117 Change of Serum r-GTP during Long-Term Treatment with Anticonvulsants Hiroshi Nagafuji, MD, Yoshifumi Fuseya, MD and Kyoji Akaishi, MD Department of Pediatrics, Tenri Hospital, Tenri, Nara Serum gamma glutamyl transpeptidase h-GTP) is elevated during long-term administration of anti- convulsants such as phenobarbitone (PB), phenytoin (PHT) and carbamazepine (CBZ). The elevation pat- tern of -r-GTP, the isozymes, and the correlation between -r-GTP and urinary 6i3-hydroxycortisol or other hepatic enzymes were studied. Materials and Methods Ninety-eight patients (aged 5-16 years) treated with anticonvulsants were analysed. Twenty-five patients had been taking valproic acid (VPA) alone, 23 PB alone, 29 CBZ alone and 21 a combination of PB and PHT (PB + PHT). Serum -r-GTP was measured by the method using l-glutamyl p-nitroaniline as the substrate. -r-GTP isozymes were determined by electrophoresis on cellulose acetate. The serum con- centration of anticonvulsants was measured by EMIT. Urinary 6/3-hydroxycortisol as an indicator of hepatic P-450 induction was measured by a paper-chromato- graphic method. Results -r-GTP was elevated in the PB alone, CBZ alone and PB + PHT groups. In the PB alone and PB + PHT groups, there was a correlation between -r-GTP and duration of treatment. The -r-GTP level fell rapidly after PHT was reduced or stopped. The -r-GTP iso- zyme pattern was not influenced by the type of drug used, but varied depending on the serum level of -r-GTP. There was a correlation between -r-GTP and some other hepatic enzymes (LAP, GOT and GPT) in the cases where serum GPT was over SOU in the CBZ and PB + PHT therapy groups. Between -r-GTP and urinary 6/3-hydroxycortisol, no definite correla- tion was detected. Key words: Anticonvulsants, -r-GTP isozyme, 6/3- hydroxy cortisol, hepatic cytochrome P-450, reduced phenytoin. 118 EEG Findings of Acute Encephalitis-Relation to Prognosis Hiroaki Shiihara, MD, Masaru Tatsuno, MD, Michiko Hayashi, MD and Hiroko Iwamoto, MD Department of Neurology, Kanagawa Children's Medical Center, Yokohama, Kanagawa Materials and Methods Two hundred and twelve electroencephalograms (EEGs) in 47 children (20 males and 27 females, aged 3 months to 11 years old) of acu te encephalitis were studied. The etiology of encephalitides were unknown in 17 cases, measles in 11, rubella in 9, varicella in 3, mumps in 3 and others in 4. Three cases died. Fifteen cases cured with sequelae (includ- ing 8 cases with postencephalitic epilepsy) and 29 cases withou t sequelae. Abnormality of EEG basic activity according to graded by Aoki-Lombroso's criterion and occurrence of paroxysmal discharges were studied respectively about their relation to prognosis. Result The grade of abnormality of EEG basic activity re- corded for the first time within one week after onset of encephalitis had no correlation with prognosis. The change of grade of basic activity between two records of EEG within one week was well correlated with prognosis. Rapid deterioration of basic activity within one week was seen in cases of death and with severe sequelae. In cases with sequelae, normalization of EEG basic activity was delayed compared with cases with- out any sequelae, in which rapid normalization was seen in several weeks after onset of encephalitis. Paroxysmal discharges were seen in 33.3% of cases of which first EEG records were taken within one week after onset of encephalitis. In a half of those cases with paroxysmal discharges sequelae of postence- phalitic epilepsy occurred. On the other hand, post- encephalitic epilepsy was seen only in one case in those with no paroxysmal discharges in the first EEG records within one week. In all but one cases with epileptic sequelae, occur- rence of paroxysmal discharges in EEG preceded to clinical attacks. There occurred no epileptic sequelae in 16 cases with no paroxysmal discharges in all the serial EEG records. It is emphasized that only one EEG record at the onset of encephalitis is not a good clue to predict its prognosis and the continuous follow-up of EEG change during and after disease is important. Key words: Acute encephalitis, electroencephalogram, postencephalitic epilepsy. Brain & Development, Vol 5, No 2, 1983 203
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Change of Serum r-GTP during Long-Term Treatment with Anticonvulsants Hiroshi Nagafuji, MD, Yoshifumi Fuseya, MD and Kyoji Akaishi, MD Department of Pediatrics, Tenri Hospital, Tenri, Nara
Serum gamma glutamyl transpeptid ase h-GTP) is elevated during long-term administration of anticonvulsants such as phenobarbitone (PB), phenytoin (PHT) and carbamazepine (CBZ). The elevation pattern of -r-GTP, the isozymes, and the correlation between -r-GTP and urinary 6i3-hydroxycortisol or other hepatic enzymes were studied.
