Prevalence of peripheral nervous system complications after major heart surgery.

We evaluated 374 consecutive patients from May 2013 to April 2014 who underwent major cardiac surgery. Each patient had an interview and a neurological clinical examination during the rehabilitation period. Patients with possible peripheral nervous system (PNS) complications underwent further electrodiagnostic tests. Among 374 patients undergoing major heart surgery (coronary artery bypass grafting, valvular heart surgery, ascending aortic aneurysm repair) 23 (6.1 %) developed 34 new PNS complications. We found four brachial plexopathies; four carpal tunnel syndromes; five critical illness neuropathies; three worsening of pre-existing neuropathies; two involvement of X, one of IX and one of XII cranial nerves; three peroneal (at knee), one saphenous, two median (at Struthers ligament), six ulnar (at elbow) mononeuropathies; two meralgia parestheticas. Diabetes is a strong risk factor for PNS complications (p = 0.002); we could not find any other relationship of PNS complications with clinical conditions, demographic data (gender, age) or type of surgical intervention. The mononeuropathies of right arms can be related to ipsilateral vein cannulation; position of body and stretching from chest wall retraction may be the cause of mononeuropathies of left arms (more frequent); the use of saphenous vein and position of the limbs may be the cause of mononeuropathies of the legs; surgical and anesthetical procedures can injure cranial nerves; respiratory failure and infection during the first days after surgery can cause critical illness neuropathies. Careful preoperative assessment and intraoperative management may reduce the risk of long-term PNS complications after cardiac surgery.

Early-onset Alzheimer disease in an Italian family with presenilin-1 double mutation E318G and G394V.

The genetic form of Alzheimer disease (FAD) accounts for about 5% of total Alzheimer disease (AD) cases, and the discovery of FAD-linked genes has shed new light on AD pathogenic mechanism. The presenilins genes (PSEN-1 and PSEN-2) carry the large majority of FAD-linked mutations. Here, we report an Italian kindred with FAD and PSEN-1 double mutation E318G+G394V that clearly cosegregates with the pathology. The E318G mutation has not an assessed pathogenic function, but some data have highlighted a role as a risk factor for AD in a predisposed familiar background. The G394V mutation was still described in association to an AD case with reported (but not demonstrated) familiar cosegregation. In an attempt to better characterize the biochemical effect of this double mutation, we assessed A beta(1-40) and A beta(1-42) concentrations in conditioned media from primary skin fibroblasts obtained from AD-affected and healthy family members. We did not find any modification of the A beta(1-42)/A beta(1-40) ratio, suggesting that this double mutation might be involved in early-onset AD etiopathogenesis by affecting a PSEN-1 function other than gamma-secretase activity.

A case of Guillain-Barrè syndrome following Staphylococcus aureus endocarditis.

Guillain-Barrè syndrome (GBS) is the most important cause of acute neuromuscular paralysis in western countries and it is preceded in almost all cases by an infectious disease such as Campylobacter Jejuni or Cytomegalovirus. However, GBS associated with previous bacterial endocarditis is very rare. We report the case of a 74-year-old man with GBS following Staphylococcus Aureus endocarditis affecting aortic valve. Although the absolute incidence of GBS is low, the present case stresses the need to consider GBS in patients developing neurological symptoms following any infectious illness, such as endocarditis, and highlights the challenging problem of rehabilitation and surgical management in these patients.

Fas-mediated T-cell apoptosis is impaired in patients with chronic inflammatory demyelinating polyneuropathy.

The Fas death receptor is expressed by activated lymphocytes and is involved in switching-off the immune response. Its inherited defects cause auto-immune lymphoproliferative syndrome. Impaired Fas function may also play a role in other auto-immune diseases, such as multiple sclerosis and type 1 diabetes mellitus. The aim of this work was to evaluate Fas function in T cells from patients with chronic inflammatory demyelinating polyneuropathy (CIDP). We evaluated Fas-induced apoptosis in T-cell lines from 27 patients with CIDP, 12 patients with acute inflammatory demyelinating polyneuropathy (AIDP), and 110 controls. CIDP patients displayed lower Fas function than both AIDP patients and controls, whereas no statistically significant difference was found between AIDP patients and controls. Moreover, Fas function was lower in CIDP patients with progressive course than in those with relapsing-remitting course and lower in CIDP patients with axonal damage than in those with pure demyelination. These data suggest that defective Fas function favours CIDP development and aggressive evolution.