Peripheral Neuropathies in Clinical Practice

Steven HerskovitzThe Saul R. Korey Department of NeurologyAlbert Einstein College of Medicine of Yeshiva UniversityMontefiore Medical CenterBronx, NY 10467Stephen ScelsaDirector, Neuromuscular Division and ALS CenterThe Hyman-Newman Institute for Neurology & NeurosurgeryNew York NY 10003Herbert SchaumburgThe Saul R. Korey Department of NeurologyAlbert Einstein College of MedicineJack and Pearl Resnick CampusForchheimer Building, Room 104 SBronx, NY 10461The authors are recognized neuromuscular experts, having published extensively on various clinical and basic science aspects of neuropathies, electrodiagnostic studies, neurotoxicology and ALS.

• 1. Basic Concepts and Glossary of Common Clinical TermsDefinition of the Peripheral Nervous System (PNS)Relationships Fundamental to an Understanding of Disease of Peripheral Nerve FibersNeuron Cell Body and AxonAxon, Schwann Cell and MyelinAxon and End OrganWallerian Degeneration and Axon RegenerationGlossary of Common Clinical Terms2. Anatomic Classification of Peripheral Nervous System DisordersSymmetric Generalized Neuropathies (Polyneuropathies)Distal Axonal DegenerationSegmental (Non-Uniform) MyelinopathyDiffuse (Uniform) MyelinopathyNeuronopathy (Ganglionopathy)Focal (Mononeuropathy) and Multifocal (Multiple Mononeuropathy) NeuropathiesIschemiaInfiltrationPhysical Injuries3. Evaluation and Management of the Patient with Peripheral NeuropathyGeneral Principles and the Algorithmic ApproachChronic Idiopathic Axonal Polyneuropathy / Small Fiber Neuropathy Treatment of Neuropathic PainDifferential Diagnoses and Work-ups for the Varied Neuropathy Phenotypes (Tables)Sensorimotor Polyneuropathies - AxonalSensorimotor Polyneuropathies - Demeyelinating or MixedSensory, Small Fiber, Painful PolyneuropathiesSensory, Large Fiber, Ataxic PolyneuropathiesMotor NeuropathiesAutonomic NeuropathiesMononeuropathy MultiplexMyeloneuropathiesNeuromyopathiesPolyneuropathy and Optic NeuropathyPolyradiculopathiesPlexopathies/RadiculoplexopathiesFacial NeuropathyTrigeminal Sensory NeuropathyUnusual Neuropathy Patterns4. Electrodiagnostic, Imaging, Nerve and Skin Biopsy Investigations in Peripheral Nerve DiseaseElectromyography and Nerve Conduction StudiesSensory Nerve Conduction StudiesMotor Nerve Conduction StudiesLate ResponsesBlink Reflex StudiesNeedle ElectromyographyStudies of Autonomic FunctionQuantitiative Sudomotor Axon Reflex TestThermoregulatory Sweat TestSympathetic Skin ResponseHeart Rate Response to Deep BreathingValsalva ManeuverQuantitative Sensory TestingDeveloping Electrophysiologic and Imaging TechniquesMotor Unit Number EstimationElectrical Impedence Myography High-Resolution Sonography of Peripheral NerveMagnetic Resonance Neurography Muscle MRIPositron Emission Tomography Nerve BiopsySkin Biopsy5. Case Presentations Illustrating the Diagnostic MethodCase 1: Painful small fiber neuropathy and dysautonomiaCase 2: Insidious onset of distal weakness in an adult with deformed feetCase 3: Lower limb paresthesias in a middle-aged adult with diabetes.Case 4: A middle-aged woman with muscle twitching and episodic numbnessCase 5: Six days of cranial neuropathies and hyporeflexiaCase 6: A two-month onset of sensory neuropathy in a woman with ovarian carcinomaCase 7: A 47-year-old man with 10 years of progressive bilateral hand weaknessCase 8: Chronic sensory loss and unsteady gait in a 59-year-old womanCase 9: An elderly man with acral paresthesias and gait unsteadinessCase 10: Foot drop in an 81-year-old womanCase 11: A middle-aged