Materials and Methods Ninety-eight patients (aged 5-16 years) treated with anticonvulsants were analysed. Twenty-five patients had been taking valproic acid (VPA) alone, 23 PB alone, 29 CBZ alone and 21 a combination of PB and PHT (PB + PHT). Serum -r-GTP was measured by the method using l-glutamyl p-nitroaniline as the substrate. -r-GTP isozymes were determined by electrophoresis on cellulose acetate. The serum concentration of anticonvulsants was measured by EMIT. Urinary 6/3-hydroxycortisol as an indicator of hepatic P-450 induction was measured by a paper-chromatographic method.
Results -r-GTP was elevated in the PB alone, CBZ alone and PB + PHT groups. In the PB alone and PB + PHT groups, there was a correlation between -r-GTP and duration of treatment. The -r-GTP level fell rapidly after PHT was reduced or stopped. The -r-GTP isozyme pattern was not influenced by the type of drug used, but varied depending on the serum level of -r-GTP. There was a correlation between -r-GTP and some other hepatic enzymes (LAP, GOT and GPT) in the cases where serum GPT was over SOU in the CBZ and PB + PHT therapy groups. Between -r-GTP and urinary 6/3-hydroxycortisol, no definite correlation was detected.
EEG Findings of Acute Encephalitis-Relation to Prognosis Hiroaki Shiihara, MD, Masaru Tatsuno, MD, Michiko Hayashi, MD and Hiroko Iwamoto, MD Department of Neurology, Kanagawa Children's Medical Center, Yokohama, Kanagawa
Materials and Methods Two hundred and twelve electroencephalograms (EEGs) in 47 children (20 males and 27 females, aged 3 months to 11 years old) of acu te encephalitis were studied. The etiology of encephalitides were unknown in 17 cases, measles in 11, rubella in 9, varicella in 3, mumps in 3 and others in 4. Three cases died. Fifteen cases cured with sequelae (including 8 cases with postencephalitic epilepsy) and 29 cases withou t sequelae.
Abnormality of EEG basic activity according to graded by Aoki-Lombroso's criterion and occurrence of paroxysmal discharges were studied respectively about their relation to prognosis.
Result The grade of abnormality of EEG basic activity recorded for the first time within one week after onset of encephalitis had no correlation with prognosis. The change of grade of basic activity between two records of EEG within one week was well correlated with prognosis. Rapid deterioration of basic activity within one week was seen in cases of death and with severe sequelae.
In cases with sequelae, normalization of EEG basic activity was delayed compared with cases without any sequelae, in which rapid normalization was seen in several weeks after onset of encephalitis.
Paroxysmal discharges were seen in 33 .3% of cases of which first EEG records were taken within one week after onset of encephalitis. In a half of those cases with paroxysmal discharges sequelae of postencephalitic epilepsy occurred. On the other hand, postencephalitic epilepsy was seen only in one case in those with no paroxysmal discharges in the first EEG records within one week.
In all but one cases with epileptic sequelae, occurrence of paroxysmal discharges in EEG preceded to clinical attacks. There occurred no epileptic sequelae in 16 cases with no paroxysmal discharges in all the serial EEG records.
It is emphasized that only one EEG record at the onset of encephalitis is not a good clue to predict its prognosis and the continuous follow-up of EEG change during and after disease is important.
CT Diagnosis of Acute Encephalitis Kimio Ogawa, MD, Shuichi Hibio, MD, Hiroshi Suzuki, MD, Osami Okubo, MD, Yasufumi Utsumi, MD and Motomizu Ariizumi, MD Department of Pediatrics, Nihon University, School of Medicine, Tokyo
To investigate the diagnostic value of brain CT scanning for acute encephalitis, we surveyed 13 patients by serial CT scanning. The 13 patients included, 6 with herpes simplex encephalitis, one each with ECHO 3 encephalitis, mycoplasma meningoencephalitis, measles encephalitis and rubella encephalitis, and three with acute encephalopathy. In the 6 patients with herpes simplex encephalitis, CT scans taken 3 or 4 days after the onset showed diffuse low density of the cerebrum. And some pictures revealed a localized low density area of the temporal lobe and/or frontal lobe, and furthermore hemispheric low density. In ECHO 3 encephalitis, on the 3rd day the CT scan showed diffuse low density, and a slightly intensified left hemisphere. The next CT scan taken on the 10th day showed the low density still more and on the 52nd day the CT picture indicated cortical and ventricular atrophy. As regards rubella encephalitis, CT scanning showed diffuse low density on the initial day of onset. And the CT finding of diffuse low density predominant in the right hemisphere was seen on the 13th day. On 24th day, the CT picture showed a localized low density area of the right frontal lobe. The other acu te encephalitides had similar changes to those described above in the serial CT findings. As to the CT findings in acute encephalopathy, there was diffuse and symmetric low density at an early time but no localized low density area. Then the follow-up CT showed cortical atrophy in some pictures but it was symmetric.
