Post-Polio Syndrome Revisited

Post-polio syndrome (PPS) is characterized by recrudescence or worsening of motor neuron disease symptoms decades after recovery from acute paralytic poliovirus infection, i.e., poliomyelitis. PPS afflicts between 25% and 40% of poliomyelitis survivors and mimics motor neuron diseases (MNDs), such as amyotrophic lateral sclerosis (ALS), due to its selective impairment, degeneration, or death of motor neurons in the brainstem and spinal cord. Herein, we report a case of PPS in a 68-year-old man with a remote history of bulbar and cervical cord involvement by poliomyelitis, review the relevant literature, and contrast the salient histopathologic features that distinguish our case of PPS from ALS

Experimental Alcohol-Related Peripheral Neuropathy: Role of Insulin/IGF Resistance

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Quality improvement in neurology: Muscular dystrophy quality measures

The muscular dystrophies (MDs) are a heterogeneous group of genetically determined myopathies. Identification of underlying genetic defects has demonstrated that MDs exhibit significant phenotypic and genetic heterogeneity. One genetic mutation can lead to a variety of phenotypes while different genetic mutations can manifest similar phenotypes; therefore MDs are challenging to diagnose. A major goal of health care reform in the United States is to replace the traditional fee-for-service model with a value-based system, which incentivizes high-quality care. Quality measurement is an integral and necessary part of this process.1,2 While standardizing care of MDs can be challenging because of their heterogeneity, common themes of management, such as the maintenance of nutrition, sustaining mobility, and management of complications, are applicable to many MDs. We report a quality measurement set for the management of MDs

A randomized, double-blind, placebo-controlled phase II study of eculizumab in patients with refractory generalized myasthenia gravis

Complement activation at the neuromuscular junction is a primary cause of acetylcholine receptor loss and failure of neuromuscular transmission in myasthenia gravis (MG). Eculizumab, a humanized monoclonal antibody, blocks the formation of terminal complement complex by specifically preventing the enzymatic cleavage of complement 5 (C5). This study was a randomized, double-blind, placebo-controlled, crossover trial involving 14 patients with severe, refractory generalized MG (gMG). Six of 7 patients treated with eculizumab for 16 weeks (86%) achieved the primary endpoint of a 3-point reduction in the quantitative myasthenia gravis (QMG) score. Examining both treatment periods, the overall change in mean QMG total score was significantly different between eculizumab and placebo (P = 0.0144). After assessing data obtained from all visits, the overall change in mean QMG total score from baseline was found to be significantly different between eculizumab and placebo (P < 0.0001). Eculizumab was well tolerated. The data suggest that eculizumab may have a role in treating severe, refractory MG