man with multifocal pain, sensory loss and weakness Case 12: Five-day onset of diffuse weakness 6. Acute Immune-mediated NeuropathiesDemyelinating Immune-mediated NeuropathiesAcute Inflammatory Demyelinating Polyradiculoneuropathy (AIDP) and Fisher Syndrome (FS)Axonal Immune-mediated NeuropathiesAcute Motor Axonal Neuropathy (AMAN) and Acute Motor and Sensory Axonal Neuropathy (AMSAN)NeuronopathiesSensory (Idiopathic, Sjogren Syndrome, Paraneoplastic) and Motor (Paraneoplastic) Neuronopathies and Autoimmune Autonomic Ganglionopathy 7. Chronic Immune-mediated NeuropathiesChronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP)CIDP Variants:Multifocal Motor Neuropathy with Conduction Block (MMN)Lewis-Sumner Syndrome (LSS)/Multifocal Acquired Demyelinating Sensory and Motor Neuropathy (MADSAM)Distal Acquired Demyelinating Symmetric Neuropathy (DADS)CIDP with CNS FeaturesChronic Immune Sensory Polyradiculopathy (CISP)Chronic Sensory Demyelinating Polyneuropathy8. Neuropathies Associated with Monoclonal Gammopathies and CancerMultiple Myeloma Osteosclerotic MyelomaPrimary Systemic Amyloidosis (AL Amyloidosis) Waldenstrom MacroglobulinemiaMonoclonal Gammopathy of Undetermined Significance9. Infectious and Granulomatous NeuropathiesHerpes Zoster/Herpes SimplexLeprosySarcoidosisLyme DiseaseHIV Hepatitis C/CryoglobulinemiaDiphtheria10. Diabetic and Other Endocrine NeuropathiesDiabetic NeuropathiesDistal Symmetric Sensorimotor/Autonomic Polyneuropathy Proximal Multifocal Neuropathies Lumbosacral Radiculoplexus NeuropathyThoracolumbar "Truncal" RadiculoneuropathyFocal Limb Neuropathies (Entrapment Sites)Isolated Cranial Neuropathies Acute Painful Neuropathy ("Diabetic Neuropathic Cachexia")Diabetic Motor-predominant NeuropathiesTreatment-induced Neuropathy ("Insulin Neuritis")Hyperglycemic NeuropathyAcromegalic NeuropathyHypothyroid Neuropathy11. Neuropathies Associated with Vitamin Deficiencies and MalabsorptionVitamin B12 (Cobalamin) DeficiencyVitamin B1 (Thiamine) DeficiencyCeliac DiseaseVitamin E (?-tocopherol) DeficiencyCopper DeficiencyOther: Cuban Epidemic Optic and Peripheral Neuropathy; Deficiencies: Riboflavin (B2), Pyridoxine (B6), Folate, Zinc; Bariatric Surgery12. Vascular/Ischemic NeuropathiesVasculitic NeuropathiesNeuropathies Associated with Peripheral Arterial Occlusive DiseaseNeuropathies Associated with Compartment Syndromes13. Neuropathies Associated with Organ FailurePulmonary FailureHepatic FailureRenal FailureUremic PolyneuropathyMononeuropathiesIschemic Monomelic NeuropathyOrgan TransplantationCritical Illness PolyneuropathyDifferential Diagnosis14. The Hereditary NeuropathiesHereditary Motor and Sensory Neuropathies Charcot-Marie Tooth Disease, Type 1 Hereditary Neuropathy with Liability to Pressure PalsyCharcot-Marie Tooth Disease, Type 2 Dejerine-Sottas Disease/Congenital Hypomyelinating Neuropathy Charcot-Marie Tooth Disease, Type 4 Charcot-Marie Tooth, Type X Charcot-Marie-Tooth Disease-Dominant IntermediateHereditary Sensory and Autonomic Neuropathies Distal hereditary Motor Neuropathy/Neuronopathy Hereditary Ataxia with NeuropathyAutosomal DominantAutosomal RecessiveX-linkedHereditary Spastic Paraplegia with Neuropathy Hereditary Brachial Plexus Neuropathy/Hereditary Neuralgic Amyotrophy Hereditary Peripheral Nerve Channelopathies Sodium ChannelopathiesPotassium Channelopathies15. Hereditary Metabolic/Multisystem Disorders with NeuropathyFamilial Amyloid PolyneuropathiesDisorders of Lipid MetabolismLysosomal DisordersFabry Disease LeukodystrophiesMetachromatic LeukodystrophyKrabbe Disease