Proteus syndrome is a rare disease manifested by progressive segmental overgrowth involving the skeletal, Cutaneous, subcutaneous, and nervous systems. We report the case of a 24-year-old female who was born with no obvious abnormality at birth. From the age of 1 year, she developed asymmetric enlargement of her left upper limb and bilateral lower limbs leading to enlargement of the right-hand phalanges with radial deviation, enlargement of the right big toe, lateral deviation of left foot, and discrepancy in the length of lower extremities and kyphoscoliosis. She had become bed-bound for the last few years due to increasing disability. She was diagnosed with Proteus syndrome based on clinical features of progressive course, mosaic distribution, and sporadic occurrence of the lesions.

A previously healthy 30-year old lady presented with 1-day history of high grade fever and drowsiness. Five days prior to presentation, she developed insomnia and visual hallucinations of seeing unknown faces. Three days prior to presentation, she suffered from 5 episodes of generalized tonic colonic fits. On admission, she had a temperature of 102oF and GCS of 10/15 with no signs of meningeal irritation, no focal neurological deficit, normal deep tendon reflexes and down-going plantar reflex bilaterally. Her brain MRI scan showed mild hyper-intense signals in right cerebellum. Her CSF was tested for Anti-NMDA receptor antibodies which were positive. She was diagnosed as having Anti-NMDA Receptor Encephalitis and started on immediate Plasma Exchange with drastic improvement.