In this study, we found that these serial CT findings of each encephalitis showed a similar course to that of herpes simplex encephalitis. On CT pictures it is difficult to differentiate acute encephalitis from acute encephalopathy. When early and follow-up CT scanning was performed and diffuse low density with assymmetry or localized low density areas is found, we should think it to be acute encephalitis.
Ultrasonography of the Brain in Cases of Herpes Simplex Encephalitis and Cytomegalovirus Encephalitis Nozomi Matsumoto, MD, Sadayuki Yano, MD, Masu· tomo Miyao, MD, Masayoshi Yanagisawa, MD, Shige· hiko Kamoshita, MD and Kohichi Itoh, MD Departments of Pediatrics (NM, SY, MM, MY, SKj, Clinical Pathology (Klj, Jichi Medical School, Tochigi
It is possible to detect intracranial lesions by ultrasonography using the anterior fontanel as the acoustic window in infants and children. Recent advances in technology have enabled us to get even more precise images of the brain structures. We report cases of herpes simplex encephalitis and cytomegalovirus encephalitis which we have followed up with the use of ultrasonography.
Methods We used an Aloka SSD 200B mechanical sector scanner for this study. For each case, coronal sections and sagittal sections of the brain were examined.
Results and Conclusion In the case of herpes simplex encephalitis of perinatal origin, using ultrasonography of the brain, we were able to observe the changes of the brain from the stage of mild ventricular enlargement to the stage of multicystic lesion of the brain.
With CT scanning, in the stage of multicystic lesion of the brain, it was difficult to differentiate the enlarged ventricles from the cystic brain lesions even by comparing the Haunsfield number. With ultrasonography, these cystic lesions were clearly distinguishable from the enlarged ventricular system. This must be due to the different characteristics of CT scanning and ultrasonography. In the case of congenital cytomegalovirus encephalitis, the characteristic periventricular calcification, enlarged ventricles and small calcification lesions of the brain were observed by ultrasonography and CT scanning confirmed these findings.
We stress the following points: Ultrasonography is a non-invasive, simple precedure that can be done repeatedly even on infants in critical conditions. Owing to the characteristics of the sound beam, contrast between the ventricles and cystic lesions of the brain which is difficult to get with CT scanning is clearly defined with ultrasonography. Calcification of the brain can be detected with ultrasonography.
A Case of Brainstem Encephalitis Caused by Herpes Hominis Type I Akiko Kosugi, MD, Kazuhiko Tanaka, MD, Hisashi Yamamoto, MD, Shosaku Yamazaki, MD, Noriaki Ohya, MD and Morimi Shimada, MD Department of Pediatrics, Shiga University of Medical Science, Ohtsu, Shiga
Herpes encephalitis typically appears as an acute necrotizing encephalitis involving the temporal lobe, extending into the orbital portion of the frontal lobe and it is known as a cause of brainstem disorders in rare cases. Herpes encephalitis has a high mortality rate and there is a high incidence of neurological sequelae in survivors.
In this study we report a rare case (7th case in the world) of brain stem encephalitis due to herpes hominis. This case was successfully treated by a combination of interferon therapy and fresh blood transfusion, the blood contained a markedly high titer of neutralizing antibody to this virus.
A 5-year-old girl was admitted to this hospital because of dyspnea, dysarthria and lethargy. On admission, she had mild disturbance of consciousness and marked bulbar paralysis. A CT scan of the. skull suggested neither space occupying lesions nor vascular lesions. An electroencephalogram showed slow wave activity, predominantly in the parieto-occipital area. CSF showed 25 mononuclear cells. She was subsequently diagnosed as having brainstem encephalitis. Detailed examination of the family history suggested an intfafamilial outbreak of herpes hominis.
On the 4th hospital day, transfusion of fresh blood with a high titer of neutralizing antibody to herpes hominis type I and intravenous administration of interferon (total 4,000,000 IU) were started. Parenteral hyperalimentation was also commenced because of difficulty in oral feeding. She recovered without notable neurological residues by these treatments and was discharged on hospital day 24.
Results for the familial serum titer of neutralizing antibody to herpes hominis type I were as follows; the patient's titer rose from 1: 14 on the 5th hospital day to 1: 6 8 on 5 days after discharge. The mother's titer was 1: 1,260. Her sister's and grandmother's titers were 1: 135 and 1: 3 80, respectively.
