Chapter 105 Peripheral Nerve Disorders E
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Chapter 105 Peripheral Nerve Disorders E. Bradshaw Bunney and E. John Gallagher ■ PERSPECTIVE oped countries, its annual incidence is just over 1 to 2 cases per 100,000 population.1 In contrast to the low incidence of Background acute peripheral neuropathies, several of which are associated The nervous system is traditionally divided into central with short-term mortality, the vast majority of peripheral nervous system (CNS) and peripheral nervous system (PNS) neuropathies seen in the emergency department (ED) are components. The PNS can be further subdivided into 12 subacute or chronic and are associated not with mortality but cranial and 31 spinal nerves. Disorders of the cranial nerves with long-term morbidity. are discussed in Chapter 103. Because diseases of the neuro- Current estimates suggest that about 1.5% of the U.S. popu- muscular junction and the myopathies are located distal to the lation suffers from peripheral neuropathy.2 Over 7% of the neuron itself, they are also considered separately in Chapter population has diabetes mellitus, with a prevalence rate of 104. Radiculopathies, which are disorders of the roots of the 20% in individuals older than 60 years. Roughly 50% of these PNS, are so commonly associated with musculoskeletal neck individuals have peripheral neuropathy.3 and back pain that they are mentioned only briefly here and are discussed in detail in Chapter 51. The simplest approach to diseases of the PNS parallels the CNS model of separating focal from nonfocal disease. In the ■ PRINCIPLES OF DISEASE PNS, the first broad category is the focal group, which can be Anatomy divided into those with evidence of single versus multiple lesions of peripheral nerves, known respectively as simple The spinal component of the PNS is shown schematically in mononeuropathies and multiple mononeuropathies (or mononeurop- Figure 105-2. The anterior and posterior nerve roots exit the athy multiplex). The second broad category, which constitutes spinal cord at each segmental level. Just distal to the dorsal the nonfocal group of peripheral neuropathies, contains the root ganglion they converge to form a mixed (motor and polyneuropathies. These tend to produce bilaterally symmet- sensory) spinal nerve, of which there are 31 pairs: 8 cervical, rical symptoms and signs, reflecting the widespread nature of 12 thoracic, 5 lumbar, 5 sacral, and 1 coccygeal. The spinal the underlying pathologic process. nerves immediately bifurcate into anterior (ventral) and pos- The evaluation of PNS disease involves a goal-directed terior (dorsal) rami. The posterior ramus travels to the back. history and physical examination targeted at answering the The anterior ramus innervates the anterolateral portion of the following three questions, each of which corresponds to a body and supplies all peripheral nerves for the upper and stratum of the algorithm presented in Fig. 105-1: lower extremities through the brachial and lumbosacral plexus, respectively. Interweaving of fibers occurs within a plexus, 1. Are the sensorimotor signs and symptoms symmetrical or producing a mixed sensorimotor innervation of peripheral asymmetrical? nerves exiting the plexus. 2. Are the sensorimotor signs and symptoms distal or both In addition to the motor and sensory modalities of the PNS, proximal and distal? the autonomic nervous system has a peripheral component. 3. Is the modality involved exclusively motor, sensory, or Anatomically and functionally, the autonomic nervous system mixed sensorimotor? is divided into two parts: a sympathetic (thoracolumbar) component and a parasympathetic (craniosacral) component. By systematically combining responses to these questions, Autonomic dysfunction may cause systemic abnormalities, one can identify seven discrete categories of peripheral neu- such as orthostasis, or local problems, such as atrophic, dry ropathy, each of which contains a finite set of possible diagno- skin. ses. Because pure motor or sensory findings tend to occur mainly in an asymmetrical, distal distribution, this is the only Pathophysiology category in Figure 105-1 subdivided into pure motor and pure sensory abnormalities. The PNS has only three basic responses to a wide array of pathologic stimuli. As shown in Figure 105-2, these are (1) the Epidemiology myelinopathies, where the primary site of involvement is limited to the myelin sheath surrounding the axon; (2) the Although Guillain-Barré syndrome (GBS) is the most com- axonopathies, where the primary site of involvement is the monly encountered emergent peripheral neuropathy in devel- axon, with or without secondary demyelination; and (3) 1398 1399 SYMMETRICAL ASYMMETRICAL Chapter Chapter 105 Figure 105-1. An approach to peripheral neuropathy in the Proximal/distal Distal Proximal/distal Distal emergency department. AIDP, acute / inflammatory demyelinating Peripheral Nerve Disorders polyneuropathy (Guillain-Barré); CIDP, chronic inflammatory Mixed Mixed Mixed Mixed Pure demyelinating polyneuropathy; DSPN, distal symmetrical polyneuropathy. *A proximal distribution of sensorimotor findings 1. AIDP/CIDP 2. DSPN 3. Plexopathy/ 6. Motor 7. Sensory may dominate the clinical picture in Primary Primary radiculopathy* neuronopathy neuronopathy patterns 3, 4, and 5, depending on myelinopathy axonopathy the location of the lesion(s). 