Nonparaneoplastic limbic encephalitis with relapsing polychondritis

• DOI: 10.1016/j.jns.2004.02.010
• Publication Date: 2004-04-03T10:19:42Z
• Authors: Yasuyuki Ohta; Isao Nagano; Daigo Niiya; Hideki Fujioka; Takumi Kishimoto; Mikio Shoji; Koji Abe
• Keywords: Male; Memory Disorders; Depression; Brain; Anxiety; Middle Aged; Magnetic Resonance Imaging; 3. Good health; 03 medical and health sciences; 0302 clinical medicine; Limbic Encephalitis; Humans; Steroids; Polychondritis, Relapsing

Relapsing polychondritis (RP), which shows pain, swelling and destruction of the affected parts, is a rare autoimmune disorder affecting cartilage. We report a patient with RP that affected skull cartilage, who subsequently developed multifocal meningoencephalitis. The patient presented with severe recent memory disturbance, anxiety and moderate depression. MRI study showed bilateral median temporal lobe lesions including hippocampi and amygdaloidal bodies, abnormal findings that disappeared after treatment with high-dose steroids. This is thought to be the first case of RP presenting amnesic syndrome and mental disorder associated with nonparaneoplastic limbic encephalitis involving bilateral hippocampi and amygdaloidal bodies detected by MRI.