with extensive involvement of the right maxillary sinus, hut the optic foramina were normal and there was no evidence of intracranial
pa-thology. The leukocyte count was normal.
The erythrocyte sedimentation rate was 35 mm/hr. Results of urinalysis and blood
tests for syphilis were negative. The
tu-berculin test result in 1:1000 and 1:100 dilutions was negative. The cerebrospinal fluid was under normal pressure, was free of leukocytes, contained 14 mg/100 ml of protein, and had a negative Kolmer re-action.
COURSE: During a 5-week hospital stay
the patient’s vision remained unchanged, and in spite of therapy with penicillin and sulfadiazine there was only slight clearing
of the bronchopneumonia. The question of
bronchiectasis was raised; however, a bron-chogram some months later revealed nor-mal findings. The patient continued to have symptoms of chronic bronchitis during the following year and had only minimal im-provement in the vision in the right eye. In June, 1951, he developed sudden visual failure of the left eye simultaneously with an exacerbation of his respiratory disease. At that time he had pain in the orbit and
pain on eye movement. His vision was then found to consist of light perception only, in both eyes. Ophthalmoscopic examination revealed marked pallor of the right eye, with blurring and elevation of the disks of the left eye. There were physical signs of bronchopneumonia, confirmed by roent-genography; and the pansinusitis persisted. Findings of cerebrospinal fluid examination
were again negative, there being no eleva-tion of the protein content, 11% of which was gamma-globulin. The infectious
proc-esses were treated vigorously with
chemo-therapy, autogenous vaccine, and drainage of purulent material. The vision improved to
3/200
on the right and 20/20 on the left.The patient remained relatively well until November, 1951, when he developed weakness of the legs together with numb-ness which gradually progressed for a week. He was readmitted to the hospital. On neurologic examination he had evidence of
a partial transverse myelopathy with a
sen-sory level on the right at the sixth thoracic
vertebra, and weakness in the legs, the left being more involved than the right. Reflexes were more active in the left leg, and the plantar responses were upward bilaterally. Concurrent with the development of these
signs there was a relapse in his vision on the left, which was then found to be 6/200.
There was bilateral optic atrophy. A repeat examination of cerebrospinal fluid disclosed no abnormality, and the gamma-globulin fraction was 10% of the total protein. He developed urinary incontinence with com-plete paraplegia in the course of hospitali-zation. A gradual spontaneous recovery en-sued during the next 3 months, and again he was able to walk and to control urinary
function. The remission continued until March, 1954, when he was lost to follow-up.
COMMENT: Within 3 years after the first attack of optic neuritis this boy showed evi-dence of disseminated involvement in the form of a partial transverse myelopathy, which subsequently cleared so that he was able to walk and to control urinary func-tion. The history of this boy resembles that of Case 1, in which early in the course there also was recurrent respiratory infec-tions and sinusitis.
Case 4 (E.F., 09-26-96)
HISTORY: A 14-year-old girl was first seen
the right eye, at which time she also had severe frontal headaches and complained of seeing dark spots before her eyes. She had received vitamins parenterally from a phy-sician, but her complaint persisted
un-changed for several weeks and then
sub-sided. She had been well until 2 months
prior to being seen, when vision on the right became blurred again, but seemed to improve after vitamins were given intra-venously. However, about 3 weeks after the onset of the recurrence on the right, she began having similar difficulty with the left eye, which persisted until the time of her visit to the Medical Center. At that time her vision was 20/30 in the right eye and 20/70 in the left eye, with normal fields. Because of pallor of both optic disks, she was admitted to the Neurological Insti-tute.
PiwsICu. EXAMINATION: There were no abnormal findings in either the general physical or neurologic examinations, except for those noted previously, and the presence of hippus bilaterally.
LABORATORY FINDINGS: Roentgenograms of the skull, including a pneumoencephalo-gram, revealed normal findings. The leuko-cyte count and results of urinalysis were within normal limits, and the erythrocyte sedimentation rate was 6 mm/hour. The cerebrospinal fluid contained 10 leukocytes, 9 of which were lymphocytes, and 43 mg of protein/100 ml. A repeat examination of cerebrospinal fluid 3 weeks after admission disclosed 16 leukocytes, 14 of which were lymphocytes. The serologic test result for syphilis was negative.
CouRsE: Shortly after the patient’s ad-mission her vision improved to 20/15 in both eyes, but pallor of the disks appeared more marked. She was discharged and was well until 3 years later when for a 3-day period she became totally blind. On exam-ination she had minimal light perception on the right and no light perception on the left. The disks were again found to be pale and flat. Visual fields were not plotted at that time; her visual impairment persisted
for an uncertain period and then improved. At the age of 22 years she had periods of diplopia shortly followed by a recurrence of bilateral visual failure, which never sig-nificantly improved. In May, 1952, at the age of 24 years she had her first major neurologic complaints and was readmitted to the Medical Center.
