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Meningoencephalitis following rubella
M E N I N G O E N C E P H A L I T I S F O L L O W I N G t~UBELLA J O t t N FRANCIS BRIGGS, M . D .
ST. PA~TL, MINN. I N A B I L I T Y to find m e n i n g o e n c e p h a l i t i s r e p o r t e d as a comMYplication of G e r m a n measles p r o m p t s this report. I t is common knowledge t h a t meningoencephalitis m a y follow a n y infectious process, and the l i t e r a t u r e contains m a n y r e p o r t s of it as a complication of measles, mumps, and other contagious diseases. Its a p p e a r a n c e , however, in such an innocuous disease as rubella, with resulting d e a t h in one case, w a r n s against d e p r e c i a t i n g the potentialities in a n y m i n o r illness. D u r i n g the m o n t h s of D e c e m b e r to F e b r u a r y of this year, this comm u n i t y suffered a w i d e s p r e a d epidemic of G e r m a n measles. Unlike most epidemics, adults were afflicted as well as children. Since the disease in most instances was so mild t h a t m a n y people failed to rep o r t its presence, the actual extent of the disease d u r i n g the epidemic remains u n k n o w n . A t the onset of the epidemic the disease was mild, b u t as it increased the a p p e a r a n c e of p u r p u r a and severe l y m p h a d e nitis became unusual complications. CASE REPORT J. S., white male, aged ten years, was seen at 9 A.~,[. on Jan. 25, 1935, at his home, and the following histolw was obtained. ]?or the previous three weeks this child had been repeatedly exposed to German measles at school. ~Ie remained perfect]y well, however, until Jan. 2~, 1936, when he complained of a nasal discharge a:nd felt feverish. ~Iis mother noted that his temperature was normal but that there was a definite swelling of the occipital glands. He felt perfectly well du~dng that day, but, on awakening on the morning of January 25, he discovered he was covered with a rash. He felt perfectly well except for some pain in the enlarged occipital lymph nodes. The family history and past history are nonessential. Physical examination at this time revealed no evidence of any constitutional defect other than the present illness of Gerinan measles. }Iis temperature was 99 ~ F. His blood pressure was 118 systolic and 90 diastolic; the pulse r~tte was 90 per minute. The course of his illness was mild~ and at no time did his temperature exceed 99 ~ F. He felt so well on January 27 that his mether permitted him ~o ski down their front lawn. At noon of this "day she called him into the house for dinner. The chiId washed himself and then began his meal. During the course of the meal the patient developed a speech defect which the family believed to be an assumed imitation of a popular radio comedian. However, as the meal progressed, it became apparent that the child had no control of his speech. When asking for meat, he said potatoes; and when asking for bread~ he said cake. When the child was furnished wifh the meat he requested~ he left the table, walked around it, and obtained the potatoes, which he actually wanted. As soon as this occurred, the father realized that the ctfild was unable to express his wants, and~ on questioning him as to the names of the foods on the table, he received incorrect names for each item of food. From the Medical Service of St. John's Hospital. 6O9
610
THE JOURNAL OF PEDIATRICS
The father obtained the impressio~ that the child realized that his answers were wrong. I again saw the child at 2 P.~. on J a n u a r y 27, when he was l y i n g in bed, apparently not acutely ill. Itis temperature was 98 ~ F.~ and his pulse, 100 per minute. He answered questions unintelligently, a n d it was obvious that the child realized that these answers were wrong: Some of the questions revealed a very marked loss of memory for certain types of words. Physical examination revealed a lateral nystagraus. The pupils were widely dilated and reacted poorly to light. The fundus examination showed one diopter of papi]ledema. Further examination of the head showed nothing of note. Examination of the neck was completely negative, except for the enlarged occipital lymph nodes. Examination of the chest revealed no abnormality. Examination of the abdomen showed ~othing of note. Examination of the skin showed that the rash was completely gone. Examinatio~ of the upper extremities revealed a definite paresis of the right arm associated with a slight increase of the reflexes of this arm. Examination of the abdominal reflexes revealed them to be markedly exaggerated. I n the lower extremities the patellar reflex of the right leg was definitely increased, and on tiffs side there were positive Bablnski, Gordon, Chaddoek~ and Oppenheim reflexes. The left leg was practically normal as to reflexes. A definite ankle clonus existed in the right