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, 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, hereditary leukodystrophies (e.g., adrenomyeloneuropathy) |
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: 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 9: 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; GBS, Guillain-Barré syndrome; HNA, hereditary neuralgic amyotrophy; HNPP, hereditary neuropathy with liability to pressure palsies; HSAN, hereditary sensory and autonomic neuropathy; SMA, spinal muscular atrophy.