Ketotic hyperglycemia with movement disorder.

Chorea, hemichorea-hemiballismus and severe partial seizures may be the presenting features of nonketotic hyperglycemia in older adults with type 2 diabetes, but cases in young adults with type 1 diabetes are rare. We hereby report a very rare case of diabetic ketosis with movement disorder in a young patient.

INTRODUCTION

A previously healthy 21-year-old girl presented with sudden onset of rapid jerky involuntary movements involving both upper limbs in emergency department (ED). Investigations revealed hyperglycemia with positive urine ketones and normal arterial blood gases. After the control of blood sugar her movement declined dramatically within 24-36 hours. The effect of hyperglycemia could be the pathogenesis of the chorea in our patient. Severe hyperglycemia without ketosis at the clinical onset of insulin-dependent diabetes mellitus (type 1) has been reported in children and adolescents as the possible cause, but ketotic hyperglycemia is an unusual cause of chorea-ballismus in children. Chorea-ballismus is also a rare manifestation of primary diabetes mellitus. Non contrast computerised tomography (CT) head was done to look for any possible neurological cause, which revealed bilateral basal ganglia calcification and magnetic resonance imaging (MRI) confirmed this finding. Our detailed research however did not reveal any other case report of type 1 diabetes with ketones associated with this condition.

CASE REPORT

A 21 years old female patient with no significant past medical history, was admitted with complaint of rapid, jerky involuntary movement of both upper limb since 4 days. There were fidgety movements of both hands, with sustained posturing of right arm and backward posturing of body. These movements were not associated with loss of consciousness, disorientation, confusion or urinary and fecal incontinence. Patient denied any history of headache, fever or rashes. There was also no history of trauma, drug abuse or any recent drug intake. She denied history of similar complains in family. Physical examination was not significant. Patient was admitted under neurology for evaluation and management of chorea. Chest radiograph, electrocardiograph (ECG) and ECHO were normal. Laboratory results showed normal blood counts, normal liver function test and a negative antinuclear antibody (ANA) profile. Non Contrast CT head showed bilateral basal ganglia calcification. Patient was started on haloperidol per oral, calcium per oral and soft gelatin capsules containing adenosylcobalamin, methylcobalamin and pyridoxine hydrochloride per oral. There was no improvement in the condition of patient.

Her random blood sugar was done which showed a reading of 535 mg/dl. Her urine ketones were positive and arterial blood gas analysis was normal. Endocrinology consultation was sought for management of diabetic ketosis and evaluation of bilateral basal ganglia calcification. She was started on intravenous (IV) insulin infusion with hourly blood sugar monitoring. Her glycated hemoglobin (HbA1C) was 18.4. MRI brain confirmed the CT findings. After the control of blood sugars (random blood sugars-150-200 mg/dl) her movement declined dramatically within 24-36 hours.

Patient was discharged on Injection human mixtard (30/70) and was advised regular follow up under endocrinology with blood sugar profiles. Repeated follow ups have shown adequately controlled blood sugars without any recurrence of previously described movements.

DISCUSSION

Chorea is a clinical symptom characterized by spontaneous involuntary movements, muscular weakness and uncoordinated movements. It can be classified as idiopathic, hereditary or symptomatic/secondary.[1] Chorea or ballismus can be caused by a wide variety of disorders affecting the basal ganglia like metabolic diseases, hypoxic-ischemic events, vascular disorders, structural abnormalities, trauma, drugs and toxins, infections and inflammatory immunological diseases (rheumatic fever–Sydenham's chorea, systemic lupus erythematosus). Chorea has been frequently associated with lesions in the basal ganglia, and in the sub thalamic nucleus. Undiagnosed diabetes mellitus should be suspected as a possible cause in patients who develop sudden onset of generalized chorea. It is possible for a patient with chorea-ballismus to have hyperglycemia at the initial presentation.[2] It may also be associated with poorly controlled diabetes and old age.[3] Chorea, hemichorea-hemiballismus and severe partial seizures may be the presenting feature of non ketotic hyperglycemia in older adults with type 2 diabetes, but cases in children with type 1 diabetes are rare.[4] Hyperglycemia could be the result of either primary or coincidental secondary diabetes mellitus stemming from a variety of diseases.[5]

The pathogenesis of chorea or ballismus associated with non-ketotic hyperglycemia is poorly understood. In nonketotic hyperglycemia, the shift to anaerobic metabolism causes brain to utilize amino butyric acid which is synthesized from acetoacetate. Unlike in ketoacidosis, acetoacetate is rapidly depleted in nonketotic hyperglycemia causing cellular dysfunction.[6] The prognosis of hemichorea-hemiballismus as a complication of nonketotic hyperglycemia is excellent.[7] When hyperglycemia is detected and corrected, the movement disorder usually resolves within two days and may not require symptomatic therapy with dopamine receptor antagonists. In our patient, hyperkinesia resolved dramatically after control of the hyperglycemia. This illustrates that acute chorea-ballismus caused by hyperglycemia is a treatable disorder with a good prognosis. In patients with chorea-ballismus associated with nonketotic hyperglycemia in primary diabetes mellitus, CT and T1-weighted MRI images show unilateral or bilateral lesions of the putamen and/or caudate. These findings may be related to petechial hemorrhage or myelin destruction. However in our patient CT head revealed basal ganglia calcification. Thus serum glucose level should be determined in all patients with new onset of chorea, as hyperglycemia is a rapidly reversible cause of extra pyramidal movement disorders.

CONCLUSION

Chorea, hemichorea-hemiballismus and severe partial seizures are more commonly associated with type II diabetes as compared with type I diabetes. It may be the presenting feature of nonketotic hyperglycemia in older adults with type 2 diabetes. Undiagnosed diabetes mellitus should always be suspected in previously normal patients who develop sudden onset of generalized chorea. Our literature search did not reveal any other case report of type 1 diabetes with ketones associated with this condition. We hereby report a very rare case of Diabetic ketosis with movement disorder in a young patient.