4. Mononeuropathy* 5. Mononeuropathy multiplex* Posterior cord Dorsal Muscle root Neuromuscular junction Dorsal root ganglion Posterior ramus Anterior ramus Ventral Peripheral root Mixed spinal nerve nerve axon Anterior Myelin sheath cord CNS PNS Figure 105-2. Schematic representation of macroscopic and microscopic anatomy of the peripheral nervous system and its interface with the central nervous system. See text for explanation. the neuronopathies, where the cell body of the neuron itself (sensory, motor, or mixed). NCSs and EMG can also identify is the primary site of involvement, ultimately affecting the the level of the neuraxis affected by the disease process (i.e., entire peripheral nerve. Although overlap occurs, each of these root, plexus, or nerve); if the nerve is affected, electrophysio- prototypes has a distinctive clinical presentation, electrophysi- logic testing can help determine whether the lesion is mono- ologic profile, and microscopic appearance. neuropathic (either caused by an isolated mononeuropathy or Electrophysiologic testing, that is, nerve conduction studies mononeuropathy multiplex) or polyneuropathic. Finally, EMG (NCSs) and needle electromyography (EMG), detects under- and NCSs can distinguish axonal from myelinopathic disease, lying pathologic abnormalities. Because neither test is readily further narrowing the differential diagnosis. Prognosis is deter- available in the acute care setting, they are discussed only mined by the nature of pathologic involvement of the PNS. briefly here. Information gathered from NCSs and EMG can Primary demyelination spares the axon and thus carries the be used to obtain objective information on the anatomic dis- best prognosis. The prognosis is worse in axonopathies because tribution of involvement (symmetrical vs. asymmetrical and reestablishing nerve function is dependent on the much slower distal vs. proximal and distal) and the modalities involved process of axonal regeneration. Neuronopathies, which begin 1400 Causes of aCute, emergent Weakness and Patterns and Prototypes of BOX 105-1 Table 105-1 Possible resPiratory ComPromise Peripheral Neuropathies Autoimmune PROTOTYPICAL Demyelinating TYPE PATTERN DISTRIBUTION DISEASE MODALITIES Neurology • Guillain-Barré syndrome 1 Proximal and distal, GBS Chronic inflammatory demyelinating polyneuropathy even symmetrical, sensorimotor S (CIDP) polyneuropathy Myasthenia gravis tion Proximal and distal Symmetrical C e Toxic Motor > sensory S / Botulism 2 Distal, symmetrical, Diabetic DSPN Buckthorn sensorimotor Seafood polyneuropathy Paralytic shellfish toxin Distal Symmetrical Tetrodotoxin (puffer fish, newts) Sensory > motor Tick paralysis 3 Proximal and distal, Brachialplexopathy Metals asymmetrical, sensorimotor Arsenic neuropathy Medicine and Surgery Thallium Proximal and distal Asymmetrical ■ Metabolic Sensory and motor III Dyskalemic syndromes 4 Distal, asymmetrical, CTS (median Acquired (especially with thyrotoxicosis) sensorimotor mononeuropathy) PART mononeuropathy Familial Distal Asymmetrical Hypophosphatemia Sensory and motor Hypermagnesemia 5 Distal, asymmetrical, Vasculitic Porphyria sensorimotor mononeuropathy Infectious mononeuropathy multiplex multiplex Poliomyelitis Distal Asymmetrical Diphtheria Sensory and motor 6 Distal, asymmetrical, pure ALS motor neuronopathy with primary destruction of the nerve cell body, produce pure Distal Asymmetrical motor or pure sensory syndromes. Eventually the entire nerve Motor is affected, resulting in the worst prognosis of the three. 7 Distal, asymmetrical, pure Pyridoxine toxicity sensory neuronopathy Distal Asymmetrical ■ CLINICAL FEATURES Sensory The differential diagnosis for any patient presenting with ALS, amyotrophic lateral sclerosis; CTS, carpal tunnel syndrome; DSPN, distal sensory, motor, or sensorimotor complaints, particularly if symmetrical polyneuropathy; GBS, Guillain-Barré syndrome. localized to the extremities, should include a peripheral neu- ropathy. Within this group, patients with focal weakness are Guillain-Barré Syndrome most concerning because they are at greatest risk for respira- tory compromise. Box 105-1 lists the causes of acute, emergent GBS is a heterogeneous and unpredictable