p-ANCA pachymeningitis presenting with isolated "optic neuropathy".
Isolated bilateral optic neuropathy is an exceedingly rare presentation of perinuclear-antineutrophil cytoplasmic autoantibody (p-ANCA) vasculitis. We report one such case with MRI documentation of a compressive mechanism mediated by pachymeningitis. A 69-year-old woman had a 6-month history of progressive visual failure caused by bilateral optic neuropathy. Cranial MRI showed diffuse contrast enhancement of the dura mater extending to the perioptic sheaths bilaterally with encasement of the optic nerves. Extensive laboratory study showed increased levels of p-ANCA titres and autoimmune markers. Corticosteroid treatment improved both visual acuity and the neuroradiological signs of active pachymeningitis. In conclusion, our case demonstrates that bilateral optic neuropathy may be the presenting symptom of p-ANCA-associated pachymeningitis, whose causative role may be demonstrated by MRI.
Salvi
Fabrizio
F
Centro per le Malattie Rare e Neuroimmuni, Unità Operativa di Neurologia, Dipartimento di Neuroscienze, Ospedale Bellaria, Bologna, Italy.
Mascalchi
Mario
M
Pasini
Elena
E
Bartolomei
Ilaria
I
Fini
Nicola
N
Marliani
Federica
F
Malatesta
Renato
R
Michelucci
Roberto
R
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000368:Aged; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000378:metabolism*; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008581:Meningitis / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy / Q000378:metabolism*; D009901:Optic Nerve Diseases / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy / Q000378:metabolism*
D000305:Adrenal Cortex Hormones; D019268:Antibodies, Antineutrophil Cytoplasmic