- Email: [email protected]
Acute cerebellar ataxia in children
ACUTE C E R E B E L L A R A T A X I A IN C H I L D R E N A REPORT OF THREE CASES CAPTAIN ARTHUR ~rOLDWYN, MEDICAL CORPS, UNITED STATES ARMY, AIND A . MARTIN WALDMAN, M.D., N E w YORK,N . Y .
cerebellar ataxia in childA CUTE hood is a syndrome whose frequency has not been generally appreciated in pediatric literature. The typical clinical picture consists of an acute onset of falling, reeling, staggering gait, and imbalance in otherwise apparently healthy children, or in the course of acute infectious disease. Additional symptoms, such as intention tremor, nystagmus, ataxic speech, and disturbances of the sensorium, may also be present. The prognosis is generally favorable. Three cases in as many years were observed on the pediatric service of a single general hospital. Each patient made a rapid and complete recovery. Although Leyden ~ first described acute ataxias in 1869, it was Griffith,2 iu 1916, who directed attention to this syndrome in children in the American literature. He collected seventeen cases and reported a case of his own. In 1920 ~ and 19214 he described three new cases from his own practice, and ten others from the literature. The ages of these thirtyone children ranged from 31/2 to 12 years. Immediately preceding or concomitant infectious disease was present in twenty-four cases. The following disease association was noted: measles, 8; influenza, 5; typhoid fever, 4; scarlet fever, 2; pertussis, 2; diphtheria, 1; poliomylitis, 1; dysentery, From the Pediatric Service of Dr. Harry D. Pasachoff at the Morrisani~t City Hospital, New York City, and the iNew York Medical College.
1. Symptoms were often severe. Unconsciousness was present in twelve cases and disturbance of the sensorium, such as mania, delirium, or apathy, occurred in nineteen. Speech was affected in twenty-two cases. Nystagmus was noted in ten. Vomiting and convulsions were common. Griffith noted that practically none of his cases showed only cerebellar symptoms and he preferred labelling the syndrome "cerebrocerebellar ataxia." He concluded that symptoms were due to an acute encephalitis affecting predominantly the c e r e b e l l u m . No deaths attributable to the syndrome were encountered, b.ut only eight patients were completely recovered in less than six months and three additional patients were well in a year. The remainder made slow gains, or remained stationary, but none showed progression. One-fourth of the patients became mentally defective. Babonneix 5 recorded five new cases in 1928 in children ranging in age from 17 months to 3 years. Three cases began spontaneously without preceding illness. Enterocolitis occurred before the fourth case and lethargic encephalitis had been present in the fifth patient. Symptoms regressed in all cases. A spinal tap was performed on one patient. The fluid was found normal except for a slight increase in the albumin content. Babonneix described the European classification of infantile ataxias into two categories: Leyden's, or pri75
76
THE JOURNAL OF PEDIATRICS
mary type, and the Westphal type occurring after known infectious disease. In 1944 Klingman and Hodges 6 described seven cases seen in a period of three years. The children were 18 months to 4 89 years old. Ataxias developed spontaneously or after simple upper respiratory infections. Lumbar taps were performed on all patients. In only two cases were more than 10 cells per cubic millimeter present in the fluid. A pleocytosis of 20 mononuclear cells in one child, and 16 cells in another child, was found. Spinal fluid proteins ranged from 16 to 38.6 mg. per cent, and the sugar varied between 49 and 73 rag. per cent. The spinal fluid Wassermann, colloidal gold curve, and cultures were all negative. X-rays of the skull, tuberculin testing, and blood Wassermanns revealed no abnormalities. Blood serum demonstrated no neutralizing a n t i b o d i e s against viruses of St. Louis encephalitis, Eastern and Western equine encephalitis, louping ill, herpes simplex, a n d lymphocytic choriomeningitis. Two of their patients were completely well within three weeks. Of two more chronic patients, one who showed no improvement had a normal pneumoeneephalogram and electroencephalogram, and a patient requiring fourteen months for clearing of symptoms had moderate cerebral atrophy on air study. A case in a 20-month-old infant was reported by Keller and Karelitz 7 in 1948. Ataxia occurred without any preceding illness, and was associated with constant movements of the head, eyes, trunk, and extremities. A pill-rolling tremor of the hands was noted. The spinal fluid contained 100 fresh red blood cells per cubic
