[First case of infantile botulism in Sweden?].
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Biomedical subjects
Publications and source records attributed to I Bjerre.
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In a long-term prospective study 46 unselected infants born before 35 completed weeks of gestational age were followed up, and compared to 26 fullterm infants. At 9 and 18 months of chronological age their height and weight were still lower than that of fullterms, but the difference disappeared when age was corrected for gestational age at birth. The motor and neurological maturity and language development was delayed in the preterms still at 18 months, which could possibly also be explained by their lower biological age. Ten of the preterm infants showed, at one or several occasions during follow up, definite neurological abnormality. At 18 months of age two of them were handicapped, one with retrolental fibroplasia, nearly blind, and another with cerebral palsy (slight spastic diplegia). Five of them had late psychomotor development, while two were borderline and one normal. We defined pre- and perinatal risk groups, but found that development at 18 months was not correlated to degree of risk. Neither was there any correlation between neurological examination at term and later handicap or psychomotor retardation. We found more illness, mostly due to common infections, during the first 18 months in the preterm group, as measured by the number of visits to a doctor and days spent in hospital.
Two cases of happy puppet or Angelman syndrome are presented. They have the typical clinical features and represent the first Swedish cases. One of the patients is a man of 75 years of age, which shows that this form of severe mental retardation is well compatible with long life. Extended EEG monitoring may identify the typical EEG abnormality when this is difficult to demonstrate in routine EEG records. The typical laughter has no specific correlate in the EEG and thus is probably not an epileptic manifestation. Regional cerebral blood flow studies were normal in the young patient (11 years of age) but in the older patient showed a reduced cerebral circulation, compatible with organic dementia.
In a long-term prospective study 46 unselected infants born before 35 weeks of gestational age were followed up, which included repeated neurological and psychological examination. 40 of them were neurologically evaluated at term conceptional age and compared with 26 full-term newborns, all the infants being considered healthy. There was no difference in mean weight or length between the two groups, but mean head circumference was greater in the preterm group. The preterm infants had lower muscle tone as judged by spontaneous posture of arms and legs, and poor resistance to passive movements and slow arm recoil. In the traction test they had more head lag. The withdrawal and Moro reflexes were weaker, while asymmetric tonic neck reflex (ATNR) was easier to elicit. Head control in the sitting position was better. All infants were evaluated with a prenatal and perinatal optimality score, which was not however significantly correlated with the neurological findings. The preterm infants with only slightly reduced optimality score (low risk group) had approximately the same birth weight and gestational age as the other preterms. They more resembled the full-term infants with good resistance to passive movements, fast arm recoil and good responses concerning the withdrawal and Moro reflexes. However, they had a semiflexed position in supine and thus in this respect were more like the other preterms.
A case of severe myasthenia gravis diagnosed in a 12-month-old girl is described. Thymectomy at 15 months of age had only a negligible effect on the course of the disease. Treatment with cholinesterase inhibitors, prednisolone and azathioprine was started. After one year of combined treatment she was symptomfree and 3 years later treatment with cholinesterase inhibitors and prednisolone was discontinued. Attempts to withdraw azathioprine have so far been unsuccessful, leading to relapse of the disease. IgG-antibodies against cholinergic receptors were lowered to near normal, whereas the levels of total plasma immunoglobulins remained normal. The proportion of T-lymphocytes in peripheral blood was reduced during the first weeks after thymectomy, but has since been normal. Lymphocyte function measured by PHA stimulation remained normal all the time. The girl grew and developed normally without complicating infectious diseases in spite of her severe disease, thymectomy and immunosuppressive treatment.
Thirty nine infants with severe asphyxia (28 affected perinatally and 11 later) were studied by electrophysiological cerebral function monitoring (CFM) for periods varying from a half to 49 days. Nineteen infants died while still in intensive care and two died later from sequelae. Eighteen survived and were followed up when aged between 8 and 36 months. The initial electroencephalogram (EEG) and the first 12 hours of CFM tracing correlated well. The type of background activity, whether continuous or interrupted, proved to be of high prognostic importance unlike the presence of seizure activity, which bore no distinct correlation to outcome in these severely asphyxiated infants.
A report of a 21-year-old female, who has had Rothmund-Thomson's syndrome since early childhood, is given. In addition to the original disease with skin and tendon manifestations, she has also developed myopathy and epilepsy. It is discussed whether these two recent manifestations are part of her original syndrome or of different pathogenesis.
