Hiroshi Ushijima

The progress of adrenoleukodystrophy as revealed by auditory brainstem evoked responses and brainstem histology

ZusammenfassungEin sechs Jahre altes Kind erkrankte im 5. Lebensjahr an Adrenoleucodystrophie (progressive metabolische Erkrankung des ZNS mit rapider Degeneration). Die Krankheit begann mit Dysarthrie, Schwerhörigkeit und Gangunsicherheit. Später kam es zur spastischen Paralyse, zu hochgradiger Schwerhörigkeit und Erblindung. Tod durch Atemlähmung.Während des Verlaufs wurden regelmäßig die Hirnstammpotentiale sowie die langsamen Rindenpotentiale abgeleitet. Dabei war mit fortschreitender Erkrankung eine deutliche Verminderung der Potentiale zu beobachten. Histologisch zeigten sich eine Entmyelinisierung des Hörnervs sowie Neuronenverluste im Verlauf der Hörbahn. Die Degeneration im Hirnstamm verlief dabei von rostral nach kaudal.SummarySerial studies of auditory brainstem evoked responses (ABR) and slow vertex responses (SVR) were obtained during the progress of adrenoleukodystrophy in a 6-year-old boy. This child was normal until 5 years of age. His illness began with a gait disturbance, dysarthria, and hearing difficulty. Later, spastic paralysis, serious deafness, and blindness appeared. He died of respiratory failure 2 years after the onset.The ABR was normal at onset but changed to an abnormal pattern. Initially, there was lengthening of the wave V-I interpeak interval. This was followed by the disappearance of the later components as his general condition deteriorated. At the terminal stage, only a prolonged wave I was recordable. The postmortem pathology revealed demyelination of auditory nerves and remarkable neuronal loss in the auditory pathways of the brainstem; in addition, there was a variety of extensive degeneration throughout the cerebrum, in particular the complete degeneration of the white matter with secondarily occurring ganglionic cell changes. These data suggest that degeneration of the brainstem from rostral to caudal levels occurred.

Epidemiology of rotavirus infection in Tokyo during two winter seasons, as revealed by analyses of recovered viral RNA

Sir, - Rotaviruses are important etiologic agents of acute gastroenteritis in young children all over the world [9]. Gel electrophoresis can be used to detect and genotype rotavirus strains [2, 3, 4, 8]. We have investigated the relationships between rotavirus RNA electropherotypes and the epidemiology of acute gastroenteritis in Tokyo. Three hundred and seventy-four diarrheal stool specimens were collected from in- and out-patients (less than 4 years old, mainly between 6 months and 2 years old) at Teikyo University Hospital in Tokyo during the winter periods of 1981-1982 and 1982-1983. Outbreaks of rotavirus infection developed in two nursery rooms during this time. Twentyeight and 11 stool specimens were obtained from children in each nursery room, respectively. The extraction of virus from feces and

Coproantibody response to rotavirus in an outbreak in a day-care nursery

13 months and 10years 8 months. Patient no. 1 was first observed at a very advanced stage of the disease and died 19 months after the diagnosis of lung metastases. Patient no. 2 is still alive 10 years and 8 months after the diagnosis. Patients 3 and 4 are still alive after 6 years 11 months and patient no. 5 is well 6 years after the diagnosis. All the patients treated in the second period are still alive. Patients 6 and 8 are still in complete remission 35 and 13 months, respectively, after diagnosis. In patient no. 7 (alveolar RMS) a local relapse was noted 2 months after the completion of initial therapy. The subject was again treated with 5000 rads to the orbit, plus chemotherapy consisting of cisplatinum and tenixposide. After 7 months of treatment there were side-effects of renal toxicity with hyperuricaemia, increase in BUN and hyaline casts in the urine. Treatment was stopped and the patient is still in complete remission. Our results do not demonstrate any significant difference in life expectancy between patients treated with radical surgery and those treated conservatively, but the advantage of the latter treatment is so impressive cosmetically that this approach must be seriously taken into consideration. In our patients, the follow-up period is obviously too short to allow evaluation of long-term evolution and to draw definitive conclusions about the most suitable chemotherapy protocol. However, taking the quality of life, vision and psychological situation into consideration, we believe that chemotherapy and radiotherapy can offer a very valuable alternative in the therapy of RMS. Further trials involving this approach must be carefully considered.

