CASE DESCRIPTION
An 86-year-old woman presented to the emergency department with involuntary movements of the left limbs and a seven-day history of polydipsia. The patient and the family denied any adittional symptoms, exposure to toxins or newly introduced medications.
The patient had previous history of arterial hypertension, hyperlipidemia, dementia and had a knee replacement surgery (date of procedure was not specified). She attended a private practice neurology clinic for her initial dementia diagnosis and was being medicated for six months with prednisolone 20mg alternating with 10mg/day. The rational for this therapy was unclear as we did not have access to her previous clinical file and neither the patient nor the family could provide any additional explanation.
She was agitated, verborrheic and with choreic movements of the left arm and foot on admission. She was hemodynamically stable with a tympanic temperature of 36,5ºC. Her blood tests revealed hyperglycemia (513mg/dL, with glycated haemoglobin of 11,9%) and excluded ketoacidosis. No evidence of acute disease was shown on head computer tomography (CT) scan.
The diagnosis of stroke of the basal nuclei versus chorea following hyperglycemia was considered, as well as newly diagnosed diabetes mellitus. Insulin therapy was strated in the emergency department and the patient was admitted to an internal medicine ward for treatment and further investigation.
At this point, corticotherapy seemed to be the cause of the patient’s hyperglycemia. After thorough investigation and careful consideration, no clear reason was found to support its need, so prednisolone was weaned.
Improvement of the clinical manifestations was observed after correction of glycemia and after eight days of treatment the involuntary movements had ceased and the patient was discharged under insulin therapy.
Brain magnetic resonance (MRI) could not be performed due to the patient’s knee prosthetics. However, clinical evolution enabled the final diagnosis of a movement disorder associated with non ketotic hyperglycemia.
After discharge, the patient attended the outpatient Diabetes clinic for 6 months and then failed the following appointments. Corticotherapy was fully weaned, and it was also possible to stop insulin. Patient maintained euglycemia for four months after discharge without any new hyperglycemic values. Thus, the diagnosis of diabetes secondary to corticotherapy was made. The patient died after some months due to non-disclosed causes.
DISCUSSION
Chorea is a type of hyperkinetic involuntary movement. Ballism is a type of choreic movement of greater amplitude that affects more proximal segments of the limbs.1
There are multiple aetiologies for chorea: hereditary, vascular, metabolic, infectious, immunologic, related to drugs and toxins or associated with tumours.2 Uncontrolled diabetes mellitus might be associated with chorea through hypo or hyperglycemia. In literature, the most reported association is between hemichorea/hemiballism and hyperglycemia, as is described in our clinical case.3
These movement disorders occur when there is a lesion in the basal ganglia as they are important structures for the control of voluntary movements, as it can cause a deactivation of the inhibitory pathways over the thalamus leading to excessive movement.[4,5] The diagnosis of chorea/ballism related to hyperglycemia includes the presence of the choreic/balistic movements, marked hyperglycemia without ketoacidosis with or without typical radiologic alterations.4,5 Gammaaminobutyric acid (GABA) and acetylcholine are the main neurotransmitters involved and hyperglycemia causes movement disorders through their depletion. With anaerobic metabolism, brain cells use GABA as a source of energy, but that does not occur in the presence of ketoacidosis, because GABA is synthesized from acetoacetate which accumulates in ketoacidosis, preventing the depletion of GABA.4,5,6 Radiologic features are usually high signal intensity images in the contralateralputamenvisualized on T1- weighted MRI, without signs of oedema, mass effect or volume loss. However, these might not be present and the diagnosis is made if the abnormal movements improve with glycemia normalization.4,5
In our case report, stroke was the main differential diagnosis considered. The neurological examination had no other alterations, and head CT scan had no evidence of acute lesions. A brain MRI was considered but not performed due to the patient’s knee prosthetics. Nonetheless, the diagnosis of chorea associated with hyperglycemia could be made by therapeutic trial. Acute chorea in adults might be a rare manifestation of cancer but it is usually associated with other neurological signs and/or symptoms and improves with the treatment of the underlying disease.7,8 Central nervous system infections might present with movement disorders, but our patient did not show fever, meningeal signs or other signs suggestive of infection. Exposure to toxins was also excluded and the patient and her family denied introduction of new medications. Therefore, the diagnosis of a movement disorder associated with non ketotic hyperglycemia was established.
In these cases, treatment consists of glycemic control.9,10 Total recovery after normalization of glycemic values is frequent, although there are refractory cases described in the literature.4,5,11 In this particular case, corticotherapy was admitted as the cause of the hyperglycemic state in a patient without a previous diagnosis of diabetes.
This diagnosis should be promptly considered in patients who have recently started glucose increasing medications and who present with acute movement disorders.Although this is a rare disease, this case shows the importance of its prompt recognition and treatment. It is a condition reversible with correction of hyperglycemia, avoiding adverse outcomes.Other important point to remember, is that the diagnosis can be made through therapeutic trail alone.12
CONCLUSIONS
Acute onset hemichorea/hemiballism might be a sign of uncontrolled diabetes or an initial manifestation of the disease. Evaluation of glycemia and/or HbA1c is essential in this clinical scenario, diagnosis can be achieved without imaging if abnormal movements improve with glycemia normalization and recognition of this condition is important because it is reversible with metabolic control.
ACKNOWLEDGMENTS
We would like to thank Dr. Pedro Pereira (Neurologist) for his contribution during the patient’s admission at the hospital.
BIBLIOGRAFIA
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