Isolation of Herpes Simplex Type 1 from a Biopsy Specimen from an Infant with Encephalitis Mariko Okamoto, MD, Hideo Nagao, MD, Mitsugi Takahashi, MD and Hiroshi Matsuda, MD Department of Pediatrics, Ehime University School of Medicine, Matsuyama, Ehime
Case Report A 8-month-old girl, who had suffered from varicella one month before, was admitted because of convulsions on June 23, 1981. Six days prior to admission, she developed fever, and the next day she had a convulsion in her right leg, which spread generally, and the level of her consciousness decreased. On admission she was comatose, the anterior fontanel was not distended, and there was no meningeal sign. The deep tendon reflexes were increased slightly but pathologic reflexes were not observed. Cerebrospinal fluid was within the normal limits and an electroencephalogram revealed periodic sharp waves. Computerized tomography showed a high density area in the left parietal lesion, surrounding a low density area, and streak linear enhancement with contrast enhancement. Left cerebral angiography showed no remarkable change. To decrease the intracranial pressure, craniotomy was performed. The brain was hyperemic and a biopsy was done for virologic studies. Then a small amount of blood was removed by puncture. We diagnosed her as herpes simplex encephalitis clinically, and adenine arabinoside therapy was started after the biopsy. After one year, she was in a state of spastic paralysis. The herpes simplex complement fixation titer in the serum was 1:32 on the 7th day of her illness, and more than 1: 4,096 on the 20th day. In the cerebrospinal fluid, it rose from less than 1: 1 to more than 1: 64.
Virologic Studies The brain biopsy specimen was submitted to immunofluorescence staining with FITC-anti herpes simplex virus and to virus isolation with 4 strains of cultured cells. The smeared specimen showed positive immunofluorescence staining for the herpes simplex antigen. For virus isolation, the homogenized biopsy specimens were inoculated with 4 strains of cultured cells (Vero, HEL etc). A few days after infection, all of the inoculums showed the typical cytopathic effect of herpes simplex virus, a rounding of cells. A neutralization test with type-specific antisera (anti-HSV type 1 and type 2) demonstrated that all of the isolates were herpes simplex virus type 1. This assignment was confirmed by the host range specificity of herpes simplex virus type 1 and type 2 toward Vero cells and chicken embryo cells.
This is the first case in Japan of a diagnosis made by virus isolation from a biopsy specimen.
A Case of Varicella Encephalitis with Low Density of the Temporal Lobe on CT and Periodic Discharge in EEG Hideo Inoue, MD Department of Pediatrics, Okayama Saiseikai General Hospital, Okayama
A low density of the temporal lobe seen on CT scanning with periodic discharge in EEG has been thought to be pathognomonic in herpes simplex virus encephalitis.
Almost the same findings on CT and EEG were seen in a 2-year-old boy, who suffered from varicella 10 days prior to admission, and developed high fever and generalized convulsion. There was nuchal rigidity, and CSF revealed moderate pleocytosis (156/3 mm3 ).
His consciousness gradually deteriorated and he became comatose with frequent myoclonic jerks on the 7th hospital day. He began to improve on the 14th hospital day without any medication including adenine arabinoside, and recovered well except for minimal left hemiparesis. A virological study revealed an elevated CF titer against varicella zoster virus, but not against herpes simplex virus in serum and CSF.
CT scanning showed a low density of the right temporal lobe on the 8th hospital day, which lasted for 3 months, and gradually disappeared thereafter. Streak-like enhancement was not demonstrated. EEG showed characteristic periodic discharges, with 1.5 to 3 seconds intervals, although transiently, at the right temporal lobe on the 8th hospital day and this disappeared within a week.
Left hemiparesis had completely improved 8 months later, while EEG still revealed frequent seizure discharges on both hemispheres, and a low density on CT scanning was minimally identified.
We can conclude that this patient had had varicella zoster virus encephalitis, whose CT scanning and EEG findings quite resembled those of herpes simplex virus encephalitis.
A Case of Nasopharyngeal Carcinoma with Intracranial Invasion in Which Anti-EB Virus Antigen and Titers of Antibodies were Detected in CSF Atsushi Nishikado, MD, Toshio Heike, MD, Tsuyoshi Maruyama, MD, Masafumi Wada, MD and Shozo Torii, MD Tazuke Kofukai Foundation Medical Research Insti· tute, Kitano Hospital, Osaka
Epstein-Barr virus (EBV) is well known for its relationship with Burkitt lymphoma and nasopharyngeal carcinoma (NPC). Recently encephalitis and meningitis caused by EBV have been reported. Nevertheless there are few reports of EB antigen and anti-EBV antibodies in CSF. We present a female patient of NPC with intracranial invasion and report the results of studies on the effect of EB nuclear antigen on the tumor cells and the titers of anti-EBV antibodies in CSF.
Case Report An ll-year-old female. Two years ago she suffered from symptoms of increased intracranial pressure and cranial nerve paralysis. Partial resection of the tumor which had invaded the cranial bone was performed and it was histopathologically demonstrated to be NPC of the undifferentiated type. Subsequently irradiation and chemotherapy were performed. Two years after the resection she complained of malaise, anorexia and headache. At that time the serum EBV anti-VCA IgG antibody and anti-EBNA antibody were 1:10,240, respectively, which were abnormally high values. In CSF they were 1: 16 and 1: 4. CSF cell counts varied from 1O/mm3 to 130/mm3
• EBNA antigen was detected in the tumor cells of NPC in the CSF. Two years after the first onset, cervical lymphnode metastasis was demonstrated by node biopsy and irradiation was performed again. The tumor cell mass got less transiently. Six months later destruction of the atlas was demonstrated by CT scan and she died soon after.
