A Difficult to Diagnose Case of Hypertrophic Pachymeningitis with Absence of Characteristic Symptoms

<p>We present a difficult-to-diagnose case of hypertrophic pachymeningitis with the absence of characteristic symptoms. A 32-year-old woman was diagnosed as having lupus erythematosus profundus in 2007. In August 2013, she was prescribed oral prednisolone at 20.0 mg/day for her condition, and cyclosporine 100 mg/day was added in July 2016. From October, she experienced headache daily. In November, the prednisolone dosage was reduced to 17.5 mg/day because her lupus erythematosus profundus showed good improvement. About two weeks after the dosage reduction, she complained of fever of over 37 °C, general malaise, and vomiting, in addition to her headache; thus, she was closely examined by her previous doctor, but no organic disease was found. Her symptoms did not improve even when the cyclosporine was stopped. She was diagnosed as having tension-type headache and was prescribed alprazolam and loxoprofen sodium as needed. However, her symptoms barely improved. On her first visit to us, she presented with medium-severity continuous headache occurring every day, but no clearly associated neurological abnormalities were found. Blood test results showed an enhanced inflammatory response and positivity for myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA). Brain magnetic resonance imaging (MRI) indicated enlargement of the pachymeninx on the right side of her head, bilateral fornices, and bilateral cerebellar tentoriums, and she was diagnosed as having hypertrophic pachymeningitis. Her symptoms rapidly improved after the administration of a corticosteroid and did not exacerbated even with the combined use of azathioprine. The diagnosis in this patient was difficult because no characteristic symptoms of hypertrophic pachymeningitis except for headache were observed. The enhanced brain MRI was helpful in determining the underlying cause of headache in the absence of any other clear neurological abnormalities and in making the final diagnosis of hypertrophic pachymeningitis.</p>