At this time, she developed partial trans-verse myelopathy, which had been pre-ceded by a respiratory infection and severe throbbing headache 3 weeks previously. The leukocyte count was 25,000, with a shift to the left; the cerebrospinal fluid con-tamed 114 lymphocytes and a protein con-centration of 60 mg/100 ml, the fraction due to gamma-globulin being 3%. Vision was reduced to counting fingers at 6 in. on the right, with absence of light perception on the left, and there was bilateral
weak-ness of the sixth nerve. Both of the upper
extremities showed moderate ataxia. During her hospital course the patient developed an unexplained, persistent fever that was considered to be out of proportion to an obviously slight infection of the urinary tract, associated with an indwelling cathe-ter. Ataxia increased in the upper extremi-ties, and she developed mild dysarthria; however, her sensory complaint and para-paresis subsided, and she was able to walk with support. She was discharged with no improvement in her vision.
COMMENT: This patient had at least four distinct episodes of optic neuritis which originally involved only the right eye.
Case 5 (M.G., 28-48-90A)
HISTORY: A 14-year-old girl was seen in August, 1956, because of pain in the right eye aggravated on eye movements, fol-lowed 4 days later by the appearance of large gray spots before her eyes. There was no previous respiratory infection.
383
elevation of the right disk. The results of general physical and neurologic examina-tions were normal.
LABORATORY FINDINGS: Lumbar puncture disclosed a normal cerebrospinal fluid pres-sure, the fluid containing nine leukocytes and 27 mg of protein/100 ml, 7% of which
was gamma-globulin. Results of blood and
cerebrospinal fluid tests for syphilis were negative; roentgenograms of the skull, in-eluding the optic foramina, revealed nor-mal findings. An electroencephalogram
contained more than the usual number of waves occurring at a frequency of 4 to 6 per second, and was therefore considered
to be abnormal.
COURSE: There was some subjective im-provement without specific treatment 2 weeks after onset of visual impairment, and in 3 weeks the visual fields showed a small relative scotoma in the left eye in addition to the central scotoma which persisted on
the right. The vision then was 15/200 on
the right and 20/15 on the left. One month
later vision and acuity were both normal,
and the patient remained in good health until April, 1958, when she again com-plained of spots before her eyes with blur-ring of vision first on the right and a week later on the left. She then noticed
unstead-mess of her gait and paresthesias in the
right arm and leg. On neurologic examina-tion there was bilateral facial paresis with weakness of all extremities, more on the right than on the left, with hypalgesia in the distal portion of all extremities. Position sense was impaired on the right, and there was marked ataxia, more pronounced on the right side. There was symmetrical hy-perreflexia with upward plantar responses. Vision was reduced to
5/200
on the right and 3/200 on the left, with central scotomas in both visual fields. On ophthalmoscopic examination the disk margins were blurred and elevated. The cerebrospinal fluid on the second admission contained lympho-cytes, 19/mm3, and protein, 34 mg/100 ml, 15% of which was gamma-globulin. Herstatus seemed to be getting progressively
worse until she was given prednisone, 40 mg/day; shortly thereafter she improved. Forty-seven days after the onset of this second episode the neurologic symptoms had markedly subsided and the vision was 20/40 on the right, with no scotoma, and
20/100 on the left, with a small central sco-toma.
COMMENT: In this patient the optic neu-ntis was bilateral but not simultaneously so. The symptoms of disseminated involvement followed about 2 years later, shortly after a recurrence of the optic neuritis, which again involved both eyes. There was no associated or preceding infection with either episode of visual failure.
Case 6 (L.M., 197-98-59)
HISTORY: A 15-year-old girl was admit-ted to the Columbia Presbyterian Medical Center in December, 1949, because of blur-ring of vision in the right eye. About a year previously she recalled an episode of numb-ness in both hands which had
spontane-ously subsided in a few days. One month
prior to admission she suddenly lost her vision in the right eye, but this had
grad-ually improved. Three weeks later she
complained of numbness of the left hand and left side of the abdomen and became aware of “funny feelings” in the right hand on the day before admission.
larger than the left. The vision was 5/200
on the right and 20/20 on the left. A 20-degree central scotoma was found on the right.
