Further learning of clinical characteristics and imaging manifestations of nonketotic hyperglycemic hemichorea.

OBJECTIVE: To summarize the clinical characteristics and imaging manifestations of patients with nonketotic hyperglycemic hemichorea (NH-HC) and to explore the possible pathogenesis, diagnosis. and treatment of the disease in order to improve the understanding of this disease and avoid misdiagnosis. METHODS: Retrospective analysis was performed on the case data of five patients with NH-HC admitted to our hospital in recent years. The patients were treated in the department of endocrinology, department of neurology, and department of neurosurgery in our hospital, respectively. Meanwhile, relevant literatures were consulted for further learning. RESULTS: NH-HC is usually presented as a triad of nonketotic hyperglycemia, lateral chorea, and typical imaging manifestations of head magnetic resonance imaging or computed tomography, but the clinical manifestations are not the same, and imaging features may also be different, presenting a diversified trend in clinical practice. All five patients were given glucose-lowering drugs and improved with or without combination of drugs to control symptoms of chorea. CONCLUSION: NH-HC is a rare complication of diabetes, characterized by hyperglycemia and hemichorea. How to identify the extreme situation and make fast judgment is a top priority. Timely and correct control of blood glucose is the key to the treatment, and when necessary, application of dopamine receptor antagonists in patients with combination therapy can accelerate improvement of the clinical symptoms. The prognosis of NH-HC is good, the clinician should strengthen comprehensive understanding of this disease to avoid missed diagnosis or misdiagnosis and enable patients to get more timely and effective treatment.

Nonketotic hyperglycemia hemichorea and hemiballismus: a case report.

BACKGROUND: Diabetic striatopathy, also known as hyperglycemic hemichorea-hemiballismus, is a rare movement disorder associated with nonketotic hyperglycemia in patients with poorly controlled diabetes mellitus. The pathophysiology is not fully elucidated but may involve hyperviscosity, ischemia, and alterations in basal ganglia neurotransmitters. CASE PRESENTATION: We present a case of a 64-year-old Asian female patient with longstanding poorly controlled type 2 diabetes mellitus who developed abrupt-onset right-sided hemichorea-hemiballismus. Laboratory results showed hyperglycemia without ketoacidosis. Neuroimaging revealed left putaminal hyperdensity on computed tomography and T1 hyperintensity on magnetic resonance imaging. With insulin therapy and tetrabenazine, her movements improved but persisted at 1-month follow-up. DISCUSSION: This case illustrates the typical features of diabetic striatopathy, including acute choreiform movements contralateral to neuroimaging abnormalities in the setting of nonketotic hyperglycemia. While neuroleptics may provide symptomatic relief, prompt glycemic control is critical given the risk of recurrence despite imaging normalization. CONCLUSION: Diabetic striatopathy should be recognized as a rare disorder that can occur with poorly controlled diabetes. Further study of its pathophysiological mechanisms is needed to better guide management. Maintaining tight glycemic control is essential to prevent recurrence of this debilitating movement disorder.

Nonketotic Hyperglycemic Hemichorea in an Elderly Male: A Case Report.

BACKGROUND: Nonketotic hyperglycemic hemichorea is a rare complication of diabetes mellitus seen in the emergency department. It is most commonly reported in elderly women, predominantly of Asian race, with poorly controlled diabetes mellitus. Patients present with a triad of nonketotic hyperglycemia, hemichorea, and contralateral basal ganglia abnormality on imaging. Its exact pathophysiology is still not known. However, it has a very good prognosis with early diagnosis and treatment. CASE REPORT: We report a case of hemichorea involving the right upper and lower limbs due to nonketotic hyperglycemia. The patient's symptoms resolved after normalization of blood glucose. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Nonketotic hyperglycemic hemichorea should be included in the differential of a patient presenting with chorea and high blood glucose levels. It has an excellent prognosis with both symptoms and imaging abnormalities typically resolving completely with restoration of normoglycemia.

Hemichorea Associated With Nonketotic Hyperglycemia.

