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ACUTE

CEREBELLAR

ATAXIA

OF CHILDHOOD

Report

of

Nine

Cases

By Geraldine King, M.D., Gabriel A. Schwarz, M.D., and Harry W. Slade, M.D.

Departments of Neurology and Neurosurgery, School of Medicine, University of Pennsylvania

(Submitted October 14, accepted November 20, 1957.) Supported in part by the Kirby-McCarthy Fund.

ADDRESS: (G.K.) 133 South 36th Street, Philadelphia 4, Pennsylvania.

PEDIATRICS, May 1958

731

I

T WILL be noted from the following brief

review of the literature that acute ataxia in children has been accorded considerable

interest by clinicians throughout the world

over the past 50 years. In the earlier reports,

the emphasis was on the fact that the con-dition followed, or occurred during, certain

of the infectious and contagious diseases. In

more recent times, attention has been

di-rected to the acute ataxias of childhood which have occurred without any specific or identifiable preceding or accompanying infection. Most observers would now seem to be agreed that the majority of these cases

are caused by unknown toxi-infectious

agents and that the cerebellum is the major target organ, although in many cases there

are other areas of involvement.

The authors, over a period of 13 years,

have studied nine such cases, which are

reported in detail.

REVIEW

OF THE

LITERATURE

In 1905 Batten’ divided the various forms

of ataxia of children into three main groups: Cases in which the ataxia was first noted

early in life and tended to improve gradu-ally, designated as congenital cerebellar

ataxia; cases in which the ataxia developed

suddenly after some acute illness in an

otherwise healthy child, acute ataxia, or

encephalitis cerebelbi; and cases in which

there was a gradually developing ataxia in a previously healthy child, progressive

cere-bellar ataxia.

He described the cases of “acute ataxia” as following a characteristic pattern: “A child perfectly healthy and of good

in-teblectual development is taken ill with some acute febrile disease, which may

assume some definite type, such as measles,

or may be assigned to the more indefinite

influenza.

A period

of unconsciousness

may

be present, but general convulsions seldom

occur. The child is kept in bed for a few

days and seems to be making a normal convalescence. When, however, the child is sat up in bed, it is found that he is unable to maintain his balance. There is marked

in-co-ordination of the hands, and, if stood

on his feet, he is wildly ataxic. It may be noticed at the same time that his speech is affected. In a few days his general health is

restored, but he remains as ataxic as ever. Recovery now gradually takes place, and

although in a few cases recovery may be complete in 2 or 3 months, in most cases the

period of recovery extends over several months, and it may be two or three years.”

Batten reported the clinical findings in

five

children

and

collected

several

examples

from the literature. Most of the cases

fol-lowed such infectious diseases as typhoid fever, measles, pertussis, scarlatina, and variola. He ventured the opinion that “the lesion is of vascular origin” . . . “similar in

origin to acute anterior poliomyelitis and

polio-encephalitis”. . . “the cerebellar

symp-toms are due to a thrombosis occurring in

the fine vessels of this region.”

Griffith,2 in a series of papers, reported

the clinical findings in

4

of his own cases

and 27 others he had collected from the literature up to the year 1921. He felt that these were forms of nonsuppurative

(2)

involved. He designated the condition as “acute cerebellar encephalitis.”

The ages of these 31 patients varied

from 3 to 12 years. Eighteen were 6 years

of age or more. In 24 of the cases, the

nerv-ous system was involved during or

immedi-ateby after an infectious disease. For

in-stance, measles immediately preceded the “encephalitis” in eight cases; “influenza” in

five, and typhoid fever in four. Trauma to the head preceded the onset of symptoms in one case. In four cases, the children had no

previous illness of any sort.

Griffith stressed the great variation in the

symptomatobogy of these cases. He noted that clouding of the sensorium and

dis-ordered mentality of varied types and de-gree occurred in most of the cases.

Uncon-sciousness, usually of short duration, was

present in 12 cases. Convulsions were

in-frequently present. Disorders of speech

were noted in 22 cases. Unsteadiness of gait, ataxia of trunk and head, and in-co-ordina-tion of the upper extremities were the signs most constantly present. The degree of

ataxia was quite variable. He further stressed the fact that the prognosis for sur-vival in “acute cerebelbar encephalitis” was good, but the outlook for complete recove.ry

was uncertain. The ataxia often cleared

slowly. Complete recovery occurred in

eight cases in less than 6 months. In some instances, recovery took place over years. In about a fourth of the cases, some “mental defect” was observed in the final reports.

Little attention was given the condition for several years subsequently. Ebey,’ in 1936, in discussing the various neurologic

complications which might arise in the course of various infectious diseases, men-tioned only that ataxia might occur with

variola, varicella and scarlatina.

Klingman and Hodges,4 in 1944,

de-scribed clinical experiences and laboratory findings concerning seven children who de-veloped acute ataxia, muscular

in-co-ordina-tion, and “intention tremor.” They

empha-sized the fact that these cases did not occur during or after the infectious diseases as mentioned by Batten,’ Griffith,2 Ebey,3 and

others. Three of the patients gave a history

of having had an acute febrile illness just

prior to the onset of the neurologic disorder, I)ut four of them were in perfectly good

health until the neurologic signs appeared. There was a slight increase in the cell

con-tent of the cerebrospinal fluid in two of the

cases. No indication of increased

intra-cranial pressure was noted. The blood sera of four children were tested for neutralizing

antibodies against the viruses of St. Louis encephalitis and Eastern equine

encephalo-myelitis. Two of these also had sera tested for the viruses of Western equine encepha-lomyelitis, louping ill, bymphocytic chorio-meningitis and herpes zoster. All serologic studies were negative. Of significance, they felt, was the complete symptomatic re-covery in five of the afflicted children and persistent minimal in-co-ordination in the

other two. In only one child did there de-vebop a marked behavior disorder

after-ward. While cases similar to these in the past had been attributed to influenza or epi-demic encephalitis, they felt that this ex-planation was not tenable, although they

had no other etiologic explanation to offer.

Shanks,5 in 1950, described 39 cases of

cerebellar ataxia in children seen at the

Royal Hospital for Sick Children in Glas-gow from 1929 to 1949. He divided the cases into two groups simply on the basis of

the rate of onset of the ataxia : Group I, sudden onset; Group II, insidious onset. He

found 17 cases which belonged to Group II.

He felt that these represented the

degen-erative ataxias of genetic origin. Most of

these cases gradually progressed and years

later presented evidence of severe

cere-bellar damage. The symptoms and signs in

cases of acute onset were felt by him to be

manifestations of post-infectious encephal-itis or encephabomyebitis. Six of the twenty-two cases of Group I followed such infec-tions as mumps, pertussis, pneumonia, ton-sillitis, nonspecific febribe illness, and febrile reaction to cold vaccine, within 3 weeks. He could not offer any satisfactory etiobogic explanation for the majority of the acute

(3)

HISTORY: T.T., a 5-year-old white boy, was percentage of complete recoveries in this

group.

Other individual case reports or larger

series of patients have since been cited; those occurring during or after exanthems and other recognized childhood diseases, as rubeola in a case reported by Cavalbotti,6 and chicken pox in a case reported by

Martin.’ Varicebba occurred in one of the

cases of Gobdwyn and Waldman;8 and St.