anIde. Kernig and Brudzinsld reflexes were absent. A diagnosis was made of meningoencephalitis following rubella. When the child was about to be removed to the hospital, he was seized with a severe convulsion. A f t e r the convulsion subsided~ exaggerated reflexes were obtainable ~n all membcrs~ and the left leg now displayed the same signs as the right leg. There also existed, at this tinl% an a l t e r n a t i n g spastic and flaccid paralysis. At the time of admission to the hospital~ the patient was in extremis. ~Iis temperature gradually increased until it reached t07 ~ F. by rectum, and definite pneumonia could be found in both lungs. :Intravenous glucose was given, as well as morphine a~d. chloral hydrate by rectum to control the convulsions. The convulsive seizures, however, were increasing in frequency~ and despite all measures taken the child died of respiratory paralys~s at :12:30 ~.~. Jan. 28, lg35. A spinal fluid puncture was ~nade by Dr. t-Ian~lcs which revealed a clear fluid under marked pressure. The cell count was 51, with 98 per cent of the Cells lymphocytes. The spinal sugar was 65 mg. per cent, and the blood Wassermann and s p i n a l ~rassermann tests were negative. A postmortem examination was conducted by Dr. Noble~ who reported as follows: The body was that of a normally developed~ well-nourished white male child measuring 42 cm. in length and weighing about 110 pounds. Postmortem rigidity was present, and there was a blotchy postmortem lividity over much of the body surface. There was no edema, cyanosis, or jaundice. The pupils were dilated, round, and equal. From the nose and mouth there was a dark bloody fluid exuding. A f t e r the abdomen was opened, peritoneal surfaces were found to be smooth and shiny with no excess fluid present. The appendix was normal in appearance. The liver was flush with the costal margin. ~ ' h e n the thorax was opened, the hmgs and the pleural cavity were found to be free~ and the pleura] cavities empty. The perica~'diaI sac showed nothing of note. The examination of the large branches of the pulmonary artery showed no evidence of thrombL The heart measured 9.5 cm. in width. The heart weighed 190 gin. The coronary arteries were norraal in appearance. The heart showed nothing of note. The myoeardium on cut section was paler than normM and definitely swollen in appearance. The left lung weighed 240 gin. and had three definite lobes. The pleural surface was smooth, and crepitation was slightly reduced throughout the lung. There were scattered small hemorrhagic areas of consolidation throughout the lung, particularly
BRIG(.~S: ME.NINGOENCJ~IPHALtTIS FOLLOWING RUBELLA
611
in the lower lobe. The right lung weighed 305 gin. and showed a picture similar to the other organ~ but here the areas of eonselidatlou appeared more pronounced in the upper lobe. The liver weighed 1,000 gm. The surfaces were smooth. On eat section, the liver showed nothing of note, save for a moderate degree of cloudy swelling. The spleen weighed 9~i gin. The organ was fairly firm~ and tile corpuscles were barely ~isible. The right kidney weighed 80 gm. The capsule of the organ stripped easily, leaving the free surface. On eat section it showed nothing of note, save for a moderate degree of cloudy swelling. The left kidney weighed 85 gm. and showed a picture similar to the right. ~]xalnination of the gastrointestinaI tract showed nothing of note, save for the fact that the P e y e r ' s patehe~ were prominent and unusually dark in color. The scalp and ealvarinm were found to be normal in appearance. Examination of the brain showed slight evidence of increased pressure with slight flattening of the convolutions. The brain ou cut section showed scattered peteehial hemorrhages throughout, most pronounced in the white substance in the base. The middle ears and mastoid processes showed nothing of note. Diagnosis: (German measles). Postmeasles encephalitis. Bilateral hemorrhagic bronchopnemnonia. Clou@ swellkag o f heart~ liver~ and kidneys. On microscopic examination sections of the brain showed a definite perivascular infiltration of lymphocytes and polymorphonuelear leucocytes at many points. In eertaln areas the smaller vessels were also surrounded by collections of red Mood cetKs. Tile splenic pulp contained many polymorphoneulear leucocytes. The myoeardium showed a moderate degree o~ cloudy swelling. The adrenals, liver, and pancreas showed nothing of note. Sections of the kidneys showed the glomeruli containing a eonslderable excess of nuclei, apparently due to a ]proliferation of endothelium. Sections of the lung showed many of the alveoli filled with blood, but very few inflammatory cells were seen. CASE 2 . - - A boy, three years old, was first seen on Feb. 2~ 1935. ~ e was the youngest of six children, all of whom were suffering with German measles. The mogher stated that the children had little difficulty with the disease and t h a t for the most part they were up and around playing. The disease had made its appearance two days before, and nothing unusual was noted about it, until the morning of ]~eb. 2, 1935~ whe~ the patient refused to get out of bed. The mother paid little attention to this until noon, when she discovered, as she attempted to arouse the child from his sleep, that he was only partially conscious. I saw the child at 5 P.5~. on that da?