RESUMO
Patients with chronic kidney disease may have neurological complications including uremic encephalopathy, stroke, neuropathy and myopathy. Rarely, acute movement disorder associated with bilateral basal ganglia lesion is seen in patients with end stage kidney disease. The hallmarks of this condition include reversible and uniform lesions of the basal ganglia on MRI which stand for decreased signal intensity on T1-weighted images and increased signal intensity on T2-weighted images, and the clinical presentation includes acute parkinsonism and/or involuntary movements. This syndrome has been reported mainly in Asian patients, typically in the setting of long-standing diabetes. We report a case of bilateral basal ganglia lesions in a patient with chronic renal failure, poorly controlled diabetes, and incidents of severe hypoglycemia. In our case, there was no evidence of acute metabolic disorders. Most reported patients with acute basal ganglia lesions in uremia also had diabetes and/or abnormal blood glucose levels. Our case had previously experienced occasional hypoglycemia before the onset of involuntary choreic movements. MRI of our patient showed acute bilateral basal ganglia lesion, corresponding to cytotoxic edema. This pattern was also observed in patients with hypoglycemic encephalopathy.
RESUMO
Involvement of central nervous system is a well- known compication in uremic patients. However, development of acute extrapyramidal symptoms with bilateral basal ganglia involvement (acute basal ganglia syndrome), especially in non-diabetic hemodialysis patient is very rare. We report a case of acute basal ganglia syndrome in a non-diabetic hemodialysis patient. A 45-year-old man with autosomal dominant polycystic kidney disease (ADPKD) on chronic hemodialysis treatment for the last 4 years was admitted due to generalized myalgia. On admission, the patient was found to have rhabdomyolysis and intractable metabolic acidosis. Nine days after admission, he suddenly developed dysarthria, lateralizing ataxia, and bradykinesia. Brain MRI demonstrated low and high signals in bilateral basal ganglia and cerebellar vermis in T1-weighted and T2-weighted images, respectively. Intensified hemodialysis treatment combined with general supportive therapy resolved the severe metabolic acidosis and the neurologic manifestations gradually improved. Follow up brain CT scan taken 3 months later showed decreased size of initial low attenuation lesions in bilateral basal ganglia and cerebellar vermis. Although no definite pathophysiology is yet established, severe metabolic disorder is believed to play an important role in development of acute basal ganglia syndrome. Correction of metabolic acidosis and hypoglycemia in our patient lead to improvement in neurologic manifestations and organic brain lesions. Our case suggests that severe metabolic acidosis and hypoglycemia in uremic patient may act as risk factors for acute basal ganglia syndrome even in non-diabetic patient.