A 27-year-old woman had normal motor and intellectual development and no family history of neurological disease. At age 22 years, she developed mood changes such as indifference and loss of impetus. Two days after an emergency appendectomy that was uneventful, she became irritable and aggressive. This was associated with transient auditory hallucinations. She was initially treated with haloperidol but developed extrapyramidal adverse effects. Within 9 months, she became mute and required electroconvulsive therapy, which lifted her mood transiently. Her extrapyramidal syndrome also improved on switching to clozapine. However, by age 24 years, her gait became shuffling and she experienced falls. She became bradykinetic and developed micrographia. She began receiving levodopa but within 7 months developed drug-induced orofacial and limb dyskinesias, which responded to treatment. At age 26 years, she had a single generalized convulsion. Anticonvulsive therapy was initiated and was effective. A progressive decline in her language ability had been noted since age 24 years, and at about the time of her seizure, she was unable to engage in conversation and could only say occasional words. She is currently dependent for most activities of daily living. General examination was unremarkable apart from hypomimia and emaciation. Higher mental functions were severely impaired on verbal and nonverbal tests of reasoning. She had weak visual perceptual and visuospatial skills, comprehension difficulties, dyscalculia, and impaired memory skills. Pursuit eye movements were jerky and vertical saccades were slow. There was marked dystonic posturing in both arms and legs. The reflexes were brisk and the plantar responses were spontaneously extensor. Biochemical and hematological test results were normal. There were no acanthocytes in her peripheral blood. Cerebrospinal fluid examination results were unremarkable. Genetic test results were negative for Huntington disease, spinocerebellar ataxia 1, 2, 3, 6, 7, 12, and 14, dentorubropallidolysian atrophy, and PANK2, DYT1, ferritin light-chain gene, and α-synuclein gene mutations. Electroencephalography showed normal activity. Magnetic resonance imaging of her brain at age 27 years showed severe cerebral atrophy with a frontotemporal predominance. The T2-weighted images demonstrated low signal intensity, indicative of abnormal iron deposition in the globus pallidus, cerebral peduncles, and substantia nigra, although no “eye of the tiger” sign was seen (Figure, A). A computed tomographic scan excluded basal ganglia calcification. Muscle biopsy showed mild nonspecific myopathic features, and results of a muscle mitochondrial assay for respiratory chain function were normal. Results of liver biopsy for copper deposition were negative. Frontal cortical biopsy was also performed.