Clinical Cases in Pediatric Peripheral Neuropathy

Abstract

There are many challenges to diagnosing peripheral neuropathy in children. While the symptoms are similar to those in adults, young children and those with developmental delays pose difficulties in extracting the appropriate history and performing a consistent and careful neurological examination. Neuropathic processes that present in childhood can be divided into those that are progressive and those that will tend improve over time. While there are exceptions, children with the latter category are those that fall into the acquired neuropathies such as vitamin deficiencies, toxicities, some immune mediated, and focal mononeuropathies. Those in the progressive category include the neuropathies that are hereditary/genetic in nature such as the heterogenous group of Hereditary Sensory Motor Neuropathies and some immune mediated neuropathies. In general, when the neuropathies of this group present earlier in childhood, the course and prognosis is worse than if they were to present in adolescence and adulthood. There are exceptions to this as some children who initially present as floppy infants due to a congenital neuropathy with respiratory difficulties can attain the ability to walk independently. A lot of the entities discussed in this chapter have been discussed in others that are dedicated to their specific mechanisms. What this chapter will try to achieve is to discuss the pediatric presentations of these disorders and to highlight, if present, differences between the adult and pediatric presentations of neuropathies. While this chapter will touch on the pathophysiology, electrodiagnostic findings, and laboratory findings, it will not try to duplicate these areas of discussion found in other chapters of this book. The intent of this chapter is to discuss pediatric presentations of peripheral neuropathy in the context of clinical cases to allow the reader to consider these diagnoses in children. A mention of performing electrodiagnostic testing is essential in any chapter discussing peripheral neuropathy. The evaluation of weakness often employs using nerve conduction studies and electromyograms. In young children and especially in those who have developmental delays, this can be difficult. In this author’s experience, performing these electrodiagnostic studies in children is more time consuming. Frequent coaching and coaxing is often needed. Without sedation available, many studies are truncated due to tolerance, inability to cooperate, and inability to follow commands. A child life specialist can be valuable in utilizing distraction techniques. Certified Child Life Specialists have been used in various clinical settings to help ease the anxiety associated with procedures (McGee, 2003). Also, use of a local anesthetic cream such as topical lidocaine may be helpful in