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Table 46-2 The principal neuropathic syndromes and their causes I Syndrome of acute motor paralysis with variable disturbance of sensory and autonomic function A Guillain-Barre syndrome (GBS; acute in ammatory polyneuropathy; acute autoimmune neuropathy); see also Table 46-3 B Acute axonal form of GBS C Acute sensory neuropathy and neuronopathy syndrome D Diphtheritic polyneuropathy E Porphyric polyneuropathy F Certain toxic polyneuropathies (thallium, triorthocresyl phosphate) G Rarely, paraneoplastic H Acute pandysautonomic neuropathy I Tick paralysis J Critical illness polyneuropathy II Syndrome of subacute sensorimotor paralysis A Symmetrical polyneuropathies 1 De ciency states: alcoholism (beriberi), pellagra, vitamin B12 de ciency, chronic gastrointestinal disease (see Chap 41) 2 Poisoning with heavy metals and solvents: arsenic, lead, mercury, thallium, methyl n-butyl ketone, n-hexane, methyl bromide, ethylene oxide, organophosphates (TOCP etc), acrylamide (see Chap 43) 3 Drug toxicity: isoniazid, ethionamide, hydralazine, nitrofurantoin and related nitrofurazones, disul ram, carbon disul de, vincristine, cisplatin, paclitaxel, chloramphenicol, phenytoin, pyridoxine, amitriptyline, dapsone, stilbamidine, trichlorethylene, thalidomide, clioquinol, amiodirione, adulterated agents such as L-tryptophan 4 Uremic polyneuropathy (see Chap 40) 5 Subacute in ammatory polyneuropathy B Asymmetrical neuropathies (mononeuropathy multiplex) 1 Diabetes 2 Polyarteritis nodosa and other in ammatory angiopathic neuropathies (Churg-Strauss, hypereosinophilic, rheumatoid, lupus, Wegener granulomatosis, isolated peripheral nervous system vasculitis); see also Table 46-3 3 Mixed cryoglobulinemia 4 Sjogren-sicca syndrome 5 Sarcoidosis 6 Ischemic neuropathy with peripheral vascular disease 7 Lyme disease C Unusual sensory neuropathies 1 Wartenberg migrant sensory neuropathy 2 Sensory perineuritis D Meningeal based nerve root disease (polyradiculopathy) 1 Neoplastic in ltration 2 Granulomatous and infectious in ltration: Lyme, sarcoid 3 Spinal diseases: osteoarthritic spondylitis 4 Idiopathic polyradiculopathy III Syndrome of chronic sensorimotor polyneuropathy A Less chronic acquired forms 1 Paraneoplastic: carcinoma, lymphoma, myeloma, and other malignancies 2 Chronic in ammatory demyelinating polyneuropathy (CIDP) 3 Paraproteinemias 4 Uremia (occasionally subacute) 5 Beriberi (usually subacute) 6 Diabetes 7 Connective tissue diseases 8 Amyloidosis 9 Leprosy 10 Hypothyroidism 11 Benign sensory form in the elderly B Syndrome of more chronic polyneuropathy, genetically determined forms (see Table 46-6) 1 Inherited polyneuropathies of predominantly sensory type a Dominant mutilating sensory neuropathy in adults b Recessive mutilating sensory neuropathy of childhood c Congenital insensitivity to pain d Other inherited sensory neuropathies, including those associated with spinocerebellar degenerations, Riley-Day syndrome, and the universal anesthesia syndrome.

Table 46-2 (Continued) The principal neuropathic syndromes and their causes C Inherited polyneuropathies of mixed sensorimotor types 1 Idiopathic group a Peroneal muscular atrophy [Charcot-Marie-Tooth; CMT types I (demyelinating) and II (axonal) and CMTX] b Hypertrophic polyneuropathy of Dejerine-Sottas, adult and childhood forms (CMT3) c Roussy-Levy polyneuropathy (CMT3) d Polyneuropathy with optic atrophy, spastic paraplegia, spinocerebellar degeneration, mental retardation, and dementia e Hereditary liability to pressure palsy (HNPP) 2 Inherited polyneuropathies with a recognized metabolic disorder (see also Chap 37) a Refsum disease b Metachromatic leukodystrophy c Globoid-body leukodystrophy (Krabbe disease) d Adrenoleukodystrophy e Amyloid polyneuropathy f Porphyric polyneuropathy g Anderson-Fabry disease h Abetalipoproteinemia and Tangier disease IV Neuropathy associated with mitochondrial diseases (see Chap 37) V Syndrome of recurrent or relapsing polyneuropathy A Guillain-Barre syndrome B Porphyria C Chronic in ammatory demyelinating polyneuropathy D Certain forms of mononeuritis multiplex E Beriberi or intoxications F Refsum disease, Tangier disease VI Syndrome of mononeuropathy or plexopathy A Brachial plexus neuropathies B Brachial mononeuropathies C Causalgia D Lumbosacral plexopathies E Crural mononeuropathies F Migrant sensory neuropathy G Entrapment neuropathies

immunization has at one time or another been reported to precede GBS (some perhaps coincidentally) In recent years, it has come to be appreciated from serologic studies that the enteric organism Campylobacter jejuni is the most frequent identi able antecedent infection but it accounts for only a relatively small proportion of cases Other antecedent events or associated illnesses include viral exanthems and other viral illnesses [cytomegalovirus (CMV), Epstein-Barr virus (EBV), HIV], bacterial infections other than Campylobacter (Mycoplasma pneumoniae, Lyme disease), exposure to thrombolytic agents, and lymphoma (particularly Hodgkin disease) The administration of outmoded antirabies vaccines and the A/New Jersey (swine) in uenza vaccine, given in late 1976, was associated with a slight increase in the incidence of GBS and at least one subsequent in uenza vaccination program has been associated with a marginal increase in cases Trauma and surgical operations may precede the neuropathy, but a causal association to them remains uncertain Historical Background The earliest description of an afebrile generalized paralysis is probably that of Wardrop and Ollivier, in 1834 Other important landmarks are Landry s report (1859) of an acute, ascending, predominantly motor paralysis with respiratory failure, leading to death; Osler s (1892) description of febrile polyneuritis ; the account by Guillain, Barre, and Strohl (1916) of