An adult man with progressive dysarthria, dysphagia and disequilibrium

The patient was a 40 year old man, who referred to our clinic because of progressive dysarthria, dysphagia and disequilibrium for the previous 15 years. According to his medical history, he had bilateral cataract surgery when he was only 2 years old and mild mental retardation since early childhood. On neurologic examination, he had severe dysarthria (anarthria) and jaw opening dystonia with drooling. Furthermore, he had bilateral hand dystonia and mild upper limb dysmetria. Deep tendon reflexes were brisk and there was bilateral Babinski and leg spasticity. Due to his severe dystonia, spasticity and ataxia, he had become bedridden and was unable to walk. The brain MRIs are presented below (Figure 1): 1What abnormalities are seen on brain MRI? 2What is the most probable diagnosis? 3Is there any treatment for this patient (other than symptomatic treatments)? Answers