The patient was a 6 year and 7 month old boy, who had suffered from measles at the age of 8months, but his mental and motor development had been normal until the onset of the disease. When he was 6 years and 4 months, the following symptoms appeared and progressively aggravated;lack of skill, mental deterioration, myoclonic seizures and extrapyramidal signs such as involuntarymovements and muscle rigidity. Characteristic diffuse, high voltage slow wave bursts appeared periodically throughout the long-run EEG; the serological study showed high titers of measles virus antibody in serum and CSF. Based on clinical and laboratory grounds as mentioned above, diagnosis of subacute sclerosingpanencephalitis was made. Oral administration of Amantadine-hydrochloride 7.5mg/kg/daily, one of the antiviral agents, seemedto be effective in alleviating neurological signs and symptoms in this case.
The patient was a 8-year-1-month old girl. Her parents are cousins. She had had measles at 6months of age and was healthy until age 7 years and 9 months, when psychotic disorders, i. e.disturbance of memory and lethargy began to appear. At age 8 years slurred speech began myoclonus of neck muscles and gait disturbance appearedand developed gradually. With chief complaints of myoclonic jerk, regressive speech, and disturbance of consciousness, shewas admitted on January 26 th, 1970. At age 8 years and 2 months, spasmo-rigidity of entire musclesdeveloped to opisthotonus, responses to painful stimuli gradually diminished and finally coma occured. In the EEG, periodic bursts of high voltage slow with sharp waves were demonstrated, the intervalsof the periodic events ranged from 5 to 6 seconds. Measles titers in serum (NT 1024→4096, HI 16→256, CF 32→128) and CSF (NT 64→612, HI 2→64, CF 8→46) had been elevated from 4 to 7 monthsafter the onset of the disease. Whereas HI and CF titers were slightly elevated, NT titers wereremarkably elevated. Electrophoresis of concentrated spinal fluid samples revealed high levels of r-globul in (39.5%).
The authors report a case of 4 year old boy with subacute sclerosing panencephalitis (SSPE), withunusual immunologic findings. The patient was well until 3 years and 4 months of age, when he developed a generalized convulsion and ataxic gait. About 3 months prior to the onset, he had had a febrile episode with exanthema, presumably of measles. Within several weeks, progressive mental deterioration was noted, and hedeveloped myoclonic movements, muscle rigidity, and pyramidal tract signs. Ophthalmologic examinationsrevealed optic and retinal atrophy. The EEG showed a paroxysmal slow wave burst with spikedischarge predominantly in frontal area. The PEG revealed slight dilatation of lateral ventricles. The measles antibody titers in serum were abnormal but curious, CF titers to one measles antigenbeing high (2048X), while that to. another antigen being not so elevated (32-64X). In addition, thereware noted several abnormal autoantibody reactions, including ANF, RA, cold hemoagglutination, and Paul-Bunnell reaction. The delayed dermal reactions to measles, mumps, rubella, mycoplasma, and SK-SD antigens were all negative, but the reaction to PPD was positive. The patient has been currently in coma with neurovegetative manifestations, including obesity, hyperhidrosis, and unexplained fever. This case has several atypical features for SSPE, such as earlier onset, acutely progressive course, suggestive but not characteristic EEG, variability of antibody titers to different measles antigens, andseveral abnormal autoantibodies.
A six-year-nine-month old boy was admitted to our hospital with complaints of personality changeand involuntary movements. He had sudden grand mal-like seizure 7 weeks prior to this admission, and since then graduallydeveloped personality changes. His initial EEG record had shown no specific abnormality. The diagnosis of SSPE was made by his clinical course, specific EEG changes since admission, colloidal gold curve of CSF, and markedly elevated serum anti-measles antibody titer. He had suffered from measles uneventfully at the age of three.According to Burnet (1968), SSPE is a condition in which the thymus-dependent system has developedspecific tolerance (non-responsiveness) to measles antigen while the antibody system remainsspecifically active. As to our case, delayed hypersensitivity to candida skin test and blastogenesis of lymphocytes to PHA were normal, and blastogenesis to measles virus showed also normal reaction compared with thenormal control. From these data, in our case, cellular immunity was considered completely intact, and no specifictolerance to measles virus was proved. He died after eight months' clinical course and the pathological examination was not performed.