% when the above history was obtained. His past history and family history are irrelevant. Physical examination revealed a child covered with a characteristic rash of Ger~ man measles, tits temperature w_us 100 .~ F . ; his pulse was 130; and his respiration rate was 26 per minute. The scalp showed nothing of note. The eyes were fixed and staring, and the fundus was 'normal. There was no abnormality in tile papillary reflex, The eyes~ ears, nose, and throat were normal. The oecipitM nodes were enlarged, but apparently not tender. The 1reek was normal. Examination of the chest revealed no pathology in the lung fields or the cardiovascular system. Examination of the abdomen showed :nothing of note. The reflexes of the right leg were essentially normal. The left leg was spastic, and the patellar reflex was exaggerated. I n the left leg there was a qnestlonable Babinski reflex. Examination of the urine revealed a trace of albmnin. The spinal fluid was under pressm'e, but clear. The cell connt was 100 with 92 per cent of the cells lymphocytes. The spinal sugar was 70 nag. per cent. A diagnosis was made of postrubella meningoe~cephalitis.
612
THE JOUR,NAL OP PEDIATI~ICS
The child's condition did not seem serious, gad on the n e x t day there was a definite imlarovement. This improvement continued until, o~ ]~eb. 9, 1935~ a complete physical examination revealed nothing abnormM. Shortly after this last examination, the family, who were ~ransients, left the city, so that the present condition of the child is unknown.
In addition to the two cases reported above, I have knowledge of at least three other cases of meningoencephalitis occurring in this epidemic, All these patients reco~vered from the illness. SUt~MARY
Two cases of rubella are reported in which meningoencephalitis appeared as a complication. In one,death ensued, and postmortem examination confirmed the clinical diagnosis. It is suggested that meningoencephalitis is not an uncommon complication of rubella when the epidemic is severe. 706 LOWRY .M~ED[CALAaTS BUILDING
ST. PA~TL, MINN. I N A B I L I T Y to find m e n i n g o e n c e p h a l i t i s r e p o r t e d as a comMYplication of G e r m a n measles p r o m p t s this report. I t is common knowledge t h a t meningoencephalitis m a y follow a n y infectious process, and the l i t e r a t u r e contains m a n y r e p o r t s of it as a complication of measles, mumps, and other contagious diseases. Its a p p e a r a n c e , however, in such an innocuous disease as rubella, with resulting d e a t h in one case, w a r n s against d e p r e c i a t i n g the potentialities in a n y m i n o r illness. D u r i n g the m o n t h s of D e c e m b e r to F e b r u a r y of this year, this comm u n i t y suffered a w i d e s p r e a d epidemic of G e r m a n measles. Unlike most epidemics, adults were afflicted as well as children. Since the disease in most instances was so mild t h a t m a n y people failed to rep o r t its presence, the actual extent of the disease d u r i n g the epidemic remains u n k n o w n . A t the onset of the epidemic the disease was mild, b u t as it increased the a p p e a r a n c e of p u r p u r a and severe l y m p h a d e nitis became unusual complications. CASE REPORT J. S., white male, aged ten years, was seen at 9 A.~,[. on Jan. 25, 1935, at his home, and the following histolw was obtained. ]?or the previous three weeks this child had been repeatedly exposed to German measles at school. ~Ie remained perfect]y well, however, until Jan. 2~, 1936, when he complained of a nasal discharge a:nd felt feverish. ~Iis mother noted that his temperature was normal but that there was a definite swelling of the occipital glands. He felt perfectly well du~dng that day, but, on awakening on the morning of January 25, he discovered he was covered with a rash. He felt perfectly well except for some pain in the enlarged occipital lymph nodes. The family history and past history are nonessential. Physical examination at this time revealed no evidence of any constitutional defect other than the present illness of Gerinan measles. }Iis temperature was 99 ~ F. His blood pressure was 118 systolic and 90 diastolic; the pulse r~tte was 90 per minute. The course of his illness was mild~ and at no time did his temperature exceed 99 ~ F. He felt so well on January 27 that his mether permitted him ~o ski down their front