Overview
What is autoimmune paraneoplastic autonomic neuropathy?
What are the diagnostic criteria for paraneoplastic neurological syndromes (PNSs)?
What is the pathophysiology of paraneoplastic neurological syndromes (PNSs)?
What is the prevalence of paraneoplastic autonomic neuropathy in the US?
What is the global prevalence of paraneoplastic autonomic neuropathy?
What is the morbidity and mortality associated with paraneoplastic autonomic neuropathy?
What are the racial predilections of paraneoplastic autonomic neuropathy?
What are the sexual predilections of paraneoplastic autonomic neuropathy?
Which age group has the highest prevalence of paraneoplastic autonomic neuropathy?
Presentation
Which clinical history findings are characteristic of paraneoplastic autonomic neuropathy?
Which physical findings are characteristic of paraneoplastic autonomic neuropathy?
What causes paraneoplastic autonomic neuropathy?
DDX
What are the differential diagnoses for Paraneoplastic Autonomic Neuropathy?
Workup
What is the role of lab testing in the workup of paraneoplastic autonomic neuropathy?
What is the role of imaging studies in the workup of paraneoplastic autonomic neuropathy?
What is the role of EMG/NCV studies in the workup of paraneoplastic autonomic neuropathy?
Which histologic findings are characteristic of paraneoplastic autonomic neuropathy?
Treatment
How is malignancy treated in patients with paraneoplastic autonomic neuropathy?
How are autoimmune processes treated in patients with paraneoplastic autonomic neuropathy?
How is autonomic failure treated in patients with paraneoplastic autonomic neuropathy?
How is paraneoplastic autonomic neuropathy treated?
What is the role of surgery in the treatment of paraneoplastic autonomic neuropathy?
Which specialist consultations are beneficial to patients with paraneoplastic autonomic neuropathy?
Which dietary modifications are used in the treatment of paraneoplastic autonomic neuropathy?
Medications
What is the role of medications in the treatment of paraneoplastic autonomic neuropathy?
Follow-up
What is included in the long-term monitoring of paraneoplastic autonomic neuropathy?
When is inpatient care indicated for the treatment of paraneoplastic autonomic neuropathy?
How is paraneoplastic autonomic neuropathy prevented?
What are the possible complications of paraneoplastic autonomic neuropathy?
What is the prognosis of paraneoplastic autonomic neuropathy?
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Paraneoplastic autonomic neuropathy. Histopathology of peripheral nerve and sympathetic ganglion from a patient with autonomic failure, oat-cell carcinoma of the lung, and positive anti-HU antibody titer. (a) Peripheral nerve in longitudinal section stained with Luxol fast blue-periodic acid-Schiff (PAS) showing scattered wallerian degeneration (arrowheads). (b) Low-power view of a paravertebral sympathetic ganglion stained with hematoxylin and eosin (H&E). Arrowhead indicates perivascular mononuclear infiltrates. (c) High-power view of the same sympathetic ganglion showing degenerating neurons (single arrowheads) and mononuclear infiltrates (double arrowhead). Magnification bars in a and b indicate 100 mm; c is 50 mm.
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Paraneoplastic autonomic neuropathy. Hematoxylin and eosin (H&E)–stained sections from dorsal root ganglion showing the hallmark histopathology of anti-HU disease; a and c are from a healthy patient; b and d are from a patient with autonomic failure, oat-cell carcinoma of the lung, and positive titer of anti-HU antibodies. The arrowheads in b and d indicate degenerating sensory neurons. Also note the interstitial hypercellularity and decreased numbers of neurons in b and d. Magnification bar in b indicates 100 mm and applies also to a. Similarly, the magnification bar in d indicates 50 mm, which also applies to c.
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Paraneoplastic autonomic neuropathy. Central nervous system sections from a patient with autonomic failure, oat-cell carcinoma of the lung, and positive titer of anti-HU antibodies stained with hematoxylin and eosin (H&E). (a) Inferior olive showing a cluster of mononuclear cells (arrowhead); (b) hippocampus showing perivascular mononuclear infiltrate (arrowhead); (c) midbrain section showing a vessel encased in a mononuclear infiltrate; (d) ventral horn of the thoracic spinal cord showing clusters of mononuclear cells around degenerating motor neurons (arrowheads). Magnification bars indicate 100 mm. The bar in b applies also to a and the bar in d also applies to c.