Treatment Two novel approaches, a small molecule, tafamidis, that prevents the aggregation of amyloid fibrils by stabilizing it in a tetrameric form (Coelho et al, 2012) and interfering RNA therapy (RNAi) that reduces the production of mutant amyloid (Coelho et al, 2013) are both promising. Both have shown promise in halting or reversing familial amyloidosis (Adams et al 2018 and Benson et al). Beneficial effects have been shown in some aspects of the neuropathy but it is not clear if these are sustained. Liver transplantation has proved curative of some of the familial amyloid polyneuropathies, but obviously it has no role in the acquired forms. According to Herlenius and colleagues, more than 500 patients at the time of their writing had received liver transplants with a 77 percent rate of survival, equivalent to liver transplantation for other diseases.
Problems in Diagnosis of the Chronic Polyneuropathies
This is the group of peripheral nerve diseases that has given the present authors the most difficulty. The cause of acute and many of the subacute and relapsing forms of nerve disease usually can be established by widely available clinical and laboratory methods. It is the early and late chronic polyneuropathies that continue to baffle the neurologist and general physician, despite the value of EMG and nerve conduction studies and the respectable advances that have been made in the field of genetic testing.
Diagnosis of Early Chronic Polyneuropathy