Serial Studies on Serum Amino Acids in Acute Encephalopathy Hideaki Sogawa, MD, Toshio Ohara, MD, Takuo Aoyama, MD and Yasunori Toyoda, MD Department of Pediatrics, Aomori Prefectural Central Hospital, Aomori
A unique serum amino acid pattern has been noted in Reye syndrome. The most striking and consistent elevations in serum concentrations are those of glutamine, alanine, o<-amino-N-butyrate and lysine. We recently encountered a patient with acute encephalopathy associated with impaired liver function, which was strictly different from Reye syndrome. Serial studies on amino acids in our patient were compared with those in Reye syndrome.
The case is a 2-year-7-month-old mentally and physically retarded female infant. Her main clinical data were; 1) sudden onset of vomiting, high fever and generalized convulsions, 2) rapid development of encephalopathy with progression to coma within 6 hours, 3) impaired liver function with elevation of SGOT and SGPT, which took 3 months to recover to normal, 4) fatty degeneration in the biopsied liver specimen on the 17th day, and 5) elevation in serum concentrations of alanine, proline, glycine and lysine on the 14th day despite a normal pattern on the third and 59th day. The serum amino acid pattern in our case resembled the hyperaminoacidemia which has been reported in Reye syndrome.
A unique pattern of hyperaminoacidemia may be specific not only to Reye syndrome, but it may also be found in acute encephalopathy with fatty degeneration of the liver.
Key words: Acute encephalopathy, R eye syndrome, h yperaminoacid em ia.
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Computerized Tomography in Acute Toxic Encephalopathy- Report of Three Cases with Unusual Features Nobuhiko Aoki, MD, Masashi Mizuguchi, MD and Eiji Kurihara, MD Departments of Neurosurgery (NA), Pediatrics (MM) , Fuchu Metropolitan Hospital, Toky o; Department of Child Neurology , Metropolitan Neurological Hospital, Tokyo (EK)
As far as the authors aware, no other CT findings for acute toxic encephalopathy other than Reye syndrome have been reported to date, and in fact, only a few workers have so far reported their CT findings on Reye syndrome in an acute stage.
We experienced three cases of acute toxic encephalopathy (case 1, a 15-month-old girl, case 2, a 5-year-7-month-old girl, and case 3, a 9-month-old girl), including one case of probable Reye syndrome (case 2), which presented similar and unique CT findings in their acute stage; symmetrical low dense areas in the thalami and the dentate nuclei, followed by changes in the cerebellar hemispheres and around the lateral ventricles.
No findings for such diseases have been reported, however, and the pathogenesis of brain damage in probable Reye syndrome and other acute toxic encephalopathies which are currently shown by common CT features may suggest a correlation between the two different diseases relating to brain damage.
The authors propose that the present cases are a new subgroup of acute toxic encephalopathy, because of their similar and unique CT features.
A Case of Reye Syndrome Complicated with Hemorrhagic Pancreatitis after Mumps Hoshun Sai. Yuji Morikawa. MD. Kyoko Miyoshi. MD. Hitoshi Izumi. Koichi Kimura. Kazumi Goto. Ryuzo Mizuta. MD. Masaharu Ochi. MD and Naoki Arizono. MD Department of Pediatrics. Kyoto Second Red Cross Hospital. Kyoto (HS. YM. KM, HI. KK. KG. RM); Departments of Pediatrics (MO) and Pathology (NA). Kyoto Medical School. Kyoto
Recently we have experienced a case of Reye syndrome, that occurred after mumps, revealing acute hemorrhagic pancreatitis at autopsy.
Case On July 14, 1981, a 2-year-old girl was diagnosed and treated as mumps. On July 24 dyspnea appeared, soon followed by loss of consciousness and generalized convulsions. She was admitted on the same day. Laboratory data recorded on admission included serum glucose 11 mg/dl, GOT 535 U, GPT 884 U, LDH 1,760 U, NH 255 mg/dl, and S-amylase 78 U. The cerebrospinal fluid contained 27 Iymphocytes/mm', glucose 9 mg/dl, and protein 222 mg/dl. After admission serum glucose rose progressively and recorded 660 mg/dl on the fourth hospital day. The titer against mumps in serum was 128 times, and that in CSF was 1 time. The patient was treated with glucose, anticonvulsants, corticosteroids, management of ICP, and other supportive cares. In spite of these therapy, she remained comatose and died 6 days after admission. Postmortem examination revealed cerebral edema without inflammation, fatty degeneration of the liver, and parotitis, as well as acute hemorrhagic pancreatitis with peritoneal hemorrhage.