Thieffrey, Martin, and Arthuis9 felt there

was a possible etiologic relationship to

varicelba in eight patients whom they studied. Other illnesses, as bronchopneu-monia, otitis1#{176}and upper respiratory

infec-1 have sometimes preceded the

de-vebopment of cerebellar symptoms.

How-ever, some patients have developed

symp-toms suddenly without preceding illness

or subsequent evidence of any of the above

conditions, and a diagnosis of

“encepha-13 has been made.

DekkerJonkerb0 reported that at the time the first four of his eight cases occurred in Haarlem, pobiomyelitis was prevalent, but

stated that the cerebrospinal fluid was not abnormal. Berglund, Mossberg, and

Ryden-stam’4 showed stronger evidence of a viral etiology in six cases with mainly cerebellar

disturbance; three were due to a

poliomye-litis virus; two were felt to be due to influ-enza virus B; and the others possibly due to influenza A. Two of the patients had

evidence of

cerebral

involvement

also,

and

another,

the

spinal

cord.

Rydenstam’5 has also described two cases of acute cerebellar ataxia during the 1953

epidemic of poliomyelitis in Stockholm in a 5-year-old girl, who became symptom-free in 3 weeks and was asymptomatic 6 months later; and in a 3%-year-old boy, who also recovered rapidly and showed only greater

emotional lability on follow-up

examina-tions.

Both

had positive serologic studies

and virus growth from stool cultures of

type I poliomyelitis virus.

Case 1

CASE

REPORTS

admitted on November 23, 1943 to the Neuro-surgical Service of the Hospital of the

Univer-sity of Pennsylvania, 14 days after the onset of illness. He was first noted to stagger while

playing, then began to stumble and fall. When he reached for objects or attempted to feed

himself, his hands were said to have shaken.

He became slower. It was felt that the symp-toms had progressed somewhat during the 2

weeks of their duration. He had had no fever

and had not vomited.

There was no history of any recent illness or head trauma.

PHYSICAL FINDINGS: The general physical

examination at the time of admission was

es-sentially within normal limits.

On neurobogic examination, he refused to

speak, but he did respond intelligently to commands. He was unable to stand without

support. He threw his lower limbs loosely and

in-co-ordinateby when attempting to walk and

had action tremors in all of his extremities.

There was a generalized hypotonicity. Chorei-form movements were noted in the upper and

lower extremities. Horizontal nystagmus was

present on lateral gaze in either direction, with the quick component to the right. There was nasal blurring of both optic nerve heads,

measured at one diopter. The other cranial nerves were intact. No sensory changes were

demonstrable. No significant alteration of his reflexes was observed.

LABORATORY AND ROENTCENOCRAPHIC

FIND-INGS: The cerebrospinal fluid was under a pres-sure of 150 mm of water. The cytologic and chemical findings in the cerebrospinal fluid were unaltered. The blood cell count,

urinaly-sis, and serologic test for syphilis were not

ab-normal.

Roentgenograms of the skull and the pneu-moencephaboventricubogram disclosed nothing unusual. A ventricular estimation indicated no increase in intracranial pressure or in the size of the lateral ventricles.

COURSE: When discharged on December

14, 1943, the gait remained ataxic; the limbs were still in-co-ordinate, but the optic nerve heads appeared normal.

A

report from his school on December 19,

1944, stated that he was in the first grade, but

that he was apathetic, and had tremors of his hands which interfered with school work. He was reported as showing average ability on psychologic testing.

(4)

reported that the patient was an apparently healthy 13-year-old boy, and was doing well

in the sixth grade. A letter from the father March 4, 1957, stated that the gait became

normal after about 3 years (in 1946) and the tremors had ceased in 1947. He passed all his

grades in school, completing the ninth grade;

he is now quite normal and is in the Navy. There has been no similar illness since, and he has had no convulsions or other evidence of neurobogic disturbance.

Case 2

HIsToRY:

G.T.,

a 9-year-old white male, was

admitted to the Neurosurgical Service of the Hospital of the University of Pennsylvania on

September 22, 1946 because of a suspected

brain tumor. On September 1, 1946 he had

be-gun to vomit. This had recurred two or three

times daily for a week. On the second day of ill-ness, he had complained of sore throat and was

found to have fever. On September 7, he corn-plained of poor vision in the left eye; and by September 1 1, he was complaining of a

simi-bar difficulty in the right eye and pain in the

left eye. He was admitted to a local hospital on September 12, 1946, where a lumbar punc-ture revealed the cerebrospinab fluid pressure to be 280 mm of water. Because of this and because of the presence of papilledema, he was transferred to the Hospital of the

Univer-sity of Pennsylvania although he was thought to be improving generally.

The only significant factor in the past medi-cal history was impaired hearing, which had first been noted when the child was in the

first grade.

PHYSICAL FINDINGS: Neurobogic

examina-tion at the time of admission revealed bilateral papilbedema, more marked in the right eye, with slight optic atrophy in the left eye, a wide-based and unsteady gait, dyssynergia in the use of his left hand, and bitemporal hemi-anopsia to gross confrontation.

LABORATORY FINDINGS: The concentration of hemoglobin was 6.9 gm/100 ml. There were 12,300 leukocytes/mm of blood. Con-centrations of urea nitrogen, sugar (fasting), and carbon dioxide in the blood, and of chloride and protein in the serum were all within nor-mal limits. Urinalysis was not abnormal. The

serologic test for syphilis was non-reactive.

The cerebrospinal fluid (September 27, 1946-from a ventricular tap) was not under increased pressure, contained 1,700 erythrocytes and 50

beukocytes/mm. The concentration of protein was 135 mg/100 ml. The serologic tests of the

cerebrospinal fluid for syphilis were

non-reac-tive.

ROENTGENOGRAPHIC FINDINGS :

Roentgeno-grams of the skull showed no evidence of in-creased intracranial pressure, and those of the chest revealed no abnormality. The

ventriculo-gram disclosed the lateral and third ventricles to be uniformly slightly dilated. The fourth ventricle and the aqueduct of Sybvius were not visualized.

OTHER STUDIES: An audiogram revealed no

hearing boss. The duration of nystagmus after

turning in the Barany chair was slightly in

excess of normal; yet, the vertigo was of av-erage duration. Both sets of vertical canals failed to respond to one minute of caloric stimulation. Both horizontal canals gave

irn-paired responses. These findings were inter-preted as suggesting a subtentorial lesion,

probably in the midline of the cerebellum.

The ophthalmologic consultant felt that the bitemporal heteronymous hemianopsia with

macubar loss was suggestive of a centrally

bo-cated lesion which was near and probably

posterior to the optic chiasm.

COURSE : After the ventricubogram had been

made on September 27, a suboccipital

craniec-tomy was performed. Careful exploration of the posterior fossa and its contents disclosed

no evidence of cerebellar tumor or of any block of the subarachnoid pathways or of the ventri-cular system.

The boy did well postoperatively. The papil-ledema subsided with some evidence of

resid-ual optic atrophy. The gait improved.

When seen on November 21, 1946, the

neu-rologic examination was within normal limits,

except that the optic discs were paler than usual. The suboccipital decompression was

soft. A follow-up better in July of 1951 con-tained no complaints. A letter March 4, 1957

stated that he is well, with no signs of any residual disturbance. There are no eye

svmp-toms, he does not wear glasses, and he is a

senior in high school. There has been no re-currence of any symptoms suggesting neuro-logic disturbance.

