ODP261 Non-Ketotic Hyperosmolar Hyperglycemia Induced Hemichorea-Dystonia Syndrome, Without Radiologic Striatopathy
Authors: Kumi Dennis , Deenadayalan Vaishali , Ramirez Marcelo , Karki Sadicchya , Patel Birju , Nissan Ninos , Shrestha Prajwal , Soon-shiong Raquel 
Published: November 2022

Background

Movement disorder is an uncommon complication of Hyperglycemic crisis. It is often associated with diabetic striatopathy which is a clinico-radiologic entity that is associated with acute changes of the contralateral basal ganglia. Isolated dyskinetic-dystonic disorder without obvious radiologic lesion is a rare clinical entity.

Case Report

A 42-years-old woman with a history of type 2 diabetes mellitus was admitted with a 1-week history of involuntary right arm and face movements and 3 weeks of polyuria. She had recurrent choreiform right arm movements without athetosis or hemiballismus lasting about 2. Additionally, she experiences right cervicofacial dystonia. There was no involvement of the lower extremities, no loss of consciousness and no other neurologic deficit between attacks. Physical examination was essentially unveiling with no focal neurologic deficits or tremors. Initial work up showed elevated; blood glucose to 620mg/dl (65–110), serum sodium of 148mEq/L (135–145) and serum osmolality of 330mmol/kg (275–295). Work up was negative for acidosis, with pH of 7.39 (7.32–7.42), bicarbonate of 23mEq/L (23–31) and no ketones on urinalysis. Other relevant test including serum bilirubin, transaminases, ammonia level, calcium, ceruloplasmin and cupper were normal. An initial non-contrast CT scan of the brain was negative for acute pathology and MRI did not show any lesions in basal ganglia. E. E. G was also normal. Movements recurred twice in hospital, and she received IV lorazepam without much response. She was diagnosed with non-Ketotic hyperosmolar hyperglycemic chorea- dystonia syndrome. She received IV normal saline and started on insulin therapy resulting in improvement in serum glucose to 255 by the next morning. She was discharge after 72 hours with no recurrence at 2 weeks follow up visit.

Discussion

Hyperglycemic crisis may rarely be associated with dyskinetic syndromes, often due to the development of diabetic striatopathy and about 80% are without ketosis. The mechanism is believed to be due to impaired GABA synthesis in the basal ganglia. Prevalence of hyperglycemia induced striatopathy stands at 1 per 100,000. The commonest manifestation is that of hemiballismus and chorea. Very few cases of dystonia have been reported. It is a clinico-radiologic diagnosis with basal ganglia abnormalities on imaging stemming from edema, microhemorrhages or calcific changes. There are very few case reports of hyperglycemic dyskinetic syndromes without CNS changes on MRI. The average time for resolution of MRI changes is 60 days thus we believe our patient had no radiologic striatopathy. In majority of cases, resolution of hyperglycemia leads to clinical resolution of dyskinetic spells. Occasionally, some patients may require medications such as antipsychotics including haloperidol, or benzodiazepines such as clonazepam to control movements. In conclusion, movement disorder related to hyperglycemia though rare, can be disabling. Prognosis is excellent if a timely clinical diagnosis is made.

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