LABORATORY FINDINGS: The leukocyte count, the serologic test for syphilis, non-protein nitrogen in serum, and results of urinalysis were within normal limits. The cerebrospinal fluid was under no increase in pressure, contained 3 leukocytes, and had a total protein value of 26 mg/100 ml,
9%
of which was gamma-globulin.COURSE: During the hospital stay there was improvement in the visual and neuro-logic status, and at the time of discharge
the vision had returned to 20/200 on the left. The central scotoma was reduced in size. Correspondence and a telephone con-versation with this patient’s family 9 years later revealed that her complaints had grad-ually subsided in the month following dis-charge, and that she had been entirely well in the interim and was presently teaching in a grade school.
COMMENT: It is possible that the epi-sode of numbness in the hand 1 year prior to the visual failure was a manifestation of what proved clearly to be multiple sclero-sis. However, the major neurologic signs in this patient followed the onset of optic neuritis by a matter of weeks. In spite of the early dissemination, the duration of complete remission is over 9 years to the time of this writing.
Case 7 (L.F., 22-93-48)
HIsToRY: An 8%-year-old girl was admit-ted to the Long Island Jewish Hospital on February 3, 1955, because of sudden loss of vision. She had been well on the previ-ous day except for blurring which she no-ticed in school. This had increased in the course of the day, and visual loss was com-plete in the evening.
At the age of 6 years she had been ad-mitted to Willard Parker Hospital because of fever, lethargy and a tremor of both arms. After admission there was spontane-ous cessation of the tremor, and she devel-oped a flaccid paraplegia with incontinence
of urine and feces. She was treated with
corticotropin (ACTH) and gradually recov-ered strength in the legs and was dis-charged after four weeks, able to walk but with marked ataxia. Three days after dis-charge she again became stuporous and was unable to speak or swallow and there-fore was readmitted. During the subse-quent two weeks she recovered, returned home and again attended school. In Jan-uary, 1953, she had developed a right-sided
hemiparesis for a few days; this subsided
spontaneously in one month. She again re-turned to school and had no further neuro-logic complaints until the time of her visual failure, for which she was hospitalized at the Long Island Jewish Hospital in Feb-mary, 1955.
PHYSICAL EXAMINATION: Results of phys-ical examination at that time were nega-tive. On neurologic examination her sen-sorium was clear. She was able to walk without difficulty and had no ataxia. The result of a Romberg test was negative. There were no sensory abnormalities. The plantar response was upward bilaterally, and there was bilateral ankle clonus. Dys-arthria was prominent, together with drooling of saliva. There was no detailed record of the degree of visual impairment except that she had searching nystagmoid movements and said she was unable to see “at all”. The optic disks were sharply out-lined and both were thought to be pale.
LABORATORY FINDINGS: Roentgenograms
of the skull were negative. The spinal fluid was under normal pressure and contained 8 leukocytes, 72 mg of sugar, and 88 mg of protein/100 ml.
Cen-ter on May 9, 1955, primarily because of vertigo and vomiting. On examination the hemiparesis was evident, together with left-sided ataxia, most conspicuous on finger-to-nose and heel-to-shin testing. The optic disks were pale, but there was no gross impairment of visual acuity, and the visual fields were normal. Results of urinalysis and the leukocyte count were within nor-mal limits. Findings from cerebrospinal fluid studies were also negative, there being 39 mg of protein/100 ml, 7% of which was gamma-globulin. An electroencephalogram
was considered abnormal, with more ab-normality noted on the left side. During her hospital course there was no change in
status, and she was discharged at the end
of 1 week.
COMMENT: The child was previously re-ported1#{176} by one of us as having multiple sclerosis in childhood. Unfortunately from the records available it is not possible to characterize in any detail the period of visual failure, lasting about 3 weeks, follow-ing which pallor of the optic disks was noted. Apparently there was simultaneous bilateral involvement.
Case 8 (JS., 09-04-08)
HISTORY: A 5-year-old boy was admitted to the Columbia Presbyterian Medical Cen-ter in October, 1936, because of blurring of his vision, photophobia and burning pain in the eyes over a 3-day period. He had been quite well except for a rather severe allergic rhinitis that had developed 3 months previously and had persisted until the time of his visual complaint. The par-ents had become aware of his difficulty in vision on observing the manner in which he would try to pick up small objects, and on the day before he was seen even large objects were not recognized.
PHYSICAL EXAMINATION: The child was unable to see a coin at 2 ft with both eyes open. A more detailed estimate of visual acuity was not recorded. There was blur-ring and elevation of both optic disks, with venous engorgement; hemorrhages were noted in the inferior nasal quadrant of the
left eye. Findings on general physical ex-amination were negative, except for evi-dence of a severe allergic rhinitis. Results of neurologic examination were negative.
