Search

Abstract: This study aims at presenting a case of symmetrical and bilateral thinning observed in a skull belonging to the skeleton of a mature woman from the medieval cemetery of Caravate (north Italy). Macroscopical, radiological, and histological analyses were performed to investigate the condition. The analyses allowed us to detect a progressive loss of both the outer table and the diploe, and the sparing of the inner table. As a controversial condition in the clinical and paleopathological literature, this case poses some difficulties in discussing the differential diagnosis. However, the sex determination, estimation of the age-at-death and different characteristics observed at the level of the postcranial bones, in particular the fractures recorded on different vertebral bodies, allowed us to correlate the biparietal thinning found in this subject to ageing and osteoporosis., Competing Interests: The authors report no conflicts of interest., (Copyright © 2021 by Mutaz B. Habal, MD.)

Purpose of Review: This article provides an up-to-date assessment of the role of skin biopsy in the evaluation of autonomic disorders. The standard methodology for completing a skin biopsy, the anatomic structures of interest detected within a skin biopsy, and the disease states in which skin biopsies may provide valuable information are reviewed., Recent Findings: Several recent advances in the studies of hereditary amyloidosis and the various degenerative synucleinopathies have demonstrated that simple skin biopsies can provide valuable pathologic evidence of neurologic disease. In addition to diagnosis of the underlying disorder, skin biopsies provide a quantitative structural measurement of the associated autonomic damage., Summary: Skin biopsies are making great inroads into the study of autonomic and peripheral nerve disorders. Complex immunohistochemical staining protocols are challenging to complete, but the rich data derived from these studies in the diagnosis and monitoring of different disease states suggest that the role of skin biopsies in the study of the autonomic nervous system will continue to expand in the years to come.

Distal symmetrical axonal polyneuropathy (DSP) is due to injury to peripheral sensory, motor, and autonomic nerve fibers, resulting in distal predominant sensory loss, pain, and gait instability. DSP occurs as a complication of multiple medical conditions including diabetes or HIV, or following exposure to various toxins such as chemotherapy. It affects at least 10% of the United States population. Few treatments for DSP are approved by regulatory agencies. Reliable and responsive outcome measures are integral to developing new DSP treatments. Multiple clinician-rated measures that incorporate neuropathy signs exist, however, it is not clear which of these measures performs best for various DSP phenotypes. This systematic review summarizes the content of 18 published measures of DSP identified using PubMed and from personal archives of the authors. The relative percentage of scoring dedicated to motor, reflex, large and small fiber sensory, and autonomic domains varied considerably among measures. The most common neurologic examination items included in the scales were (1) vibration perception (n = 18, 100%), (2) reflexes (n = 16, 89%), (3) pinprick perception (n = 14, 78%), (4) muscle strength (n = 11, 61%), (5) touch-pressure perception (n = 9, 50%), and (6) joint position perception (n = 8, 44%). This review can be used to inform decisions regarding which of the available clinician-rated sign outcome measures would be most appropriate for use in a particular DSP population, based on the domains most affected by that neuropathy or on the domains most likely to be affected by a particular experimental therapy., (© 2019 American Academy of Neurology.)

Objective: To evaluate long-term lenalidomide neurotoxicity and correlation with cumulative dose and hematologic response., Methods: Nineteen myeloma patients (7 men, mean age 63.2 years) underwent clinical and neurophysiologic assessment at baseline and at 2 (8 patients, group A) or 5 years (11 patients, group B) after starting lenalidomide therapy for relapsed/refractory multiple myeloma. Neuropathy was scored with Total Neuropathy Score clinical version (TNSc). Lenalidomide cumulative dose was correlated with severity of neuropathy and hematologic response., Results: At enrollment, 7/19 patients (3 in group A, 4 in group B) had neurophysiologic signs of neuropathy secondary to previous chemotherapy, in 2 of them subclinical. Neurophysiologic evidence of sensory axonal neuropathy occurred in 4/8 patients at 2 years follow-up (group A) and in 3/11 patients at 5 years follow-up (group B). Dorsal sural nerve sensory action potential amplitude was the earliest neurophysiologic abnormality. No relevant (≥4) clinical changes were found in TNSc score. Hematologic overall response was 62% in group A and 100% in group B. No correlation was found between lenalidomide cumulative dose and neuropathy or between neuropathy and hematologic response., Conclusions: In our study, up to 50% of myeloma patients on long-term lenalidomide therapy developed sensory axonal neuropathy. Reduced dorsal sural nerve sensory action potential amplitude was the first neurophysiologic alteration. Neuropathy was usually subclinical or mild, however. Neurotoxicity was independent of lenalidomide cumulative dose and hematologic response., (© 2016 American Academy of Neurology.)