Hemichorea-Hemiballism Syndrome Caused by Nonketotic Hyperglycemia in a Newly Diagnosed Diabetes Mellitus Type 2 Patient with Euglycemia at Presentation

@#INTRODUCTION: Nonketotic hyperglycemia among type 2 diabetic patients have recently been documented to cause the rare movement disorder called Hemichorea-hemiballism syndrome which is a hyperkinetic movement disorder presenting as a continuous, non-patterned, involuntary movements caused by a basal ganglia dysfunction. METHODS: A 76-year-old male with a known history of hypertension and no history of stroke and diabetes presented with a 10-day history of increasingly persistent involuntary movements of the right extremities. On admission, the patient was conscious with stable vital signs and unremarkable neurologic findings except for the involuntary flailing movements of the right extremities. Diagnostic testing revealed first documentation of hyperglycemia with brain MRI changes on T1 hyperintensity signals on the basal ganglia and T2/FLAIR weighted imaging showing mixed hypointense and hyperintense signals which is a classical MRI finding in patients with HC-HB syndrome caused by nonketotic hyperglycemia. The patient was treated for diabetes and was maintained on anti-dopaminergic medications for the uncontrollable involuntary movements. After five months, resolution of the hemiballism-hemichorea syndrome was noted after appropriate treatment. CONCLUSION: This case report highlights hemichoreahemiballism syndrome in a newly diagnosed type 2 diabetic patient who had normal glucose levels at presentation. The prompt recognition and correction of uncontrolled newly diagnosed diabetes and administration of anti-dopamine agents lead to a rapid improvement of symptoms, less neurologic sequelae and an overall favorable prognosis.

Clinical and neuroimaging characteristics of patients with hemichorea-hemiballismus induced by nonketotic hyperglycemia / 第二军医大学学报

Objective To study the clinical manifestations and neuroimaging characteristics of patients with hemichorea-hemiballismus (HC-HB) induced by nonketotic hyperglycemia. Methods The clinical data, laboratory findings and neuroimaging findings of 5 patients with HC-HB induced by nonketotic hyperglycemia who were treated in Changhai hospital of Second Military Medical University were retrospectively analyzed. The 5 patients included 4 females and 1 male, ranging 65-83 years old and averaging (76. 6±7. 2) years old. Results All the five patients presented an acute onset, with four of them having chorea or ballismus involuntary movement in unilateral limbs and face and one having generalized chore. The highest levels of blood glucose in patients at onset were 18. 6-44. 6 mmol/L (averaging[26. 6±10. 5] mmol/L), with negative urine ketone. T1-weighted imaging showed hyperintensity in contralateral basal ganglia in 4 cases and in bilateral basal ganglia in 1 case, with no edema or mass effect; most T2-weighted imaging was of isointensity. Increased protein levels and normal number of cells were observed in 3 cases in cerebrospinal fluid examination, and two of three cases had increased IgG index or 24 h intrathecal synthesis rate, which was relieved by effective control of blood glucose combined with pharmacotherapy, such as haloperidol. Conclusion Nonketotic hyperglycemia and HC-HB are the characteristics of HC-HB induced by nonketotic hyperglycemia, with hyperintensity on T1-weighted MRI imaging in the contralateral basal ganglia or in bilateral basal ganglia occasionally. Early diagnosis and proper treatment of those patients can achieve good prognoses.

Oromandibualr Dystonia Associated with Diabetic Hyperglycemia

Oromandibular dystonia (OMD) is a focal dystonia that is characterized by repetitive or sustained spasms of the masticatory, facial, or lingual muscles. The etiology is idiopathic in most cases. A patient presenting with OMD associated with diabetic hyperglycemia is reported herein. A 74-year-old woman with a history of diabetes developed OMD. Brain MRI revealed a high signal intensity in the bilateral putamen on T1-weighted imaging. Nonketotic hyperglycemia was detected. The OMD gradually subsided with normalization of the hyperglycemia and medication with haloperidol over 10 days.

Two Cases of Hemichorea Associated with Nonketotic Hyperglycemia in Type 2 Diabetes Mellitus Patient

Hemichorea-hemiballism is a rare complication of nonketotic hyperglycemia in type 2 diabetes mellitus (T2DM). It can be complicated in long-standing type 1 diabetes mellitus or T2DM, and has been described as a presenting symptom of new-onset diabetes. Rapid correction of diabetic ketoacidosis may also cause the delayed hemichorea. Although hyperglycemic hemiballism rarely causes generalized chorea due to bilateral basal ganglia involvement, patients typically present with hemichorea developing over days to months in the setting of elevated serum glucose. On T1-weighted brain magnetic resonance imaging and computed tomography scan a high signal intensity lesion at the basal ganglia is characteristic. After the correction of hyperglycemia, the movements generally disappear within hours, but atypical cases with delayed onset after the resolution of hyperglycemia, unremitting severe movements, and late recurrence are also reported. We report two cases of female T2DM patients who presented with hemichorea. One patient presented with hemichorea in nonketotic hyperglycemia, and the other with delayed onset hemichorea after the resolution of hyperglycemia.

Visual Seizure: A Reversible Complication of Non-Ketotic Hyperglycemia

A 65-year-old man with diabetes mellitus was presented with left visual aura, followed by a versive seizure, each lasting approximately 3 minutes. Neurological examination showed an intermittent left homonymous hemianopsia. Brain magnetic resonance imaging (MRI) showed right occipital lobe lesion, with cytotoxic edema. Blood glucose was 593 mg/dL and serum osmolarity was 309 mOsm/kg. The seizures were controlled by normalization of blood sugar and short-term anticonvulsant, and the lesions were resolved in a follow-up MRI. We report a case of visual seizures associated with non-ketotic hyperglycemia.

A Case of Nonketotic Hyperglycemia as a Manifestation of Generalized Convulsive Status Epilepticus / 대한내과학회지

Seizure is one of the manifestations of nonketotic hyperglycemia (NKH). Partial motor seizures are observed in most cases and, occasionally, with epilepsia partialis continua. Generalized convulsive status epilepticus caused by NKH is rare. In this report, we present a case of a 68-year-old man who developed generalized convulsive status epilepticus as an initial manifestation of NKH.

Acute Onset Of Nonketotic Hyperglycemic Hemichorea Associated With Putaminal Hypointensity On Gradient Echo Magnetic Resonance Image

No abstract available.

MR Signal Change in a Patient with Partial Seizure and Nonketotic Hyperglycemia / 대한간질학회지

Seizures are a common presenting manifestation in patients in a nonketotic hyperglycemic(NKH) hyperosmolar state and the MR findings are typically hyperintense on T2 weighted (T2-W) and fluid attenuated inversion recovery (FLAIR) images. We recently experienced a case of the patient with partial seizure and nonketotic hyperglycemia who showed T2-W and FLAIR hypointensities with T1 weighted (T1-W) contrast enhancement.

One Case of Reflex Focal Seizures Associated with Nonketotic Hyperglycemia / 대한간질학회지

BACKGROUND & SIGNIFICANCE: Focal seizures are usually due to an underlying structural lesion of the brain but occasionally caused by metabolic disturbances such as nonketotic hyperglycemia (NKH). Reflex focal seizures associated with NKH have been reported. CASE: The 69 year-old man was admitted in our hospital due to clonic movements of the right leg. His seizures did not occur spontaneously but were provoked by active or passive movements and tactile stimulation of the affected leg. The convulsive movements of the right leg often spread to the left leg without involving any other parts of his body. The seizures regressed with the control of hyperglycemia only. COMMENT: We report a patient presented with reflex focal seizures as the initial manifest-ation of NKH.