CONTEXT: Hemichorea associated with nonketotic hyperglycemia (HC-NH) is a rare diabetic complication for which the pathogenesis remains unclear. OBJECTIVE: This study reported 16 cases of HC-NH to improve the understanding of the disease and avoid misdiagnosis and missed diagnosis. METHODS: Data of 16 patients with HC-NH in a single center from 2000 to 2021 were analyzed retrospectively, and the relevant literature was reviewed. RESULTS: The participants (8 men and 8 women) had a mean age of 67.6 ± 16.4 years. Bilateral limbs were involved in 2 cases, and the others had hemichorea (6 in the left side and 8 in the right side). The average random blood glucose level was 17.51 ± 7.67 mmol/L, and the glycated hemoglobin A1c level was 11.9%±3.1% at admission. Eleven patients had a history of diabetes, and the other 5 patients were diagnosed with new-onset diabetes mellitus, but no remarkable differences were observed in the presentation or treatment of chorea. Ketonuria was detected in 7 patients. The basal ganglia (putamen, globus pallidus, and caudate nucleus) of 9 cases had typical hyperdensity on computed tomography and/or hyperintensity signals from magnetic resonance imaging. The chorea symptoms of 15 patients improved within 5.0 ± 1.9 days after treatment. CONCLUSION: This study provides additional valuable information about the clinical and neuroimaging features of HC-NH. We hypothesize that chronic ischemia of the basal ganglia due to cerebral atherosclerosis combined with hyperglycemia is associated with HC-NH.

Non-Ketotic Hyperglycemic Hemichorea-Hemiballismus in the Setting of Antipsychotics and Methamphetamine.

The management of common chronic conditions presenting in uncommon ways is an important facet of medical education and patient care. We report a 54-year-old patient who developed left arm dystonia precipitated by several potential factors. These include laboratory findings notable for significant hyperglycemia, methamphetamine positive on urine drug testing and patient history consistent with bipolar II managed with Lurasidone, a second-generation antipsychotic medication. The potential etiology of this uncommon presentation is discussed below.

Hyperglycemic hemianopia: A case report.

BACKGROUND: Nonketotic hyperglycemia (NKH) is characterized by hyperglycemia with little or no ketoacidosis. Diverse neurological symptoms have been described in NKH patients, including choreoathetosis, hemiballismus, seizures, and coma in severe cases. Homonymous hemianopia, with or without occipital seizures, caused by hyperglycemia is less readily recognized. CASE SUMMARY: We describe a 54-year-old man with NKH, who reported seeing round, colored flickering lights with right homonymous hemianopia. Cranial magnetic resonance imaging demonstrated abnormalities in the left occipital lobe, with decreased T2 signal of the white matter, restricted diffusion, and corresponding low signal intensity in the apparent diffusion coefficient map. He responded to rehydration and a low-dose insulin regimen, with improvements of his visual field defect. CONCLUSION: Patients with NKH may present focal neurologic signs. Hyperglycemia should be taken into consideration when making an etiologic diagnosis of homonymous hemianopia.

Bilateral striatum with high-signal intensity on T1-weighted MRI: A case of hemichorea induced by nonketotic hyperglycemia.

Hemichorea induced by nonketotic hyperglycemia is a rare complication of diabetes mellitus. Here we present a case of 80-year-old female patient. She had a history of involuntary movement of the left body. Imaging examination showed abnormal signs of bilateral basal ganglia. After relevant treatment was conducted, the patient's hemichorea symptoms were significantly improved. Imaging features of nonketotic hyperglycemia Hemichorea plays a very important role in diagnosing and treatment of this disease. Introduction: Hemichorea refers to the involuntary movements of one or more limbs of the same side. These movements are continuous and irregular, variable amplitude, and usually involve the arms and legs together. Stroke is the most common reason, hyperglycemia is relatively rare. Characteristic imaging findings can be very suggestive of diagnosis, and also closely related to the development of disease. Here we present a case of hemichorea induced by nonketotic hyperglycemia which was confirmed by bilateral abnormal signals of basal ganglia in MRI.

A Case Report of Nonketotic Hyperglycemic Seizures: A Diagnostic Dilemma.