millimeter, 6 lymphocytes per cubic millimeter, and presented normal chemical, serological, and negative bacteriological findings. Symptoms persisted up to six months after the acute episode. Pneumoeneephalogram demonstrated slight cortical atrophy and the electroencephalogram showed diffuse activity with the greatest concentration in the mesencephalon. CASE REPORTS
CASE 1. (684548).--A 26- month-old white female child was admitted to the hospital on July 6, 1948, because of tremor of the hands and inability to walk without support for four days. Birth and developmental history were normal. The patient had previously walked well and without any difficulty. Rubeola was contracted at the age of 22 months and ran an uneventful course. Two weeks before admission to the hospital, the patient had varicella. The course was uncomplicated until four days prior to admission when the mother noted that the child had tremors of the hands and was unable to walk because of unsteadiness. On admission, the patient was alert, afebrile, and cheerful, but showed marked unsteadiness and staggering both on standing and attempting to walk. Ambulating with support, she maintained a wide base. The patient did not fall in any particular direction and did not have nystagmus. Paresis was not demonstrable, and the superficial and deep tendon reflexes were normal. There was no evidence of meningeal irritation, and no abnormal reflexes could be elicited. The cranial nerves functioned normally. A lumbar tap revealed a normal pressure and no cells. The proteins were 26 mg. per cent, the sugar 54 mg. per cent (blood sugar 98 rag. per cent), the chlorides 6.98 rag. per cent. The culture was sterile and serology was negative. A blood count showed 20,350 white blood cells: 54 per cent lymphocytes, 42 per cent polymorpho-
GOLDWYN AND W A L D M A N :
nuclears, 2 per cent monocytes, and 2 per cent eosinophils. A urine specimen showed no abnormalities. The patient improved rapidly without medication, and by J u l y 11, 1948, five days after hospital admission, signs of ataxia had disappeared. She was discharged two days later asymptomatic. CASE 2. (1250648).---A 3 89 old Negro girl was admitted to the hospital on Dec. 3, 1948, because of convulsions. She had been well until the previous day when she became ill with rhinitis, cough, and fever. Past history revealed that the patient was subject to convulsions on sudden elevation of temperature. On admission the seizure was noted to be mainly left-sided, clonic in nature, and was rapidly controlled by 11/2 grains of sodium phenobarbital. Physical examination revealed acute pharyngitis and bilateral catarrhal otitis media. No pathological neurological signs were found. Her temperature was 103.8 ~ F. Blood count showed hemoglobin 13 grams, red blood count 4,000,000, white blood count 5,600 with 52 per cent ]ymphocytes, 40 per cent polymorphonuclears, and 8 per cent monocytes. Urine analysis and blood serology were normal. The respiratory infection improved rapidly; the patient was treated with penicillin and aspirin, and she was afeb.rile in forty-eight hours. On the same day, the patient was found to be irritable and ataxic. She could neither sit nor stand without falling. Truncal ataxia was most pronounced. No nystagmus or physical findings of meningeal irritation were present. There were no paresis or pathological reflexes. Deep and superficial tendon reflexes were normal. No abflormality of the cranial nerves was found. A spinal tap at this time revealed clear fluid under normal pressure and no cells. The sugar was 66 rag. per cent, and the chlorides 726 rag. per cent. Culture was sterile. On Dec. 6, 1948, one day later, personality changes became manifest.