Discussion Although a large number of viruses have been associated with Reye syndrome, there are very few reports on Reye syndrome following mumps, and complication with hemorrhagic pancreatitis is also rare. The combination of these two is interesting. As the causes of acute hemorrhagic pancreatitis infection, drugs, Reye syndrome must be considered. Generally the infection is bacterial, and pancreatitis complicating mumps is often mild, rarely becoming hemorrhagic. In Reye syndrome changes in the pancreas are very slight or not detectable. Hemorrhagic pancreatitis in Reye syndrome usually occurs in the course and in our case it occurred after the second hospital day. For this reason, iatrogenic causes must be considered, too. Hemorrhagic pancreatitis caused by steroids is usually observed with much larger doses and longer periods. A combination of abnormal glucose metabolism in Reye syndrome, mumps infection, and steroid therapy may be operative to produce severe pathologiC changes in the pancreas.
A Case of Acute Encephalopathy with Symmetric Hypodense Areas in Bilateral Thalami onCT Eiji Kurihara. MD. Yoshihiko Mizuno. MD. Kazuhiko Komiya, MD and Yasuko Hasegawa. MD Department of Pediatrics. Tokyo Metropolitan Neurological Hospital. Toky o (EK. YM. KK); Department of Pediatrics, Kyorin University. School of Medicine. Tokyo (YH)
Abstract not received
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Cerebral Infarction in Purulent Meningitis Shigeru lino, MD, Katsuhiko Fujiwara, MD, Masaharu Ochi, MD, Akira Yoshida, MD, Hiroshi Yoshioka, MD, Tomoichi Kusunoki, MD, Yasuo Kasubuchi, MD, Hiroshi Takada, MD and Ryuzo Mizuta, MD Department of Pediatrics, Kyoto Prefectural University of Medicine, Kyoto (SI, KF, MO, A Y, HY, TK); Department of Pediatrics, Ohtsu City Hospital, Oh tsu , Shiga (HT); Department of Pediatrics, Kyoto Second Red Cross Hospital, Kyoto (RM)
We have already reported that meningitis patients with cerebral infarction developed serious neurological sequelae_ Here we describe the clinical and CT features of purulent meningitis complicated by cerebral infarction_
Material and Method Forty-one children with purulent meningitis were examined by CT in the acute stage of the illness_ According to CT findings in the acute stage, patients were divided into 4 groups: group 1, normal (14); group 2, subdural effusion and/or ventricular dilatation (16); group 3, cerebral infarction (9); group 4, miscellaneous (2). In the clinical course, group 3 was compared with group 1 and group 2. Furthermore, in the 9 cases in group 3 evolution of cerebral infarction was studied by serial CT.
Nine cases (22%) had cerebral infarction. Clinical features of the cases with cerebral infarction were as follows: the age of onset was below one year. In the majority of cases the cause was group B Streptococcus. Convulsions, especially those persisting for more than 24 hours after adequate treatment, were seen frequently. Unilateral convulsions and/or paresis were seen frequently. CT manifestations in the cases with cerebral infarction were as follows: cerebral infarctions were often seen in the supply area of the middle cerebral artery and the so-called "border zone" in the fronto-parietal region. CT usually failed to demonstrate any abnormal finding in the first week after the onset. The infarcted lesion appeared on CT in 2-3 weeks as hyper- or hypodense areas. Thereafter, they were revealed as homogenous hypodense areas.
Deafness and Ataxia in Children due to Meningitis Sadayasu Akai, MD, Kimitaka Kaga, MD and Yoshisato Tanaka, MD Department of Oto-Rhino-Laryngology, Teikyo University, Tokyo
We investigated the auditory and vestibular function of 18 deaf children who suffered from meningitis and visited the Department of Otorhinolaryngology of Teikyo University Hospital from 1975 to 1981. Their auditory function was tested by conditioned orientation reflex audiometry and auditory brainstem response. The vestibular function tests undertaken were; 1) the damped rotation test for 12 cases, 2) the optokinetic pattern test for 5 cases, and 3) the caloric test for 2 cases.
The results obtained were as follows: 1) Severe hearing loss was found in all cases. 2) The cases who had suffered from meningitis in
early infancy lost articulation and language because of loss of hearing.
3) The damped rotation test revealed vestibular disorders (hypofunction in 8 cases, afunction in 2 cases and normal function in 2 cases). The optokinetic pattern test revealed normal function for all cases and caloric irrigation was unresponsive in two cases. These results suggest that the ataxia of the patients was caused by vestibular dysfunction of the labyrinth. Surprisingly it was more than 6 months after most
cases had suffered from meningitis that the complication of deafness was found. Meningitis should be considered to have complications of auditory and vestibular disorders. The early diagnosis of such disorders is expected for patients by physicians.
