Proximal limb weakness is almost as severe as distal limb weakness, and the upper and lower extremities are equally affected but muscle wasting is rarely pronounced. These signs provide helpful clinical clues to separate these patients from those with axonal neuropathies. Deep tendon reflexes are invariably depressed or absent at some stage of the disease. Sensory symptoms of stocking glove distribution described as numbness, asleep or tingling sensation implicating large fiber involvement occur frequently, but pain is much less common. Additional findings, listed in decreasing order of frequency, are postural tremor of the upper extremities, palpable thickening of peripheral nerve trunks, papilledema and bulbar and facial weakness.
Autonomic dysfunction is rare.
Some patients may present with predominantly multi- focal motor signs masquerading as motor neuron disease. Electrophysiological studies demonstrating multi- focal conduction block confined to motor axons distinguish this acquired, demyelinating, multifocal motor neuropathy from motor neuron disease. The latter may be associated with high serum antibody titers to gangliosides and has been reported to respond to immunosuppression with cyclophosphamide. A CIDP-like syndrome may develop in cases of inherited neuropathy. These patients typically have a positive family history of affected kin, and have bony abnormalities such as pes cavus and hammer toes since an early age, but develop subacute deterioration with proximal muscle weakness and raised CSF protein.