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Neuromuscular Disorders of Infancy, Childhood, and Adolescence

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Neuromuscular Disorders of Infancy, Childhood, and Adolescence Chapter 1 Introduction: Historical Perspectives Darryl C. De Vivo, Basil T. Darras, Monique M. Ryan, and H. Royden Jones, Jr. INTRODUCTION infancy, childhood, and adolescence. Nevertheless, these traditional tests remain useful in certain situations: to The role of the clinician in the diagnosis and treatment of evaluate patients in whom molecular genetic analyses fail a weak child is as important today as it was in the 19th to confirm the initial clinical impression, to facilitate the century, when pediatric neuromuscular diseases were first 1 rapid electrophysiologic diagnosis of a neuropathy or being recognized by such luminaries as Meryon (1852), spinal muscular atrophy, or to focus more precisely on Duchenne (1861),2 Werdnig (1891),3 and Hoffmann 4 5 molecular mechanisms before ordering relatively expen- (1893), and later by Batten (1903). The clinician needs sive molecular genetic tests. The dramatic advances in to react to the concerns of the patient and their parents at DNA diagnostics have added to the complexity of a chal- the first encounter. A detailed medical history and care- lenging clinical field and have left physicians occasion- fully performed physical examination remain the funda- ally uncertain about the relative indications for traditional mental tools and starting point for assessing various tests such as EMG and muscle biopsy. Clearly, all these symptoms and signs. This initial clinical assessment has diagnostic tests remain useful, and it is up to the modern three possible immediate outcomes: (1) the concerns of clinician to make informed decisions, after the initial clin- the patient and family appear to be unfounded, and the ical evaluation, to facilitate an accurate biomolecular clinician can be reassuring. A follow-up visit is advised to diagnosis as quickly and as economically as possible. substantiate this outcome and to give the parents peace of mind that all, indeed, is well; (2) the clinician shares the concerns of the patient and family but decides to use time as the first test to determine the natural history of the pro- TRADITIONAL DIAGNOSTIC TESTS cess; or (3) the clinician recognizes the importance of the The technique of muscle biopsy was first introduced by clinical symptoms and realizes the need to perform diag- Duchenne in 1868, using a harpoon-like device to sample nostic procedures as soon as possible. the affected tissue during life.12 This crude device was This clinical approach places great demands on the the harbinger of the biopsy needle. Earlier and later inves- physician at the first and subsequent encounters. Here, the tigators used muscle tissues obtained after the patient’s difference between the 19th-century clinician and the death. These techniques have continued to be refined, and 21st-century clinician is enormous. This difference is a analysis of involved tissue has become increasingly ele- measure of the scholarly advances of the past century, gant and precise, as demonstrated by advances in electron particularly over the past three decades, since the advent microscopy, enzyme histochemistry, immunocytochemis- À of molecular neurogenetics.6 11 Examples of such try, and single myofiber analysis. Today, a biopsied speci- advances include major discoveries in the genetic analysis men can be processed for several studies, including light of the muscular dystrophies, congenital myasthenic syn- microscopy, electron microscopy, enzyme histochemistry, dromes, spinal muscular atrophies, and hereditary neurop- immunohistochemistry, biochemistry, tissue culture, and athies. These advances in molecular neurogenetics have molecular studies,13 and protein expression patterns can led to new diagnostic tests, which may confirm the clini- be assessed in single 8-micron sections taken from muscle cal diagnosis accurately and noninvasively. Traditional biopsies.14 Advances in electrophysiology have been diagnostic tests such as nerve conduction studies, electro- equally dramatic. The first application of these physio- myography (EMG), and open muscle biopsy are less often logic principles to neuromuscular diseases dates to the required in the evaluation of neuromuscular disorders in latter part of the 19th century.2 However, the practical use B.T. Darras, H. Royden Jones, Jr., M.M. Ryan & D.C. De Vivo (Eds): Neuromuscular Disorders of Infancy, Childhood and Adolescence, Second edition. DOI: http://dx.doi.org/10.1016/B978-0-12-417044-5.00001-9 © 2015 Elsevier Inc. All rights reserved. 