INTRODUCTION Among the hereditary systemic amyloidosis, transthyretin (TTR) neuropathies (OMIM #176300) are devastating disorders with an autosomal dominant transmission, expressed mainly as a progressive fibre length dependent sensorimotor polyneuropathy and life threatening autonomic dysfunction. Initially, the condition was recognised in northern Portugal, in the area of Povoa de Varzim, whe...

PURPOSE OF REVIEW To summarize the current understanding of clinical assessment, pathophysiology, and treatment of pain in neuropathies, focusing on selected entities in which the understanding of the mechanisms underlying pain has advanced recently. RECENT FINDINGS Ongoing studies are classifying the symptoms and signs of painful neuropathies, assuming that this approach may indicate particu...

BACKGROUND Lower cranial neuropathies are a late effect of radiotherapy (RT), typically reported in nasopharyngeal cancer survivors. Limited data examine these neuropathies after oropharyngeal cancer, particularly as it relates to late radiation-associated dysphagia (late-RAD). METHODS Two cases were examined over 4 to 6 years. Late-RAD was assessed per MBS impairment profile (MBSImP™©), Pene...

The pathological differential diagnosis between potentially treatable autoimmune neuropathies and degenerative neuropathies is often difficult if major T-cell infiltrates are absent in sural nerve biopsies. Since it is suggested that macrophages play a central pathogenetic role in inflammatory neuropathies, we investigated the expression of macrophage differentiation antigens associated with ac...

Electrodiagnostic (EDX) patterns of neuropathic dysfunction have been based on axonal/demyelinating criteria requiring prior assumptions. This has not produced classifications of desired sensitivity or specificity. Furthermore, standard nerve conduction studies have limited reproducibility. New methodologies in EDX seem important. Recurrent Quantification Analysis (RQA) is a nonlinear method fo...

The inflammatory demyelinating neuropathies constitute a significant proportion of the acquired polyneuropathies. Major progress in finding the causes and in the treatment of these neuropathies has been made over the last decade. Early recognition is of paramount importance, because timely and appropriate treatment can largely reduce morbidity and disability. Electrodiagnosis plays a key role i...

There has been tremendous progress in the detection and diagnosis of hereditary neuropathies and while the genes responsible for these rare neuropathies have improved our understanding of the development of the sensory system, they remain clinical enigmas with rare occurrences and diverse presentations. In 1963, Swanson first described two brothers with congenital insensitivity to pain, anhidro...

UNLABELLED Post-surgical neuropathies are usually attributed to mechanical factors, such as compression, stretch, contusion or transection. The role of inflammatory mechanisms in neuropathies occurring after surgeries is poorly appreciated and not well characterized, and may provide a rationale for immunotherapy. A total of 23 selected patients with post-surgical neuropathies received nerve bio...