Neurology Case Challenge: A Man With Buttocks Pain, Bladder and Bowel Incontinence

Xuan Kang, MD


July 21, 2022

Vasculitic neuropathy is a multifocal painful neuropathy that results from inflammatory cells infiltrating the vasa nervorum and causing peripheral nerve ischemia. Patients frequently develop weakness and sensory loss that follows the distribution of individual peripheral nerves, with subsequent involvement of other nerves. The disorder can be isolated to the peripheral nervous system (nonsystemic vasculitic neuropathy), or it can be associated with systemic vasculitis. A laboratory workup is necessary to detect systemic vasculitis or viral infections, and electrodiagnostic testing demonstrates multifocal and asymmetric severe sensorimotor axonal changes. Sensory nerve and neighboring muscle biopsies are helpful to establish the diagnosis. Treatment begins with a combination of a high-dose corticosteroid and cyclophosphamide,[28,29] followed by methotrexate, azathioprine, or rituximab as maintenance therapy.[30]

Sensory neuronopathies are generalized neuropathies with diffuse dorsal root ganglia damage. Patients present with early ataxia, multifocal sensory loss, intact strength, and absent deep tendon reflexes. The etiology is diverse and includes paraneoplastic causes (small cell lung cancer is the most common malignancy), Sjögren syndrome, autoimmune hepatitis, lupus, and association with anti-fibroblast growth factor receptor 3 antibodies.[31,32] Electrodiagnostic testing demonstrates non–length-dependent sensory neuropathy, especially when upper-extremity sensory responses are disproportionately affected.[33] Unfortunately, this group of disorders has a poor response to immunotherapy.


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