A case of subacute sclerosing panencephalitis (SSPE), 12-year-old boy was reported. The exactdate of measles infection was not known but said to be around 2 to 4 years of age. About 10 yearslater, he developed indifference to the surroundings followed by myoclonic seizures. He had mentalretardation since infancy but it became more marked prior to the onset of these symptoms. EEG findings showed changes with the course of the disease and became gradually typical withperiodic high voltage burst. He had extraordinary high levels of measles antibody titers both in serumand CSF. He also had high IgG level in CSF though his IgG level in serum remained normal. Heshowed positive delaped skin reaction against purified measles antigen but negative result against Schwartz live vaccine. Thymus biopsy revealed the evidence of old inflammation suggesting that thepatient had some immunological abnormalities.
The case histories of 20 infants, in 14 of whom purulent meningitis developed during the first 4weeks of life and in other 6 during the following 2 weeks, were reviewed. Gram-negative intestinal organisms were the most frequent etiologic agents. In 3 cases of low-birth-weight infants, meningitis was one of the manifestions of septicaemia andthe prognosis were very poor (two of the 3 infants died and another one developed hydrocephlus). In full-term infants with meningitis, the course and prognosis differed according to the time of onsetof the disease. In 3 infants in whom meningitis developed during the first week of life, the signswere non-specific, the course was fulminant, and all of them died. But, when the disease developedafter the first week of life, the diagnostic signs suggesting nervous system involvement were observed, and by early treatment, a half of the patient recovered without neurologic sequelae. Non-specific and subtle signs such as lethargy and pcor sucking were very important to suggestmeningitis when other diseases were excluded. Early diagnosis by cerebrosinal fluid examination and intensive treatment would improve theprognosis of the patient with purulent meningitis in the neonatal period.
A 4 2/12-year old girl with fucosidosis was described who is the first documented case in Japan. Itwas noted that a-L-fucosidase activity was completly absent in liver tissue, white blood cells, andcultured skin fibroblasts. In the liver tissue obtained by biopsy, fucose rich glycolipids as well as glycoprotein was found tobe markedly increased, whereas amounts of acid mucopolysaccharide estimated as uronic acid contentwas only slightly elevated. Amounts of α-L-fucosidase activity in white blood cells from parents were within normal range.However, the calculated ratio of α-L-fucosidase to α-D-mannosidase activity of the parents was intermediate between that of the affected patient and the control. This result might suggest that the parentsare phenotypically normal heterozygote and the disease is transmitted as an autosomal recessivetrait.
The first case, T. O., was 10 year old girl whose parents were consanguineous. Clinical findings of T. O. revealed the following: bilateral dislocated lenses without cataracts, fine hair, myopia, poordevelopmental milestones, mental retardation, pes valgus and abnormal EEG patterns. The second case, S. M., 6 year old boy, showed developmental retardation, fine hair, myopia, ectopialentis and hypertension. Routine laboratory studies disclosed no abnormality of urinalysis, hematologic findings and bloodchemistry. Renal function test was abnormal. Cyanide-nitroprussid tests on their urine were positive. Patient T. O. and S. M. found to excrete37.9mg/dl and 10.7 to 16.4mg/dl of homocystine in urine by the estimation with use of an automaticamino acid analyzer. Their serum levels of homocystine and methionine were elevated whereas thoseof cystine were diminished. We made methionine loading experiments on two affected sibs, their families and two unrelatedadult males. The results were shown in Fig. 3 & 4. By the administration of Vitamin B6 500mg per day, both serum and urinary homocystine levels ofthe patient S. M. decreased remarkably. Methionine level in serum also diminished. In the case of T. O., Vitamin B6 showed no influence on homocystine concentration in blood and urine. A diet low in methonine or a lowmethionine diet to which high cystine was added was effectivefor keeping serum homocystine in low level.