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.
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Familial amyloid polyneuropathy (FAP) is an autosomal dominant genetic disorder with systemic deposition of amyloid fibrils, and is characterized by progressive sensory, motor, and autonomic polyneuropathy. FAP was considered a rare endemic disease; however, its worldwide incidence is much higher than previously recognized. ⋯ Furthermore, a large number of the patients are not good transplant candidates because of their age and/or advanced disease status. Recently, the clinical effects of two transthyretin tetramer stabilizers, diflunisal and tafamidis, were demonstrated in randomized clinical trials, and tafamidis was approved for the treatment of FAP in European countries in 2011 and Japan in 2013.