ACUTE CEREBELLAR ATAXIA
77
The child lay quietly in bed, inattentive, and apparently oblivious to her surroundings. She obeyed simple commands, but carried on no voluntary activity. Marked ataxia on attempting to stand or walk persisted. Another spinal tap was performed on the seventh hospital day. Clear fluid under normal pressure was obtained with 365 cells per cubic millimeter present. A differential count showed 60 per cent lymphocytes, 40 per cent polymorphonuclears. The proteins were 20 mg. per cent, the sugar 66 mg. per cent, and the chlorides 718 rag. per cent. Culture was sterile. Four days later, a lumbar tap revealed no cells in the fluid. The proteins were 18 mg. per cent, the sugar 75 rag. per cent, and the chlorides 701 mg. per cent. By December 16, the patient w~s more alert, and the ataxia was noted to have diminished. Gradual improvement took place thereafter, and by Jan. 5, 1949, the patient was asymptomatic. The clinical course of the illness was approximately one IIlonth.
CASE 3. (158752).--A 15-monthold Negro female was admitted Feb. 10, 1952, because of ataxia of five hours' duration. The child had had a normal birth and developmental course and had previously walked well. She had been asymptomatic until two days before when she developed a mild upper respiratory infection characterized by rhinitis and cough. The patient was afebrile. On awakening February 10, her mother noted that the child was unable to walk or maintain a sitting or standing position. She vomited once on the morning of admission. On admission the patient appeared well developed and well nourished, alert, afebrile, and in no distress. On being placed in a standing or sitting position, she swayed jerkily from side to side and quickly fell. The child ground her teeth intermittently. Physical examination revealed a mildly injected pharynx. No nystag-
78
THE
JOURNAL
mus or signs of meningeal irritation were noted. Pathological reflexes were absent. No muscle weakness was apparent. Deep tendon and superficial reflexes were equal and active. The cranial nerves were unimpaired. A spinal tap revealed clear fluid under normal pressure containing 90 cells per cubic millimeter; 85 per cent were mononuclear and 15 per cent polymorphonuclear. The proteins were 15 mg. per cent, the sugar 45 mg. per cent, and the chlorides 655 mg. per cent. Culture was sterile and serology was negative. A blood count showed hemoglobin 13 grams, white blood count 9,000 with a differential of 80 per cent polymorphonuclears and 20 per cent lymphocytes. A urinalysis was negative. The patient was placed on terramycin, 200 mg. every six hours. Her ataxia gradually cleared and she was asymptomatic ten days after hospital admission. She was afebrile during her entire hospital stay. On Februa r y 20 the spinal fluid contained 6 mononuclear cells per cubic millimeter. The proteins were 58 mg. per cent, the sugar 46 mg. per cent, and the chlorides 668 mg. per cent. COMMENT
In contrast to the early reports, our three cases represent a relatively isolated involvement of the cerebellure. Evidence of diffuse involvement of the brain, such as coma, fever, persistent vomiting, paresis, or paralysis, and signs of meningeal irritation, were notably absent. Cases 1 and 3 were completely asymptomatie except for the ataxia. Our second case presented convulsions on admission associated with a high temperature and an upper respiratory infection. This patient, however, had a history of previous febrile convulsions. Since ataxia did not develop until the child was asymptomatic two
OF P E D I A T R I C S
days later, with a normal spinal fluid, it may be assumed that the convulsions and fever were not specifically associated with the subsequent cerebellar clinical picture. As noted in the majority of previously reported cases, the ataxia in our children followed infectious disease; in one, varicella, and in two, upper respiratory infections had been present. Although intention tremor and nystagmus have been reported as common associated manifestations, neither of these signs was present in our three eases. Spinal fluid findings in previous reports have been normal, with only two patients demonstrating up to 20 cells per cubic millimeter. The more marked pleocytosis in two of our cases suggests an infectious etiology. It is interesting to note that in the second case the initial lumbar tap revealed no cells, while four days later 365 cells per cubic millimeter were present. Four days later all cells had disappeared. These findings suggest that several spinal taps may be indicated if pleocytosis is to be found in a larger number of cases. F u r t h e r viral studies may disclose a causative agent. Our three patients made a uniformly rapid and complete recovery, confirming the prevailing impression of a favorable outcome in the great majority of children afflicted with acute cerebellar ataxia. However, in only four previously reported cases in the American literature have all symptoms similarly cleared within one month. It is not believed that the use of terramycin in the third case altered the outcome in any way.
