Introduction Leprosy is a chronic infectious disease caused by Mycobacterium leprae ( M. leprae ), an acid-fast, Gram-positive, rod-shaped, obligate intracellular parasite which has the unique propriety of systematic invasion and multiplication inside peripheral nerves. Although mostly known due to…
Introduction In a sense, all peripheral nervous system (PNS) disorders caused by toxic molecules are toxic neuropathies. Even normal PNS proteins can have deleterious effects if overexpressed. For example, Charcot-Marie-Tooth syndrome type 1A (hereditary motor and sensory neuropathy type 1A)…
Introduction There is a clear distinction between a peripheral neuropathy that is clinically significant and which is a presenting feature of the underlying condition (e.g. those associated with diabetes mellitus, lymphoma, and uremia) and a peripheral neuropathy that is asymptomatic…
Epidemiology Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) comprises a small but important subset of chronic childhood polyneuropathies. One series identified childhood CIDP in 11/125 (9%) of all children with a chronic polyneuropathy confirmed by sural nerve biopsy. A second series reported…
Introduction The acute polyneuropathies are a group of acquired, generally immune-mediated or infectious disorders causing dysfunction and sometimes degeneration of peripheral nerves, spinal sensory and motor nerve roots, and occasionally the cranial nerves. The most common of these conditions are…
Introduction Peripheral neuropathy is associated with numerous inherited metabolic diseases, such as Fabry disease (trihexosylceramide lipidosis) where it may be the presenting complaint, or adrenomyeloneuropathy, in which it may play a relatively minor role in contrast to the more prominent…
Introduction The hereditary sensory and autonomic neuropathies (HSANs) are a group of rare disorders caused by different genetic mutations, each affecting specific aspects of development, function, or survival of small myelinated and unmyelinated neurons resulting in variable phenotypic expression. Affected…
Definition and Nomenclature This chapter will focus on those hereditary neuropathies that can present in late childhood and adolescence. Inherited neuropathies are often referred to collectively as Charcot-Marie-Tooth (CMT) disease, an eponym used in recognition of the three men who…
Introduction The congenital and infantile neuropathies are an uncommon and complex group of conditions with broad phenotypic and genetic diversity. The overwhelming majority have a genetic basis, and will be the main focus of this chapter. Rarely, acquired inflammatory causes…
You’re Reading a Preview Become a Clinical Tree membership for Full access and enjoy Unlimited articles Become membership If you are a member. Log in here