THREE CASES OF ENCEPHALITIS
LETHARGICA By V. M. BHAT, b.a., m.b., Yeola, Dist. Nasik
b.s.
Dr. Kundu's article in the July 1949 issue of the Indian Medical Gazette prompts me to publish three cases of Encephalitis lethargica which occurred in one family at Yeola, an outof-the-way town in Nasik District, Bombay
Presidency.
First two
cases
deceptive
On 28th May, 1949, the son and daughter of one of our colleagues were, all of a sudden, attacked with what appeared, at first sight, to be symptoms of cholera or food poisoning. Both were aged 9 and 7, respectively. (Cholera was somewhat prevalent in the adjoiningvillages.) Both had one or two watery motions each and both vomited twice. They had nausea also and were complaining of giddiness. This had occurred in the afternoon. Yet the children were moving about till the evening. Their mother got alarmed at their increasing giddiness and nausea, and sent for their father. He, being a doctor, found them somewhat uneasy, and thought that they were possibly suffering from a ijiild attack of cholera or food poisoning, and administered some gastric sedatives. Coma and
pulmonary oedema
In the evening at about 6-30 p.m. the
suddenly became comatose,
His
boy
temperature
486
THE INDIAN MEDICAL GAZETTE
?F. But the pulse and respiration rates much disturbed?16 respirations with a pulse rate of 120 per minute. The girl became drowsy and completely comatose at 8 p.m. and I was becoming worse and worse every hour. was called in at about 9 p.m. The girl was acutely ill. Her temperature was 102?F. The respirations were slow. Her chest was full of rales and she showed all the symptoms of pulmonary oedema, and was getting cvanosed. The heart was flickering. She was at once given atropine 1/50 gr. by injection and coramine also by the same route. Yet there was no improvement. Lobeline was also administered along with coramine subcutaneously. As the respiration was failing, artificial respiration was resorted to. At about 10 p.m. blood-coloured foam was coming out of the mouth on compressing the chest and the child was gasping. We had almost given her up as dead, although artificial respiration was being continued. Again atropine 1/20 gr. with coramine 1 cc. was given. For a minute or two, respirations were stopped altogether, it seemed. But on compressing the chest, the child heaved a sigh, and our hopes were again revived. We continued the same treatment till about 2 a.m., when respirations and heart resumed their rhythm, and were somewhat steadied. The temperature rose to 104?F. at that time.
was 101 were
Her brother's condition was not so bad. But the rate of respiration was slower than normal, we administered atropine 1/50 gr. and 1 cc. coramine to him also, once or twice, during the night. Glucose water with a little brandy was given to both the children in sips. The patient's father with Dr. P. S. Mahajan and myself were struggling for the whole night, and had the satisfaction that the girl was almost rescued from the jaws of death. The next morning, the boy became conscious, and Avas prancing about in the afternoon. The girl continued to be comatose, but her heart and respirations had resumed their rhythm. The temperature was 103?F. in the morning, and she was able to take sips of glucose wrater. As the temperature was high, cold compresses were put on the abdomen and forehead. as
No diagnosis : Treatment symptomatic Although we were treating both the children symptomatically, still none of us was sure about the diagnosis. Cholera or food poisoning theories did not fit in the picture. But as the boy had apparently recovered?I say apparently because his case had become chronic, and he was to cause constant worry arid anxiety to all concerned for 3 months?our minds were somewhat at ease, and we hoped' that the girl too would recover soon like her brother.
Masking meningeal symptoms morning the girl began to show symptoms meningitis. Her neck was stiff, eyes were
Next of
[Nov., 1949
Kernig's sign was present. The 104?F., and the coma continued. At once we decided upon penicillin, and gaye her 3 lakhs units during the 24 hours. Glucose was administered intravenously twice a day, 111 addition to glucose water and milk by mouthcongested,
and
temperature
^
was
time she could not swallow anyand the liquids trickled down the cheeks. Glucose-saline enemata were also sometimes rejected. Urine had sometimes be drawn by catheter. Bowels were emptiedv But many
a
thing by mouth,
^
was
by glycerine syringe or enema. Cylotropin sometimes given on the theory of meningeal infection. The girl continued in this comatose condition for full 9 days in spite of the treatment, with cibazol, coramine and sometimes digitalis by mouth or parenterally in additionOn the 10th day she showed some signs o* returning consciousness. She began to cry and wince if pricked, and opened her eyes and cried for her mother. She began to take her feeds regularly. Penicillin was continued in smaller doses till the temperature dropped down to normal. Protein hydrolysate was given f?r protein deficiency, and she made a rapid recovery in about 4 weeks.
