Estimating costs and benefits of advanced control for wastewater treatment plants--the MAgIC methodology.

This paper discusses a methodology to estimate the costs and benefits of advanced control for wastewater treatment plants. The methodology has been applied to four wastewater treatment plants, representing four standard types of plants built in Flanders, Belgium. The paper outlines the methodology and illustrated results from one of the four design cases. General results are shown and contrasted with full-scale experience. The methodology appears to give realistic results and will be used for further refinement of default control algorithms for certain types of plants. A preliminary analysis indicates that on-line control can become cost-effective for plant sizes above 50,000 population equivalents.

Dementia, myoclonus, peripheral neuropathy, and lipid-like material in skin biopsy during psychotropic drug treatment.

Chronic treatment of humans with several drugs is associated with lesions resembling lipidosis in different tissues. Recently, a Creutzfeldt-Jacob-like syndrome has been observed during tricyclic antidepressant therapy, but no evidence of interaction of these drugs with lysosomal function has been reported during such treatment. We report a case of dementia, myoclonus, peripheral neuropathy, and lipid storage in the skin due to antidepressant drug therapy, in which the discontinuation of drugs resulted in an improvement of clinical and electrophysiologic signs together with reduction of morphological evidence of lipid lysosomal storage.

Unusual clinical features and early brain MRI lesions in a family with cerebral autosomal dominant arteriopathy.

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a recently described inherited disorder. The pathologic gene maps on chromosome 19. The clinical spectrum of the disease consists of recurrent strokes, migraine, transient ischemic attacks, mood changes, and dementia. We report a genetically assessed CADASIL family with atypical clinical presentations of epileptic seizures. In two asymptomatic family members there were early brain abnormalities on MRI. Our report expands the clinical spectrum of CADASIL and suggests that it is possibly an undiagnosed disorder.

Ataxia, mental deterioration, epilepsy in a family with dominant enamel hypoplasia: a variant of Kohlschütter-Tönz syndrome?

We describe 3 sibs, their father, and paternal grandfather with amelogenesis imperfecta. In 2 sibs and the father the defect is associated with a neurological syndrome which has a wide range of phenotypic variability. The proposita has ataxia, EEG abnormalities, moderate dementia, and enamel hypoplasia. This case and the affected relatives are discussed in relation to Kohlschütter-Tönz syndrome and neuroectodermal diseases. The syndrome described here, characterized by the association of a genetic enamel defect and neurological impairment, may be of considerable interest in advancing genetic and clinical knowledge on ectodermal tissues and their development.

Potential triggering factors of intracerebral hemorrhage.

The distributions of intracerebral hemorrhage (ICH) according to place of onset, degree of physical activity at onset and potential triggering factors were analyzed in 848 patients with ICH. Patients were grouped according to the presumed cause of ICH: hypertensive ICH, secondary ICH and ICH of undetermined origin. The influence of demographic and temporal factors on the relative frequency of events was also assessed. In 30% of the cases, ICH occurred during inactivity or sedentary activity, in 50% during light exertion and in 20% during moderate/vigorous exertion. During inactivity or sedentary activity, hypertensive ICH was significantly less frequent than secondary ICH (OR 0.32; 95% CI 0.21-0.47) and undetermined ICH (OR 0.36; 95% CI 0.23-0.55), whereas during moderate or vigorous exertion hypertensive ICH was more frequent than secondary (OR 1.88; 95% CI 1.16-3.05) and undetermined ICH (OR 2.29; 95% CI 1.31-4.00) Potential triggering factors were observed in 27% of patients and were significantly more frequent in patients with hypertensive ICH than in patients with secondary ICH (OR 2.90; 95% CI 1.85-4.54) or undetermined ICH (OR 2.44; 95% CI 1.54-3.87). Our findings suggest that many potential external triggers that act mainly by raising blood pressure may interact, and their concurrence may favor cerebral hemorrhage, particularly in hypertensive patients. In many cases, these circumstances of increased risk may be mitigated by preventive measures.

Differing temporal patterns of onset in subgroups of patients with intracerebral hemorrhage.

BACKGROUND AND PURPOSE: The purpose of this study was to further analyze the temporal patterns of onset of intracerebral hemorrhage (ICH) and to determine whether or not subgroups with specific clinical characteristics exhibit different patterns of onset. METHODS: The daily, weekly, and yearly variations in occurrence of ICH together with the relationship between ICH occurrence and changes in air temperature were evaluated in 1018 patients. Patients were grouped according to the presumed etiology of ICH: hypertensive ICH, secondary ICH, and ICH of undetermined origin. The contribution of demographic and clinical factors to the temporal distributions of ICH was also evaluated. RESULTS: Marked differences in seasonal and diurnal patterns of ICH onset were observed in the different groups. The incidence of hypertensive ICH reflected seasonal and circadian changes in blood pressure, whereas the latter did not seem related to the onset of nonhypertensive ICH. The seasonal pattern was more evident in elderly patients with hypertensive ICH than in younger subjects. No significant weekly variations were observed; however, risk was greater on Monday in the working population. CONCLUSIONS: Our results suggest that the higher incidence of ICH in the colder months is due to the effect of low temperatures on blood pressure and that the clustering of ICH events in the morning is due to the increase in sympathetic tone, and consequent increase in blood pressure, on awakening.

Further evidence of involvement of central and peripheral sensory pathways in olivopontocerebellar atrophy.

