ACUTE CHOREO-DYSTONIA IN A NEWLY DIAGNOSED PATIENT WITH DIABETES MELLITUS: A CASE REPORT AND REVIEW OF LITERATURE

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T.O. Akande1, O.V. Olalusi¹, D.I. Olulana²

1. Department of Medicine, University College Hospital, Ibadan, Nigeria.
2. Department of Surgery, University of Ibadan and University College Hospital, Ibadan, Nigeria.

Abstract

Introduction: Diabetes mellitus is a disease with diverse macrovascular and microvascular consequences. One of the unusual effects of hyperglycemia is involuntary movement, termed hyperglycemia-induced involuntary movement. This could range from hemibalismus, chorea, choreo-atethosis, tremors to dystonia. Chorea associated with dystonia is a less commonly reported manifestation. When it is focal, it can be misdiagnosed as stroke or seizure disorder. To the best our knowledge, there is hitherto no case report in sub-Saharan Africa describing the occurrence of focal choreo-dystonia in type 2 Diabetes Mellitus.

Case presentation: Here, we present a case of a middle-aged Nigerian woman with focal choreo-dystonia of the right upper limb accompanying the diagnosis of type 2 diabetes. Achieving euglycemia with insulin resulted in complete resolution of the choreo-dystonia.

Conclusion: Doctors in resource-constrained settings should be aware of this presentation to avoid misdiagnosis and to provide prompt and goal-oriented management with a view to reducing morbidity and attendant health-care costs.

Keywords: Diabetes mellitus, Choreo-dystonia, Case report, Hyperglycemia, Involuntary movement

Background

Diabetes mellitus (DM) is a disease of public health concern with rapidly worsening epidemiologic indices.¹ It affects over 400 million patients worldwide with devastating macrovascular and microvascular complications.² However, involvement of the nervous system and the spectrum of movement abnormalities in patients with DM are often under-recognised and under-reported.³ These neurologic features can result from hyperglycemia, vascular complications or the treatment of the DM itself. Early recognition and diagnosis however remain a challenge in resourcelimited settings. One of the unusual effects of hyperglycemia is involuntary movement, termed hyperglycemia-induced involuntary movement (HIIM).⁴

Chorea is a hyperkinetic movement disorder characterized by rapid non-purposeful dance-like movements of distal limbs and can involve the face and trunk while dystonia is a sustained contraction of both agonistic and antagonistic muscles giving rise to abnormal posturing.⁵ Chorea, dystonia, athetosis, and ballism are some of the involuntary movements of the choreiform spectrum that have been described in literature^6,7. Bedwell⁸, in 1960, first reported chorea as a rare clinical entity but it is now known to encompass a triad of chorea, hyperglycemia, and basal ganglia hyperintensity. The prevalence of hyperglycemia induced chorea in resource-limited settings is unknown and this could be due to under-recognition and late diagnosis. Dystonia associated with chorea is rare and, to the best of our knowledge, yet unreported in subSaharan Africa. Here, we present a case of a middleaged woman with focal choreo-dystonia of the right upper limb heralding the diagnosis of type 2 diabetes. Prompt recognition and attainment of euglycemia with insulin resulted in complete resolution of the choreodystonia.

CASE PRESENTATION
A 64-year-old right-handed Nigerian female was admitted on account of sudden onset of involuntary movement of the right upper limb a week prior to presentation. She described involuntary movement of the right upper extremity with associated abnormal posturing. The abnormal movement was precipitated by attempts to use the hand, starting as abnormal twisting involving the wrist and then jerky movements of the forearm, resolving spontaneously within five minutes. Symptoms occurred during the day and she had no preceding numbness and paresthesias. She had no prior history of similar symptoms, involvement of other body parts, limb weakness or gait abnormality. There was no loss of consciousness during episodes, alteration in sensorium or personality change. She had received a diagnosis of diabetes mellitus at a private health facility two weeks before presentation following a month’s history of increased urination and thirst. She had no known family history of DM. She had been commenced on tabs metformin 500mg three times daily and glibenclamide 5 mg daily to which she was not compliant.

Vital signs were normal on admission. Other than moderate dehydration and choreo-dystonic hyperkinetic movement involving the right hand, the other aspects of the physical findings were unremarkable. Admitting fasting blood glucose was 400mg/dl. The Hemoglobin A1c was 14.5%. The complete blood count was normal. Assessment was acute focal choreo-dystonia from severe hyperglycemia to exclude a stroke and focal-onset seizure disorder. An axial section of the computed tomography (CT) scan of the brain showed hyperdense signals within the Globus pallidi, the falx and choroid plexus in keeping with calcification (likely age-related) figure 1. A brain MRI was not done due to financial constraints, the electroencephalogram (EEG) was otherwise not remarkable. Serum electrolytes, calcium, magnesium as well as albumin were essentially normal. Dipstick urinalysis showed 1+ ketonuria, 2+ glycosuria and trace leucocytes. The serum osmolality was 343mosm/l.

She was placed on intravenous fluids normal saline (one litre every 8 hours), basal-bolus insulin regimen (soluble insulin 8 units 30 minutes before meals and Glargine 14 units at night) and tabs levetiracetam (Keppra) 500 mg daily. She was managed by the endocrinology and neurology teams while on admission. Following control of the hyperglycemia the abnormal movements completely resolved on day five of in-patient care. She was discharged home 9 days after admission. Medications at discharge were to tabs gliclazide 30mg daily, sitagliptin 50mg daily, metformin 1000 mg twice daily, glargine insulin 14 units at bed time and levetiracetam 500 mg twice daily. She was followed up at the clinic 2 weeks, 1 month, 3 months and 6 months post-discharge and had no repeat dystonia. The glycemic control remained satisfactory. Blood pressure remained normal, fasting plasma glucose ranged between 111-141 mg/dl, 2 hours post prandial ranged between 119-148mg/dl. Levetiracetam was discontinued and there was still no recurrence of the abnormal movements. She continued to do well on anti-diabetic medications till date. In one of her clinic sessions, she was thankful that the diagnosis was not stroke and particularly glad that her symptoms had resolved without any neurologic sequalae or disability.