Just before the girl was rallying round, the idea struck me that these may be cases of Encephalitis lethargica, and I expressed my opinion to the father of the patients. But as the girl's case was masked by meningeal symptoms, I could not arrive at definite diagnosis. The cases were occasionally discussed in consultation with my colleagues here, but none could express a positive opinion about encephalitis, as there were no clear-cut signs and symptoms of that disease. Third case clears up diagnosis. Unfortunate^, on 6th June, 1949, possibly by prolonged mental strain and vigil for so many days and nights, the mother of the children began to show symptoms of illness. Sbc developed diplopia, ptosis, insomnia, giddiness, garrulity and symptoms of mental aberration-
An affectionate mother as she was, she began to show complete apathy towards her ailing daughter, who wanted to cling to her. She railed against her near and dear relatives and her lady friends. She refused food and drinkShe developed something like paranoia. She was however conscious and could recognizc others. Her temperature was slightly above normal.
The mother's symptoms clinched the diagnosis which was agitating my mind for about two to three weeks. I pored over all the available authorities, Osier, Price, Savill and various medical annuals from 1925 to 1937, and cam6 to the definite conclusion that all the three wei'c cases of Encephalitis lethargica. Her syrup' toms definitely pointed to that. Hysteria suggested itself, but was ruled out, as she had never showi} $ny of its symptoms before-
Nov., 1949] Resides,
three
THREE CASES OF ENCEPHALITIS LETHARGIC A
cases,
in
occurring
the
histories of the three room for any doubt.
Subsequent
Relapses
and laboratory
cases
left
no
investigations
attack, with
as
the
all
symptoms
diowsiness, ptosis, giddiness
She took
Parkinsonism.
lor
recovery.
After
a
month
and
or so
of
diplopia,
even some
traits
nearly six weeks
after her recovery, the
Sul also had one relapse on 17th August, 1949. She had ut it was very mild in character. blurred vision, ptosis, drowsy look and giddiness. She, however, recovered within 16 hours. Chronic
cases
whose sudden in the first ourselves we Recovery congratulated on 2nd relapse severe distance, had a very diplopia, August, 1949. He became drowsy, had furred vision, ptosis, giddiness and Parkinsonism. But when not drowsy, he was very and Violent, kicking and biting his mother that others, sometimes he was so uncontrollable Even !us hands and legs had to be tied down. this condition, he was rolling himself to catch 'lls mother and have a bite of any of her part could reach. This relapse lasted for 3 days, aud he recovered from it completely on the 4th
Unfortunately
the
boy,
on
ju
487
Appearance
..
Albumin
..
Sugar
..
Globulin Cell count T.B.
j
19-8-49
Girl,
Mother,
Clear nil nil
Clear nil nil
Negative
.
.
.
..
i
!
19-8-49
Boy,
*
The mother was treated'on the same lines the girl. In addition, she was given nervous sedatives. But as she was in a suicidal mood for some days, she point blank refused to take be anY treatment or nourishment. She had to we and coaxed to take homoeopathic treatment, it. succeeded in smuggling some barbitones into is there as And we had to be content with it, She no for encephalitis. specific treatment a recovered for a time, but again relapsed into third a into few days second attack and after a
BHAT
C.S.F. examination
same
family, one after another, with initial drowsiness, third case, ??ma, and definite symptoms in the and absence of any history of hysteria befoie, pointed to encephalitis and not to hysteria.
:
84 per
c.mm.
Negative
20-8-49
Negative
Clear nil nil Trace
5 per c.mm.
3 per c.mm.