Nonketotic hyperglycemia (NKH) is a rare but serious complication of uncontrolled diabetes mellitus that occurs acutely with a mortality rate of more than 50%. This condition presents with a clinical syndrome consisting of profound hyperglycemia, hyperosmolality, and dehydration. Infrequently, the patients also present with seizure activity. The most common types of seizures observed in this condition are focal seizures, as opposed to the generalized seizures observed in hypoglycemia-induced seizures. Though various hypotheses tried to explain NKH-induced seizure activity, the actual mechanism remains unknown. The treatment modalities include the management of hyperglycemia and circulatory collapse. However, the role of anti-epileptics is controversial. We herein illustrate an atypical case of focal faciobrachial seizures in a young female patient, which occurred as a rare complication of NKH. A 21-year-old female was admitted with multiple jerking and spasmodic movements of the right upper limb and face, with no significant neurological findings. Past medical history was significant for uncontrolled type 2 diabetes mellitus and multiple episodes of focal seizures. On laboratory examination, serum osmolarity was 309 mOsm/L, blood glucose was 364 mg/dL, HbA1c was 12.1%, and ketone bodies were absent. MRI brain showed large subtle T2 FLAIR (T2-weighted fluid-attenuated inversion recovery) cortical hyperintensities in the left frontal, temporal, parietal, and occipital regions with subcortical hypointense areas. The EEG illustrated a background slowing and generalized spikes, polyspikes, and sharp-wave discharges with post-ictal slowing. The patient's seizures were initially refractory to insulin therapy and resolved with the use of dual anti-epileptics. Thus, to conclude, our case represents a diagnostic dilemma with MRI findings pointing towards NKH as the underlying etiology of focal seizures, with the resolution of seizures only occurring with the addition of anti-epileptics to insulin therapy.

Non-Ketotic Hyperglycemia Causing a Transient Unilateral Homonymous Hemianopia: A Manifestation of Occipital Lobe Seizure.

Focal seizures related to non-ketotic hyperglycemia (NKH) are rare in clinical practice. Plasma glucose levels are usually above 16.6 mmol/L and with normal or slightly elevated serum osmolality. The occurrence of focal seizures may be augmented by the absence of ketoacidosis. Electroencephalogram (EEG) during seizures usually confirms the diagnosis, however, the absence of epileptiform discharges does not rule out seizures. A non-ketotic hyperglycemia-associated occipital lobe seizure can manifest itself as color flashes, blurry vision with periodic confusion, and usually resolves with insulin treatment and rehydration. We are reporting a 65-year-old male patient who presented with intermittent confusion and left-sided visual disturbances, found to have a blood glucose of 33.7 mmol/L with a normal anion gap of 10 and calculated serum osmolality of 303 mOsm/L. The patient's visual disturbances responded very well to rehydration and insulin treatment.

Reversible Homonymous Inferior Quadrantanopia in a Nonketotic Hyperglycemic Patient.

Homonymous hemianopia is frequently associated with retrochiasmal lesions. Vascular etiology is the most common and usually evident on magnetic resonance imaging. When the results of neuroimaging are normal, there are other etiologies that we should consider, like nonketotic hyperglycemia (NKH). We report a 62-year-old female diabetic patient with headache, colour vision and sudden homonymous inferior quadrantanopia and elevated blood sugar levels with normal pH. The neuroimaging was normal and the visual lost improved after the correction of the hyperglycemia. NKH should be considered in patients with sudden and transient hemianopia and normal neuroimaging.

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.

Unusual Magnetic Resonance Imaging Abnormality in Nonketotic Hyperglycemia - related Epilepsia Partialis Continua.

Epilepsia partialis continua (EPC) is a rare epileptic syndrome, presenting as continuous focal motor seizures for a period of minutes, hours, or days. EPC may develop in patients with cerebral cortical lesions and occasionally may develop in patients with metabolic disorders, such as nonketotic hyperglycemia (NKH). Here, we report a case of EPC following NKH, showing an unusual magnetic resonance imaging (MRI) finding of concurrent hypointensity on susceptibility-weighted image (SWI) and T2-weighted image (T2WI) with leptomeningeal and cortical enhancement, which have never been reported. A 68-year-old woman presented to our emergency department with a 3-day history of involuntary repeated contraction of the right side of the face and upper limb. Laboratory data revealed NKH of diabetes mellitus. Electroencephalography (EEG) was unremarkable. Brain MRI revealed focal cortical and leptomeningeal enhancement together with subcortical T2 shortening and SWI hypointensity of the left frontal operculum. She responded well for hyperglycemia and antiepileptic drug therapy. Follow-up brain MRI performed 1 week later showed complete resolution of the abnormal signal and enhancement in the same region. Although EPC caused by NKH occurs rarely, it may result in an MRI abnormality of subcortical hypointensity on SWI and T2WI with leptomeningeal and cortical enhancement, which may be misinterpreted as other brain pathologies. Rapidly recognition is important because timely treatment with hydration and correction of hyperglycemia can lead to better outcome. We recommend such cases of metabolic disorder (such as hyperglycemia) for early consideration, particularly in the elderly.

Hemichorea caused by nonketotic hyperosmolar state: A case report and review of the literature.