3 4 PART | I Clinical and Laboratory Approach to the Infant and Child with a Neuromuscular Problem of nerve conduction studies and needle EMG did not neuromuscular junction. Additionally, precise microelec- emerge until the mid-20th century. At that time, observa- trode analysis of a biopsied neuromuscular junction is still tions by Lambert at the Mayo Clinic in Rochester, necessary for diagnosis of some conditions. Minnesota, Gilliatt at Queens Square in London, and Infant motor unit potentials (MUPs) are typically quite Buchthal at the Institute of Neurophysiology in small, mimicking the size of abnormal adult “myopathic” Copenhagen defined the electrical parameters of the nor- motor units. This maturational distinction sometimes chal- mal peripheral motor and sensory unit as well as the lenges the clinical neurophysiologist who is attempting to potential for widespread application of these findings to differentiate a myopathy in a newborn from a normal the evaluation of neuromuscular disorders. These three response. The rapid acquisition of a full recruitment clinical neurophysiologists, among others, also recognized pattern encompassing very small MUPs may provide the the importance of carefully defined normal neurophysio- initial clue to the presence of a myopathy. On some logic values, which are still used today.15À20 These stud- occasions, it is equally important for the clinical neuro- ies defined the significant maturational changes during physiologist to advise the referring physician that even early development that remain important to the perfor- though the MUPs may appear to be normal in a floppy mance and interpretation of pediatric EMG, as detailed infant, such a finding does not exclude a myopathy. in Chapter 3. The more universal application of EMG Similarly, the finding of fibrillation potentials may not to pediatric motor unit disorders had attracted increasing define a neuromuscular disorder as either neurogenic or interest during the two decades that preceded the mapping myopathic. Generally, these potentials signify a denervat- and discovery of the SMN gene mutated in classic spinal ing process, but similar abnormalities are sometimes seen muscular atrophy.20,21 Byers’ and Banker’s classification in myopathies.29 In reality, the fibrillation potential results of the spinal muscular atrophies at Boston Children’s from the disconnection of all or part of a myofiber from Hospital was an important early contribution22 later the nerve, so the principle remains intact; that is, fibrilla- expanded by Dubowitz.23 tion potentials signify denervation of the myofibers. It is Other seminal reports continued to appear. Dyck important to emphasize that the MUP is the key to making and Lambert separated Charcot-Marie-Tooth disease into the neurophysiologic distinction between a primary ante- demyelinating and axonal forms, presaging a similar rior horn cell process and one that relates to a dysfunction DNA classification that followed a quarter century later.24 of the muscle fiber.29 Differentiating a normal immature Gutrecht and Dyck performed important anatomic studies MUP from a congenital myopathy or early dystrophy is a defining the maturation of peripheral nerve myelination.25 bigger challenge for the clinical neurophysiologist. These findings mirrored the normal increase in the DNA analysis now frequently resolves the confusion speed of peripheral nerve conduction from infancy to age in these clinical settings. The benefit of DNA analysis is 5 years.16 Appreciation of these normal ranges for motor apparent in the evaluation of Duchenne muscular dystro- nerve conduction velocities also led to the definition of a phy (DMD), where EMG and muscle biopsy were state- subacute acquired polyneuropathy: chronic inflammatory of-the-art diagnostic studies three decades ago. Today, the demyelinating polyneuropathy (CIDP). These observa- clinical diagnosis of children with suspected DMD or tions were important both because of CIDP’s predominant spinal muscular atrophy can be quickly confirmed by spe- proximal presentation, often mimicking a myopathy, but cific DNA testing. Similar changes are occurring in a particularly because CIDP is a treatable condition. These number of the other hereditary neuromuscular disorders, maturational distinctions also emphasize the importance as reviewed elsewhere in this book. of traditional testing modalities such as nerve conduction Clinical chemistry methods emerging after World War studies to enable diagnosis in complex cases with dual II provided another important means of distinguishing pathologies, such as the rare instances of CIDP superim- between neuropathies and myopathies. The serum transa- posed upon genetic neuropathies.26,27 minases and aldolase measures were introduced first, but Engel and Lambert applied microelectrode methodol- measurement of serum creatine kinase (CK) activity has ogy in vitro, extending our understanding of the various proved to be more tissue specific, CK expression being uncommon congenital neuromuscular transmission defects
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