Some authorities are favourable to put together postvaccinal encephalitis, post-measles encephalitis and some encephalopathies after prophylactic treatments under the concept of secondary enceph alitis. But I preferas a virologist to regard postvaccinal encephalitis as a modified infection with vaccinia virus, in another hand, postmeasles encephalitis as a type of infections of nervous system with measles virus. Because of the dissociation between etiology and pathogenesis, the morphological concept of secondary encephalitis is not so good for making clear the etiology of these encephalitides.
This report was based on 32 cases with involvement of the central nervous system-encephalitis or encephalopathy in association with an attack of measles, which were observed at the Department of Pediatrics of Keio University and Tokyo Women's Medical College f rom January 1959 to March 1970. 1) The diasease was more frequent at a younger age. 20 cases (62.5%) were in the 7 months-4 years of age group. 2) The onset of central nervous complications occurred most commonly between the third and fourth day after the first apperance of the rash. 3) The material was classified according to the clinical types, which had been previously shown by Ford, F. R. Symptoms of diffuse cerebral involvement of brief duration was observed in 12 cases (37.5%), signs of multiple focal or diffuse lesions in the nervous system in 8 cases (25.0%), signs of single focal cerebral lesions in 8 cases (25.0%), cerebellar syndromes in 1 case (3.1%), and spinal syndromes in 3 cases (9.1%), optic neuritis was none. 4) All of 11 cases with signe of single focal cerebral lesions and spinal syndromes were under 4 years of age. 5) The most frequent signs and symptoms in the acute stage of the disase were loss of consciousness (90.6%), convulsions (65.6%), pathological reflexes (65.6%), and exaggerated deep reflexes (43.8%). Other signs and symptoms noted, but less frequent, were vomiting, Kernig's sign, absent or sluggish deep and superficial reflexes, and ankle clonus. Hemiplegia was observed in 8 cases (25.0%). 6) The spinal fluid cell count and protein content were nearly always normal under 3 years of age, but extremely variable above 3 years of age. 7) There was one death in our series of 32 cases, of the 31 survivors 21 cases (43.8%) recovered completely, 17 cases (53.1%) had neurological sequelae. The prognosis was good in symptoms of diffuse cerebral involvement of brief duration, but poor in signs of multiple focal or diffuse lesions in the nervous system and single focal cerebral lesions. The commonest sequelae were paralysis (11 of 17 cases, 64.7%), mental deterioration (6 cases, 35.3%), and convulsion (4 cases, 23.5%). 8) The clinical features of prognostic significance regarding neurological sequelae were: (i) patients under 3 years of age; (ii) prolonged deep loss of consciousness; (iii) continuous or recurrent convulsions; (iv) Hemiplegia. Whereas the onset of central nervous complications in relation to the rash, hyperpyrexia, and spinal fluid findings seemed to bear no relation to the prognosis. 9) An analysis of the relationship between the sequelae and treatment (ACTH or steroids, γglobulin) was not possible because of the relatively small number of cases in each group.
Asu survey was made on 63 patients of the secondary encephalitides admitted to our department during the ten years from the clinical and immunological view points. Etiologically, sixty-three patients consist from 13 of measles, 12 of mumps, 11 of varicella, 5 of rubeo1a infantum, 2 of herpes simplex, 1 of rubella, 5 of other exanthematous infection, and 14 of vacclnatlon. Although the incidence, age of onset, latent period, prognosis, and sex ratio were variable dependingon the each antecedent infection, most patients be1onged to the age between 4 and 5 years An interesting relationship was found between the latent period, sex incidence, and the clmical forms. The cases with latent period over 8 days were predominantly female and encephalitic form and those within 8 days were male and meningitic form. In some recent cases including 2 of subacute sclerosing encephalitis and one varicella encephalitis, immunoglobulin-G (Ig-G) concentration, Ig-G/albumin ratio, and the activity of macrophage inhibition factor (MIF) were determined in CSF.Cases with SSPE and varicella encephalitis had higher level of Ig-G, Ig-G/albumin ratio, and MIF in CSE These findings suggested that some immunological reaction took place in the central nervous system as a cause of these encephlitides.
During 1967 to 1970, 65 cases of postvaccinal central nervous system diseases were reported to Smallpox Vaccination Committee. Among these, 40 cases were classified as postvaccinal encephalitis or encephalopathy. Incubation periods of these cases were between 4 to 17 days (mean 8.7 days). 31 cases were under 1 year of age, 8, 1 year and 1, 12 years of age, respectively. These case distribution accounted for age were thought to be paralleled to age distribution of smallpox vaccination performed Though it would be often difficult, from the clinical grounds, to distinguish encephalopathy from encephalomyelitis, 14 cases were thought to be typical encephalopathy, and many of the others showed only slight cerebrospinal fluid abnormality. It was suggested that the clinical manifestations of those very young children were belonged to encephalopathy. The prognosis were relatively good, 20 were completely recovered, 3 died and 16 left central nervous system sequeles. It was thought that few severe or fulminant cases were reported here. The other types reported were, mild transient encephalopathy 3, acute infantile hemiplegia, 3, aseptic meningitis type, 11, myelitis, 1 and neuropathy, 2.