lawn. At noon of this "day she called him into the house for dinner. The chiId washed himself and then began his meal. During the course of the meal the patient developed a speech defect which the family believed to be an assumed imitation of a popular radio comedian. However, as the meal progressed, it became apparent that the child had no control of his speech. When asking for meat, he said potatoes; and when asking for bread~ he said cake. When the child was furnished wifh the meat he requested~ he left the table, walked around it, and obtained the potatoes, which he actually wanted. As soon as this occurred, the father realized that the ctfild was unable to express his wants, and~ on questioning him as to the names of the foods on the table, he received incorrect names for each item of food. From the Medical Service of St. John's Hospital. 6O9
610
THE JOURNAL OF PEDIATRICS
The father obtained the impressio~ that the child realized that his answers were wrong. I again saw the child at 2 P.~. on J a n u a r y 27, when he was l y i n g in bed, apparently not acutely ill. Itis temperature was 98 ~ F.~ and his pulse, 100 per minute. He answered questions unintelligently, a n d it was obvious that the child realized that these answers were wrong: Some of the questions revealed a very marked loss of memory for certain types of words. Physical examination revealed a lateral nystagraus. The pupils were widely dilated and reacted poorly to light. The fundus examination showed one diopter of papi]ledema. Further examination of the head showed nothing of note. Examination of the neck was completely negative, except for the enlarged occipital lymph nodes. Examination of the chest revealed no abnormality. Examination of the abdomen showed ~othing of note. Examination of the skin showed that the rash was completely gone. Examinatio~ of the upper extremities revealed a definite paresis of the right arm associated with a slight increase of the reflexes of this arm. Examination of the abdominal reflexes revealed them to be markedly exaggerated. I n the lower extremities the patellar reflex of the right leg was definitely increased, and on tiffs side there were positive Bablnski, Gordon, Chaddoek~ and Oppenheim reflexes. The left leg was practically normal as to reflexes. A definite ankle clonus existed in the right anIde. Kernig and Brudzinsld reflexes were absent. A diagnosis was made of meningoencephalitis following rubella. When the child was about to be removed to the hospital, he was seized with a severe convulsion. A f t e r the convulsion subsided~ exaggerated reflexes were obtainable ~n all membcrs~ and the left leg now displayed the same signs as the right leg. There also existed, at this tinl% an a l t e r n a t i n g spastic and flaccid paralysis. At the time of admission to the hospital~ the patient was in extremis. ~Iis temperature gradually increased until it reached t07 ~ F. by rectum, and definite pneumonia could be found in both lungs. :Intravenous glucose was given, as well as morphine a~d. chloral hydrate by rectum to control the convulsions. The convulsive seizures, however, were increasing in frequency~ and despite all measures taken the child died of respiratory paralys~s at :12:30 ~.~. Jan. 28, lg35. A spinal fluid puncture was ~nade by Dr. t-Ian~lcs which revealed a clear fluid under marked pressure. The cell count was 51, with 98 per cent of the Cells lymphocytes. The spinal sugar was 65 mg. per cent, and the blood Wassermann and s p i n a l ~rassermann tests were negative. A postmortem examination was conducted by Dr. Noble~ who reported as follows: The body was that of a normally developed~ well-nourished white male child measuring 42 cm. in length and weighing about 110 pounds. Postmortem rigidity was present, and there was a blotchy postmortem lividity over much of the body surface. There was no edema, cyanosis, or jaundice. The pupils were dilated, round, and equal. From the nose and mouth there was a dark bloody fluid exuding. A f t e r the abdomen was opened, peritoneal surfaces were found to be smooth and shiny with no excess fluid present. The appendix was normal in appearance. The liver was flush with the costal margin. ~ ' h e n the thorax was opened, the hmgs and the pleural cavity were found to be free~ and the pleura] cavities empty. The perica~'diaI sac showed nothing of note. The examination of the large branches of the pulmonary artery showed no evidence of thrombL The heart measured 9.5 cm. in width. The heart weighed 190 gin. The coronary arteries were norraal in appearance. The heart showed nothing of note. The myoeardium on cut section was paler than normM and definitely swollen in appearance. The left lung weighed 240 gin. and had three definite lobes. The pleural surface was smooth, and crepitation was slightly reduced throughout the lung. There were scattered small hemorrhagic areas of consolidation throughout the lung, particularly