GOLDWYN AND ~,VALDMAN: SUMMARY
1. T h r e e cases of a c u t e c e r e b e l l a r a t a x i a in c h i l d r e n a r e r e p o r t e d . 2. T h e s y m p t o m s of t h i s c l i n i c a l s y n d r o m e a r e a n a c u t e onset of a t a x i a w h i c h m a y be a s s o c i a t e d w i t h n y s t a g mus, i n t e n t i o n t r e m o r , a n d c e r e b r a l manifestations. The syndrome may o c c u r s p o n t a n e o u s l y o r f o l l o w i n g inf e c t i o u s illness. T h e e t i o l o g y is unknown. 3. A n u n u s u a l f i n d i n g of a r e l a t i v e l y m a r k e d p l e o c y t o s i s in t h e s p i n a l fluid w a s n o t e d i n t w o of o u r cases. 4. P r o g n o s i s as to l i f e is g e n e r a l l y favorable. No p r o g r e s s i o n of cereb e l l a r s y m p t o m s has been s e e n ; however, c h r o n i c cases h a v e b e e n r e p o r t e d .
ACUTE CEREBELLAR ATAXIA
79
Our three patients made a complete recovery. The longest course was one m o n t h . REFERENCES 1. Leyden, E.: Verlangsamte motorische Leitung, Arch. f. Path., Anat. u. Physiol., u.f. kiln. Med. 46: 476, 1869. 2. Griffith, J. P. C.: Acute Cerebellar Ataxia in Children, Am. J. M. Sc. 151: 24, 1916. 3. Griffith, J. P. C.: Acute CerebrocerebelJar Ataxia, Am. J. Dis. Child. 20: 82, 1920. 4. Griffith, J. P. C.: Acute Cerebellar Ataxia, Am. J. M. Sc. 162: 781, 1921. 5. Babonneix, L.: Acute Infantile Ataxias, Riform~ reed. 44: 694, 1928. 6. Klingman, W. O., and Hodges, 1r G.: Acute Ataxia of Unknown Origin in Children, 5. PEDIAT. 24: 536, 1944. 7. Keller, M. 5., and Karelitz, S.: Acute Ataxia in a 20-Month-Old Female, Pediatrics 1: 756, 1948.
cerebellar ataxia in childA CUTE hood is a syndrome whose frequency has not been generally appreciated in pediatric literature. The typical clinical picture consists of an acute onset of falling, reeling, staggering gait, and imbalance in otherwise apparently healthy children, or in the course of acute infectious disease. Additional symptoms, such as intention tremor, nystagmus, ataxic speech, and disturbances of the sensorium, may also be present. The prognosis is generally favorable. Three cases in as many years were observed on the pediatric service of a single general hospital. Each patient made a rapid and complete recovery. Although Leyden ~ first described acute ataxias in 1869, it was Griffith,2 iu 1916, who directed attention to this syndrome in children in the American literature. He collected seventeen cases and reported a case of his own. In 1920 ~ and 19214 he described three new cases from his own practice, and ten others from the literature. The ages of these thirtyone children ranged from 31/2 to 12 years. Immediately preceding or concomitant infectious disease was present in twenty-four cases. The following disease association was noted: measles, 8; influenza, 5; typhoid fever, 4; scarlet fever, 2; pertussis, 2; diphtheria, 1; poliomylitis, 1; dysentery, From the Pediatric Service of Dr. Harry D. Pasachoff at the Morrisani~t City Hospital, New York City, and the iNew York Medical College.