Peroxisomal DisordersRefsum DiseaseAdrenomyeloneuropathyLipoprotein DeficienciesTangier DiseaseAbetalipoproteinemiaFamilial HypobetalipoproteinemiaCerebrotendinous XanthomatosisDisorders of Heme Metabolism - PorphyriaDisorders of Defective DNA RepairMitochondrial DisordersNeuroacanthocytosis SyndromesChorea-AcanthocytosisMcLeod SyndromeNeurofibromatous NeuropathyGlycogen Storage DiseasesAdult Polyglucosan Body Disease16. The Toxic Neuropathies: Principles of General and Peripheral Neurotoxicology; Pharmaceutical AgentsPrinciples of General NeurotoxicologyPrinciples of Peripheral NeurotoxicologyPeripheral Neurotoxicity Associated with Pharmaceutical AgentsAmiodaroneBortezomibColchicineDapsoneDisulfiramEthambutolEthanolIsoniazidMetronidazoleMisonidazoleNitrous OxideNucleoside AnaloguesPhenytoinPlatinumPyridoxineSuraminTacrolimusTaxanesThalidomideVinca Alkaloids17. The Toxic Neuropathies: Industrial, Occupational and Environmental AgentsPeripheral Neurotoxicity Associated with Industrial, Occupational and Environmental AgentsArsenicEthylene OxideHexacarbons (n-hexane)LeadMethyl BromideOrganophosphatesThallium18. Focal neuropathies: Nerve injuries, Entrapments and Other MononeuropathiesNerve InjuriesAnatomy and Pathophysiology of Nerve InjuriesClinical Classification or Nerve InjuriesElectrodiagnostic Features of Nerve InjuriesNerve Regeneration and RepairFocal Neuropathies: The Upper ExtremityMedian NerveUlnar NerveRadial NerveOther Upper Extremity MononeuropathiesFocal Neuropathies: The Lower ExtremitySciatic nervePeroneal NerveTibial NerveFemoral NerveLateral Femoral Cutaneous NerveOther Lower Extremity MononeuropathiesFocal Neuropathies: Cranial NeuropathiesIdiopathic Facial Nerve Paralysis (Bell's palsy)19. PlexopathiesBrachial PlexopathyAnatomyEtiologyTraumaThoracic Outlet SyndromeNeoplastic Brachial PlexopathyRadiation-induced Brachial PlexopathyImmune Brachial Plexus Neuropathy (Neuralgic Amyotrophy)Lumbosacral PlexopathyAnatomyEtiologyTrauma and IschemiaRetroperitoneal HemorrhageNeoplastic Lumbosacral PlexopathyRadiation-induced Lumbosacral PlexopathyNon-diabetic Lumbosacral Radiculoplexus Neuropathy20. Disorders of Peripheral Nerve HyperexcitabilityGeneralizedNeuromyotonia CrampsFasciculationsTetanyLocalizedFacial MyokymiaLocalized or Focal MyokymiaHemifacial SpasmHemimasticatory SpasmHypothenar DimplingIndex

"...a much welcomed book...The authors successfully present a practical approach to peripheral neuropathies...Numerous tables and figures help integrate the knowledge and form a quick reference when evaluating a patient with peripheral neuropathy. The book is affordable and small, making it easy to carry during the neuromuscular rotation or in the clinic...I enjoyed reading Peripheral Neuropathies in Clinical Practice and would recommend it to bothtrainees and practicing neurologists." --Chafic Karam, MD"Peripheral Neuropathies in Clinical Practice is well written, up-to-date, and well illustrated, providing a logical and comprehensive approach to peripheral nerve diseases. Any clinician would be able to use it to find specific facts as well as understand how to approach a category of disorders. Therefore, this book would prove useful to any practitioner or practitioner-in-training who would want an easily accessible and comprehensive textbook onperipheral neuropathy. I strongly recommend this textbook as a reference work as well as a clinical manual, because it contains all of the information necessary for the physician treating patients who haveperipheral nerve disease." --Daniel L. Menkes, MD, JAMA