BRIG(.~S: ME.NINGOENCJ~IPHALtTIS FOLLOWING RUBELLA
611
in the lower lobe. The right lung weighed 305 gin. and showed a picture similar to the other organ~ but here the areas of eonselidatlou appeared more pronounced in the upper lobe. The liver weighed 1,000 gm. The surfaces were smooth. On eat section, the liver showed nothing of note, save for a moderate degree of cloudy swelling. The spleen weighed 9~i gin. The organ was fairly firm~ and tile corpuscles were barely ~isible. The right kidney weighed 80 gm. The capsule of the organ stripped easily, leaving the free surface. On eat section it showed nothing of note, save for a moderate degree of cloudy swelling. The left kidney weighed 85 gm. and showed a picture similar to the right. ~]xalnination of the gastrointestinaI tract showed nothing of note, save for the fact that the P e y e r ' s patehe~ were prominent and unusually dark in color. The scalp and ealvarinm were found to be normal in appearance. Examination of the brain showed slight evidence of increased pressure with slight flattening of the convolutions. The brain ou cut section showed scattered peteehial hemorrhages throughout, most pronounced in the white substance in the base. The middle ears and mastoid processes showed nothing of note. Diagnosis: (German measles). Postmeasles encephalitis. Bilateral hemorrhagic bronchopnemnonia. Clou@ swellkag o f heart~ liver~ and kidneys. On microscopic examination sections of the brain showed a definite perivascular infiltration of lymphocytes and polymorphonuelear leucocytes at many points. In eertaln areas the smaller vessels were also surrounded by collections of red Mood cetKs. Tile splenic pulp contained many polymorphoneulear leucocytes. The myoeardium showed a moderate degree o~ cloudy swelling. The adrenals, liver, and pancreas showed nothing of note. Sections of the kidneys showed the glomeruli containing a eonslderable excess of nuclei, apparently due to a ]proliferation of endothelium. Sections of the lung showed many of the alveoli filled with blood, but very few inflammatory cells were seen. CASE 2 . - - A boy, three years old, was first seen on Feb. 2~ 1935. ~ e was the youngest of six children, all of whom were suffering with German measles. The mogher stated that the children had little difficulty with the disease and t h a t for the most part they were up and around playing. The disease had made its appearance two days before, and nothing unusual was noted about it, until the morning of ]~eb. 2, 1935~ whe~ the patient refused to get out of bed. The mother paid little attention to this until noon, when she discovered, as she attempted to arouse the child from his sleep, that he was only partially conscious. I saw the child at 5 P.5~. on that da?% when the above history was obtained. His past history and family history are irrelevant. Physical examination revealed a child covered with a characteristic rash of Ger~ man measles, tits temperature w_us 100 .~ F . ; his pulse was 130; and his respiration rate was 26 per minute. The scalp showed nothing of note. The eyes were fixed and staring, and the fundus was 'normal. There was no abnormality in tile papillary reflex, The eyes~ ears, nose, and throat were normal. The oecipitM nodes were enlarged, but apparently not tender. The 1reek was normal. Examination of the chest revealed no pathology in the lung fields or the cardiovascular system. Examination of the abdomen showed :nothing of note. The reflexes of the right leg were essentially normal. The left leg was spastic, and the patellar reflex was exaggerated. I n the left leg there was a qnestlonable Babinski reflex. Examination of the urine revealed a trace of albmnin. The spinal fluid was under pressm'e, but clear. The cell connt was 100 with 92 per cent of the cells lymphocytes. The spinal sugar was 70 nag. per cent. A diagnosis was made of postrubella meningoe~cephalitis.
612
THE JOUR,NAL OP PEDIATI~ICS
The child's condition did not seem serious, gad on the n e x t day there was a definite imlarovement. This improvement continued until, o~ ]~eb. 9, 1935~ a complete physical examination revealed nothing abnormM. Shortly after this last examination, the family, who were ~ransients, left the city, so that the present condition of the child is unknown.
In addition to the two cases reported above, I have knowledge of at least three other cases of meningoencephalitis occurring in this epidemic, All these patients reco~vered from the illness. SUt~MARY
Two cases of rubella are reported in which meningoencephalitis appeared as a complication. In one,death ensued, and postmortem examination confirmed the clinical diagnosis. It is suggested that meningoencephalitis is not an uncommon complication of rubella when the epidemic is severe. 706 LOWRY .M~ED[CALAaTS BUILDING