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Table 196-1

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.