A study of adenovirus gastroenteritis in the Tokyo area

Probable serotypes of faecal adenoviruses 40, 41, 31, 2 and 1 were found in the Tokyo area by rapid DNA restriction endonuclease analyses directly of stool specimens or after primary culture in Graham 293 cells. Some variations were found in both adenoviruses 40 and 41 by this method.

Evaluation of four tests for detecting human rotavirus in feces

Sir, Infantile diarrhea during the winter is mostly due to viruses and the majority of pathogenic viruses prove to be rotavirus. Various methods for diagnosing infections by rotavirus have been described. The migration patterns of RNA segments ascertained by polyacrylamide gel electrophoresis (PAGE) are sensitive for the detection of rotavirus and effective for the analysis of the molecular epidemiology of rotavirus infections [9]. A simple and specific technique for rapidly detecting rotaviruses in feces is still needed. Enzyme-linked immunosorbent assay (ELISA) kits for detecting rotaviruses (Abbott Laboratories, North Chicago, Ill., USA) and latex agglutination test kits (Orion Diagnostica, Helsinki, Finland) are commercially available and have recently been evaluated [1-5, 7, 10]. In the present study, four different techniques to detect rotaviruses in feces are compared. Sixty-two fecal specimens were obtained from patients with infantile diarrhea. Specimens were kept at -20~ Samples were suspended in phosphatebuffered solution (PBS), and centrifuged at 3000 rpm for 15rain at room temperature. Supernatant was used for all four techniques. Migration patterns of rotavirus RNA segments by PAGE were determined as described previously [9]. Virus isolation: MA104 cells and primary cynomolgus monkey kidney (PMK) cells were used. The isolation methods have been described previously [6, 8]. Latex agglutination (LA): The Rotalex kit was used according to the manufacturers instructions. The results were read under the inverted microscope (x 40, Chiyoda Kogaku). ELISA was carried out according to the manual of the commercially available Rotazyme kit. Results were read by measuring absorbance at 492nm (Quantum II, Abbott). Of 62 samples, 31 were positive and 10 were negative by all four techniques (Table 1). Thirteen samples were positive by three techniques and negative by one, while five samples were negative by three techniques and positive by one. The results for the 62 samples tested are as follows: 79% (49/62) for PAGE, 65% (40/62) for virus isolation, 73% (45/62) for LA, and 61% (37/62) for ELISA. Several different migration patterns of RNA segments can be ascertained by PAGE [8]. However, there were no distinct correlations between migration patterns and the results obtained in this study. The commercially available

Chlamydia trachomatis infection in a premature infant.

Sir, We read with interest the report of a lung infection with Chlamydia trachomatis in a premature infant by Mfirdh et al. (1). It seems plausible that the infant had acquired the lung infection in utero and died due to lack of C . trachomatis antibodies. A male premature infant, born after 24 weeks and weighing 806 g was referred to our newborn intensive care unit with tachypnea, cyanosis and cough. There was no conjunctivitis or fever. X-ray films showed bilateral diffuse interstitial infiltration. He improved during the first days after admission but then became worse again and died from a nosocomial lung infection (Pseudomonas aeruginosa) 43 days after admission. Antigens related to C . trachomatis were detected in the lungs at autopsy and in samples from the mothers cervix using monoclonal antibodies to C . trachornatis. P . aeruginosa was recovered from the lungs of the infant. No viruses were recovered from the autopsied samples. Serum IgM and IgG antibodies to C . trachomatis and P . aeruginosa were not found to be increased by enzyme-linked immunosorbent assay. Anti-chlamydia1 drugs were not used. Chlamydia trachomatis causes lung infection in infants (2) but death due to C . trachomatis is very rare. Lack of immune defence andlor mixed infection with bacteria e.g. P . aeruginosa or viruses may lead to death in infants, especially premature infants, as in the case described here and by Mfirdh et al. (1).

