Brain and nerve = Shinkei kenkyū no shinpo
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Review Case Reports
[Multiple cranial neuropathies in a patient with IgG4-related hypertrophic pachymeningitis: a case report].
We describe the case of a 75-year-old woman who presented with acute loss of vision. She experienced subacute headache, hearing loss on the left side, hoarseness, and dysphagia during the previous 10 months. On admission, she had bilateral loss of vision, without any ophthalmological abnormalities, and multiple cranial nerve palsies, including left hearing loss and right IX, X, and XI nerve palsies. ⋯ However, it should be recognized that these conditions might be underdiagnosed. The possibility of central nervous system involvement in IgG4-related disorders should be considered in patients with multiple cranial nerve neuropathies associated with hypertrophic pachymeningitis, even in the absence of systemic sclerosis symptoms. In our case, early treatment with corticosteroids showed immediate effectiveness in correcting the visual symptoms.