Diabetic Striatopathy: A New Challenge in Type 1 Pediatric Diabetic Patients

Rai, Seema; Kaul, Varun; Singh, Sulena; Kaur, Simranpreet; Thenmurugan, Palani

doi:10.5001/omj.2021.47

Cited by 7 publications

(5 citation statements)

References 10 publications

(12 reference statements)

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“…In most cases, HCHB resolves fully, although recurrence was reported in 14% of patients [ 72 ]. While elderly patients with type 2 DM are most predisposed, there are recent reports of this syndrome also occurring in pediatric patients with type 1 DM [ 73 ]. Females have a higher predilection than males at a ratio of 1.7:1 [ 72 ].…”

Section: Resultsmentioning

confidence: 99%

“…72 While elderly patients with type 2 DM are most predisposed, there are recent reports of this syndrome also occurring in pediatric patients with type 1 DM. 73 Females have a higher predilection than males at a ratio of 1.7:1. 72 Apart from being more frequent among Asians, there are no differential clinical features that distinguish HCHB among Asians compared to non-Asian ethnic groups.…”

Section: Hemichorea-hemiballism Associated With Hyperglycemiamentioning

confidence: 99%

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Historical and More Common Nongenetic Movement Disorders From Asia

Ibrahim¹,

Jagota²,

Pal³

et al. 2023

JMD

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Nongenetic movement disorders are common throughout the world. The movement disorders encountered may vary depending on the prevalence of certain disorders across various geographical regions. In this paper, we review historical and more common nongenetic movement disorders in Asia. The underlying causes of these movement disorders are diverse and include, among others, nutritional deficiencies, toxic and metabolic causes, and cultural Latah syndrome, contributed by geographical, economic, and cultural differences across Asia. The industrial revolution in Japan and Korea has led to diseases related to environmental toxin poisoning, such as Minamata disease and β-fluoroethyl acetate-associated cerebellar degeneration, respectively, while religious dietary restriction in the Indian subcontinent has led to infantile tremor syndrome related to vitamin B12 deficiency. In this review, we identify the salient features and key contributing factors in the development of these disorders.

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Section: Resultsmentioning

confidence: 99%

Section: Hemichorea-hemiballism Associated With Hyperglycemiamentioning

confidence: 99%

Historical and More Common Nongenetic Movement Disorders From Asia

Ibrahim¹,

Jagota²,

Pal³

et al. 2023

JMD

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“…This implies that DS may be among the first presentations of DM [ 9 ]. DS may also, but rarely, be seen in pediatric patients with type 1 diabetes [ 21 ].…”

Section: Discussionmentioning

confidence: 99%

“…Although a number of mechanisms can lead to striatal hyperintensity in T1-weighted MRI—including hypertensive hemorrhages, calcification, genetic diseases (e.g., Tay–Sachs’s disease, tuberous sclerosis, neurofibromatosis, and Fahr’s disease), metabolic disorders (e.g., Wilson’s disease, hypoglycemic comas, and chronic hepatic encephalopathy), toxicity (e.g., manganese toxicity and carbon monoxide poisoning), and brain ischemia (e.g., lenticulostriate infarctions, post-cardiac arrest encephalopathy)—the striatal lesions in DS are mostly bilateral [ 21 ]. One of the distinctive features of a DS-associated striatal anomaly to differentiate it from a hypertensive hemorrhage is the absence of a mass effect and the sparing of the internal capsule [ 37 ], as shown here.…”

Section: Discussionmentioning

confidence: 99%

Diabetic Striatopathy: Parenchymal Transcranial Sonography as a Supplement to Diagnosis at the Emergency Department

