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Few reports have focused on the unusual phenotypes in dystonia-ataxia syndrome, and those studies proposed new entities for these phenotypes, such as predominant dystonia with marked cerebellar atrophy (DYTCA) or slowly progressive cerebellar ataxia and cervical dystonia. 1,2 We performed pathologic and genetic examinations on a patient presenting a unique dystonia-ataxia phenotype.Case report. The patient was born to healthy, nonconsanguineous parents and had a medical history of atopic dermatitis and asthma. He had a younger brother, who exhibited slight unsteadiness and mild cerebellar atrophy (appendix e-1 on the Neurology ® Web site at Neurology.org). At age 42 years, the patient noticed an uncomfortable sensation in the larynx and experienced a slight speech disturbance. At age 43 years, he started to drag his left leg and presented to us with walking difficulties. On neurologic examination, he exhibited spasmodic dysphonia, lower limb dystonia, and limb and truncal ataxia. Brain MRI demonstrated severe cerebellar atrophy (figure, A and B). He did not present nystagmus, hyperreflexia or hyporeflexia, pathologic reflexes, parkinsonism, autonomic disturbances, or cognitive impairment. The results of blood examinations, including hemogram and albumin, a-fetoprotein, copper, lactate, and pyruvate levels, were within normal ranges. At age 46 years, his dystonia symptoms spread rapidly to other body parts, including the neck, upper limbs, eyes, and trunk (video). However, his ataxia remained static. Trihexyphenidyl and L-dopa had only a modest effect on his dystonia. At age 47 years, he underwent bilateral deep brain stimulation of the globus pallidus pars interna (GPi-DBS), which partially alleviated his dystonia symptoms (his preoperative Burke-Fahn-Marsden Dystonia Rating Scale score of 88 was decreased to 66). Twenty-two days after the operation, he was found in cardiac arrest in the prone position.A macroscopic analysis revealed hemostasis in multiple organs, which suggested positional asphyxia as the cause of death. The cerebellum was markedly

We studied 50 Japanese patients who were first diagnosed with type 2 diabetes mellitus. Psychological functioning was assessed by using the Rorschach test and the Yatabe-Guilford Personality test. Analysis indicated that patients who discontinued clinic attendance during the 2-yr. study period had more depression, lower general activity and less extraversion, more negative self-attitudes, and more concern about being helpless than those who continued having regular followups. This study also found that patients who had worse glycemic control 2 yr. after being first diagnosed reported more inferiority feelings, lower general activity, more need for closeness to others, and more emotional demands than those who had better glycemic control. In conclusion, understanding the psychological functioning would be helpful in the management of Japanese patients with type 2 diabetes mellitus.