Case 3

HISTORY: S.B., a 17-month-old white

(5)

history, she awakened from a nap on March 6, 1948 and was noted to be “shaky.” She was especially unsteady when she tried to walk. The parents noted that when she was lying down, no shaking was present. The

tremulous-ness was at first confined to the lower

extremi-ties, but soon involved the trunk and head. There was no fever and no vomiting. Indeed, she did not even appear to be ill.

It was pointed out that she had had a mild

upper-respiratory infection, “a cold,” about 6 weeks previousb, but had recovered without difficulty from this illness.

PHYSICAL FINDINGS: An exammation done

by

one of us (G.A.S.) on March 11, 1948, re-veabed no papilledema, but there were some

nystagmoid movements of the eyeballs. She

was unable to walk unless supported, and even then showed marked ataxia. When she sat un-supported, there were tremors of the trunk and head. There was an action tremor of the upper extremities, but no dysmetria. There were no abnormal movements at rest. There were no weaknesses of muscles, no hypotonia, no re-flex abnormalities, and no sensory disturbances.

At the time of admission, March 12, 1948, the clinical findings were essentially as noted above.

LABORATORY FINDINGS: Examination of the

cerebrospinal fluid on March 15, 1948, dis-closed normal pressure and concentrations of protein, sugar and chlorides of 35 mg/100 ml,

87

mg/100 ml and 120 mEq/b, respectively. Serologic tests for syphilis were non-reactive.

A second examination on March 22, 1948, showed no cells, a concentration of protein of 28 mg/100 ml and a concentration of sugar of 64 mg/100 ml.

The blood cell count, urinalysis, serologic

tests of the blood for syphilis, and routine

chemical examination of the blood disclosed nothing abnormal. Roentgenograms of skull

and chest were not abnormal.

Virus studies (of serum and cerebrospinal fluid) were done by Dr. M. Michael Sigel at the Virus Diagnostic Research Laboratory of

the Children’s Hospital of Philadelphia.

Com-plement fixation tests revealed no serologic evidence of infection with the viruses of bymphocytic choriomeningitis, mumps or East-em equine encephabomyelitis. Intracerebral in-oculations of mice showed no evidence of virus in the cerebrospinal fluid.

An electroencephalogram made on March

24, 1948 disclosed a general level of activity

of 75 to 300 microvolts. Bilaterally symmetrical

trains of 3-4 cps. waves were most prominent from the central and parietal regions. A second test on April 7, 1948, showed the same fea-tures, but the voltage was lower and the 3-4 cps. waves were bess persistent. A third test

done on April 21, 1948, showed even bess

slow wave activity and the presence of other usual frequencies so that the record was con-sidered to be within the normal range for the age.

COURSE: The hospital course was

compli-cated by a feeding problem due to the severe

tremors and ataxia, considerable irritability and, at times, bow-grade fever. She remained in the hospital for 6 weeks; and at the time of discharge, she was weak and unable to sit alone, but tremors were less marked.

This patient was followed for several years. When last seen on April 3, 1952 (4 years after the onset of illness), she still needed a little support to walk, and had a markedly ataxic

gait with a steppage-like quality. There was still a lack of co-ordination of all movements. She was beginning to talk a little and had a bess

negativistic attitude.

The last electroencephalogram, made on March 18, 1950, was within normal limits for children sleeping with sedation.

In April, 1957, the family physician

in-formed us that she still has impaired speech and attends a school for retarded children.

Case4

HISTORY:

GA.,

a 10-year-old white boy, was

admitted to the Neurosurgical Service of the Hospital of the University of Pennsylvania on March 11, 1950. There was no family history of neurobogic disease; two siblings and four half-sibs were living and well. Two weeks be-fore admission, he complained of weakness and tingling of the left arm and hand. On March 4,

1950, a series of 10 convulsions occurred dur-ing a 5-hour period. The seizures were char-acterized by drowsiness, but not unconscious-ness, with opisthotonus. He did not respond during the % to 1 minute they lasted. He ex-perienced a similar episode the next day, and

this was followed by vomiting, fever, apathy, headache, and ataxia.

He was hospitalized elsewhere on March 5, 1950, with an oral temperature of 38.5#{176}C. There was nuchal rigidity and the Kemig test

(6)

lumbar puncture disclosed a cerebrospinal fluid pressure of 240 mm of water. It was stated that the cerebrospinal fluid contained 5 cells/mm’, the globulin test was “positive,” the concentra-tion of sugar was 85 mg//100 ml and that of chloride 660 mg/100 ml; culture was nega-tive, and the Kolmer serologic test for syphilis was non-reactive. The sedimentation rate was 20 mm in 1 hour.

Because of persistent vomiting and increas-ing drowsiness, he was transferred to the Chib-dren’s Hospital of Philadelphia on March 10,

1950. He was found to have left hemiparesis,

considerable ataxia, especially truncab ataxia, questionable early papilledema in the right eye, alid horizontal coarse nystagmus with the (1uick component to the left. Lethargy in-creased and the temperature remained moder-ately elevated. Because of the apparent progres-sion of the illness, he was transferred to the Hospital of the University of Pennsylvania on March 11, 1950.

PHYSICAL FINDINGS: At the time of admission,

the blood pressure was 130/90, the pulse rate 120/mi: and rectal temperature 38.5#{176}C. He was somnolent and, at times, restless. He could

be

aroused, but did not respond well to ques-tions. The pupils were large, equal, and reacted sluggishly to light. There was a slow horizontal nvstagmus to the left. Early papilledema was noted on the right. Brudzinski and Kernig signs were present. There was truncal ataxia and left hemiparesis with hperreflexia.

LABORATORY STUDIES AND CoutiSE: A

yen-tricubogram was made on March 13, 1950. The ventricular system was not abnormal. The ventricular fluid was tinder normal pressure and contained no cells, concentrations of pro-tern and sugar of 22 and 72 mg/100 ml, re-spectively.

Clinical improvement followed the ventric-ulogram. A lumbar puncture done on March

12, 1950, revealed a pressure of 60 mm of water. The cerebrospinal fluid was clear and colorless, and contained 6 leucocvtes/mm’.

At the time of discharge on March 28, 1950, the boy was alert, responsive, and fully ori-ented. He was able to walk without any ab-normality of gait. The neurologic status was not abnormal.

He was readmitted to the hospital on May 30, 1952 at the age of 12 years, because of con-tinued somnolence and right-sided weakness

for 24 hours after the cessation of convulsions.

On May 28, 1952, right-sided seizures had begun, but had soon become generalized and were accompanied by vomiting. Seizures oc-curred about every 10 to 15 minutes through-out the night. A previous seizure was said to have occurred with vomiting in November, 1951.

At the time of admission, he was drowsy and had right hemiparesis with generalized hporeflexia and absent gag reflex.

Laboratory studies of blood, urine and cere-brospinal fluid revealed nothing of significance. Roentgenograms of chest and skull were with-in normal limits. A pneumoencephalogram showed slight dilation of the entire ventricular system. These changes were interpreted as representing a basal arachnoiditis associated with some brain atrophy.

