Neurodegeneration With Brain Iron Accumulation

NBIA is a group of inherited disorders characterized by excessive iron deposition in the basal ganglia. Clinical features include dystonia, parkinsonism, spasticity, and cognitive decline. Pathophysiology involves defective iron metabolism leading to oxidative stress and neuronal degeneration. Diagnosis relies on MRI showing basal ganglia iron accumulation and genetic testing. Management is supportive, including symptomatic therapy and iron chelation in experimental contexts; prognosis is progressive.