A Case of Infantile Tuberculous Meningitis Who Recovered on Internal Treatment with INH and RFP Koichi Kimura, MD, Kazumi Goto, MD, Hoshun Sai, MD, Hitoshi Izumi, MD, Yuji Morikawa, MD, Kyoko Miyoshi, MD, Ryuzo Mizuta, MD and Yasumasa Yamamoto, MD Departments of Pediatrics (KK, KG, HS, HI, YM, KM, RM), Neurological Medicine (YY), Kyoto Second Red Cross Hospital, Kyoto
We have experienced a case of a female infant, 9 months of age, who recovered from tuberculous meningitis on internal treatment with INH and RFP. The patient's family history revealed no tuberculous disease. Since 5 days before admission a remittent fever of 38-39°C had continued, simultaneously membranous desquamation of palmar skin and swelling of cervical lymph nodes were noted, and the patient was admitted on suspicion of MCLS. The blood sedimentation rate was 24 mm/hour, CRP 2 (+), Hb 9.2 g/dl, leukocyte count 13,300/mm3 , biochemical tests showed no abnormalities, blood and urine cultures were negative, tuberculin reaction was positive (10 x 10 mm) and chest X-rays revealed no abnormality . The fever also continued after admission, and 3 days after admission right hemispasm and paresis of the right upper limb appeared. The EEG showed lazy activity of the left cerebral hemisphere, and the CT scan a hemorrhagic-like infarction in the area of the left middle cerebral artery . In the initial examination of spinal fluid, the cell count was 52/3, but in the second examination the cell count was 692/3 (polynuclear 136/3, mononuclear 556/3), protein 59 mg/dl, glucose 29 mg/dl, tryptophan reaction (-), smear test of Myco. tuberculosis negative, fever continued without spasms, tuberculous meningitis was suspected on account of a positive tuberculin reaction for the second time (12 x 14 mm), and the internal treatment with INH and RFP was started from the 12th day after admission. Clinical symptoms improved soon after the administration, and CT and spinal fluid findings improved slowly , too. One month and a half later, Myco. tuberculosis was detected in the cultures of spinal fluid and gastric juice, and the diagnosis of tuberculous meningitis was established. It is difficult to diagnose tuberculous meningitis early, considering that it is difficult to check the adverse reactions of SM and EB in children; when this disease is suspected, we think at least internal treatment with INH and RFP should be started early, and one should wait for the results of cultures of Myco . tuberculosis.
Key words: Tuberculous meningitis, RFP.
210 Brain & Development, Vol 5, No 2,1983
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The Mechanisms of Brain Damage due to Postmeningitic Communicating Hydrocephalus Hiroshi Suzuki, MD, Kimio Ogawa, MD, Shuichi Hibio, MD, Kazuhiko Goto, MD, Hiroaki Shiihara, MD, Osami Okubo, MD, Yasufumi Utsumi, MD, Motomizu Ariizumi, MD and Saburo Nakamura, MD Department of Pediatrics (HS, KO, SH, KG, HS, 00) and Neurosurgery (SN), Nihon University, School of Medicine, Tokyo
We experienced 78 cases of bacterial meningitis from 1972 to 1981. Of the 24 cases of below 1 month of age, 8 e xpired and 5 had hydrocephalus as a sequela. Out of 53 cases aged 2 months and more, 5 expired and 4 had hydrocephalus as a sequela. Communicating hydrocephalus ensued in 11 cases out of 13 including 4 cases of postmeningitic hydrocephalus from other hospitals.
CT pictures obtained from 1 to 4 years after a shunt operation could be classified according to the severity of brain damage into 5 groups; (1) normal-slit ventricle, (2) cortical atrophy, (3) ventricular atrophy , (4) focal ventricular dilatation, and (5) hydroencephalodysplasia. This classification fairly well coincided with the neurologic prognosis. The cases belonging to groups (1) to (4) showed the possibility of further motor development but the cases belonging to group (5) remained quadriplegic. Out of 6 cases in groups (4) and (5), 5 were below 1 month of age.
The cases with hydrocephalus generally experienced convulsions and consciousness disturbance at an early period of the disease and had pyramidal signs in the course of the disorder. The nystagmus and sunset phenomenon meant grave neurological sequelae and involuntary movements were the preceding sign of minor motor seizures.
The protein value in the CSF of the cases of below 1 month of age was higher throughout the total course of the disease than that of the cases aged 2 months and more. The serial variation of the chlorine value in CSF was different between the cases of below 1 month of age and those aged 2 months and more.
The reasons for the inclination toward hydrocephalus and severe brain damage in the cases of below 1 month of age were: (1) the lack of typical clinical features which masked the disease, (2) the scarce diffusibility into CSF of the antibiotics used, and (3) the immature physiological functions of the brain such as the blood brain barrier by which the inflammatory reaction in the brain is facilitated.
Because the prognosis was determined from the severity of brain damage in the acute phase, the early diagnosis of bacterial meningitis below 1 month of age and the appropriate selection of antibiotics could lessen the motor disability resulting from communicating hydrocephalus although it is not the case about mental retardation and intractable epilepsy.
Key words: Hydrocephalus, bacterial meningitis.