LABORATORY FINDINGS: The leukocyte count was within normal limits, and the Mazzini test result was negative. The cere-brospinal fluid, which was under normal pressure, contained 6 leukocytes and gave a negative result of a Pandy test. Roent-genograms of the skull were considered normal.
COURSE: At the end of the first week of hospitalization the vision improved, as judged from reaction to nearly objects. The child was given a ketogenic diet and sent home; 2 weeks later the vision had re-turned to normal, as judged from observa-tions of the child in play. The duration of impairment could not be estimated, but there seemed no question that 2 months later his vision was as good as it had been prior to the episode of visual failure. He continued to suffer from severe allergic rhinitis during the next 3 years, during which time he had no other illnesses.
was quite well for the next 12 years except for recurrent asthmatic attacks.
In April, 1952, he was readmitted to the hospital, following a respiratory infection, with numbness and weakness of the legs and difficulty in passing urine. During the first few days of hospitalization he devel-oped complete transverse myelopathy at the level of the eighth thoracic vertebra. The cerebrospinal fluid was found to con-tam 108 leukocytes/mm3, 95 of which were lymphocytes; the sugar content was 59 mg and protein 60 mg/100 ml, 21% of which was gamma-globulin. Subsequently the boy showed partial improvement in his motor function.
COMMENT: The boy’s optic neuritis re-sembles that of Case 7, in being bilateral and simultaneous. It antedates the diagnosis of multiple sclerosis by more than 15 years. The undiagnosed encephalopathy at the age of 9 years may well have been a manifesta-tion of the disseminated process, although it was atypical, convulsions usually being a very late symptom of the disease.
COMMENT
On the basis of a 15-year follow-up of patients with optic neuritis, Taub and Rucker have suggested that the likelihood of the later development of multiple sclero-sis varies with age.1#{176}While 40 to 50% of those who had visual involvement between
the ages of 20 and 44 years showed signs
of dissemination within a 10 to 15-year period, this was true of a much smaller percentage when the optic neuritis occurred before the age of 20 years. The eight cases of multiple sclerosis cited here are from among 30 instances of optic neuritis of un-known cause in children seen at the Co-lumbia Presbyterian Medical Center during a 25-year period.2 The proportion of one quarter would seem to bear out the pre-viously mentioned impression. However, because the mean follow-up period in the children (8.0 years) was somewhat shorter than that of Taub and Rucker, and also be-cause the interval between involvement of the optic nerve and the later development
of other symptoms may be prolonged,21 it is reasonable to assume that the propor-tion with multiple sclerosis among those in whom optic neuritis occurred in childhood will eventually be larger.
There were no distinctive clinical or
labo-ratory findings at the time of the attack of
optic neuritis by which one might predict the subsequent development of multiple sclerosis, although the frequency of simul-taneous bilateral involvement (3 of 8 cases) and of papillitis (4 of 8 cases) is in contrast to the reports of adults indicating that uni-lateral retrobulbar neuritis is far more com-monly seen.11 17, 21 The evolution of
symp-toms and signs of dissemination was fairly typical in these children, the only unusual features being the encephalitic-like findings
noted in Case 7. Conceivably, the
encepha-lopathy in Case 8, in which convulsions
occurred, was also a manifestation of the
demyelinating process.
Lethargy is an infrequent but well
rec-ognized 223 Convulsions are most
unusual except as a late manifestation,24
although a few reports have appeared in which seizures have occurred early in the course of the disease.22 The progression to a state of rather severe disability when last seen in six of the eight children indicates that this group may have a more malignant form of multiple sclerosis than usual. How-ever, it is noteworthy that one of the
chil-dren (Case 6) has been in complete re-mission for 9 years; in this connection Mac-kay has called attention to a case in which there was unilateral visual failure at 4 years of age with recovery in a few months and then recurrence 29 years later. This patient was 46 years of age before any significant disability developed as a result of her mul-tiple sclerosis.25 Probably, therefore, the variability in the progress of the disease is about the same whether the visual involv-ment occurs during childhood or in adult life.
SUMMARY
cause were found to have developed multi-ple sclerosis in variable periods after the episode of visual failure. The clinical find-ings are reported here in detail. There were
no distinctive characteristics of the ocular
findings to indicate those who would
event-tially show evidence of multiple sclerosis,
although simultaneous bilateral involve-ment and papillitis would appear to be seen more frequently in the children than in the adults who later developed multiple sclero-sis. Six of the eight children developed evi-dence of dissemination within a 2%-year period. In the other two, dissemination did not occur until 12 and 16 years later.
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