POLYNEUROPATHY + NEURONOPATHY: Points in Differential Diagnosis
Selective Functional Involvement
Motor Motor Neuropathy: Multifocal (MMN); Distal Motor Neuron Disorders   Lower Motor Neuron (LMN): BVvL; Fazio-Londe; Madras   Spinal Muscular Atrophies (SMA): 5q; BSMA; Infantile; HMN   ALS: Sporadic; Hereditary Toxic: Pb; Dapsone; Botulism; Tick Acute: Porphyria; Axonal GBS (AMAN); Polio; West Nile Diabetic amyotrophy	Autonomic Diabetes Mellitus; Amyloid; Paraneoplastic Immune: Acute; GBS; Vasculitis Multisystem: Shy-Drager; IOH; Mitochondrial Hereditary: Riley-Day; Sensory PN; HSAN II Infections: Chagas; Leprosy; HIV Toxic: Vacor; Perhexiline; Vincristine   Hyperhidrosis: Arsenic; Hg; Acrylamide Localized: RSD; Horner's	Treatable neuropathies Diabetes; Vasculitis; CIDP; Lymphoma; Infection; Toxic; Compression Deficiency: B12; E; Cu Antibody: GM1; TS-HDS; MAG;   Neurofascin; Contactin-1; GALOP   ? FGFR3; Cryoglobulin; M-protein
Sensory: Large fiber & Ataxic Tabes dorsalis; Vitamin deficiency: B12; E Toxic: Vitamin B6; cis-platinum; Taxol; MeHg Hereditary: an-α-lipoproteinemia; Friedreich's; CANVAS   Ataxia telangectasia; Biemond; OPCA; HSAN Immune: MAG; GALOP; GD1b; Miller-Fisher Neuronopathies: Hu; Sjögren's Rule out: Myelopathy; HTLV1; HIV	Sensory: Small fiber Leprosy; Amyloid; Idiopathic Toxic: Kepone; Ciguatera Metabolic: Diabetes; Increased Triglycerides Hereditary   HSN I & IV; Tangier's, Fabry's;   Indifference; Thermoanalgesia; Navajo   HSN + Paraparesis (Cavanagh)	Neuropathy + Pain Diabetic amyotrophy; Vasculitis; Idiopathic; Polio; HIV; Ca meningitis; Toxic: EtOH; Arsenic; Ifosfamide   cis-Platinum; Thallium; Thalidomide Hereditary: Fabry's; HSN I; Erythermalgia Focal: Median & Post tibial n.   Other: Roots; Plexus

Selective Anatomical Distribution: Most are symmetric and maximal distally in the lower extremities
Extraocular muscle Botulism Diabetes Miller-Fisher Diphtheria Rule out:   MG; Myopathy	Proximal Motor Immune Demyelinating:   GBS; CIDP SMA; Porphyria Plexopathy: Brachial; Lumbar Rule out: Joint pain; Myopathy	Proximal Sensory Hereditary: Porphyria; Tangier Neuronopathy: Hu; Sjögren's Thoracic neuropathy Rule out: Myelopathy ___________________________ Skin temperature-related Leprosy	Upper extremity Immune: MMN; Vasculitis   CIDP variant Amyloid: Carpal tunnel Entrapment: HNPP; Other Toxic: Lead; Vincristine ALS; LMN Rule out: Spinal; CNS	Asymmetric Mononeuritis multiplex Neuronopathy: ALS; Sensory Entrapments Plexopathies Toxic Mononeuritis Multiplex Vasculopathy; Amyloid; Leprosy; Diabetes; CMV Waldenström; Perineuritis Demyelinating: HNPP;   Multifocal CIDP; MMN Compression: Multiple Lymphoma: Intraneural Wartenberg
CNS Spinal: Organophosphate; Hexacarbon; AMN; MLD; Lymphoma; Cuban; Vernant's Optic: Disulfiram; CS2; Hg; Drugs; NARP; CMT6; Post col & RP; Cuban; Vernant's Hearing loss: HMSN X, 1A, 1B, 4D, 6; Mitichondrial; Sarcoid Cerebellum: FA; AT; MLD; Refsum; A-β-lipoproteinemia; SCA 2, 3, 4; IOSCA; CANVAS; Hu & CV-2 Supratentorial: Mitochondrial; Thyroid; Hu; B12; Vasculitis; Neoplastic; Sarcoid   Infection: Lyme; HIV; Rabies; Syphilis; West Nile   Hereditary: Polyglucosan; Fabry; HexA; Porphyria; Prion; ALS; Cowchock; NAD; Krabbe; MLD			Face Bell's Palsy Melkersson; Tangier Polyradiculopathies:   Sarcoid; Lyme; GBS Motor neuron disorders:   ALS; Kennedy's; Möbius Rule out: MG; Myopathy