An ophthalmologic consultation disclosed anisocoria, 1)ilateral reduction of corneal sensi-tivity, and “insufficiency type” of nvstagmus with bilateral absence of opticokinetic re-sponse. Barany and caloric tests of the vesti-bubbar apparatus gave responses that were within normal limits. A psychiatric consultant felt that there was evidence of mental retarda-tion and schizophrenia, possibly secondar to previous encephalitis. It was felt that some

type of special school might be required.

At the time of discharge from the hospital

on June 11, 1952, he was more alert and co-operative. There was no neurobogic deficit.

He was to take anticonvulsive medication

(diphenlhydantoin sodium and

phenobar-bital).

A series of convulsions were said to have

occurred on July 10, 1952, and were followed

by

an inability to walk for 2 to 3 weeks. When seen on September 24, 1952, he exhibited some nvstagmus and mild bilateral disability in doing the finger-to-nose test.Otherwise, the

neurobogic examination was within normal

limits. It was felt that an unusual home situa-tion was probably contributing somewhat to the continuing maladjustment. He was ad-mitted to Trenton State Hospital October 23, 1952, and a diagnosis was made of chronic

brain syndrome associated with intracranial

infection other than syphilis with psychotic reaction (basal ganglion disease). He expired on November 5, 1954. The causes of death were recorded as bronchopneumonia and basal

(7)

Case

5

HIsToRY:

O.L.,

an 11-year-old white boy,

was admitted to the Neurosurgical Service of

the Hospital of the Universit of Pennsylvania on April 9, 1951.

Ten days previousl’ he had become nause-ated and vomited. The oral temperature was 38.9#{176}C. On the following day, he continued

to vomit and began to complain of frontal

headache and transitory double vision. Four days after the appearance of the initial svmp-toms, he was allowed out of bed, and was

found to be unsteady on his feet. He was

noted to be very weak, especially in his right extremities.

PHYSICAL FINDINGS: At the time of

admis-sion the rectal temperature was 38#{176}C. The general physical examination disclosed nothing of significance. The neurologic examination re-vealed questionable blurring of the nasal mar-gin of the right disc. There was coarse

hori-zontal nvstagmus On lateral gaze with the slow

component to the left. Slight weakness of the

right lower facial muscles and slight weakness

of the right extremities were noted. The tendon reflexes seemed to be slightl\? more active on

the right. The gait was characterized by

cir-cumduction of the right lower extremits’ and slight ataxia. He swayed in the Romberg po-sition. There was dysmetria, adiadokokinesia, and dssvnergia of the extremities, more marked on the right side.

LABORATORY FINDINGS: The blood cell count, routine chemical analyses of the blood, serologic test for syphilis, and urinalysis were not al)Ilormal. The Wassermann and colloidal mastic tests of the cerebrospinal fluid were

nor-mal.

OTHER STUDIES AND COURSE: Tests of the

vestibular mechanism revealed normal re-sponses to turning. The right vertical canals showed no response after 3 minutes of caloric stimulation; while the right horizontal canal and all of the left canals responded. Hearing was good in both ears.

Electroencephalogram revealed a

well-de-veloped alpha pattern of 10 cycles/see, and occasional normal beta rhythm. Three cps.

slow waves were seen from the left central, temporal, and parietal leads.

The patient’s course in the hospital was characterized by a fluctuating mental status, ranging from somnolence to unconsciousness,

and a marked bradycardia at one point.

How-ever, ventricular taps never revealed any in-crease in intraventricular pressure.

The ventricubogram showed the lateral yen-tricules to he symmetrical and slightly

en-barged, as was the third ventricle. The aque-duct of Svlvius and the fourth ventricle were

riot visualized.

After ventricubographv, clinical

improve-ment began. When discharged from the hos-pital on April 17, 1951, nystagmus was less

marked, but he still had signs of bilateral

cerebellar deficit and right hemiparesis.

Follow-up examination on June 8, 1951, demonstrated continued awkwardness in the use of the right upper extremity. On May 15,

1952, mild right hemiparesis and awkwardness

persisted. The left side of the face was said to appear flatter than the right in repose. On November 21, 1952, he still had a slight right hemiparesis, but he walked well and used the right upper extremity quite satisfactorily. He attended the eighth grade and was reported to

be somewhat irritable and restless in school. Our most recent information was received

in a letter from the referring physician on April 2, 1957. The boy had not graduated from high school, but had stopped to go to work,

and is working regularly. There was no

descrip-tion of the neurobogic status.

Case 6

HIsToRY: M.S., a 21-month-old white

fe-male, was first seen by one of us (G.A.S.) on January 22, 1953, because of inability to walk unaided and the presence of tremors of the upper extremities.

On January 3, 1953, she began to vomit and developed fever. These were said to have been

controlled by the administration of penicillin and dimenhvdrinate. However, soon after this, a tremor was noted. She was clumsy and spilled things, especially with the left hand. She “mis-judged distances,” “staggered,” and often fell.

Because the symptoms persisted, she was hospitalized locally on January 13, 1953. Blood

cell count and sedimentation rate were said

to be normal. The cerebrospinal fluid was not under increased pressure and contained 12 cells/mm3; the concentrations of protein, sugar

and chloride were 30 mg/100 ml, 60 mg/100 ml and 123 mEq/l, respectively. Serologic tests for syphilis were non-reactive.

(8)

She was discharged from the hospital unim-proved on January 16, 1953. She continued to have intermittent vomiting; walking became more unsteady. She slept more and was more irritable.

PHYSICAL FINDINGS : The neurobogic exami-nation on January 22, 1953, showed the cranial

nerves to be intact. No definite muscular weak-ness was noted. She could walk only with sup-port and even then had a markedly ataxic gait with truncal tremor. In a sitting position, the

truncal ataxia was less marked, and the bower limbs could be held outstretched quite well.

There was an action tremor of the left upper

extremity. No sensory evaluation could be

done. The tendon and periosteal reflexes were

active and equal in the upper extremities. The

patebbar reflex was a little more active on the left than the right, and the left Babinski sign

was equivocal.

COURSE AND LABORATORY FINDINGS: When

seen again on February 19, 1953, the parents

felt that she had improved, for she could use her hands better and now attempted to stand up alone in her crib. She was irritable and

had temper tantrums. The neurobogic

exami-nation revealed a terminal tremor and in-co-ordination of the left upper extremity, truncal ataxia in the sitting position, and an ataxic

gait requiring support to walk. There was an

element suggestive of extensor thrust in her

gait.

On February 25, 1953, roentgenogram of the skull showed no evidence of increased

intra-cranial pressure. Funduscopic examination

showed the optic discs to be of normal color

and outline. The cerebrospinal fluid was clear and colorless and contained 1 lymphocyte/mm3

and a concentration of protein of 10 mg/100 ml. Examination on March 12, 1953, found her

standing and walking alone, but with a wide-based, ataxic gait. There was still past-pointing of the left upper extremity and difficulty in

picking up small objects with this extremity.

She was able to stoop over to pick up objects

from the floor without falling.

By April 30, 1953, she was much improved.

She walked alone, but was still a little unsteady

when standing. She was able to pick up

ob-jects without trouble, and there was no ataxia

on reaching for objects. There was no

nystag-mus. The tendon and periosteab reflexes were active and equal, except that the left pateblar

reflex was a bit more active than the right. She

was no longer irritable, and she did not have temper tantrums. Subsequent reports from the family indicated that she made a complete re-covery. The mother informed us on June 6, 1957 that she is active and energetic, able to use her hands well, rides a bicycle, climbs trees, et cetera. She was “slow” in beginning

to speak but speech is clear. Some

over-de-pendence on her mother is attributed to the fact that her father died quite suddenly several months ago.

