A novel approach to the control of experimental environments: the ESCA microscopy data-acquisition system at ELETTRA.

An efficient control system is today one of the key points for the successful operation of a beamline at third-generation synchrotron radiation sources. The high cost of these ultra-bright light sources and the limited beam time requires effective instrument handling in order to reduce any waste of measurement time. The basic requirements for such control software are reliability, user-friendliness, modularity, upgradability, as well as the capability of integrating a horde of different instruments, commercial tools and independent pre-existing systems in a possibly distributed environment. A novel approach has been adopted to implement the data-acquisition system of the ESCA microscopy beamline at ELETTRA. The system is based on YASB, a software bus, i.e. an underlying control model to coordinate information exchanges and networking software to implement that model. This 'middleware' allows the developer to model applications as a set of interacting agents, i.e. independent software machines. Agents can be implemented using different programming languages and be executed on heterogeneous operating environments, which promotes an effective collaboration between software engineers and experimental physicists.

Mitochondrial respiratory failure in skeletal muscle from patients with Parkinson's disease and multiple system atrophy.

We studied mitochondrial respiratory chain function in skeletal muscle taken from 27 patients with idiopathic Parkinson's disease (PD; 21 Dopa-treated PD patients and 6 de novo patients), 5 patients with multiple system atrophy (MSA) and from 43 age-matched controls in order to determine the occurrence of mitochondrial respiratory chain abnormalities in parkinsonian syndromes. In our control subjects, we found a significant age-related decrease in the activity of respiratory chain complex I. As compared to carefully age-matched control subjects, activity of complex (NADH:ubiquinone reductase) was significantly lower in muscle mitochondria from patients with PD and MSA and a mean remaining activity < 30% of controls was observed. Mean activities of complexes III (ubiquinol:cytochrome c reductase) and IV (cytochrome c oxidase) were also lower in PD patients than controls, but a low activity (remaining activity < 30% of controls) was observed in only 5 PD patients for complex I and III or I and IV. No deficit in complex II activity (succinate:ubiquinone reductase) was observed. Our results support the hypothesis of a wide-spread mitochondrial complex I deficiency in PD and MSA as compared to age-matched controls, who showed age-related deficiency. This deficit can be found in de novo PD patients as well as in treated patients. The observed respiratory enzyme chain deficiency could not be explained by the dose and duration of L-Dopa or dopaminergic agonist treatment, the severity of the disease, anxiety or depression since no significant correlation was found between these parameters and enzyme complexes activities.

Coexisting kappa light chain multiple myeloma and primary hyperparathyroidism.

The authors report the case of an 82 year old woman hospitalized for hypercalcemia associated with low serum phosphate. Multiple myeloma was first diagnosed. However, despite chemotherapy, hypercalcemia persisted and she was subsequently diagnosed as primary hyperparathyroidism; eucalcemic state was then obtained after parathyroidectomy. Fifteen similar cases are reported in the literature and the mechanisms and implications of such an association are discussed.

Unilateral loss of facial flushing and sweating with contralateral anhidrosis: harlequin syndrome or Adie's syndrome?

A 45-year-old woman presented with a 10 year history of asymmetrical facial flushing and sweating after exertion or in hot weather. During these episodes the right side of her face remained dry and white, while the left side normally flushed. Sweating was impaired on the left side in the limbs and trunk. She also had areflexia in the lower limbs and slow pupillary reactions to light and darkness, as seen in Adie's syndrome. The topography of the sweating disorder suggested that the lesion involved the sympathetic pathways at the level of spinal cord. The relationship with the harlequin syndrome and related disorders is discussed.

Astasia-abasia revealing a primary Sjögren's syndrome.

A 63-year-old woman presented with mild arthralgias and inability to stand and walk without other significant neuropsychiatric abnormality. Neurological explorations showed intrathecal oligoclonal immunoglobulin production in CSF and numerous bright foci at brain MRI. A primary Sjögren's syndrome was concomitantly evidenced and was thought to be the cause of the astasia-abasia symptom by multiple brain involvement. Patient's condition gradually improved with hydroxychloroquine and corticosteroid therapy. Central nervous system involvement by primary Sjögren's syndrome is discussed.