76 cases with neurological complications following Japanese B. encephalitis vaccination were examined. Age of onset of the neurological manifestations varied from 9 months to 61 years, 31 of 76 cases were under 5 years. 44 cases were male and 32 were female. Among 76 cases with neurological complications, 66 had signs and symtoms of lesions of the central nervous system and 10 had clinical features of lesions of the peripheral nervous system. 66 cases with lesions of the central nervous system were classified into three groups; 13 cases with transitory convulsion, 38 cases with encephalopathy or meningoencephalomyelitis and 15 cases with occurrence or exacervation of preexisting illness. Among 38 cases with clinical features of encephalopathy or meningoencephalomyelitis, 14 cases hadonset of the symptoms within 2 days after inoculation and 24 cases had onset from 3 to 19 days after inoculation. Neurological symptoms occurred with first inoculation in 10 cases and with second or subsequent inoculation in 13 cases. 13 cases recovered completely, 19 were left with sequelae and 6 died. 13 cases with transitory convulsion had no history of epilepsy or febrile convulsion. Time interval from inoculation to the onset of convulsion was within 2 days in 8 cases. 13 cases with transitory convulsion had no history of epilepsy or febrile convulsion. Time interval from inoculation to the onset of convulsion was within 2 days in 8 cases. 10 cases with clinical features of lesions of the peripheral nervous system had all polyneuritis. In 5 cases whose injection order was given, all had neurological symptoms with second or subsequent injection.
Acute encephalomyelitis following vaccination and measles is representative of secondary encephalomyelitides which appear after acute general infection. In their pathomorphology focal proliferation of microglia and histiocyte around the vene Predominates in the whole central nervous system together with scarse lympho-histiocytic perivenous cuffs. Myelin sheath is also vulnerable within the lesion. Based on the characteristics of these pathological changes, secondary encephalomyelitides have been considered as caused by an abnormal immune response allergic reaction of the host, to the primary infection. This concept seems to be supported by the facts that the same lesion can be evoked irrespective of a variety of causative agents without success in recovery of infective virus from nervous tissue of the patient, and that experimental allergic encephalomyelitis has pathological features almost similar to those of secondary encephalomyelitis Recent research progress on etiopathogenesis of subacute sclerosing panencephalitis clearly indicates that merely morphological identification of various pathological conditions should be made cautiously. Aneffort of further investigation would be required from virological view point.
A case of 9-days-baby, who was born from a normal delivery, with a unilateral subdural hematoma was reported. He was suffered from the persisted jaundice, repeated vomiting and general malaise. He showed the biparietal cephalohematoma and tense bulging of the anterior fontanel, but neurologically only a right-sided facial weakness. The subdural hematoma was diagnosed with the procedure of subduraltap, subdural pneumography and the Echoencephalography. The Echoencephalography and skull percussion test were most important and beneficial in the diagnosis of the space-taking intracranial hemorrhage in the newborn. He was successfully treated by the trephination operation. Various types of the intracranial hemorrhage in the newborn were reviewed in literature.
A case of Kleeblattschadel syndrome (clover leaf skull) was described. The case was a 4 month old infant, who was born from healthy young parents. He had a grotesque trilobed skull with bulging through the sagittal suture and squamosal sutures. Further, exophthalmos, downward displaced ears, ankylosis of both elbows, deeply recessed nasal root were associated. Skull films revealed craniolacuniae, synostosis of coronal and lambdoid sutures, widely opened sagittal suture and hypoplasia of orbits. Ventriculogram showed marked dilatation of lateral ventricles. Ventriculoatrial shunt was performed. At 1 year and 8 month old, the patient visited the out patient clinic. He was mentally and physically retarded. The OFC was smaller than that of 4 month old. He was unable to stand by himself, but able to walk with assistance. The authors reviewed 19 cases of this syndrome previously reported. This case may be the first case in Japan. A number of cases have associated anomalies in the extremities. This syndrome might be an association of severe acrocephaly and chondrodystrophy. The cause of hydrocephalus is unknown, but it is thought that the synostosis of the basilar cranial bones may obstruct the outlets of the fourth ventricle and produce hydrocephalus in the intrauterine period.
A case of a girl with acquired sensory aphasia of obscure origin was reported. Her family history was noncontributory and physical and mental development was almost normal until the age of 4 years and 7 months, when she developed difficulty in comprehension of spoken language 5 months later of attack of measles. Two days prior to the onset of measles, she was administered on killed measles virus vaccine. Thereafter, spontaneous speech was also lost gradually. No focal neurological abnormalities such as paralysis, agnosia and apraxia were observed and her intelligence seemed to be entirely normal. She was impressed to be very friendly and rather euphoric with anosognosic tendency. Laboratory studies including cerebrospinal fluid, carotid angiography, pneumoencephalography and brain scanning revealed no specific findings. Electroencephalogramm showed right temporal dominant single spike. Repeated audiological examinations revealed almost normal hearing. Recovery of comprehension and expression in speech was very poor, so that special education by written language, and lip reading was commenced. Learning of written language was satisfactory, while lip reading was very diffcult for her. Several problems concerning diagnosis, etiology neurological consideration, and education for the patient were discussed.