1. Symptoms were often severe. Unconsciousness was present in twelve cases and disturbance of the sensorium, such as mania, delirium, or apathy, occurred in nineteen. Speech was affected in twenty-two cases. Nystagmus was noted in ten. Vomiting and convulsions were common. Griffith noted that practically none of his cases showed only cerebellar symptoms and he preferred labelling the syndrome "cerebrocerebellar ataxia." He concluded that symptoms were due to an acute encephalitis affecting predominantly the c e r e b e l l u m . No deaths attributable to the syndrome were encountered, b.ut only eight patients were completely recovered in less than six months and three additional patients were well in a year. The remainder made slow gains, or remained stationary, but none showed progression. One-fourth of the patients became mentally defective. Babonneix 5 recorded five new cases in 1928 in children ranging in age from 17 months to 3 years. Three cases began spontaneously without preceding illness. Enterocolitis occurred before the fourth case and lethargic encephalitis had been present in the fifth patient. Symptoms regressed in all cases. A spinal tap was performed on one patient. The fluid was found normal except for a slight increase in the albumin content. Babonneix described the European classification of infantile ataxias into two categories: Leyden's, or pri75
76
THE JOURNAL OF PEDIATRICS
mary type, and the Westphal type occurring after known infectious disease. In 1944 Klingman and Hodges 6 described seven cases seen in a period of three years. The children were 18 months to 4 89 years old. Ataxias developed spontaneously or after simple upper respiratory infections. Lumbar taps were performed on all patients. In only two cases were more than 10 cells per cubic millimeter present in the fluid. A pleocytosis of 20 mononuclear cells in one child, and 16 cells in another child, was found. Spinal fluid proteins ranged from 16 to 38.6 mg. per cent, and the sugar varied between 49 and 73 rag. per cent. The spinal fluid Wassermann, colloidal gold curve, and cultures were all negative. X-rays of the skull, tuberculin testing, and blood Wassermanns revealed no abnormalities. Blood serum demonstrated no neutralizing a n t i b o d i e s against viruses of St. Louis encephalitis, Eastern and Western equine encephalitis, louping ill, herpes simplex, a n d lymphocytic choriomeningitis. Two of their patients were completely well within three weeks. Of two more chronic patients, one who showed no improvement had a normal pneumoeneephalogram and electroencephalogram, and a patient requiring fourteen months for clearing of symptoms had moderate cerebral atrophy on air study. A case in a 20-month-old infant was reported by Keller and Karelitz 7 in 1948. Ataxia occurred without any preceding illness, and was associated with constant movements of the head, eyes, trunk, and extremities. A pill-rolling tremor of the hands was noted. The spinal fluid contained 100 fresh red blood cells per cubic
millimeter, 6 lymphocytes per cubic millimeter, and presented normal chemical, serological, and negative bacteriological findings. Symptoms persisted up to six months after the acute episode. Pneumoeneephalogram demonstrated slight cortical atrophy and the electroencephalogram showed diffuse activity with the greatest concentration in the mesencephalon. CASE REPORTS
CASE 1. (684548).--A 26- month-old white female child was admitted to the hospital on July 6, 1948, because of tremor of the hands and inability to walk without support for four days. Birth and developmental history were normal. The patient had previously walked well and without any difficulty. Rubeola was contracted at the age of 22 months and ran an uneventful course. Two weeks before admission to the hospital, the patient had varicella. The course was uncomplicated until four days prior to admission when the mother noted that the child had tremors of the hands and was unable to walk because of unsteadiness. On admission, the patient was alert, afebrile, and cheerful, but showed marked unsteadiness and staggering both on standing and attempting to walk. Ambulating with support, she maintained a wide base. The patient did not fall in any particular direction and did not have nystagmus. Paresis was not demonstrable, and the superficial and deep tendon reflexes were normal. There was no evidence of meningeal irritation, and no abnormal reflexes could be elicited. The cranial nerves functioned normally. A lumbar tap revealed a normal pressure and no cells. The proteins were 26 mg. per cent, the sugar 54 mg. per cent (blood sugar 98 rag. per cent), the chlorides 6.98 rag. per cent. The culture was sterile and serology was negative. A blood count showed 20,350 white blood cells: 54 per cent lymphocytes, 42 per cent polymorpho-
