Pediatric Hemichorea-Hemiballismus Associated with Nonketotic Hyperglycemia: A Case Report and Review of the Literature.
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SUBMITTER: Kumar N
PROVIDER: S-EPMC8490195 | biostudies-literature |
REPOSITORIES: biostudies-literature
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Project description:Objective: To explore the clinical manifestation, diagnosis, therapy, and mechanism of hemichorea associated with non-ketotic hyperglycemia (HC-NH) so as to enhance awareness and avoid misdiagnosis or missed diagnosis of the disease. Methods: A case of HC-NH was reported and reviewed in terms of the clinical features, diagnosis and treatment. Results: Hemichorea associated with non-ketotic hyperglycemia is a rare complication of diabetes mellitus, which is commonly seen in elderly women with poorly-controlled diabetes. The condition is characterized by non-ketotic hyperglycemia, unilateral involuntary choreiform movements, and contralateral basal ganglia hyper-intensity by T1-weighted MR imaging or high density on CT scans. Blood glucose control is the basal treatment, in combination with dopamine receptor antagonists and benzodiazepine sedative, in controlling hemichorea. Conclusion: In clinical practice, the possibility of unilateral chorea should be considered for diabetic patients with poor blood glucose control.
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Project description:Background and purposeNonketotic hyperglycemia often causes transient visual field defects, but only scattered anecdotes are available in the literature.MethodsWe report a patient with homonymous superior quadrantanopsia due to nonketotic hyperglycemia and provide a systematic literature review of the clinical features of 40 previously reported patients (41 in total, including our case) with homonymous visual field defects in association with nonketotic hyperglycemia.ResultsThe typical visual field defect was congruous (84.6%), homonymous hemianopsia (87.8%) with macular splitting (61.5%) or sparing (38.5%). It was transient and repetitive in 54.5% of the patients, but it developed as a persistent form in the remainder. Positive visual symptoms such as hallucinations and phosphenes developed in 73.2% of patients. Brain MRI revealed corresponding abnormalities in most patients (84.8%), characterized by a low-intensity white-matter signal or a high-intensity gray-matter signal on T2-weighted or fluid-attenuated inversion recovery images with diffusion restriction or gadolinium enhancement. Most (97.0%) patients recovered completely, with 48.5% treated by glycemic control alone and the remainder also receiving antiepileptic agents.ConclusionsNonketotic hyperglycemia should be considered a possible cause of transient visual field defects, especially when it is associated with repetitive positive visual symptoms and typical MRI findings in hyperglycemic patients.
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Project description:IntroductionHemichorea-hemiballismus, which spans a spectrum of involuntary, continuous, nonpatterned movement involving one side of the body, can emerge as the initial manifestation of acute ischemic stroke. However, because of its rarity in the community, the diagnosis and treatment are often delayed.Patient concernsWe report a unique case of a 47-year-old female who presented with acute onset hemichorea-hemiballismus. No obvious focal sign apart from involuntary, continuous, nonpatterned movement of her left arm and leg was presented.DiagnosisInitial diffusion-weighted magnetic resonance imaging (MRI) was negative but significant increase of blood flow velocity in the right middle cerebral artery (MCA) stem was revealed by transcranial doppler sonography. Repeated MRI showed acute infarction in the contralateral globus pallidus. Isolated dissection of the right MCA typified by intimal flap with double lumen was identified by digital subtraction angiography and high-resolution magnetic resonance imaging (HR-MRI).InterventionsThe patient was initially treated with dual antiplatelet agents but the uncontrollable movement deteriorated during hospitalization. Antithrombotic therapy was then intensified with combination of tirofiban and low-molecular-weight heparin. Other symptomatic treatment included volume expansion with colloidal fluid to improve cerebral perfusion. Her involuntary movement gradually diminished and the patient was discharged with rivaroxaban 15 mg/daily.OutcomesThe patient had recovered with significant reduction in her hemichorea-hemiballismus. Three-month follow-up HR-MRI showed complete resolution of the MCA dissection lesions.ConclusionPrompt recognition of acute onset hemichorea-hemiballismus as the manifestation of acute ischemic stroke in appropriate clinical setting may reduce diagnostic delay. Multiple imaging techniques including cerebral digital subtraction angiography and HR-MRI can be applied to diagnosis and further clarify the mechanism of stroke, which facilitate in selection of secondary prevention therapies.
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Project description:ObjectiveTo determine whether neuroanatomically heterogeneous strokes causing hemichorea-hemiballismus localize to a common functional network.MethodsWe identified 29 cases of lesion-induced hemichorea-hemiballismus from the literature and mapped each lesion volume onto a reference brain. Using a recently validated technique termed lesion network mapping, we tested whether these lesions belonged to the same functional network. To accomplish this, the network of brain regions functionally connected to each lesion was identified using a connectome dataset from healthy participants. Network maps were overlapped to identify any region functionally connected to our set of lesions. Specificity was evaluated using a case-control design; control cohorts included a group of similar lesions randomized to different brain locations and a second group of lesions causing a separate movement disorder, asterixis. Reproducibility was evaluated using an independent cohort of 10 additional hemichorea-hemiballismus cases.ResultsLesions showed heterogeneity in anatomical location, consistent with prior reports. However, at least 90% of these lesions showed network overlap in the posterolateral putamen. This result was specific to lesions causing hemichorea-hemiballismus and reproducible in an independent cohort. The putaminal overlap site was itself connected to a broader motor network that predicted the distribution of lesions causing hemichorea-hemiballismus.ConclusionsStrokes causing hemichorea-hemiballismus, while anatomically heterogeneous, localize to a common functional network. Specifically, lesions occur in regions functionally connected to the posterolateral putamen, a region previously implicated in hyperkinetic movement disorders. Lesion network mapping may be useful in identifying the neuroanatomical substrates of heterogeneous lesion-based disorders.
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Project description:Hemichorea-hemiballism can be the solely presentation of a wide range of non-neurological clinical pictures, such as metabolic or hydro-electrolyte derange-ments. Hemichorea-hemiballism as the first presentation of type 2 diabetes mellitus has been described. The case depicted herein reinforces this association highlighting that especially in elder patients with new-ly diagnosed hemichorea-hemiballism, non-ketotic hyperglycemia should promptly be recognized.
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