Acute and delayed neurologic reaction to inoculation with attenuated live measles virus.

A Japanese boy developed a high fever and a prolonged convulsion 11 days after inoculation with live measles vaccine. He had 4 more seizures during the next 6 mos. His EEG became transiently abnormal 14 mos later. Antimeasles complement fixation, hemagglutination, and neutralization titers were elevated.

Diagnostic significance of specific IgA coproconversion in rotavirus infection

early functional abnormalities within a few weeks, can occur at any time after the onset of diabetes, and are reversible by improvement in diabetic control. Later, the metabolic disturbances are followed by structural and irreversible lesions. At this stage, clinical neuropathy is related to the duration of diabetes. So, the suggested explanation for the discrepancies between various studies could be: (1) the number of patients with clinical signs of diabetic neuropathy (higher in Lorinis study); (2) the search for late components indicating low conduction velocity in some nerve fibres, even in patients in whom the PMNCV is normal (not mentioned in Lorinis study). Recently, we have found that the femoral nerve conduction velocity also is unrelated to the duration of diabetes [7]. In contrast to subclinical peripheral neuropathy, we have shown previously that the prevalence of incipient retinopathy, as diagnosed by fluorescein angiography, clearly was related to the duration of diabetes [2]. However, the mechanisms responsible for retinal microangiopathy are different, including changes in the vascular wall as well as haemorrheological perturbations. These microvascular modifications seem to need more time for installation than the neuropathic abnormalities [3]. On the other hand, the breakdown of the bloodretineal barrier may be precocious and is dependent on metabolic control even in short time periods [1, 5]. The discrepancies concerning the relationship between PMNCV and age could be explained by the young age of the patients included in some studies, and may be biased by the duration of diabetes and by metabolic control.

Nosocomial Infection by Rotavirus and Adenovirus Within One Month

We experienced a small outbreak of acute gastroenteritis in December 1985 in hospitalized children. Two different serotypes of rotavirus and two serotypes of adenovirus were found by molecular epidemiological and serological studies. Four patients were considered to have two serotypes of rotavirus. The results showed that a single strain of a virus was not always the cause of nosocomial infection.

Frequency of successive rotavirus infections among infants in a nursery home measured by coproantibody conversion

Sir: Dysplasia epiphysealis hemimelica is a developmental disorder of childhood in which there is a symmetrical cartilaginous overgrowth of one, or more than one epiphysis, or of a tarsal or carpal bone [1, 5]. The disorder is usually limited to the medial or lateral half of a single extremity (4). Dysplasia epiphysealis hemimelica is microscopically indistinguishable from an osteocartilaginous exostosis. We report a patient in whom the disease involved the ulna. This is very rare. H .A. , a girl aged 6 years, was brought in for consultation because of swelling over the ulnar aspect of the right wrist without pain of limitation of movement. Radiographs of the right wrist showed a very large lower ulnar epiphysis (figure). The height of the distal ulnar epiphysis was twice that of the medial portion of the distal radial epiphysis. Medially, the epiphysis overlapped the ulnar diaphysis. The metaphysis was dense and somewhat irregular. The right ulna was somewhat shortened. There was no abnormality of the carpal bones. The left distal ulnar epiphysis had not appeared. Bone age was about 6 and a half years in both hands. The remaining skeleton was normal. A diagnosis of dysplasia epiphysealis hemimelica was made. Our observation is very unusual because the whole epiphysis was affected, the metaphysis was slightly irregular. This was also seen by Connors [3]. The most similar publication is that of Buckwalter [2] in a three year old boy. There were multiple areas of ossification adjacent to the distal left ulna whereas the distal right ulnar epiphysis was unossifted. This was associated with shortening of both bones of the right forearm, bowing of the ulna and abnormal development of the lumate. In the literature, improvement followed excision of all or part of the lesion when the patient had symptoms. Excision of the lesions is indicated when it interferes with function. In our case, surgery was not indicated up to now.