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Table 193-1

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, 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.