Nelson Textbook of Pediatrics 22nd Edition | Group of inherited disorders with iron deposition in basal ganglia. PKAN (PANK2, most common, eye-of-the-tiger sign), PLAN (PLA2G6, infantile neuroaxonal dystrophy), MPAN (C19orf12), BPAN (WDR45, X-linked, SENDA syndrome), FAHN (FA2H, spastic paraplegia). Clinical: progressive dystonia, parkinsonism, spasticity, cognitive decline. MRI: T2 hypointensity in globus pallidus/substantia nigra. Treatment: supportive (dystonia management, DBS).
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