Case 7

HISTORY:

M.C.,

a 23k-year-old Negro boy, was admitted to the Neurosurgical Service of

the Hospital of the University of Pennsylvania

on August 28, 1953. Four days prior to ad-mission, it was first noted that he staggered when he walked. This gait disturbance wors-ened. On the second day of illness, he began

to vomit and this continued at varying

inter-vals until admission to the hospital. He had

no fever, and did not seem to have headache or other pain. He was irritable and somnolent on the day of admission.

There was no history of a preceding infec-lion, of contact with a contagious disease, or of trauma.

PHYSICAL FINDINGS: Other than a rectal temperature of 38#{176}Cthe general physical ex-amination was within normal limits at the time

of admission to the hospital. The neurobogic examination showed him to be very irritable.

He screamed when disturbed, but would go to

sleep readily when quieted by his mother. He recognized his mother and spoke appropriately

to her with no apparent speech impairment.

There were no Kernig or Brudzinski signs.

He was unable to walk without support and tended to fall more often toward the left.

There was no muscle weakness nor sensory disturbances. The tendon reflexes were equally active. There were no pathologic reflexes. The cranial nerves were intact.

LABORATORY FINDINGS: The concentration

of hemoglobin was 61% of normal and the

leu-cocyte count 10,000/mm3. Urinalysis was not abnormal. Serologic tests for syphilis were non-reactive.

Roentgenograms of skull, chest, right tibia, and left radius revealed nothing abnormal.

(9)

abnormalities of the ventricles were noted.

The cerebrospinab fluid contained 44

erythro-cytes and 1 lymphocyte/mm3, and the

con-centration of protein was 24 mg/100 ml. No bacteria were seen or grown on culture media.

COURSE : Within the next few days, an action

tremor of the upper extremities was noted,

more marked on the left side. He continued to fall more often to the left. Questionable

Babinski signs developed. He remained

irrita-ble and the reflexes became hpo-active. Five days after admission to the hospital, he would not even attempt to stand. He was

even unsteady in a sitting position, tended to lean forward, and the hands were ataxic. He preferred to lie quietly on either side. His re-flexes were hypo-active, and there were no pathologic reflexes.

On the ninth day after admission, he was

more cheerful and co-operative. He could stand alone but was hesitant about walking. The gait was uncertain and wide-based. In walking, he

tended to deviate more often to the left.

By the fifteenth day after admission, he was markedly improved. He walked alone. The gait was uncertain, but without true ataxia.

There was no action tremor or ataxia of the

upper extremities. The reflexes were now quite

active and equal.

He was discharged from the hospital on September 11, 1953, 18 days after the onset of

his illness. When seen in the Neurology Clinic on October 30, 1953, there were no residual signs or symptoms. Our last contact with the family in February, 1957, revealed the boy to be a normal and active child with no evi-dence of retardation, and no seizures. There

has been no recurrence of symptoms.

Case

8

HISTORY: J.D., a 4-year-old white male, was admitted to the Pediatric Service of Dr. Harold Medoff in the Hospital of the University of

Pennsylvania on August 12, 1954, because of

the onset of staggering gait the day previously. Approximately 1 week before, he had had a sore throat with slight fever. Otherwise, he had been in good health.

On August 11, 1954, about 3 P.M., he was given a new puppy, and was quite excited.

Some minutes later, he was first noted to

stag-ger when he walked. His appetite at dinner

was good. The next morning he seemed to “shiver all over,” and then vomited after

drink-ing orange juice. He had little interest in his surroundings. There was no complaint of

head-ache or other pain.

There was no history of recent exanthem or exposure to contagious disease. There had been no trauma.

PHYSICAL FINDINGS: At the time of

admis-sion the general physical examination was es-sentially negative. The rectal temperature was 37.2#{176}C. Neurologic examination revealed a

child who preferred to lie quietly in bed, who did not object to being moved, and was fairly co-operative, but who would not talk very much. He was unable to sit up without sup-port. There was marked swaying of the trunk

and head. He was unwilling to stand or walk

alone, but with reassurance and support, he took a few unsteady steps with marked truncal ataxia. There was dysmetria and dysdiadokoki-nesia of the upper and lower extremities, more

marked on the right side.

There was no apparent disturbance of

speech, nystagmus or papilledema. The func-tions of the other cranial nerves seemed intact.

There were no abnormal involuntary

move-ments. There was no disturbance of muscle

power, tone or volume. There were no sensory

disturbances. The tendon reflexes were slightly

hyperactive, particularly the biceps and

Achil-les reflexes. The plantar reflexes were predomi-nately extensor. There was no evidence of

meningeal irritation.

LABORATORY FINDINGS:

The

concentration of hemoglobin was 82% of normal. The

leuko-cyte count was 3700/mm3, of which 36% were neutrophiles and 64% lymphocytes. Urinalysis was within normal limits. Heterophile

anti-bodies were not present in significant concen-trations. The cerebrospinal fluid was under a

pressure of 160 mm of water. It was clear and

colorless, and contained many erythrocytes and

11 bvmphocvtes/mm. Concentrations of sugar, protein and chlorides were 60 mg/100 ml, 32 mg/100 ml and 124 mEq/l, respectively. Tu-berculin skin test was negative.

Virus could not be demonstrated from fecal and nasal swabs as tested by tissue culture inoculation.

Roentgenograms of the skull showed slight exaggeration of the digital markings on the in-ner table of the cranium and a slight widening of the coronal sutures, but there were no intra-cranial calcifications and the hypophyseal fossa

(10)

inter-preted as early indications of increased intra-cranial pressure.

CouRSE: The patient began to improve on

the fourth day of hospitalization. He became

more alert and talkative. First, there was im-provement in the use of the upper extremities.

The disturbance of gait and truncal ataxia cleared last.

By August 20, 1954, he was walking about the ward unaided, but with obvious ataxia.

When discharged from the hospital on August

26, slight ataxia was still present. He received

no therapy during the hospitalization.

Slight ataxia of gait persisted about 1 week after discharge.

He has been followed by his pediatrician,

and the only subsequent illness has been an episode of bronchitis in January, 1955. There

have been no sequelae of the illness in the form of personality changes or other

disturb-ances.

When last seen on January 16, 1957, he was

6 years old, and doing well in the first grade

at school.

The neurobogic examination was within

nor-mal limits.

Case 9

HISToRY: D.M., a 3-year-old white female was admitted to the Pediatric Service of Dr. David Baker of the Hospital of the University of Pennsylvania on September 14, 1956, be-cause of shaking of the lower extremities. She

had had rhinorrhea, slight cough and fever 2

days previously, and had been restless during

the early morning of September 13. When she awoke from sleep, uncontrollable shaking movements of the lower extremities were

noted. When examined by her pediatrician 15 to 20 minutes later, she was semi-stuporous, but responded. The eves deviated upward and to the right. There was some injection of the conjunctivae and nasopharvnx. There were no

meningeal signs or other abnormal neurobogic findings. Sedatives and antibiotics were

ad-ministered.

