Exploring the use of the Swiss medical tariffication codes (TARMED) in the establishment of the frequency of radiodiagnostic examinations.

In population surveys of the exposure to medical X-rays both the frequency of examinations and the effective dose per examination are required. The use of the Swiss medical tariffication system (TARMED) for establishing the frequency of X-ray medical examinations was explored. The method was tested for radiography examinations performed in 2008 at the Lausanne University Hospital. The annual numbers of radiographies determined from the "TARMED" database are in good agreement with the figures extracted from the local RIS (Radiology Information System). The "TARMED" is a reliable and fast method for establishing the frequency of radiography examination, if we respect the context in which the "TARMED" code is used. In addition, this billing context provides most valuable information on the average number of radiographs per examination as well as the age and sex distributions. Radiographies represent the major part of X-ray examinations and are performed by about 4,000 practices and hospitals in Switzerland. Therefore this method has the potential to drastically simplify the organisation of nationwide surveys. There are still some difficulties to overcome if the method is to be used to assess the frequency of computed tomography or fluoroscopy examinations; procedures that deliver most of the radiation dose to the population. This is due to the poor specificity of "TARMED" codes concerning these modalities. However, the use of CT and fluoroscopy installations is easier to monitor using conventional survey methods since there are fewer centres. Ways to overcome the "TARMED" limitations for these two modalities are still being explored.

Cerebrovascular complications of L-asparaginase therapy in children with leukemia: aphasia and other neuropsychological deficits.

Children with acute lymphoblastic leukemia (ALL), treated with L-asparaginase are at risk for cerebral thrombosis or hemorrhage because of coagulation protein deficiencies. The results and the importance of serial neuropsychological examinations after such complications in three children with ALL are presented. All patients showed marked aphasia and other cognitive deficits. Recovery was complete in two of three children.

Splitting of the ego, as the central phenomenon in neurosis. Ritske le Coultre. 1966.

The phenomenon of a split in the ego is not confined to fetishism, but is central to all neuroses. The author demonstrates this with regard to several specific neurotic types. He then goes on to show that the attempt to establish an absolute distinction between ego and id postulates an artificial split between--in fact--mutually interdependent parts. Traditional distinctions between primary and secondary process are critically examined. The id is an abstraction, while the essence and strength of the ego reside not in its defensive functions, but in the extent to which it accepts the drives; whereas the repressing ego weakens or even eliminates itself. The child who cannot resolve psychosexual conflict uses a makeshift solution, whereby that portion of the ego engaged in the conflict is split off and ceases to participate in further development. This part, emotionally rich, may again blossom and mature in the transference.

Acute cerebellar ataxia in a child with transient pontine lesions demonstrated by MRI.

A case of acute cerebellar ataxia with discrete signs of pyramidal and tegmental involvement is reported, several days after recovery from an upper respiratory infection of unknown etiology. Magnetic resonance imaging showed transient pontine lesions, disappearing in the convalescence phase. Laboratory tests established the etiologic diagnosis of post-infectious encephalitis. An allergic autoimmune response with subsequent demyelinisation is assumed. The important role of MR-imaging in similar acute syndromes is emphasized.

[Selected data from childhood epilepsies. ACTH treatment and ketogenic diet: a critical evaluation]. / Capita selecta uit de kinderepilepsieën. ACTH-kuur en ketogeen dieet: een kritische evaluatie.

The authors have evaluated critically the results of ACTH and ketogenic diet in 122 cases of 'malignant' childhood epilepsies, especially West-syndrome (WS) and Lennox-Gastaut-syndrome (LGS). In agreement with data from the literature about 10% of the idiopathic forms recovered with or without ACTH. In the other cases ACTH caused a transient amelioration of the EEG and/or the spasms; a high-dosage regimen of ACTH was not significantly better than a low-dosage regimen. Fatal complications only occurred in the high-dosage ACTH group. Ketogenic diet in 10 children with LGS seems to have an influence on the LGS-specific seizures but not on the other signs and symptoms of the syndrome. The diet is difficult to maintain and is not free of side-effects.

Mevalonate kinase deficiency in a child with cerebellar ataxia, hypotonia and mevalonic aciduria.