Our report involves a case of hemichorea caused by the nonketotic hyperosmolar state. We have analyzed the clinical data and relevant features of a patient who presented herself to the Affiliated Hospital of Xuzhou Medical University. The patient had unilateral involuntary movements for 1 month. We discovered that her blood glucose levels were very high. The patient underwent computed tomography (CT), magnetic resonance imaging (MRI), and magnetic resonance angiography (MRA), indicating right basal ganglia lesion. Control of the patient's blood glucose plus supportive treatment resulted in a significant improvement of her clinical state.

T2*-based MR imaging of hyperglycemia-induced hemichorea-hemiballism.

INTRODUCTION: Hyperglycemia can induce hemichorea-hemiballism, especially in elderly type II diabetics. CT and MRI findings include hyperdensity and T1-shortening in the contralateral lentiform nucleus, respectively. This study explores the associated imaging findings on T2*-based sequences. METHODS: Six patients with clinically documented hyperglycemia-induced hemichorea-hemiballism who had undergone MR imaging with a T2*-based sequence (T2* gradient echo or susceptibility-weighted imaging) were included in this retrospective case series. RESULTS: All six patients demonstrated T1-shortening contralateral to their hemichorea-hemiballism. T2*-based sequences demonstrated unilateral hypointense signal within the striatum in four patients. One patient had mild bilateral striatal hyperintensities, while another did not show significant signal changes. CONCLUSION: It is important for the radiologist to be aware of the signal changes that can be seen on T2*-based sequences in hyperglycemia-induced hemochorea-hemiballism.

Irreversible Hemichorea-Hemiballism in a Case of Nonketotic Hyperglycemia Presenting as the Initial Manifestation of Diabetes Mellitus.

BACKGROUND: Hemichorea-hemiballism (HCHB) is a hyperkinetic movement disorder with features of both chorea and ballism occurring on the same side. CASE REPORT: We present a case of HCHB due to nonketotic hyperglycemia (NKH) that was the initial presentation of diabetes and was irreversible clinically even after 6 months of optimal blood sugar control. DISCUSSION: Although HCHB due to hyperglycemia is a potentially reversible condition in the majority of patients, prolonged uncontrolled hyperglycemia may cause ischemic insult and persistent symptoms. Hyperglycemia should always be kept in the list of differentials while dealing with patients who are newly diagnosed with HCHB.

Occipital seizures and persistent homonymous hemianopia with T2 hypointensity on MRI in nonketotic hyperglycemia.

INTRODUCTION: Nonketotic hyperglycemia (NKH) is known to cause focal motor or secondarily generalized seizures. Occipital seizures in NKH are seldom reported, especially with visual hallucinations and persistent homonymous hemianopia (HH) with characteristic radiological and electroencephalographic (EEG) findings. SUMMARY: Our patient was a middle-aged man who presented with a new onset, single episode of generalized tonic-clonic seizure and NKH. He complained of seeing intermittent colorful stripes in his right visual field. Examination revealed persistent complete right HH and he was observed to have complex partial seizures. Magnetic resonance imaging (MRI) showed subcortical T2 hypointensity within the left occipital lobe in T2W and FLAIR images. The EEG showed electrographic seizures originating from the left occipital region. Random blood glucose at presentation was 581 mg/dl with HbA1c of 11.4%. The seizure and visual field deficits were successfully terminated by the introduction of antiseizure medication and glycemic control. CONCLUSION: Occipital seizures with visual field deficits can occur in hyperglycemic states. These can be associated with specific MRI brain and EEG changes. The HH is reversible with apt treatment primarily including glycemic control with or without antiseizure medication.

Nonketotic Hyperglycemia Presenting as Monoballism.

BACKGROUND: Monoballismus is rarely seen clinically, but when observed, it is usually a manifestation of an acute cerebrovascular accident (CVA). We report a case of monoballismus observed in a patient without evidence of a CVA. CASE REPORT: We observed a case of monoballismus in a 60-year-old diabetic patient who had not had a stroke. The movement disorder resolved with improvement of the patient's hyperglycemia. Nonketotic hyperglycemia is an uncommon cause of ballismus. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Emergency physicians should include the movement disorder of ballismus among the potential clues that a patient may be suffering an acute CVA. However, noncerebrovascular causes of ballismus exist. The movements manifest by a patient with ballismus should also lead the physician to consider the possibility not only of a CVA, but also neuroleptic malignant and serotonin syndromes.

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.