Sporadic Creutzfeldt-Jakob disease: a case report of long disease duration and difficulties in confirming the diagnosis with short literature review.

Creutzfeldt-Jakob disease (CJD) is a spongiform encephalopathy with the fatal outcome, caused by the accumulation of pathological prion protein in the central nervous system (CNS). CJD is classified into four types: sporadic (sCJD), familial or genetic (fCJD), iatrogenic (iCJD) and variant form (vCJD). The recognition of CJD is based on the clinical presentation, neuroimaging, electroencephalography and biochemical tests. The hyperintense signals in basal ganglia on brain magnetic resonance imaging (MRI), periodic sharp and slow wave complexes (PSWCs) in the electroencephalogram as well as presence of neuronal proteins such as protein 14-3-3 in the cerebrospinal fluid (CSF) support the diagnosis. The definite diagnosis of CJD still demands neuropathological confirmation. We report the case of a 56-year-old woman with the rapidly progressive cognitive impairment, motor dysfunctions and the fulminant neurological deterioration to akinetic mutism during the five weeks' hospitalisation. The probable diagnosis of sCJD was based on medical history and characteristic findings in MRI. The positive result of the real-time quaking-induced conversion (RT-QuIC) test and presence of protein 14-3-3 were obtained post-mortem and definite diagnosis was confirmed by neuropathological examination. In this paper we would like to emphasize the difficulties in reaching the diagnosis and the need for a series of diagnostic examinations in different points of time to obtain the confirming results.

A Correction Method for Wet Gas Flow Metering Using a Standard Orifice and Slotted Orifices.

Flow measurements that utilize differential pressure meters are commonly applied in industry. In such conditions, gas flow is often accompanied by liquid condensation. For this reason, errors occur in the metering process that can be attributed to the fluctuations in continuous phase parameters in the flow. Furthermore, the occurrence of a dispersed phase results in flow disturbance and dynamic pressure pulsations. For the above reasons, new methods and tools are sought with the purpose of performing measurements of gas-liquid flows providing measurement results that can be considered as fairly accurate in the cases when flow involves a liquid phase form. The paper reports the results of a study involving measurement of wet gas flow using differential pressure flowmeters. The experiments were conducted for three constant mass air flow rates equal to 0.06, 0.078 and 0.086 kg/s. After stabilization of the air flow rates, water was fed into the pipe with flow rates in the range from 0.01 to 0.16 kg/s. The research involved a standard orifice and three types of slotted orifices with various slot arrangements and geometries. The analysis focused on the effect of orifice geometry on the flow metering results. On the basis of the results, it was found that the slotted orifice generates smaller differential pressure values compared to the standard orifice. The water mass fraction in the gas leads to overestimated results of measurements across the flowmeter. Regardless of the type of the orifice, is necessary to undertake a correction of the results. The paper proposes a method of gas mass flow correction. The results were compared with the common over-reading correction models available in the literature.

Repetitive transcranial magnetic stimulation for the treatment of cognitive impairment in frontotemporal dementia: an open-label pilot study.

BACKGROUND: Frontotemporal dementia (FTD) is one of the most frequent dementia types in patients under 65 years of age. Currently, no therapy can effectively improve the cognitive deficits associated with FTD. Repetitive transcranial magnetic stimulation (rTMS) is a noninvasive method of inducing brain plasticity with therapeutic potential in neurodegenerative diseases. The purpose of this study was to evaluate the effect of rTMS on cognitive, behavioral, and emotional function in FTD. METHODS: Nine patients (seven women, four men, mean age 61.7±10.1 years) with the behavioral variant of FTD, one with nonfluent/agrammatic variant primary progressive aphasia, and one with progressive nonfluent aphasia (subtypes of FTD) underwent 10 daily sessions of 10 Hz rTMS over the bilateral dorsolateral prefrontal cortex. Cognitive and behavioral assessments were administered before and after therapy. RESULTS: After rTMS, the Montreal Cognitive Assessment and letter and digit cancellation test scores, as well as reading time and error number in the Stroop test improved. The caregivers' impression of the daily functioning of patients improved in the Frontal Behavioral Inventory scores. These changes were not paralleled by an improvement of mood. CONCLUSION: The results indicate that rTMS may improve the cognitive performance of patients with FTD and warrant sham-controlled trials.