Negative
Negative
* On 1st September, 1949, again the C.S.F. examined and the cell count was 1 or 2 per c.mm.
was
When they all arrived here, the boy got a second violent relapse on 2nd September, 1949, with all the symptoms described above. Parkinsonism was more evident now. But one peculiar feature in these two relapses was that his neck muscles became as if partially paralysed. Due to this and Parkinsonism, he used to fall on his face, and sustain minor injuries. Then on 9th September, 1949, he was taken to Bombay where he was thoroughly examined over again by Drs. Coelho, Tulpule and Wad. They confirmed the diagnosis but could not suggest any specific treatment against the virus. They expressed the fear that the boy might develop epilepsy and suggested prominal and other sedatives with siolan-iodine 2 cc. twice a week for 4 times only. Then this chronic patient started getting the attacks every 10th day or sometimes even sooner than that. According to Price, the course is variable, and Savill-says that 1/3 of the cases die, and 1/3 seriously disabled. I conjectured that this patient would fall in this category of disablement. The boy was taken to Poona also with a view to showing him to Dr. Bhagvat of the mental asylum. He also suggested sedatives like luminal or prominal to be given in small doses daily. His blood sugar was 83.3 mg. per cent.
The mother of the two children affected had to Ujjain as her father had an attack of paresis, about a month previously. She took These relapses left no room for any doubt both her children with her, as they were free from relapses. She and her children returned ?llt the diagnosis. On Saturday here on the 3rd November, 1949. exhausted, As our resources of treatment were the 5th instant, both the children had severe examinathe to a.ud confirm the diagnosis by relapses or fresh attacks, I can't say which. C.S.F., we decided on the 18th August, These attacks of encephalitis resembled the to Ahmednagar first to send the patients attacks and were so severe that the the under original where all the facilities were available in comatose condition after 20 hours girl expired the of Evangelical direction of Dr. Anderson due to of respiratory failure primarily. Her lungs every isooth Hospital. He took the C.S.F. Patient by lumbar puncture and made a thorough were so much cedematous that venous blood She was trickling down her nose and mouth. examination of all the patients. had been given atropine, lobeline, coramine, Blood of all was negative for malarial para- adrenaline by injections along with glucose sites. Yet one doctor thought that the boy might no purpose. to but saline, e suffering from cerebral type of malaria, he boy's case was causing us more anxiety, The boy was also affected on the 5th instant well for an hour or so, e confirmed my diagnosis and advised his at night. He had slept her to try procain penicillin 1 cc. contain- but as was subsequently known, then become ln? 3 lakhs and some sedatives. unconscious, and could not be roused. His for 10
la?
^, Jble
?
units
days
to go
488
THE INDIAN MEDICAL GAZETTE
stertor
drew our attention to him. He was the same treatment as was given to his sister. His pulse was good. Next morning he woke up, and was quite conscious till 2 p.m. He took his food also with his own hands. But at 2 p.m. while asleep, his respiration began to fail, and he became comatose. Again the same gamut of treatment was gone through, and penicillin was started as his temperature was raised. Two lakhs units were given initially, and the same dose was repeated at night. His lungs too became (edematous, and venous blood came out every time when artificial respiration was resorted to. Gradually the number of respirations began to increase from 8 to 16, and then to 34 at night, with a pulse rate of 140 to 160, and temperature hovering between 102 to 105. Cerebral congestion was fully evident as his eyes were red on the next day. Artificial respiration had to be resorted to every time when the number of respirations fell below 12. Tuesday and Wednesday {i.e. the 8th and the 9th November) passed in the same condition without any improvement. Nothing could be given by the mouth, and dehydration was combated with glucose saline intravenously and subcutaneously. On Thursday morning some pus trickled down the nares, and blood had to be cleared out of his throat every few minutes. Eyes were very red, and both the corneas resembled ground glass. On the 10th instant respiratory failure again supervened, and the boy died at 6-30 p.m. in the evening.
given
who had a mild of these shocks.
Fortunately the mother, attack, has survived in spite Points
for
consideration
(1) whether few months were fresh ones or mere severe relapses. There is some ground for suspicion on this point; because the children were infected at their grandfather's house at Indore in the first instance, when they had been there in last May. Later also they had been to their grandfather, but this time he was at Ujjain. Possibly his attack of paresis may be one to the infection, and he was the carrier. (2) I have never come across an instance of death in relapses as no authority mentions it anywhere in the literature. And it came a few months after ! these attacks after nearly
are :
a
[This account was received in two instalments which have been combined. The second instalment gave the fatal ending. The diagnosis of polio also needs a consideration.?Editor, l.M.G.]