Three subsequent episodes of shaking oc-curred with deviation of the eyes of almost one minute’s duration. She was drowsy most of the day and vomited twice.

When examined about midnight on

Septem-ber 14, she showed essentially the same find-ings as first noted. Rectal temperature was

37.8#{176}C,abdominal reflexes were absent, and

a Babinski sign was present on the left, and was questionable on the right.

She was admitted to the Hospital of the Uni-versitv of Pennsylvania for studs’. In the

his-tory, it was noted that there were two para-keets at home which were well, and a dog who limped and had been drowsy for a few days.

PHYSICAL FINDINGS: At the time of adniis-sion the rectal temperature was 37.9#{176}C, the pulse rate 120/mm and the respirator rate 20/mm. The patient slept throughout the ex-amination (she had been sedated). There was grinding of the teeth and some lacerations of the buccal mucosa were present. On the left lateral portion of the tongue there was a small ulcerated area with a vesicle. There was slight lvmphadenopathv of the neck. Lichenification of the antecubital area was attributed to allergy

to milk.

Neurobogic examination showed a drowsy, sedated child who responded to stimuli, with hyperactive tendon reflexes of the lower limbs bilaterally, bilateral Babiuski signs, and no

abdominal reflexes.

During the first day in the hospital, she had two seizures and was somnolent. She was noted to sit iii an unstable manner, refusing to stand or walk; hand movements were awk-ward, but no involuntary movements were noted. Deep tendon reflexes were diminished

or absent bilaterally, with definite Babinski

sign on the right, questionable on the left.

The dosage of phenobarbital was reduced.

The next morning the patient was hyperactive, and seemed euphoric. On neurobogic examina-tion, however, she was alert, attentive, and fairly co-operative. There was no papilledema

or other impairment of the cranial nerves. She

moved all extremities vell with no evidence of weakness or impaired tone. She stood with a broad base, and walked with an ataxic gait. There was some ataxia of the upper extremi-ties, especially the left. There was no truncal

ataxia when sitting. Tendon and periosteal re-flexes were present, and equally active bilater-ally; abdominal reflexes were present. She had a suggestive Babinski sign on the right.

LABORATORY FINDINGS: The concentration

of hemoglobin and the leukocvte count and differential count were within the normal

range on several occasions. The concentrations

of urea nitrogen, sugar, calcium, phosphorus,

(11)

carbon dioxide were normal. The concentra-tion of lead in the blood on September 19 was 3.0 p.g/gm of hemoglobin. The cerebrospinal fluid findings were not abnormal: The fluid was under increased pressure (200 mm of water) but the child was crying; it was clear and colorless with 1 leukocyte/mm3; concen-trations of protein, sugar and chloride were 20 mg/100 ml, 87 mg/100 ml and 115 mEq/b, respectively; there was no growth on culture. The Kolmer and colloidal mastic tests were negative. Tuberculin (O.T.) test was negative.

There was no evidence of virus in the

cere-brospinal fluid by inoculation of Hela, done

by

the Virus Diagnostic Research Laboratory of Children’s Hospital. Other virus studies showed no serologic evidence of infection caused by lymphocytic choriomeningitis, mumps, psittacosis, or bymphogranuboma yen-ereum. Viral studies were negative for influ-enza A and B,

Q

fever, adeno-pharyngeab-con-junctivitis, and coxsackie B; complement fixa-tion studies were negative for poliomyelitis,

types I, II, and III. Complement fixation was

negative for herpes simplex, and the cold ag-glutination titer was 1:40.

Roentgenograms of the skull showed no abnormal calcifications; those of the long bones revealed no lead line.

An electroencephalogram made on Septem-ber 17 revealed no evidence of cortical ab-normality. A second examination on October

9 also revealed no definite abnormality. The record was felt to indicate that the patient was approximately 1 year behind the average ma-turation schedule.

COURSE: The subsequent course in the hos-pital was characterized by continued seizures which were poorly controlled by administra-tion of phenobarbital and later chborab hydrate. Paraldehvde seemed to give most effective con-trol of the seizures.

The ataxia became more severe by Septem-ber 24, at which time the neurobogic examina-tion revealed ataxia of the trunk and of all ex-tremities. The truncal ataxia was present when sitting as well as when standing. There was dvsmetria on reaching for objects, especially with the right hand. There was no nystagmus. The reflexes were depressed with no pathologic reflexes. Speech was limited but intelligible. She seemed slow in responses.

Ophthabmoscopic examination on September 20 showed a coboboma in the right eye

involv-ing the inferior portion of the disc, one disc diopter below the inferior pole.

She was still ataxic when examined on Oc-tober 2 but there had been no seizures after September 28.

By October 6, when she had had no seizures for 7 days, the dosage of phenobarbital was decreased and later discontinued. She was alert at that time, and bess in-co-ordinated. There was some evidence of emotional insta-bility with thrashing of the arms and throwing of objects.

Several seizures occurred on October 1 1 and 12 and administration of phenobarbital and diphenylhydantoin was restarted.

At the time of discharge from the hospital

on October 19, 1956, she had become symp-tom-free, but continued to receive diphenyl-hvdantoin and phenobarbitab.

She has been followed by her pediatrician and was seen for follow-up neurobogic exami-nation on February 25, 1957, 4 months after discharge.

The mother felt that the child was no longer

ataxic, and stated that there had been no change in intellectual achievement or per-sonality; the speech was clear and she was adding more and more words. There had been

no more seizures, but she was still taking

phe-nobarbital, 15 mg, three times daily, and diphenylhydantoin, 50 mg, twice daily.

On neurologic examination, she was bright,

alert and fairly co-operative. Speech was clear and distinct. There was no weakness; reflexes were active and equal bilaterally, with no ab-normal reflexes. There was no sensory disturb-ance, dysmetria or past-pointing, but there was a slight ataxia of gait especially on turning, somewhat wide-based. There was no nystag-mus. The cranial nerves were normal.

ANALYSIS

OF

CASES

Of the nine patients, six were males. The majority of the patients were 5 years of age or younger. Only one patient was more than 11 years of age.

Four of the patients had onset of illness

in the months from January through March, and five began in the months from August to October. While this is indeed too small a

group from which to draw conclusions,

some significance to these times of onset

(12)

dis-orders of childhood are due to different

sea-sonally-prominent pathogens.

Only a few of the patients gave a history

suggesting a preceding infection. None

were exposed to known contagious

dis-eases. As a matter of fact, the children were

quite healthy when the acute ataxia began.

None of them had a history of head trauma. The most common early symptoms were “staggering gait” and vomiting. Seizures occurred in two patients. Action tremors of

the extremities, trunk or head were often noted with associated awkwardness. In four

of the patients, the early symptoms

sug-gested involvement of other portions of the

central nervous system as well as the cere-bebbum. Fever was present in five.

Seven of the patients had some degree of

disturbance of personality and state of con-sciousness as part of the illness. Such

changes as irritability, moroseness, or nega-tivism were seen, while the sensorium

varied from somnolence to unconsciousness.

Residual disturbances in behavior were

reported in four of the children. In two of these, this remaining effect was quite ex-tensive: Simple mental retardation in one, and a schizoid personality change

requir-ing institutionalization in the other. Yet in two, only mild apathy and irritability at school were reported.

