Abstract

A 42-year-old female presented with a two-week history of difficulty walking, multiple falls and urinary hesitancy. Relevant past medical history included undiagnosed recurrent lymphadenopathy and immunogenic thrombocytopenic purpura. Several episodes of ‘eye inflammation’ were also reported. Examination revealed pyramidal weakness in the lower limbs with hypereflexia, upgoing plantars and a spastic gait. Widespread lymphadenopathy was also found. Autoimmune serology was negative including ANA and anti ds-DNA. CSF analysis revealed a mild elevation in protein (0.56) only. MRI demonstrated a longitudinally extensive myelitis throughout most of the thoracic spinal cord. CT of the thorax revealed asymptomatic pneumonitis. Lymph node biopsy showed partial effacement of normal architecture with fibrosis, focal areas of necrosis and numerous CD68+ histiocytes. A diagnosis of Kikuchi-Fujimoto Syndrome was made. This is a syndrome of relapsing lymphadenitis with negative autoimmune serology and characteristic histopathological features on lymph node biopsy. Rare extranodal manifestations include ITP, pneumonitis and uveitis. There are isolated case reports of neurological involvement, including acute disseminated encephalomyelitis. The syndrome generally responds well to treatment, and our patient had resolution of all clinical features after methylprednisolone induction and hydroxychloroquine maintenance.