[Nov., 1949
LETHARGICA By V. M. BHAT, b.a., m.b., Yeola, Dist. Nasik
b.s.
Dr. Kundu's article in the July 1949 issue of the Indian Medical Gazette prompts me to publish three cases of Encephalitis lethargica which occurred in one family at Yeola, an outof-the-way town in Nasik District, Bombay
Presidency.
First two
cases
deceptive
On 28th May, 1949, the son and daughter of one of our colleagues were, all of a sudden, attacked with what appeared, at first sight, to be symptoms of cholera or food poisoning. Both were aged 9 and 7, respectively. (Cholera was somewhat prevalent in the adjoiningvillages.) Both had one or two watery motions each and both vomited twice. They had nausea also and were complaining of giddiness. This had occurred in the afternoon. Yet the children were moving about till the evening. Their mother got alarmed at their increasing giddiness and nausea, and sent for their father. He, being a doctor, found them somewhat uneasy, and thought that they were possibly suffering from a ijiild attack of cholera or food poisoning, and administered some gastric sedatives. Coma and
pulmonary oedema
In the evening at about 6-30 p.m. the
suddenly became comatose,
His
boy
temperature
486
THE INDIAN MEDICAL GAZETTE
?F. But the pulse and respiration rates much disturbed?16 respirations with a pulse rate of 120 per minute. The girl became drowsy and completely comatose at 8 p.m. and I was becoming worse and worse every hour. was called in at about 9 p.m. The girl was acutely ill. Her temperature was 102?F. The respirations were slow. Her chest was full of rales and she showed all the symptoms of pulmonary oedema, and was getting cvanosed. The heart was flickering. She was at once given atropine 1/50 gr. by injection and coramine also by the same route. Yet there was no improvement. Lobeline was also administered along with coramine subcutaneously. As the respiration was failing, artificial respiration was resorted to. At about 10 p.m. blood-coloured foam was coming out of the mouth on compressing the chest and the child was gasping. We had almost given her up as dead, although artificial respiration was being continued. Again atropine 1/20 gr. with coramine 1 cc. was given. For a minute or two, respirations were stopped altogether, it seemed. But on compressing the chest, the child heaved a sigh, and our hopes were again revived. We continued the same treatment till about 2 a.m., when respirations and heart resumed their rhythm, and were somewhat steadied. The temperature rose to 104?F. at that time.
was 101 were
Her brother's condition was not so bad. But the rate of respiration was slower than normal, we administered atropine 1/50 gr. and 1 cc. coramine to him also, once or twice, during the night. Glucose water with a little brandy was given to both the children in sips. The patient's father with Dr. P. S. Mahajan and myself were struggling for the whole night, and had the satisfaction that the girl was almost rescued from the jaws of death. The next morning, the boy became conscious, and Avas prancing about in the afternoon. The girl continued to be comatose, but her heart and respirations had resumed their rhythm. The temperature was 103?F. in the morning, and she was able to take sips of glucose wrater. As the temperature was high, cold compresses were put on the abdomen and forehead. as
No diagnosis : Treatment symptomatic Although we were treating both the children symptomatically, still none of us was sure about the diagnosis. Cholera or food poisoning theories did not fit in the picture. But as the boy had apparently recovered?I say apparently because his case had become chronic, and he was to cause constant worry arid anxiety to all concerned for 3 months?our minds were somewhat at ease, and we hoped' that the girl too would recover soon like her brother.
Masking meningeal symptoms morning the girl began to show symptoms meningitis. Her neck was stiff, eyes were
Next of
[Nov., 1949
Kernig's sign was present. The 104?F., and the coma continued. At once we decided upon penicillin, and gaye her 3 lakhs units during the 24 hours. Glucose was administered intravenously twice a day, 111 addition to glucose water and milk by mouthcongested,
and
temperature
^
was
time she could not swallow anyand the liquids trickled down the cheeks. Glucose-saline enemata were also sometimes rejected. Urine had sometimes be drawn by catheter. Bowels were emptiedv But many
a
thing by mouth,
^
was
by glycerine syringe or enema. Cylotropin sometimes given on the theory of meningeal infection. The girl continued in this comatose condition for full 9 days in spite of the treatment, with cibazol, coramine and sometimes digitalis by mouth or parenterally in additionOn the 10th day she showed some signs o* returning consciousness. She began to cry and wince if pricked, and opened her eyes and cried for her mother. She began to take her feeds regularly. Penicillin was continued in smaller doses till the temperature dropped down to normal. Protein hydrolysate was given f?r protein deficiency, and she made a rapid recovery in about 4 weeks.
