Quantitative, functional MRI and neurophysiological markers in a case of Gerstmann-Sträussler-Scheinker syndrome.

Gerstmann-Sträussler-Scheinker syndrome (GSS) is an inherited autosomal dominant prion disease, caused by a codon 102 proline to leucine substitution (P102L) in the prion protein gene (PRNP). We describe the case of a 40-year-old male, affected by a slowly progressive gait disturbance, leg weakness and cognitive impairment. Genomic DNA revealed a point mutation of PRNP at codon 102, resulting in P102L, and the diagnosis of GSS was confirmed. Somatosensory evoked potentials showed alterations of principal parameters, particularly in the right upper and lower limbs. Laser-evoked potentials were indicative of nociceptive system impairment, especially in the right upper and lower limbs. Conventional magnetic resonance imaging (MRI) revealed marked atrophy of the vermis and cerebellar hemispheres and mild atrophy of the middle cerebellar peduncles and brainstem, as confirmed by a brain volume automatic analysis. Resting-state functional MRI showed increased functional connectivity in the bilateral visual cortex, and decreased functional connectivity in the bilateral frontal pole and supramarginal and precentral gyrus. Albeit limited to a single case, this is the first study to assess structural and functional connectivity in GSS using a multimodal approach.

Experience in Italy in the development and application of clinical guidelines for low back pain.

UNLABELLED: Clinical Guidelines (CG) reflect the up to date scientific knowledge in the treatment of Low Back Pain (LBP). The diffusion of CG and their everyday application by health care professionals is a significant problem. As most CG are developed in English, the concerns are obviously greater in non English-speaking countries. The first CG on LBP by the Quebec Task Force (1987) was introduced in 1990 by the Gruppo di Studio della Scoliosi (GSS). Some studies where planned to verify their everyday application. The first one was carried on in Mantua, and evaluated the assessment of patients by General Practitioners (GPs): there is a clear tendency to over-prescribe examinations in acute cases, while in chronic cases under-prescription is sometimes seen. An educational approach was then proposed through a number of meetings, with fable RESULTS: A third experience verified the help GPs could receive through two different educative interventions such as a booklet and a direct access to a classical Back School. In acute patients a Booklet is useful, while Back School is not; at long term follow-up, chronic cases were significantly reduced only by the Back School approach. Finally, the Abruzzo Study's results on GPs management through computer-assisted evaluation is reported. The second part of the paper deals on the new experiences that are underway on the application of Diagnostic-Therapeutic Pathways (DTP) to Low Back Disorders.

Management of the peritoneal dialysis patient receiving radioactive iodine 131.

The patient met all expected outcomes while receiving I-131 and performing CAPD. As anticipated, the multidisciplinary approach proved to be effective in meeting this patient's needs. Careful planning and supportive care by the nephrology professionals resulted in a positive influence on F.A. and his family's acceptance of the prescribed treatment. Providing education for the nurses and patient decreased anxiety levels, and enabled F.A. and the nephrology nurses to cope with this difficult situation. The optimal delivery of nursing care was enhanced through patient and staff education, open communication and proper preparation of the patient, family, nurses, and environment for the safe administration of radioactive I-131. The nursing care of this patient was indeed an extraordinary experience. The purpose of this case study is to serve as a useful reference to other health care professionals who might face a similar dilemma.