Mevalonate kinase deficiency has been documented in an 8-year-old child who presented with cerebellar ataxia, hypotonia and mevalonic aciduria. The activity of mevalonate kinase in extracts of cultured skin fibroblasts derived from the patient was approximately 2% of the mean value for controls. Family studies were carried out on the mother, the father and a sister, all of whom were clinically well. Mevalonate kinase activity in extracts of cultured skin fibroblasts and transformed lymphoblasts derived from the parents of the patient were 43%-52% of the mean control values. These data are consistent with an autosomal recessive mode of inheritance for mevalonate kinase deficiency.

Early infantile form of neuronal ceroid lipofuscinosis. Four Dutch cases and review of the literature.

In this paper four Dutch cases of early infantile neuronal ceroid lipofuscinosis (NCL) are described, all being boys. NCL is a group of diseases morphologically characterized by accumulation of autofluorescent ceroid lipofuscin-like pigment. Psychomotor deterioration, impairment of vision, and epileptic manifestations are the major clinical features. Onset in early infantile age is rare. We reviewed 71 cases reported in the literature, and compared the symptoms and signs with the findings in our patients. Psychomotor deterioration, visual impairment and myoclonic jerks are often observed early in the disease. Eventually, every patient shows psychomotor deterioration. The outcome is always lethal, usually within a few years. Special attention is paid to diagnostic procedures.

Signs of focal posterior cerebral abnormality in early subacute sclerosing panencephalitis.

In 16 cases of subacute sclerosing panencephalitis (SSPE), praxis and visual spatial capacities were more impaired early in the disease than were language functions. Together with the electroencephalographic findings, this suggests that the early lesions in SSPE are more pronounced in the parietooccipital area than in the classic language areas. Other reports also support such a localization. Detection of the disease in its early phase when dressing apraxia and visual impairment predominate is important in conducting clinical trials of different therapeutic agents.

Progressive poliodystrophy (Alpers' disease) with a defect in cytochrome aa3 in muscle: a report of two unrelated patients.

We present two unrelated patients, a boy and a girl, with a progressive neurologic disorder, characterized by psychomotor retardation, seizures and paresis, the illness being exacerbated during stressful periods. Lactate levels in serum and cerebrospinal fluid were elevated in both patients. Histopathologic studies of muscle tissue revealed mitochondrial abnormalities in the boy; in the girl, slight neuronal degeneration was observed. A cerebral biopsy in the girl showed abnormalities compatible with progressive poliodystrophy. Autopsy in the boy demonstrated progressive poliodystrophy. Biochemical studies in muscle tissue showed a defect of cytochrome aa3 in both patients, connected with a defect of cytochrome b in the girl. The association of defective pyruvate metabolism and progressive poliodystrophy is discussed.

Infantile neuroaxonal dystrophy and giant axonal neuropathy: are they related?

Light and electron microscopic findings from two sural nerve biopsies obtained at a one-year interval from a patient with the clinical features of Seitelberger's disease are described. Ballooned axons with accumulations of membranous profiles, vesicles, mitochondria, and a homogeneous center were present, and there were masses of 90 A filaments in endothelial, endoneurial, perineurial, and Schwann cells. These pathological alterations were less prominent in the second nerve biopsy, which showed a more pronounced decrease in myelinated fibers. The case shows that a generalized increase of 90 A filaments in structures of the peripheral nervous system is not a phenomenon exclusively occurring in patients with giant axonal neuropathy and, furthermore, that it may be a transitory feature.

Cuirass respirator treatment of chronic respiratory failure in scoliotic patients.

The results are reported of domiciliary cuirass respirator treatment, using tailor-made shells, in four patients with severe thoracic scoliosis. Three of the patients had suffered from poliomyelitis. All complained of increasing dyspnoea on exertion, ultimately interfering with almost every activity of daily life; three patients had severe acute respiratory failure necessitating urgent admission to the Respiratory Care Unit. Right heart failure was present in two. Two patients required mechanical treatment via an endotracheal tube. All the patients were discharged home with a cuirass respirator. Standard type shells were used initially with low efficiency due to the poor fit of the cuirass shell to the deformed thoracic cage. Tailor-made shells were constructed from polyester reinforced with glass fibre, modelled on plaster casts of the thoracic cage. Subjectively the patients improved greatly and were able to resume and increase many activities. One patient committed suicide for reasons unconnected with treatment but the other three patients have been doing well from the time the cuirass respirator treatment was started, respectively, 3, 6, and 10 years ago. This treatment seems particularly effective in younger patients with severe paralytic scoliosis and cardiorespiratory failure, although the possibility of using it in older patients suffering from scoliosis of other aetiology should certainly be explored.