Just before the girl was rallying round, the idea struck me that these may be cases of Encephalitis lethargica, and I expressed my opinion to the father of the patients. But as the girl's case was masked by meningeal symptoms, I could not arrive at definite diagnosis. The cases were occasionally discussed in consultation with my colleagues here, but none could express a positive opinion about encephalitis, as there were no clear-cut signs and symptoms of that disease. Third case clears up diagnosis. Unfortunate^, on 6th June, 1949, possibly by prolonged mental strain and vigil for so many days and nights, the mother of the children began to show symptoms of illness. Sbc developed diplopia, ptosis, insomnia, giddiness, garrulity and symptoms of mental aberration-
An affectionate mother as she was, she began to show complete apathy towards her ailing daughter, who wanted to cling to her. She railed against her near and dear relatives and her lady friends. She refused food and drinkShe developed something like paranoia. She was however conscious and could recognizc others. Her temperature was slightly above normal.
The mother's symptoms clinched the diagnosis which was agitating my mind for about two to three weeks. I pored over all the available authorities, Osier, Price, Savill and various medical annuals from 1925 to 1937, and cam6 to the definite conclusion that all the three wei'c cases of Encephalitis lethargica. Her syrup' toms definitely pointed to that. Hysteria suggested itself, but was ruled out, as she had never showi} $ny of its symptoms before-
Nov., 1949] Resides,
three
THREE CASES OF ENCEPHALITIS LETHARGIC A
cases,
in
occurring
the
histories of the three room for any doubt.
Subsequent
Relapses
and laboratory
cases
left
no
investigations
attack, with
as
the
all
symptoms
diowsiness, ptosis, giddiness
She took
Parkinsonism.
lor
recovery.
After
a
month
and
or so
of
diplopia,
even some
traits
nearly six weeks
after her recovery, the
Sul also had one relapse on 17th August, 1949. She had ut it was very mild in character. blurred vision, ptosis, drowsy look and giddiness. She, however, recovered within 16 hours. Chronic
cases
whose sudden in the first ourselves we Recovery congratulated on 2nd relapse severe distance, had a very diplopia, August, 1949. He became drowsy, had furred vision, ptosis, giddiness and Parkinsonism. But when not drowsy, he was very and Violent, kicking and biting his mother that others, sometimes he was so uncontrollable Even !us hands and legs had to be tied down. this condition, he was rolling himself to catch 'lls mother and have a bite of any of her part could reach. This relapse lasted for 3 days, aud he recovered from it completely on the 4th
Unfortunately
the
boy,
on
ju
487
Appearance
..
Albumin
..
Sugar
..
Globulin Cell count T.B.
j
19-8-49
Girl,
Mother,
Clear nil nil
Clear nil nil
Negative
.
.
.
..
i
!
19-8-49
Boy,
*
The mother was treated'on the same lines the girl. In addition, she was given nervous sedatives. But as she was in a suicidal mood for some days, she point blank refused to take be anY treatment or nourishment. She had to we and coaxed to take homoeopathic treatment, it. succeeded in smuggling some barbitones into is there as And we had to be content with it, She no for encephalitis. specific treatment a recovered for a time, but again relapsed into third a into few days second attack and after a
BHAT
C.S.F. examination
same
family, one after another, with initial drowsiness, third case, ??ma, and definite symptoms in the and absence of any history of hysteria befoie, pointed to encephalitis and not to hysteria.
:
84 per
c.mm.
Negative
20-8-49
Negative
Clear nil nil Trace
5 per c.mm.
3 per c.mm.
