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Tumor necrosis factor receptor–associated periodic syndrome (TRAPS) is an autosomal dominantly inherited autoinflammatory disorder caused by mutations in the TNFRSF1A gene (MIM 191190). Clinical features of TRAPS include recurrent inflammatory episodes with variable symptoms like fever, abdominal pain, myalgia, arthralgia, exanthema, and ocular involvement.1 In addition, a few cases of CNS involvement have been described in association with TRAPS. In one report, a patient with TRAPS developed a single inflammatory, MRI contrast-enhancing lesion in the cerebellum.2,3 Histopathologic examination of a stereotactic brain biopsy showed a dense infiltration with polymorphonuclear leukocytes without demyelination. The authors suggested that this was a case of TRAPS with CNS manifestation (CNS-TRAPS).2,3 We report a patient with TRAPS carrying the R92Q substitution encoded by exon 4 of the TNFRSF1A gene, who presented with a demyelinating disorder and whose symptoms and histology were highly reminiscent of multiple sclerosis (MS).
Case report.
Course and diagnosis of the MS-like demyelinating disease.
At age 16, the patient experienced a facial nerve palsy, and 8 months later an episode with hypesthesia of the left arm …
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