Five patients made a complete recovery, sometimes within a few weeks after the

on-set of the illness. However, persistent cere-bellar deficits and residua of cerebral

de-struction in three of the patients attest to the fact that the possibility of continued morbidity in some of these patients must never be forgotten. The three patients who

fared so badly had the onset of the illness

in the winter.

The neurobogic sign of most clinical

sig-nificance was the ataxic gait. This occurred in varying degrees of severity in eight of

the children. Truncal ataxia, action tremor,

and dysmetria were also frequently noted.

But

nystagmus,

hypotonia

and

hypo-reflexia were infrequently encountered.

Choreiform movements, and hemiparesis

with hyperreflexia occurred in a few

in-stances and indicated that the pathologic

process was by no means limited to the cerebellum in some of the patients.

The most pressing diagnostic problems

presented were the degree of increased intracranial pressure and the possibility of a posterior fossa neoplasm. In four of the patients, changes of the optic nerve heads

suggestive of increased intracranial pres-sure were noted; two had bilateral early

evidence of papilledema; two others had

blurring of one optic nerve head. Also, in two cases, lumbar punctures done in other

hospitals revealed increased pressure of the

cerebrospinal fluid (280 mm and 240 mm of water). The indications were such that

ventricular tap and ventricubography were performed in five of the nine cases. In none

was the intraventricular pressure increased,

despite other signs suggesting increased

in-tracranial pressure. In one case, there seemed to be progression of the illness and the ventricubogram showed a slight dilation of the lateral and third ventricles. A sub-occiptal craniectomy was performed on this

child, and exploration of the posterior fossa

disclosed no cause for the findings. He made

a complete functional recovery.

DISCUSSION

As can be seen from these cases and those

previously reported in the literature, infants and children can develop an acute ataxia in which the signs suggest a disorder

pre-dominantly of the cerebellum. There occurs

a markedly ataxic gait, truncal and head ataxia, action tremor, and in-co-or(lination

of the extremities, nystagmus, and scanning

speech. Some of the children have also had involvement of the cerebrum, brain stem and/or spinal cord, but in these cases the predominant dysfunction has always been

due to the cerebellar deficit. We have been impressed in our cases and in those

re-ported in the literature by the lack of fever,

the often normal cerebrospinal fluid, and

the rapid and complete spontaneous

re-covery of many of these children. Yet it

must be stated that this is not altogether

(13)

and, particularly, residual behavior dis-orders, have been seen. In general, the

prognosis is felt to be favorable.

It would seem to us that it is important

to distinguish between the acute cerebellar ataxias which occur during or following certain of the infectious and contagious diseases (measles, chicken pox, mumps, pertussis, scarlet fever, diphtheria, and typhoid fever) and those which have, as yet, no certain etiology. The prognosis for recovery seems to be more favorable in the latter. While early observers tended to as-sign the etiology of these unknown cases to “influenza” and “epidemic encephalitis,”

there has been a tendency recently to book toward viruses, as the poliomyelitis virus,

as possible etiologic agents. With the ad-vent of tissue culture techniques, cases like those reported here might well have suc-cessive serologic titers determined

through-out the course of the illness. Such studies

by Glaser, Stark, and Godennel6 have

mdi-cated the vast clinical panorama possibly attributable to the pobiomyelitis virus.

Future serologic studies of acute cerebeblar disorders in children may well reveal many

different causative agents.

The cerebellum is generally a vulnerable

division of the nervous system.1’ Various toxins or noxious states have been described

as damaging portions of this structure. For instance, heat hyperpyrexia or “heat stroke”

can cause extensive degeneration of the cerebebbum.’8”#{176} Alcohol, bead, and many un-related organic toxins can damage the Purkinje cell bayer of the cerebellum.20 Magnesium-deficient diets have been re-ported as damaging the Purkinje cells in

chickens, rats, and rabbits.2’ Chboropheno-thane can chiefly injure the cerebebbum.2223 Ingestion of agenized protein has been said to affect the cerebelbum.24 Carcinoma with-out metastases has been shown to damage

the Purkinje cells of the cerebellum.25 While

this peculiar delicacy of the cerebellar corti-cal cells and of the intrinsic nuclei of the

cerebellum is not specifically applicable to

the present problem of etiology of the acute

cerebellar disorders of childhood, it must

be emphasized that infections are not the

only factors that need to be considered in

these cases. Acute demyelinizing processes, as multiple sclerosis with cerebellar

involve-ment, are known to occur in childhood as well as in adults. Various therapeutic

agents,

notably

barbiturates and

diphenyl-hydantomn, cause ataxia in toxic dosages.

Thus, since the cerebellum is such a

sus-ceptible structure and since many agents,

known and unknown, can damage it, atten-lion in future cases of acute cerebellar dis-order of childhood might well be directed

toward such various possibilities.

The major problem in the differential

diagnosis of this relatively benign condition is from one of the more common and too

often disastrous causes of cerebellar ataxia, the medulboblastoma. It is well recognized

that the tumor may manifest itself quite

abruptly and in the early stages there may

be fever.

In many of the cases reported here such a

possibility could not be excluded until

ventricular taps showed no increased intra-cranial pressure and no enlargement of the ventricles. The electroencephalogram was of little help. The one significant differential feature was the course of the illness.

In

the

cerebellar

medulloblastoma,

the

course is inevitably progressive with in-creasing papibledema or hydrocephalus. In acute idiopathic cerebeblar disorders, the

papilbedema does not increase; there is

usually no progression of signs, and in

several weeks the signs begin to recede.

Hence, in such cases, a period of careful,

watchful waiting would seem to be the best procedure, but ventricular tap and

yen-tricubography may be the methods of choice in settling the problem finally. Our limited

experience would suggest that this pro-cedure can be performed quite safely.

SUMMARY

The clinical reports of nine children who developed an acute cerebeblar disorder are

recorded. The etiology was not determined in any of the cases.

(14)

744

literature as “acute cerebellar encephalitis” or “acute cerebellar ataxia,” a form of

non-suppurative encephalitis.

The concept is developed that these cases

represent a toxi-infectious process of van-able etiology with the cerebellum or its sys-tems as the major or only target organ. The main significance of these relatively benign forms of cerebellar disorder in children is their differentiation from cerebellar tumor; the major features of the disorder are out-lined to help in the differential diagnosis.

ADDENDUM

Since the paper was prepared for pubbica-tion, Cottom reported seven cases of acute cerebelbar ataxia and analyzed these and 33 from the literature (Cottom, D.C. : Acute cerebellar ataxia. Arch. Dis. Childhood, 32: 181, 1957).

REFERENCES

1. Batten, F. E. : Ataxia in childhood. Brain, 28:484, 1905.

2. Griffith,

J.

P. C. : (a) Acute cerebellar ataxia in children: Report of a case with rapid and complete recovery. Am.

J.

M. Sc., 151:24, 1916; (b) Acute

cerebro-cerebellar ataxia. Am.

J.

Dis. Child., 20:82, 1920; (c) Acute cerebellar en-cephalitis (acute cerebeblar ataxia). Am.

J.

M. Sc., 162:781, 1921.

3. Elev, R. C.: Neurobogic conditions in in-fants and children.

J.

Pediat., 9:797, 1936.

4. Klingman, W. 0., and Hodges, R. G..: Acute ataxia of unknown origin in chil-dren.

J.

