INC Featured Speakers

INC Featured Speakers
23 June, 2019

Shirley D'Sa, MD, FRCP, FRCPath, University College London Hospitals, NHS Foundation Trust, London, United Kingdom 
Title: Monoclonal Gammopathies of Neurological Significance

 

Plasma cell disorders are characterised by an abnormal clone of plasma cells, identifiable as monoclonal gammopathy. Monoclonal gammopathy of undetermined significance (MGUS) is a benign process with the potential to progress to a malignant condition. A low tumour burden monoclonal entity with resulting organ damage has been termed monoclonal gammopathy of clinical significance (MGCS), based on the mechanism of tissue injury rather than per organ involvement. Diverse mechanisms such as deposition of monoclonal immunoglobulin, development of autoimmune disorders, activation of the complement pathway, absorption of active molecules, or secretion of cytokines. An update on the diagnosis and management of such plasma cell and lymphoproliferative disorders including Waldenström's macroglobulinaemia, Bing-Neel syndrome, AL amyloidosis and POEMS syndrome will be discussed. The advent and role of biological and targeted treatments will be described, including how they measure up to existing more conventional chemoimmunotherapeutic approaches.

 


Francesco de Assis Gondim, MD, MSc, PhD, MD, Universidade Federal do Ceará, Fortaleza, Brazil
Title:
Peripheral Neuropathy and Gastrointestinal Disease

          The link between gastrointestinal and neurological diseases is extensive. Peripheral neuropathy stands as one of the most common neurological manifestations. Peripheral nerve involvement (neuropathy, autonomic neuropathy, CIDP and mononeuritis multiplex) has been linked to celiac disease in a nationwide population-based study. Inflammatory bowel disease (IBD) patients are also affected by a wide range of peripheral nerve diseases, varying from small fiber, to axonal and demyelinating neuropathies. A small cohort study reported that neuropathy is 5-7 times more common in patients with IBD than controls. The mechanisms of nerve injury in IBD are complex and include nutritional/vitamin deficiencies, neurotoxicity from IBD therapies and immune-mediated disorders. Neuropathy in IBD is frequently subclinical or mildly symptomatic, only detected by neurophysiological tools. Although not linked to disease activity, recent data support a decreased incidence of neuropathy (especially more severe phenotypes) with the advent of new therapies and nutritional status.

 


Yusuf Rajabally, MD, FRCP, Aston University, Birmingham, United Kingdom
Title: 
An Overview of Inflammatory Neuropathies in Diabetes

   

A number of inflammatory neuropathies may occur during diabetes. Misdiagnosis is not uncommon in view of the presumed responsibility of diabetes causing an untreatable neuropathy. On the other hand, diagnostic difficulty may also result in consideration of inappropriate therapies. Electrophysiology in the context of diabetes may be affected by the presence of an underlying diabetic neuropathy, resulting in further complicating the diagnostic process. This talk summarises the main forms of inflammatory neuropathy that need to be promptly recognised for adequate management in the setting of underlying diabetes.


24 June, 2019


Luca Padua, MD, PhD, Università Cattolica del Sacro Cuore - Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy &
Simonetta Gerevini, MD, IRCCS San Raffaele Hospital, Milano, Italy
Title: Ultrasound of Peripheral Nervous System: Usefulness for Diagnosis and Therapeutic Indication

 

Electrodiagnosis is the main tool in assessing nerve function and hence is crucial in the diagnosis of nerve involvement. In the last years, an improved resolution, an increased portability and a wider access to ultrasound (US) have made this tool useful in assessing peripheral nerve impairments as entrapments, tumors, extrinsic compressions, traumatic lesions, immune-mediated and inherited neuropathies. A wide literature shows that US integrates neurophysiological assessment in routine practice: by a combined use of electrodiagnosis and US we obtain more information than we do if they are separately used. If neurophysiology allows detecting nerve function, US provides detailed imaging of nerve morphology and size and of the surrounding structures. A multidimensional evaluation of peripheral nervous system impairments improves diagnostic precision and therapeutic accuracy, even providing data about prognosis and disease evolution. The main quantitative measure that US provides, owing to its sensitivity, is the nerve cross-sectional area (CSA), which is the area of the nerve structure visualized by a transverse US scan, calculated using the ellipse or the tracing method. Usually, an increase CSA indicates nerve involvement. Besides this, we can evaluate echogenicity, alteration of nerve elements (e.g. fascicles), and variations in nerve shape. US report is based on characterization of nerve abnormalities and localization and extension of these findings.

 

Haruki Koike, MD, PhD, Nagoya University Graduate School of Medicine, Japan
Title: 
Neuropathology of Inflammatory Neuropathies

   

Recent advances in the search for autoantibodies against components expressed at nodal regions, such as the nodes of Ranvier and the paranodes, have significantly contributed to clarifying the pathogenesis in a subpopulation of chronic inflammatory demyelinating polyneuropathy (CIDP) patients. In particular, IgG4 antibodies to paranodal junction proteins, including neurofascin 155 and contactin 1, have attracted the attention of researchers. By contrast, the mechanisms of neuropathy in cases with classical macrophage-induced demyelination remain unclear despite the long-standing recognition of this process in Guillain-Barré syndrome and CIDP. The site that macrophages select to initiate myelin breakdown is located around the nodal regions in some patients and internode in others. Hence, it seems that the components that distinguish between the nodal regions and internode play a pivotal role in the behavior of macrophages that initiate phagocytosis of myelin. I will present pathological features of inflammatory neuropathies, focusing on the ultrastructure of Guillain-Barré syndrome and CIDP.