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Fisher Syndrome with CSF Pleocytosis and EEG Abnormalities Yoichi Sakakihara, MD, Akiko Hosaka, MD, Yoshiko Nomura, MD and Masaya Segawa, MD Department of Pediatrics, Faculty of Medicine, The University of Tokyo, Tokyo (YS, AH); Segawa Neurological Clinic for Children, Tokyo (YN, MS)
A previously healthy 3-year-old boy suffered from moderate pyrexia and parotid swellings 6 days before admission. He then developed ptosis of the left eye and bilateral ophthalmoplegia. He was admitted to our hospital because he had developed gait disturbance 2 days before admission.
The patient was a pyrexic, mentally clear boy, without any sign of speech disturbance. He showed an ataxic gait which was not accentuated by closing of the eyes. His bilateral parotid glands were moderately swollen. The eyes, which were in the midposition, were able to move only to the left. The pupils were isocoric, and the light reflex was present. However, convergent eye movement and Bell's phenomena were not elicited. Subtle horizontal jerky nystagmus was observed when the eyes rotated to the left. All cranial nerves except the 3rd, 4th and 6th were intact. Deep tendon reflexes were not elicited at all . Mild hypotonia of the muscles of the extremities was present, but muscle weakness was not observed. There was no meningeal irritation, sensory disturbance, or sphincter dysfunction.
Routine laboratory studies were normal except for nonspecific inflammatory changes (leukocytosis, positive CRP). A Tensilon test revealed no improvement of the blepharoptosis. Nerve conduction studies and auditory brain stem responses were normal. Right parietal sporadic spikes were observed on EEG, but the basic activity was normal. The cavum septi pellucidi was seen to be delineated on CT. A cerebrospinal fluid examination was performed on the 8th hospital day , and marked pleocytosis (174/3) with mononuclear cell dominance was present . Protein, glucose and chloride were within normal limits. An 8-fold rise of the Coxsackie Bl virus titer was observed in paired sera of the patient.
The symptoms of the patient were compatible with the diagnosis of Fisher syndrome. But the findings, such as CSF pleocytosis and EEG abnormality, suggested involvement of the brain parenchyma. Considering the 8-fold rise of Coxsackie B 1 virus titer as a matter of significance, it may be possible to diagnose the patient's illness as brain stem encephalitis. There has been much dispute on the lesion of Fisher syndrome, but the precise lesion is not known yet because there have been few autopsy cases. Our case suggests probable involvement of the brain parenchyma in Fisher syndrome.
Cerebrovascular Disease in Childhood Kenji Yazawa, MD, Ryota Hosoya, MD; Tatsuo Ohya, MD, Kozo Nishimura, MD, Ryoichi Ishikawa, MD, Isao !toh, MD, Kazuhiko Iwashita, MD and Kiyotaka Tomiwa,MD Department of Pediatrics (KY, RH, TO, KN) and Neurosurgery (RI, II, KI), St. Luke's International Hospital, Tokyo; Department of Pediatrics, Faculty of Medicine, Kyoto University, Kyoto (KT)
Cerebrovascular disease (CVD) is relatively rare in childhood. And the clinical course and prognosis in children whose CNS is still maturing are different in many aspects from those of adult. Clinically acute hemiplegia is a major characteristic symptom of CVD as well as seizures. The term "acute infantile hemiplegia" has been used for this disorder for a long time.
Thirty-two cases of CVD or possible CVD with underlying diseases admitted to our hospital from 1967 to 1981 were the subjects for the analysis; acute infantile hemiplegia (AIH) 10, arteriovenous malformation (AVM) 2, moyamoya disease (MMD) 5, brain embolism (EMB) 4, postictal hemiplegia (PIH) 5, post traumatic hemiplegia (PTH) 3 and encephalopathy (ENC) 2. The mean age for each disease varied. AIH showed the youngest mean age of 14 months, and MMD had the oldest mean age of 9 yrs 7 mos. Of these 32 cases, 30 cases showed hemiplegia, 19 episodes on the right side and 13 episodes on the left side. The course of hemiplegia, EEG, brain CT scan and CAG were analyzed.
AIH is defined as hemiplegia of acute onset without any known perinatal and neurodevelopmental abnormalities, and PIH as that with possible underlying disorders before. AIH had relatively good prognosis with few clinical sequelae, although this was not seen in laboratory findings including EEG and brain CT scan. About 40% of the brain CT scans of the AIH group showed some abnormalities later inspite of normal findings at the acute phase. PIH and other hemiplegias with possible underlying disorders are worse than AIH in prognosis and laboratory findings.
The definition of AIH seemed different from study to study in the past. And the difference between AIH and PIH is stiU unclear. In order to get a definite prognosis of AIH, clear-cut criteria for this disease will be definitely necessary in the future. MMD had a variety of symptoms at the onset. Angiography was the only definitive study for diagnosis. A suspicious history for MMD with abnormal EEG characterized by abnormal build-up in hyperventilation is the indication for angiography even if the brain CT scan is normal.
