Eosinophilic granulomatosis with polyangiitis (neurological manifestations)

Neurological manifestations of eosinophilic granulomatosis with polyangiitis (EGPA) are common, more often in patients who are ANCA positive and most frequently manifesting as peripheral neuropathy, although central nervous system involvement is also encountered ^1,2.

Epidemiology

For a general discussion, please refer to the main article on eosinophilic granulomatosis with polyangiitis.

Involvement of the peripheral nervous system is seen in approximately 50% of patients with EGPA, whereas central nervous system involvement is far less common seen only in approximately 5% of individuals ¹.

Clinical presentation

Clinical presentation of neurological involvement will depend on the location and severity of involvement ^1-4.

• peripheral nervous system (most common overall)

  • mononeuropathy or multiple mononeuropathies

  • polyneuropathy

• ophthalmological

  • optic neuritis

  • central retinal artery occlusion

• other cranial nerves

• central nervous system vascular events

  • cerebral infarction (most common CNS manifestation) ⁴

  • subarachnoid hemorrhage

  • cerebral hemorrhage

  • posterior reversible encephalopathy syndrome

• psychiatric symptoms

Radiographic features

There are no specific features distinguishing the various neurological sequelae of involvement by EGPA from numerous other causes of the various manifestations ^3,4. As such the imaging features will depend on the specific condition and are discussed separately (see above) and CNS vasculitis for futher details.

Treatment and prognosis

For a general discussion, please refer to the main article on eosinophilic granulomatosis with polyangiitis.

Central nervous system involvement by EGPA confirs a somewhat worse prognosis ^1,4.