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Obstetric factors were studied in an unselected series of mothers of LBW-children and compared with those in a corresponding control series. Both groups consisted of 161 women with single pregnancies and 16 with twin pregnancies. Of the 188 LBW-children, 14% were twins. Of the single infants, 42% were appropriate for gestational age (AGA), i.e. within the 10th to 90th percentile according to Swedish curves for intrauterine growth, and 58% small for gestational age (SGA). The commonest causes of low birthweight (LBW) in the AGA-group were bleeding (34%) and premature rupture of the membranes (28%). In the SGA-group toxaemia was found in 15%, but in 50% of the SGA-group no cause of LBW was demonstrable. In this latter group the average placental weight was low. The mortality was noted and 89% of the living children were examined neurologically at 5 years of age. The prognosis of the child depended on the duration of pregnancy. Bleeding during pregnancy was a prognostically unfavourable sign, but even serious bleeding complications did not exclude a normal development of the child. The prognosis was best for those infants in whom no cause of LBW was demonstrable.
Drooling involves great practical problems in the care of patients with cerebral paralysis and mental deficiency. Different types of medical and surgical treatments have been used. In 17 disabled patients chorda tympani has been cut bilaterally in order to reduce the production of saliva, especially from the submandibular and sublingual glands. The loss of taste on the anterior part of the tongue seems to contribute to the reduction of salivation. Fifteen patients have been observed postoperatively during two to four years. Five patients have been cured, two are considerably improved and seven partially improved. The effect of nerve section was illustrated by Tcm99 scintigraphy in two additional cases before operation and six months postoperatively. No complications were observed.
Of 3841 children born alive in Malmö in Sweden in 1966, 188, 4.9% had a birth weight of larger than 2500 g. We studied these children with their families and a control in order to form an opinion on the role played by certain social and biological factors in the aetiology of LBW in a country with a relatively high average standard of living. Biological factors were analysed, such as the mother's age, parity, stature, and weight, as well as purely socio-economic factors such as the mother's civil staatus, the social group of the mother and father, income, mother's allowance in case of illness, social help or investigations regarding one or more members of the family, as well as the frequency of immigrants. The combined effect of various socio-economic factors was judged by help of a "social score". The results were analysed for 3 different groups of low birth weight, appropriate for gestational age, small for gestational age and multiple births. Judging from our investigation, biological factors probably play the greatest role in the question of LBW but these factors are difficult to distinguish from socio-economic factors which probably still play a certain role in the group preterm children, appropriate for gestational age.
139 children with a low birthweight (LBW), i.e. not more than 2 500 g, were examined at 5 years of age in respect of their neurological status, with special reference to motor coordination according to Touwen & Prechtl. 5 (3.6%) children had cerebral palsy; 13 (9.4%), minimal brain dysfunction; 38 (27.3%), delayed motor maturation; and 83 (59.7%), normal motor development and normal neurological status. Impairment of hearing and of vision, epilepsy and mental retardation were more common in children with cerebral palsy and MBD. Children with MBD and delayed motor maturation had a lower IQ, as judged from the draw-a-mantest according to Goodenough than children with normal neurological status. Cerebral palsy was found in children with a very low birthweight and short gestational age. Other findings were equally distributed among groups classified by weight and duuration of gestation.
In a prospective investigation of the physical growth of children with a low birth weight, 143 unselected children who weighed less than 2500 g at birth were reviewed at 5 years of age. The children were grouped according to weight relative to gestational age. Stature and weight were compared with normal curves for Swedish children and with a control series. The circumference of the head was compared with a normal curve. Osseous development was assessed by the method of Eklöf & Ringertz and was compared with their normal values. Growth of the LBW-children was slightly retarded in respect of stature, weight and osseous development, but not regarding head circumference. The values found for stature and osseous development were low in the group small for gestational age and for twins. Those LBW-children who were appropriate for gestational age developed at a normal rate. This is true even for those with the lowest birth weight and those born long before term. Osseous development was correlated with stature, which may be due to the measuring method used. Neither maternal disease nor feeding difficulties during the first few months of life had any demonstrable effect on the physical development of the children. Socio-economic factors now surely play at most a subordinate role. Physical development of the children varied most with the mothers' stature and weight and probably with hereditary factors.
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