3/3/2017-   Dr. Ted Burns interviews Drs. Adam Loavenbruck and Phillip Low about their article "Transthyretin amyloid neuropathy has earlier neural involvement but better prognosis than primary amyloid counterpart: an answer to the paradox?" One hundred one cases of amyloidosis with peripheral neuropathy were identified, 60 primary and 41 transthyretin. Twenty transthyretin cases were found to have Val30Met mutations; 21 had other mutations. Compared to primary cases, transthyretin cases had longer survival, longer time to diagnosis, higher composite autonomic severity scale scores, greater reduction of upper limb nerve conduction study amplitudes, more frequent occurrence of weakness, and later non-neuronal systemic involvement. Four systemic markers (cardiac involvement by echocardiogram, weight loss > 10 pounds, orthostatic intolerance, fatigue) in combination were highly predictive of poor survival in both groups. Their findings suggest that transthyretin has earlier and greater predilection for neural involvement and more delayed systemic involvement. The degree and rate of systemic involvement is most closely related to prognosis. Ann Neurol 2016;80:401-411.

2/8/2016-  Dr. Justin Willer interviews Dr. Divisha Raheja about her Muscle & Nerve article on paraproteinemic neuropathies. When the neuropathy is the presenting feature, neuromuscular specialists can play a particularly important role, possibly uncovering clinical, laboratory, radiologic, electrodiagnostic, or biopsy findings that lead to identification of the underlying paraproteinemia. Dr. Raheja offers suggestions on when testing should be done, elaborating on the various types of testing as well as treatments. Muscle Nerve. 2015 Jan;51(1):1-13. doi: 10.1002/mus.24471.