Time Course

Acute Immune: GBS; Vasculitis Toxic: Botulism; Thallium; Vacor;   Drugs; Org-phos Infections: Tick; Lyme; HIV; West Nile   Leptospirosis; Rabies; CMV; Zika Porphyria; Paraneoplastic (Anti-Hu) Rule out: Myopathy; MG; Spinal Relapsing CIDP ± GBS Hereditary: Porphyria; HNPP; HNA;   Thermosensitive; Refsum Childhood Onset Immune: CIDP; GBS Hereditary: NAD; HMSN I & III; MLD   Giant Axonal; Lethal neonatal;   Tangier; CCFDN; Congen Hypomyelin;Krabbe

Hereditary CMT Demyel Dominant: IA PMP-22; IB & IE P0; IC LITAF; ID EGR2; IF NFL   HNPP PMP-22, KARS; YARS; Thermosensitive; SOX10; DNM2; 10q24   III (PMP-22; P0; EGR2); CMT + Intermed NCV: 10q24; DNM2; YARS; INF2; GNB4; P0; GJB1 CMT Demyel Rec: III; 4A GDA P1; 4B MTMR2; 4B2 SBF2; 4B3 SBF1; 4C SH3TC2; 4D NDRG1;   4E EGR2; 4F Prx; 4G HK1; 4H FGD4; 4J FIG4; CCFDN CTDP1 CMT X-linked: Connexin-32; Xp22.2; Xq26; Cowchock AIFM1; PRPS1; PDK3; AUNX1; Xq23 CMT Axon, AD: 2A MFN2; 2B RAB7; 2C TRPV4; 2D GARS; 2E NFL; 2F HSPB1; 2G 12q12; 2I P0; 2K GDAP1; 2L: HSPB8;   2M DNM2; 2N AARS; 2O DYNC1H1; 2P LRSAM1; 2Q DHTKD1; HMSN-P TFG; HNA1 SEPT9; VCP; 2T DNAJB2; 2U MARS   2V NAGLU; 2W HARS; 2Y MORC2; 2Z ; MORC2; 2CC NEFH; DGAT2; TFG; MME CMT Axon AR: 2A LAM A/C; 2B MED25; F HSPB1; 2K GDAP1; P LRSAM1; R TRIM2; S IGHMBP2; T MME; X SPG11;     P0; HINT1; HSJ1; MFN2; PNKP; MCM3AP   CMT + CNS: Andermann KCC3; Deafness; Optic Δ; NAD; GAN Gigaxonin;       AR-CMT2 + Pyramidal 8q21; Lethal Neonatal; TDP1; CANVAS (RBC1) Myelin, Recessive: Cockayne; Refsum (PAO & Peroxin-1); Krabbe; MLD; CMT 4E; P0; EGR-2 ALS: AD SOD; SETX; VAPB; ANG; NFH; TDP-43; FIG4; VCP; Ataxin-2; GRN; HNRNPA1; BSCL2; Matr3; TUBA4A; DAO;     GLE1; SS18L1; ANXA11; ATXN2; BSCL2; FUS; c9orf72; PFN1; UBQLN2; SQSTM1; ErbB4; CHMP2B; CHCHD10; 20p13   AR NF-H; Alsin; PRPH; FUS; Spatacsin; OPTN; SIGMAR1; PNPLA6; c19orf12; PNPLA6; OPTN SMA: AR Xq12; SMN; SCO2 & TK2 (Mito Δ); ATP7A; P 3q13; VAPB; DYNC1H1; TRPV4 HMN: HSPB 1,3 & 8; GARS; BSCL2; ATP7A; DCTN1; SETX; PLEKHG5; FBLN5; IGHMBP2 Sensory, Dominant: HSAN1 SPTLC1 & 2; ATL1 & 3; DNMT1; GJB3; ARHGEF10; RNF170; PRNP; SCN9A & 11A Sensory, Recessive: HSAN2 HSN2; HSAN3 (Riley-Day) IKBKAP; Tangier; SCN9A; Dystonin; FLVCR1; MPV17; PRDM12   HSAN4 NTRK1; HSAN5; NGF-β; ATM; FA FRDA; Fabry; DHH; NARP; HSN2; FAM134B; ATSV; CCT5; CLTCL1