Pediat., 24:536, 1944.

5. Shanks, R. A.: Notes on cerebellar ataxia in childhood. Arch. Dis. Childhood, 25:389, 1950.

6. Cavallotti, A. M.: Un caso di atassia

cere-bebbare acuta. Minerva pediat., 2:510, 1950.

7. Martin,

J.

K.: The ataxic child. Canad.

M.A.J., 69:601, 1953.

8. Goldwyn, A., and Waldman, A. M.: Acute cerebelbar ataxia in children; a report of

three cases.

J.

Pediat., 42:75, 1953.

9. Thieffrev, S., Martin, C., and Arthuis, M.: L’ataxie c#{233}r#{233}belleuseaigu#{235}curable de

l’enfant. Arch. franc p#{233}diat., 10:14, 1953.

10. Dekker-Jonker, A.: Acute cerebrale tremor

en ataxie bij jonge kinderen. Nederl. tijdschr. v. geneesk., 92:2912, 1948. 11. James, T. : Acute ataxia of cerebeblar type

in children; report of a case with rapid and complete recovery. Glasgow M.

J.,

33:455, 1952.

12. Keller, M.

J.,

and Karelitz, S. : Acute ataxia in a twenty-month-old female.

PEDIATRICS, 1:754, 1948.

13. Beyer, P., and Eichler, A. : Ataxie aigu#{235} curable avec troubles du langage et du comportement chez un enfant de 5 aiis. Arch. franc. p#{233}diat., 10:765, 1953. 14. Berglund, G., Mossberg, H. 0., and

Rv-denstam, B.: Acute cerebellar ataxia in

children. Acta paediat., 44:254, 1955. 15. Rydenstam, B. : Some atypical cases of

polioencephalitis. Acta med. scandinav., 154:suppl. 316, p. 50, 1956.

16. Glaser, G. H., Stark, L., and Godenne,

M. 0. : The diagnosis of atypical polio-virus infection. Read before the Amen-can Neurological Association Meeting, June, 1956.

17. Freedman, D. A., and Rourke,

J.

C. : Se-lective sensitivity of the Purkinje cells of cerebellum. Am.

J.

Med., 17:861, 1954.

18. Freeman, W. , and Dumoff, E. : Cerebelbar syndrome following heat stroke. Arch. Neurol. & Psychiat., 51:67, 1944.

19. Kramer, L. : Lamellar atrophy of Purkinje cells following heat stroke. Arch. Neurol. & Psychiat., 61:441, 1949. 20. Lhermitte,

J.

: Cortical cerebellan

degen-enation. Proc. Roy. Soc. Med., 28:379, 1935.

21. Barrow, G. P., Brown, S. 0., and Pearson,

P. B. : Histological manifestations of a

magnesium deficiency in the rat and rabbit. Proc. Soc. Exper. Biol. & Med., 70:220, 1949.

22. Haymaken, W., Ginzler, A. M., and Fer-guson, R. L.: The toxic effects of

pro-longed ingestion of DDT on dogs with special reference to lesions in the brain.

Am.

J.

M. Sc., 212:423, 1946.

23. Onifer, T. M., and Whisnant,

J.

P.: Cere-belbar ataxia and neuronitis after ex-posure to DDT and lindane. Proc. Staff

Meet., Mayo Clin., 32:67, 1957.

24. Silver, M. L.: Carbon dioxide, cerebellum, chboramines and convulsions. Science,

108:685, 1948.

(15)

SUMMARIO

IN

INTERLINGUA

Acute

Ataxia

Cerebellar

in Juveniles

Es descnibite de novo le condition designate

per Batten como ataxia acute o encephalitis cerebellar de juveniles. Es presentate un breve

revista del casos de iste condition reportate in be curso del passate 50 annos. In be reportos plus ancian, be accento esseva placiate super be relation con previe 0 concomitante morbos infectiose, sed be occurrentia del condition in juveniles sin previe morbo esseva etiam notate. In be reportos plus recente, plus attention esseva prestate a investigationes virobogic con be objec-tivo de identificar un possibile etiologia virusab.

Le condition in question es vidite le plus

frequentemente in patientes de etates de infra 6 annos, sed iblo occurre etiam in juveniles de etates plus matur. Le complexo de svmptomas pare esser similissime in be casos con exanthema o altere infectiones e in be casos in que be patientes se trovava perfectemente ben al

tem-pore quando be ataxia se declarava. Tamen, in be secunde de iste gruppos, be prognose es apparentemente plus favorabibe.

Es reportate 9 casos. In be majoritate, be

etates del patientes esseva 5 annos o minus.

Sobmente paucos habeva historias que

sugge-reva be occurrentia de infectiones; nubbos, in tanto que cogrioscite, habeva essite exponite a morbos contagiose. Omnes se trovava ben al tempore del disveboppamento de ataxia o altere

manifestationes neurobogic. Le plus commun symptoma precoce esseva un ambulatura titu-bante e vomito. Tremores de action in be extremitates, be trunco, o be capite esseva notate frequentemente in association con vane grados

de malhabibitate. Accessos convulsive occurreva

in duo del patientes. Febre esseva presente in

cinque. Septe habeva un certe grado de dis-turbation characterobogic e in Ion stato de

conscietate. Cinque patientes se restabliva

compbetemente, a vices intra abicun septimanas

post le declaration del morbo. Un esseva ancora ataxic e mentalmente retardate 9 annos plus tarde. Un altere habeva probabibemente ancora tin leve deficit residue; post 7 menses ille manifestava un leve hemiparese.

Es disveboppate be concepto que iste casos repnesenta processos toxico-infectiose de vane etiobogias, con be cerebeblo como principal

organo de attacco. Es delineate be principal tractos que differentia iste condition ab tumores cenebellar.

THE TONSIL-ADENOIDECTOMY ENIGMA, Harry Bakwin.

(J.

Pediat., 52:339, March

1958.)

This extensive commentary on the literature concerning an age-old enigma de-serves the thoughtful consideration of every pediatrician. It is an analysis of the horrors and confusion which stilb prevail concerning the results of, and indications for, removal of the tonsils and adenoids in children. As it is written by a pediatrician, a suspicion of bias would he understandable, hut this will have to be demonstrated by cogent reasons from the advocates of the procedure who wish to argue for con-tinuance of its present widespread usage. Condemnation of the present employment of the operation is so complete that one is still left confused as to the proper

indica-tions for tonsillectomy, if any. Also left unexplained is the reason for the present situation after so many years of preaching of a conservative point of view. Will another expos#{233} bring improvement or must some more compelling tactic be em-ployed? The author states “The operation retains its vogue in the face of well-docu-mented evidence that, in the overwhelming majority of cases, it is useless.” This sets

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1958;21;731

Pediatrics

Geraldine King, Gabriel A. Schwarz and Harry W. Slade

ACUTE CEREBELLAR ATAXIA OF CHILDHOOD: Report of Nine Cases

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(17)

1958;21;731

Pediatrics

Geraldine King, Gabriel A. Schwarz and Harry W. Slade

ACUTE CEREBELLAR ATAXIA OF CHILDHOOD: Report of Nine Cases

http://pediatrics.aappublications.org/content/21/5/731

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American Academy of Pediatrics. All rights reserved. Print ISSN: 1073-0397.

References

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