We report the electrophysiological findings of the central and peripheral somatosensory pathways in 20 patients with olivopontocerebellar atrophy. Changes in sensory action potentials of the median nerve were observed in 14 patients and consisted of reduced sensory potential amplitudes associated in 10 cases with an increase in distal latencies. Sixteen out of 20 patients also showed changes in somatosensory evoked potentials after stimulation of the median nerve, consisting of decreased amplitude of N13 (13 cases) and N20 (16 cases) components, associated with increased N9-N13 interpeak latency in 9 and N13-N20 in 14 patients. The origin of these alterations is discussed.

Sensory and motor peripheral neuropathy in olivopontocerebellar atrophy.

We report the findings of an electrophysiological study in 9 patients affected by olivopontocerebellar atrophy, 4 with a dominant form and 5 with a sporadic form. Superficial peroneal nerve biopsy was obtained from 2 patients. The electrophysiological alterations were signs of collateral reinnervation and loss of motor units, decrease in sensory potential amplitude and increase in distal motor latency. Only a slight reduction in motor and sensory conduction velocity was observed in some cases. Nerve biopsy showed slight reduction of the number of myelinated fibres. In the first case, fibre diameter distribution was unimodal, due to reduction of myelinated fibres of large diameter, in the second case there was no significant alteration of the fibre distribution. In both cases short internodes were present with no signs of segmental demyelination, remyelination or axonal degeneration. The alterations observed in the peripheral nervous system are probably secondary to a lesion of the posterior root ganglion and the anterior horn cell in the spinal cord.

Exercise-induced bilateral anterior tibial compartment syndrome without pain.

We report the case of a 41 year old man who complained of a severe bilateral deficit of the anterior tibial compartment two hours after prolonged exercise. On admission there was no spontaneous or evoked pain, no objective sensory deficit but total loss of dorsiflexion of feet and toes. Electrophysiological investigation showed no voluntary or evoked electrical activity in tibial and extensor digitorum muscles; while peroneus longus and gastrocnemius muscles showed a near-normal pattern. On the other hand a weak motor response was obtained by direct stimulation of the anterior tibial muscle belly. An early bilateral fasciotomy was followed by almost complete recovery of the spontaneous and evoked motor activities of the tibial anterior muscles. On the basis of these findings we discuss the possible pathogenetic role of a neuroapraxic block of the deep peroneal nerve where it crosses the anterior fibular septum and supply a possible interpretation of the absence of pain.

Aperiodic alternating nystagmus: report of two cases and treatment by baclofen.

We report two cases of non periodic alternating nystagmus, one of vascular origin (ischemia of the vertebrobasilar territory) and a second of traumatic origin (whiplash injury) with otoneurological signs, typical of lesions in posterior cranial fossa and in particular of vestibulum-cerebellum and brainstem: gaze paretic nystagmus, rebound nystagmus, saccadic dysmetria, vestibular hyperreflexia and impaired visual suppression test. In one case it was possible to give baclofen therapy, which yielded positive results. Suspension of drug administration resulted in the worsening of clinical signs. The mechanism of action of the drug will be discussed.

Cherry-red spot myoclonus syndrome (type I sialidosis).

The authors report the sequence of the clinical symptoms in type I sialidosis or cherry-red spot myoclonus syndrome, derived from the cases personally observed and from the literature. They also report neuropathological and neurochemical data. A serial EEG study in a case shows the tendency to a progressive deregulation of cerebral electric activity. Therapeutic attempts to reduce myoclonus, which is one of the more disabling symptoms in this syndrome, are described.

Late recurrence of thymoma and myasthenia gravis.

In a case of thymoma associated with myasthenia gravis, symptoms of relapse appeared 14 years after thymectomy. Tumour tissue from repeat resection showed the same histologic pattern and aneuploidy as in the original specimen. The case illustrates the necessity of wide surgical exposure to permit maximal thymectomy, though recurrence remains possible.

Choreo-acanthocytosis like phenotype without acanthocytes: clinicopathological case report. A contribution to the knowledge of the functional pathology of the caudate nucleus.

Detailed clinical and neuropathological findings in two unrelated patients with a chorea-acanthocytosis-like phenotype (CA) are reported. One case met all the diagnostic criteria of CA and had a deceased brother with the same disease. The second case had a virtually identical phenotype to the former but without acanthocytes. These findings suggest that both patients are affected by the same disease and that acanthocytes are not essential to the diagnosis. Neuropathological autopsy studies on the brain of the second case showed selective atrophy of the caudate nucleus that seemed to correspond to the movement disorder and behavioural abnormalities prominent in this patient. In both subjects, morphometric and ultrastructural examination of the peripheral nerve showed loss of myelinated fibres, more accentuated distally, and cytoskeletal changes in the axoplasm. These findings support the hypothesis that peripheral neuropathy in CA is caused by distal axonopathy.

Type I sialidosis: a clinical, biochemical and neuroradiological study.

We report biochemical, morphological and neuroradiological findings in a 40-year-old woman affected with type I sialidosis. The clinical symptoms, consisting of a cerebellar syndrome, were first noted at the age of 17 years. The macular cherry-red spot was first observed after 23 years of disease. A CT scan performed at 21 years of age showed enlargement of the fourth ventricle. Nuclear magnetic resonance imaging of the brain performed at the age of 40 showed severe atrophy of the cerebellum and pontine region; atrophy of cerebral hemispheres and of the corpus callosum was also observed. We emphasize the prolonged course of illness in this patient, observed over a long period of time. Of particular interest is the neuroradiological study showing our findings both at the beginning of the disease and after 20 years.