Other Features
Paraneoplastic Myopathy: RIIM; DM-VP; Type II atrophy;   Decorin Antibody; Rippling muscle; Metabolic; Scleromyxedema NMJ: MG (Thymoma); LEMS (Small cell Ca) Nerve: Hu; Motor neuronopathy; Isaac's; M-protein   Neuromyopathy (wt loss > 15%); Autonomic (Hu) Spinal: Necrotizing; Stiffman (Breast +) Cerebellar: Yo (Gyn); Tr (Lymphoma); Hu; LEMS; Ma   Opsoclonus/Myoclonus - Child (Neuroblastoma), Adult, Ri Encephalopathy: Hu; Thymoma; Ma2 (Ta)		GI disorders Neuropathy   Hereditary: Hirschsprung; MEN; Pseudoobstruction   Infection: Polio; Chagas   MNGIE; Diabetes; Amyloid; Hu; POEMS; Porphyria   Vitamin ↓: B12; E Myopathy: Inflammatory; ↑ Thyroid; Visceral   Dystrophy: Myotonic; Duchenne; Oculopharyngeal;     Oculopharyngodistal Spinal: Syringomyelia	Large nerves Leprosy; CIDP; Amyloid; Acromegaly; Entrapment Hereditary: Refsum;   HMSN I & III; Noonan Neoplasm: Focal;   Diffuse: Lymphoma Local: Entrapment; Tumors;   Mononeuropathy
Demyelination Immune: GBS; CIDP; MMN; MAG;   Sulfatide; GALOP; POEMS Toxic: Amiodarone; Perhexiline;   Diphtheria; Na cyanate; Buckthorn Hereditary:   HMSN I, III, IV; HNPP;   Krabbé; MLD; Refsum Mitochondrial: MNGIE; NARP	Spontaneous Activity Complex repetitive Dystonia Fasciculations   ALS; MMN; Normal Hyperekplexia (Startle) Myokymia   Isaac's; Radiation Neuromyotonia Myotonia Stiffman Syndrome Tetanus	Antibodies: For M-protein use immunofixation IgM vs GM1, NS6S or GalNAc-GD1a: Motor neuropathy IgG vs GM1, GM1b, GalNAc-GD1a: AMAN IgM vs GM2: Sensory > Motor; Demyelinating IgM vs GD1b: Sensory; Axonal; CANOMAD IgM vs TS-HDS, IgG vs FGFR3 or PD1: Sensory; Pain; Axonal IgG vs GQ1b: Miller-Fisher; Ophthalmoplegia IgM vs MAG: Sensory > Motor; Ataxia; Tremor IgM vs Sulfatide or GALOP: Sensory; Gait Δ IgG vs Hu: Sensory neuron; IgG vs Yo, Tr, Hu: Cerebellar IgM vs Tubulin or IgG vs NFASC, CNTN1: Demyelin PN IgG vs CRMP5: Polyneuropathy + Cerebellar	Nerve biopsy: ↑ Utility Asymmetric PN Age > 65 + Disabled Sensory loss Abnormal NCV Diagnoses   Vasculitis; ± CIDP   Systemic: Leprosy;       Amyloid; Sarcoid   Hereditary: MLD     Giant axonal;     Fabry; Krabbé

Alan Pestronk 7/2/2019 More information at Neuromuscular Website: http://neuromuscular.wustl.edu