Vasculitic neuropathy systemic

Genetic testing

NCV/EMG

Laboratory

Imaging

Biopsy

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Fig. 4. Sural nerve biopsy from a patient with isolated peripheral nerve vasculitis. A Infiltration of a perineurial vessel wall by multiple inflammatory cells including lymphocytes and macrophages (black arrows). There is also evidence of pink fibrin deposits consistent with the presence of fibrinoid necrosis. B Teased fiber preparations showing multiple axon balls (white arrows) and evidence of empty strands consistent with axonal degeneration

Fig. 4. Sural nerve biopsy from a patient with isolated peripheral nerve vasculitis. A Infiltration of a perineurial vessel wall by multiple inflammatory cells including lymphocytes and macrophages (black arrows). There is also evidence of pink fibrin deposits consistent with the presence of fibrinoid necrosis. B Teased fiber preparations showing multiple axon balls (white arrows) and evidence of empty strands consistent with axonal degeneration

Fig. 5. Dorsal root ganglion biopsy from a patient with severe sensory ataxia due to dorsal root ganglionitis. There are clusters of inflammatory cells (white arrows) surrounding the dorsal root ganglion neurons (black arrows). Many of the neurons show evidence of degeneration

Vasculitis Neuropathy

Fig. 6. Hand in a patient with vasculitis. Atrophy of the small hand muscles and vasculitic changes at the nailbed

Fig. 7. Wegener's granulomatosis. This patient had right orbital involvement A. Vasculitic neuropathy was heralded by vasculitic skin changes B

Nerve and muscle pathology relates to destruction of blood vessels. Anatomy/distribution

Proximal and distal weakness, pain, and sensory loss occur in a multifocal Symptoms distribution.

May affect isolated nerves (45% of cases), overlapping nerves (40%), or cause Clinical syndrome/ symmetric neuropathy (15%). Patients typically present with a mixture of motor signs and sensory signs. Associated signs of systemic vasculitic disease include: fever, weight loss, anorexia, rash, arthralgia, GI, lung, or renal disease. Usually the neuropathy presents in patients that have already been diagnosed with a specific vasculitic disease (Fig. 6).

Pathogenesis Several immune-mediated mechanisms have been identified that lead to destruction of vessel walls. The various mechanisms result in ischemic necrosis of axons (see Figs. 4 and 5).

Systemic disease that can involve vasculitic neuropathy can be divided into the following categories:

Peripheral Neuropathy Natural Treatment Options

Peripheral Neuropathy Natural Treatment Options

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