Negative
Negative
* On 1st September, 1949, again the C.S.F. examined and the cell count was 1 or 2 per c.mm.
was
When they all arrived here, the boy got a second violent relapse on 2nd September, 1949, with all the symptoms described above. Parkinsonism was more evident now. But one peculiar feature in these two relapses was that his neck muscles became as if partially paralysed. Due to this and Parkinsonism, he used to fall on his face, and sustain minor injuries. Then on 9th September, 1949, he was taken to Bombay where he was thoroughly examined over again by Drs. Coelho, Tulpule and Wad. They confirmed the diagnosis but could not suggest any specific treatment against the virus. They expressed the fear that the boy might develop epilepsy and suggested prominal and other sedatives with siolan-iodine 2 cc. twice a week for 4 times only. Then this chronic patient started getting the attacks every 10th day or sometimes even sooner than that. According to Price, the course is variable, and Savill-says that 1/3 of the cases die, and 1/3 seriously disabled. I conjectured that this patient would fall in this category of disablement. The boy was taken to Poona also with a view to showing him to Dr. Bhagvat of the mental asylum. He also suggested sedatives like luminal or prominal to be given in small doses daily. His blood sugar was 83.3 mg. per cent.
The mother of the two children affected had to Ujjain as her father had an attack of paresis, about a month previously. She took These relapses left no room for any doubt both her children with her, as they were free from relapses. She and her children returned ?llt the diagnosis. On Saturday here on the 3rd November, 1949. exhausted, As our resources of treatment were the 5th instant, both the children had severe examinathe to a.ud confirm the diagnosis by relapses or fresh attacks, I can't say which. C.S.F., we decided on the 18th August, These attacks of encephalitis resembled the to Ahmednagar first to send the patients attacks and were so severe that the the under original where all the facilities were available in comatose condition after 20 hours girl expired the of Evangelical direction of Dr. Anderson due to of respiratory failure primarily. Her lungs every isooth Hospital. He took the C.S.F. Patient by lumbar puncture and made a thorough were so much cedematous that venous blood She was trickling down her nose and mouth. examination of all the patients. had been given atropine, lobeline, coramine, Blood of all was negative for malarial para- adrenaline by injections along with glucose sites. Yet one doctor thought that the boy might no purpose. to but saline, e suffering from cerebral type of malaria, he boy's case was causing us more anxiety, The boy was also affected on the 5th instant well for an hour or so, e confirmed my diagnosis and advised his at night. He had slept her to try procain penicillin 1 cc. contain- but as was subsequently known, then become ln? 3 lakhs and some sedatives. unconscious, and could not be roused. His for 10
la?
^, Jble
?
units
days
to go
488
THE INDIAN MEDICAL GAZETTE
stertor
drew our attention to him. He was the same treatment as was given to his sister. His pulse was good. Next morning he woke up, and was quite conscious till 2 p.m. He took his food also with his own hands. But at 2 p.m. while asleep, his respiration began to fail, and he became comatose. Again the same gamut of treatment was gone through, and penicillin was started as his temperature was raised. Two lakhs units were given initially, and the same dose was repeated at night. His lungs too became (edematous, and venous blood came out every time when artificial respiration was resorted to. Gradually the number of respirations began to increase from 8 to 16, and then to 34 at night, with a pulse rate of 140 to 160, and temperature hovering between 102 to 105. Cerebral congestion was fully evident as his eyes were red on the next day. Artificial respiration had to be resorted to every time when the number of respirations fell below 12. Tuesday and Wednesday {i.e. the 8th and the 9th November) passed in the same condition without any improvement. Nothing could be given by the mouth, and dehydration was combated with glucose saline intravenously and subcutaneously. On Thursday morning some pus trickled down the nares, and blood had to be cleared out of his throat every few minutes. Eyes were very red, and both the corneas resembled ground glass. On the 10th instant respiratory failure again supervened, and the boy died at 6-30 p.m. in the evening.
given
who had a mild of these shocks.
Fortunately the mother, attack, has survived in spite Points
for
consideration
(1) whether few months were fresh ones or mere severe relapses. There is some ground for suspicion on this point; because the children were infected at their grandfather's house at Indore in the first instance, when they had been there in last May. Later also they had been to their grandfather, but this time he was at Ujjain. Possibly his attack of paresis may be one to the infection, and he was the carrier. (2) I have never come across an instance of death in relapses as no authority mentions it anywhere in the literature. And it came a few months after ! these attacks after nearly
are :
a
[This account was received in two instalments which have been combined. The second instalment gave the fatal ending. The diagnosis of polio also needs a consideration.?Editor, l.M.G.]
[Nov., 1949
