Patterns of Neuropathic Disorders | |||
Pattern 1: Symmetric proximal and distal weakness with sensory loss Consider: inflammatory demyelinating polyneuropathy (GBS and CIDP) Pattern 2: Symmetric distal sensory loss with or without distal weakness Consider: cryptogenic or idiopathic sensory polyneuropathy (CSPN), diabetes mellitus and other metabolic disorders, drugs, toxins, familial (HSAN), CMT, amyloidosis, and others Pattern 3: Asymmetric distal weakness with sensory loss With involvement of multiple nerves Consider: multifocal CIDP, vasculitis, cryoglobulinemia, amyloidosis, sarcoid, infectious (leprosy, Lyme, hepatitis B, C, or E, HIV, CMV), HNPP, tumor infiltration With involvement of single nerves/regions Consider: may be any of the above but also could be compressive mononeuropathy, plexopathy, or radiculopathy Pattern 4: Asymmetric proximal and distal weakness with sensory loss Consider: polyradiculopathy or plexopathy due to diabetes mellitus, meningeal carcinomatosis or lymphomatosis, sarcoid, amyloid, hereditary plexopathy (HNPP, HNA), idiopathic Pattern 5: Asymmetric distal weakness without sensory loss With upper motor neuron findings Consider: motor neuron disease Without upper motor neuron findings Consider: progressive muscular atrophy, juvenile monomelic amyotrophy (Hirayama's disease), multifocal motor neuropathy, multifocal acquired motor axonopathy Pattern 6: Symmetric sensory loss and distal areflexia with upper motor neuron findings Consider: Vitamin B12, vitamin E, and copper deficiency with combined system degeneration with peripheral neuropathy, chronic liver disease, hereditary leukodystrophies (e.g., adrenomyeloneuropathy) HSP-plus Pattern 7: Symmetric weakness without sensory loss With proximal and distal weakness Consider: SMA With distal weakness Consider: hereditary motor neuropathy (distal SMA) or atypical CMT Pattern 8: Focal midline proximal symmetric weakness Neck extensor weakness Consider: ALS Bulbar weakness Consider: ALS/PLS, isolated bulbar ALS (IBALS), Kennedy's syndrome (X-linked, bulbospinal SMA), bulbar presentation GBS Diaphragm weakness (SOB) Consider: ALS Pattern 9: Asymmetric proprioceptive sensory loss without weakness Consider causes of a sensory neuronopathy (ganglionopathy): Cancer (paraneoplastic) Sjögren's syndrome Idiopathic sensory neuronopathy (possible GBS variant) Cisplatin and other chemotherapeutic agents Vitamin B6 toxicity HIV-related sensory neuronopathy Pattern 10: Autonomic symptoms and signs Consider neuropathies associated with prominent autonomic dysfunction: Hereditary sensory and autonomic neuropathy Amyloidosis (familial and acquired) Diabetes mellitus Idiopathic pandysautonomia (may be a variant of Guillain-Barré syndrome) Porphyria HIV-related autonomic neuropathy Vincristine and other chemotherapeutic agents |
Abbreviations: CIDP, chronic inflammatory demyelinating polyneuropathy; CMT, Charcot-Marie-Tooth disease; CMV, cytomegalovirus; GBS, Guillain-Barré syndrome; HIV, human immunodeficiency virus; HNA, hereditary neuralgic amyotrophy; HNPP, hereditary neuropathy with liability to pressure palsies; HSAN, hereditary sensory and autonomic neuropathy; HSP-plus, hereditary spastic paraplegia plus neuropathy; SMA, spinal muscular atrophy; SOB, shortness of breath.