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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.