Godani¹,

Lanza

2022

Diagnostics

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Background: Diabetic striatopathy (DS) is a rare condition with a debated pathophysiology; a local metabolic dysfunction is the most likely hypothesis. We present a case of DS mimicking an acute stroke, outline a few uncommon/atypical features, and report for the first time the parenchymal transcranial sonography (pTCS) findings. Case Report: An 86-year-old man, treated for insulin-dependent diabetes, presented at an emergency department because of the occurrence of isolated choreo-athetotic movements in his left limbs with fluctuations in the location, frequency, and duration. The blood glucose level was 569 mg/dL. Both urgent and follow-up brain computed tomography (CT) were negative for recent lesions whereas pTCS revealed hyperechogenicity in the right lenticular nucleus. Subsequent magnetic resonance imaging (MRI) showed T1-weighted hyperintensity in the right putamen with negative diffusion-weighted imaging. The symptoms were responsive to glucose control and haloperidol administration, although they persisted during sleep. Conclusions: Unlike previously described cases characterized by hemichorea and/or hemiballism, our patient presented with a stroke-like onset of unilateral irregular choreo-athetotic movements. Notably, based on CT alone, it would not have been possible to distinguish DS from a stroke. In this scenario, the pTCS hyperechogenicity of the right lenticular nucleus helped to hypothesize a metabolic disorder, which was subsequently confirmed by MRI.

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“…Changes in Gamma Amino Butyric Acid (GABA) or dopamine receptors in post-menopausal women following estrogenic changes have also been considered [ 22 ]. Hemichorea does not only affect elderly type 2 diabetics with non-ketotic hyperglycemia with an average onset age of 67.6 years old [ 2 ] but several cases are reported during type 1 DM in the pediatric population [ 26 – 31 ].…”

Section: Diabetic Striatopathy: Rare But Undervalued Diseasementioning

confidence: 99%

Diabetic striatopathy: an updated overview of current knowledge and future perspectives

Arecco

Ottaviani

Boschetti

et al. 2023

J Endocrinol Invest

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Purpose Diabetic striatopathy (DS) is a rare complication of poorly controlled diabetes mellitus (DM), characterized by hyperglycemia associated with chorea/ballism and characteristic reversible basal ganglia abnormalities on computed tomography (CT) and/or magnetic resonance imaging (MRI). We propose a narrative review of the literature on this topic, currently unknown to most, and about which physicians should be aware. We intend to summarize, critically review, and take to mean the evidence on this disorder, describing its typical features. Methods We searched Pubmed for English-language sources using the following keywords in the title and the abstract: diabetic striatopathy, hyperglycemic non-ketotic hemichorea/hemiballism, chorea/hemichorea associated with non-ketotic hyperglycemia, diabetic hemiballism/hemichorea, chorea, hyperglycemia, and basal ganglia syndrome. We collected scientific articles, including case reports, reviews, systematic reviews, and meta-analyses from the years 1975 to 2023. We eliminated duplicate, non-English language or non-related articles. Results Older Asian women are more frequently affected. Suddenly or insidiously hemichorea/hemiballism, mainly in the limbs, and high blood glucose with elevated HbA1c in the absence of ketone bodies have been observed. Furthermore, CT striatal hyperdensity and T1-weighted MRI hyperintensity have been observed. DS is often a treatable disease following proper hydration and insulin administration. Histopathological findings are variable, and no comprehensive hypothesis explains the atypical cases reported. Conclusion DS is a rare neurological manifestation of DM. If adequately treated, although treatment guidelines are lacking, the prognosis is good and life-threatening complications may occur occasionally. During chorea/hemiballism, we recommend blood glucose and HbA1c evaluation. Further studies are needed to understand the pathogenesis.

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Diabetic Striatopathy: A New Challenge in Type 1 Pediatric Diabetic Patients

Cited by 7 publications

References 10 publications

Historical and More Common Nongenetic Movement Disorders From Asia

Historical and More Common Nongenetic Movement Disorders From Asia

Diabetic Striatopathy: Parenchymal Transcranial Sonography as a Supplement to Diagnosis at the Emergency Department

Diabetic striatopathy: an updated overview of current knowledge and future perspectives

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