Clinical short communication
Polyglucosan bodies in intramuscular nerves: Association with muscle fiber denervation atrophy

https://doi.org/10.1016/j.jns.2015.11.053Get rights and content

Highlights

  • Polyglucosan bodies are occasionally found in intramuscular nerves of the elderly.

  • The presence of polyglucosan bodies may be age-related, but for related diseases.

  • The finding of polyglucosan bodies is associated with muscle denervation atrophy.

Abstract

Polyglucosan bodies (PB) in the intramuscular nerves have been rarely studied, and their presence particularly in subjects without neurologic disorders has been thought to be age-related. We examined, by using light and electron microscopy, 204 consecutive muscle biopsies. PB was found in 5 quadriceps intramuscular nerves (2.45% of all biopsies). All 5 quadriceps containing PB exhibited varying degrees of muscle fiber denervation atrophy with or without fiber type grouping. These quadriceps with PB, compared with the other 119 quadriceps without PB, showed a significantly greater association with muscle fiber denervation atrophy (5/5 versus 55/119; p = 0.02, by two-tailed Fisher's exact test), for which aging is not confounding. Electron microscopy identified PB in intramuscular nerve myelinated fibers along with ongoing degenerative changes. Our observation suggests that PB in intramuscular nerves may be pathologic and associated with muscle fiber denervation atrophy.

Introduction

Polyglucosan bodies (PB) are small ellipsoid structures, consisting of glucose polymers, found especially in the central and peripheral nervous system (PNS) [1]. PB has been reported in sural nerves of patients with neurologic diseases [2], [3], [4], [5] and elderly people without neurologic diseases [6], [7], [8], as well as in intramuscular nerves of patients with adult PB disease [2], [3], Lafora's disease [2], [8], burning paresthesias [10], spastic paralysis [11], and elderly people without neurologic diseases [1], [8]. Only one large series found PB in intramuscular nerves of 55 out of 1822 (3.02%) muscle biopsies, although electron microscopy (EM) examination was not performed [1]. That study concluded that the presence of PB was nonspecific and correlated with aging, since the presence of PB increased with aging and showed no differences with respect to the diagnoses of various disorders [1].

It is well-known that age-related changes occur in PNS and skeletal muscles [12]. The aging PNS with a reduced number of motor units contributes to the muscle atrophy and lower muscle power [12], while physical activity may adapt to age-related denervation and promote reinnervation of muscle fibers [13]. With aging, muscle biopsies may exhibit “disseminated neurogenic atrophy” with progressive accumulation and clustering of small angulated fibers [13].

Although an increasing number of studies has suggested the association of PB with aging, the presence of PB may have neurotoxic potential and cause neuronal-selective apoptosis [14], [15]. Since the clinical significance of PB particularly in intramuscular nerves deserves further investigation [1], [8], [9], [10], [11], this study examines the intramuscular nerve PB in relation to neuromyopathic changes.

Section snippets

Methods

We prospectively examined 204 consecutive muscle biopsies that were reported by one neuropathologist from 2011 to 2014 at the University of Alberta Hospital. Open muscle biopsies were taken from the quadriceps (n = 124), deltoids (n = 45), biceps (n = 11), gastrocnemius (n = 9), and others (n = 15). The age of patients ranged from 0.25 to 88 years. The fresh biopsy specimens (transversely larger than 4 × 4 mm at minimum) were snap frozen in isopentane precooled in liquid nitrogen at − 80 °C. Serial transverse

Results

The intramuscular nerves, composed of 1–20 nerve fibers, were seen in approximately one quarter of all biopsies and identified mostly in the perimysium. PB was occasionally found in the intramuscular nerves but not in muscle fibers. Only the following 5 biopsies showed PB.

Discussion

This study is the first to demonstrate an association between the presence of PB and muscle fiber denervation atrophy in the quadriceps with ultrastructural and histochemical evidence. As the finding of PB in intramuscular nerves [1], [4], [8] and muscle fiber denervation atrophy are both associated with aging [12], there was a concern about confounding by aging in the present study. However, there are three criteria that need to be fulfilled to determine whether a variable could be considered

Financial disclosure

The authors are not receiving any financial or material support in conjunction with the generation of this submission.

Conflict of interest

The authors report no conflict of interest.

Acknowledgments

The authors thank Mr. Richard Vriend and Mrs. Lesley Burnet for their help with electron microscopy examination.

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