GOLDWYN AND W A L D M A N :
nuclears, 2 per cent monocytes, and 2 per cent eosinophils. A urine specimen showed no abnormalities. The patient improved rapidly without medication, and by J u l y 11, 1948, five days after hospital admission, signs of ataxia had disappeared. She was discharged two days later asymptomatic. CASE 2. (1250648).---A 3 89 old Negro girl was admitted to the hospital on Dec. 3, 1948, because of convulsions. She had been well until the previous day when she became ill with rhinitis, cough, and fever. Past history revealed that the patient was subject to convulsions on sudden elevation of temperature. On admission the seizure was noted to be mainly left-sided, clonic in nature, and was rapidly controlled by 11/2 grains of sodium phenobarbital. Physical examination revealed acute pharyngitis and bilateral catarrhal otitis media. No pathological neurological signs were found. Her temperature was 103.8 ~ F. Blood count showed hemoglobin 13 grams, red blood count 4,000,000, white blood count 5,600 with 52 per cent ]ymphocytes, 40 per cent polymorphonuclears, and 8 per cent monocytes. Urine analysis and blood serology were normal. The respiratory infection improved rapidly; the patient was treated with penicillin and aspirin, and she was afeb.rile in forty-eight hours. On the same day, the patient was found to be irritable and ataxic. She could neither sit nor stand without falling. Truncal ataxia was most pronounced. No nystagmus or physical findings of meningeal irritation were present. There were no paresis or pathological reflexes. Deep and superficial tendon reflexes were normal. No abflormality of the cranial nerves was found. A spinal tap at this time revealed clear fluid under normal pressure and no cells. The sugar was 66 rag. per cent, and the chlorides 726 rag. per cent. Culture was sterile. On Dec. 6, 1948, one day later, personality changes became manifest.
ACUTE CEREBELLAR ATAXIA
77
The child lay quietly in bed, inattentive, and apparently oblivious to her surroundings. She obeyed simple commands, but carried on no voluntary activity. Marked ataxia on attempting to stand or walk persisted. Another spinal tap was performed on the seventh hospital day. Clear fluid under normal pressure was obtained with 365 cells per cubic millimeter present. A differential count showed 60 per cent lymphocytes, 40 per cent polymorphonuclears. The proteins were 20 mg. per cent, the sugar 66 mg. per cent, and the chlorides 718 rag. per cent. Culture was sterile. Four days later, a lumbar tap revealed no cells in the fluid. The proteins were 18 mg. per cent, the sugar 75 rag. per cent, and the chlorides 701 mg. per cent. By December 16, the patient w~s more alert, and the ataxia was noted to have diminished. Gradual improvement took place thereafter, and by Jan. 5, 1949, the patient was asymptomatic. The clinical course of the illness was approximately one IIlonth.
CASE 3. (158752).--A 15-monthold Negro female was admitted Feb. 10, 1952, because of ataxia of five hours' duration. The child had had a normal birth and developmental course and had previously walked well. She had been asymptomatic until two days before when she developed a mild upper respiratory infection characterized by rhinitis and cough. The patient was afebrile. On awakening February 10, her mother noted that the child was unable to walk or maintain a sitting or standing position. She vomited once on the morning of admission. On admission the patient appeared well developed and well nourished, alert, afebrile, and in no distress. On being placed in a standing or sitting position, she swayed jerkily from side to side and quickly fell. The child ground her teeth intermittently. Physical examination revealed a mildly injected pharynx. No nystag-
78
THE
JOURNAL
