Clinical and imaging features of Hallervorden-Spatz syndrome and its deep brain stimulation treatment / 中华神经医学杂志

ABSTRACT

Objective To explore the clinical and imaging features of Hallervorden-Spatz syndrome (HSS) and its deep brain stimulation (DBS) treatment.Methods A 12-year-old boy with HSS,admitted to our hospital from June 2010,was chosen in our study; the clinical and imaging features and the DBS therapy outcome were retrospectively analyzed.Results The patient (12 years old) was at an early onset,predominantly presented with dystonia,cognitive impairment,dysarthria and pyramidal signs; the symptoms were rapidly progressive.Brain MRI revealed a typical "eye-of-tiger" sign.Significant improvement of motor function after DBS of the internal globus pallidus (Gpi) and no significant improvement of cognitive dysfunction and dysarthria were noted; and the benefit of surgery was maintained during the 1 year follow-up.Conclusion The characteristic imaging manifestations and clinical features are very important for HSS diagnosis; DBS of the internal globus pallidus (Gpi) can improve the motor function; and DBS is one of the limited optional therapies for HSS.