Association of Anti N-methyl-D-aspartate (NMDA) Receptor Encephalitis with Chediak-Higashi Syndrome

Background: Neurological manifestations of Chediak-Higashi syndrome mainlyinclude peripheral neuropathy, ataxia, tremors, cranial nerve palsies, intellectual declineand seizures. Case Characteristics: A 2 years 10 month old girl with silvery hair syndromepresented with sub-acute onset behavioral issues, ataxia and multiple type abnormalmovements. Cerebrospinal fluid examination was positive for Anti NMDA receptorantibodies. Hair shaft examination and peripheral blood film findings were suggestive ofChediak Higashi syndrome. Message: Anti NMDA receptor encephalitis may beassociated with Chediak Higashi Syndrome.