mus or signs of meningeal irritation were noted. Pathological reflexes were absent. No muscle weakness was apparent. Deep tendon and superficial reflexes were equal and active. The cranial nerves were unimpaired. A spinal tap revealed clear fluid under normal pressure containing 90 cells per cubic millimeter; 85 per cent were mononuclear and 15 per cent polymorphonuclear. The proteins were 15 mg. per cent, the sugar 45 mg. per cent, and the chlorides 655 mg. per cent. Culture was sterile and serology was negative. A blood count showed hemoglobin 13 grams, white blood count 9,000 with a differential of 80 per cent polymorphonuclears and 20 per cent lymphocytes. A urinalysis was negative. The patient was placed on terramycin, 200 mg. every six hours. Her ataxia gradually cleared and she was asymptomatic ten days after hospital admission. She was afebrile during her entire hospital stay. On Februa r y 20 the spinal fluid contained 6 mononuclear cells per cubic millimeter. The proteins were 58 mg. per cent, the sugar 46 mg. per cent, and the chlorides 668 mg. per cent. COMMENT
In contrast to the early reports, our three cases represent a relatively isolated involvement of the cerebellure. Evidence of diffuse involvement of the brain, such as coma, fever, persistent vomiting, paresis, or paralysis, and signs of meningeal irritation, were notably absent. Cases 1 and 3 were completely asymptomatie except for the ataxia. Our second case presented convulsions on admission associated with a high temperature and an upper respiratory infection. This patient, however, had a history of previous febrile convulsions. Since ataxia did not develop until the child was asymptomatic two
OF P E D I A T R I C S
days later, with a normal spinal fluid, it may be assumed that the convulsions and fever were not specifically associated with the subsequent cerebellar clinical picture. As noted in the majority of previously reported cases, the ataxia in our children followed infectious disease; in one, varicella, and in two, upper respiratory infections had been present. Although intention tremor and nystagmus have been reported as common associated manifestations, neither of these signs was present in our three eases. Spinal fluid findings in previous reports have been normal, with only two patients demonstrating up to 20 cells per cubic millimeter. The more marked pleocytosis in two of our cases suggests an infectious etiology. It is interesting to note that in the second case the initial lumbar tap revealed no cells, while four days later 365 cells per cubic millimeter were present. Four days later all cells had disappeared. These findings suggest that several spinal taps may be indicated if pleocytosis is to be found in a larger number of cases. F u r t h e r viral studies may disclose a causative agent. Our three patients made a uniformly rapid and complete recovery, confirming the prevailing impression of a favorable outcome in the great majority of children afflicted with acute cerebellar ataxia. However, in only four previously reported cases in the American literature have all symptoms similarly cleared within one month. It is not believed that the use of terramycin in the third case altered the outcome in any way.
GOLDWYN AND ~,VALDMAN: SUMMARY
1. T h r e e cases of a c u t e c e r e b e l l a r a t a x i a in c h i l d r e n a r e r e p o r t e d . 2. T h e s y m p t o m s of t h i s c l i n i c a l s y n d r o m e a r e a n a c u t e onset of a t a x i a w h i c h m a y be a s s o c i a t e d w i t h n y s t a g mus, i n t e n t i o n t r e m o r , a n d c e r e b r a l manifestations. The syndrome may o c c u r s p o n t a n e o u s l y o r f o l l o w i n g inf e c t i o u s illness. T h e e t i o l o g y is unknown. 3. A n u n u s u a l f i n d i n g of a r e l a t i v e l y m a r k e d p l e o c y t o s i s in t h e s p i n a l fluid w a s n o t e d i n t w o of o u r cases. 4. P r o g n o s i s as to l i f e is g e n e r a l l y favorable. No p r o g r e s s i o n of cereb e l l a r s y m p t o m s has been s e e n ; however, c h r o n i c cases h a v e b e e n r e p o r t e d .
ACUTE CEREBELLAR ATAXIA
79
Our three patients made a complete recovery. The longest course was one m o n t h . REFERENCES 1. Leyden, E.: Verlangsamte motorische Leitung, Arch. f. Path., Anat. u. Physiol., u.f. kiln. Med. 46: 476, 1869. 2. Griffith, J. P. C.: Acute Cerebellar Ataxia in Children, Am. J. M. Sc. 151: 24, 1916. 3. Griffith, J. P. C.: Acute CerebrocerebelJar Ataxia, Am. J. Dis. Child. 20: 82, 1920. 4. Griffith, J. P. C.: Acute Cerebellar Ataxia, Am. J. M. Sc. 162: 781, 1921. 5. Babonneix, L.: Acute Infantile Ataxias, Riform~ reed. 44: 694, 1928. 6. Klingman, W. O., and Hodges, 1r G.: Acute Ataxia of Unknown Origin in Children, 5. PEDIAT. 24: 536, 1944. 7. Keller, M. 5., and Karelitz, S.: Acute Ataxia in a 20-Month-Old Female, Pediatrics 1: 756, 1948.