Hyperkinetic and Hypokinetic Movement Disorders in SSPE: A Systematic Review of Case Reports and Case Series.

Background: Subacute Sclerosing Panencephalitis (SSPE) typically presents with periodic myoclonus; however, a spectrum of movement disorders including dystonia, chorea, tremor, and parkinsonism have also been described. This review aims to evaluate the array of movement disorders in SSPE, correlating them with neuroimaging findings, disease stages, and patient outcomes. Methods: A comprehensive review of published case reports and case series was conducted on patients with SSPE exhibiting movement disorders other than periodic myoclonus. PRISMA guidelines were followed, and the protocol was registered with PROSPERO (2023 CRD42023434650). A comprehensive search of multiple databases yielded 37 reports detailing 39 patients. Dyken's criteria were used for SSPE diagnosis, and the International Movement Disorders Society definitions were applied to categorize movement disorders. Results: The majority of patients were male, with an average age of 13.8 years. Approximately, 80% lacked a reliable vaccination history, and 39% had prior measles infections. Dystonia was the most common movement disorder (49%), followed by parkinsonism and choreoathetosis. Rapid disease progression was noted in 64% of cases, with a disease duration of ≤6 months in 72%. Neuroimaging showed T2/FLAIR MR hyperintensities, primarily periventricular, with 26% affecting the basal ganglia/thalamus. Brain biopsies revealed inflammatory and neurodegenerative changes. Over half of the patients (56%) reached an akinetic mute state or died. Conclusion: SSPE is associated with diverse movement disorders, predominantly hyperkinetic. The prevalence of dystonia suggests basal ganglia dysfunction.

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.

A case of hemichorea associated with nonketotic hyperglycaemia: A new magnetic resonance spectroscopy (MRS) finding and possible future implications.

BACKGROUND: Diabetic Striatopathy (DS) is a rare complication of a poor-controlled Diabetes Mellitus consisting of sudden onset of movement disorders. To date, there is still poor knowledge about the pathogenesis. CASE: We describe a 79 year old men affected by sudden onset hemichoreic movements whose cause was a non-ketotic hyperglycaemia diagnosed despite the normal blood glucose levels thanks to brain CT and magnetic resonance imaging. Then, we introduce a new magnetic resonance spectroscopy (MRS) finding never described until today which allowed us to produce a new pathogenetic theory of a phenomenon still without definitive explanations. LITERATURE REVIEW: We performed a review of DS cases using the Medline database and we extracted main data regarding imaging findings. CONCLUSIONS: Thanks to our MRS we show new imaging findings never described until today, with a new pathogenetic explanation, since all the causative hypotheses produced during the past years have never found evidence.

Striatal Hyperperfusion Observed in Dual-Phase 18F-FP-CIT PET Imaging of Hyperglycemic Chorea.

ABSTRACT: A 76-year-old woman with a history of diabetes mellitus presented with right-side dominant generalized chorea. At presentation, her blood glucose level was 500 mg/dL with an HbA1C of 11%. Because the patient had been on levodopa treatment from her primary physician, a dual-phase 18F-FP-CIT PET scan was performed. The early-phase images showed increased perfusion in the bilateral striatum, and the delayed-phase images revealed decreased uptake in the left caudate. Hyperperfusion in the striatum may indicate the acute phase of hyperglycemic chorea. This image illustrates the advantage of adding early-phase scans in 18F-FP-CIT PET in differentiating various hyperkinetic and hypokinetic disorders.

Deep Brain Stimulation for the Treatment of Hemichorea: Case Series and Literature Review.

Background: Hemichorea (HC) and its severe form hemiballismus (HB) are rare movement disorders which can be medically refractory to treatments and may need surgical intervention. Case Report: We report 3 patients with HC-HB who had meaningful clinical improvement with unilateral deep brain stimulation (DBS) of the globus pallidus interna (GPi). We identified 8 prior cases of HC-HB treated with GPi-DBS, and a majority of these patients experienced significant improvement in their symptoms. Discussion: GPi-DBS can be considered in medically refractory HC-HB in carefully selected patients. However, data is limited to small case series and further studies are needed.

Investigation of the factors associated with hemichorea/hemiballismus in post-stroke patients.

Classical knowledge highlights the role of lesions of the subthalamic nuclei (STN) in the pathophysiology of hemichorea/hemiballismus (HH). However, the published reports indicate various other lesion regions in the majority of post-stroke cases with HH. Ergo, we aimed to investigate the significance of the lesion site and clinical features for developing HH in post-stroke patients. Overall, we retrospectively scanned all the patients with stroke who were hospitalized between 01/06/2022 and 31/07/2022 in our neurology clinic. The data regarding the demographic features, comorbidities, stroke etiologies, and laboratory findings, including serum glucose and HBA1C were retrospectively recruited using the electronic-based medical record system. The cranial magnetic resonance imaging (MRI) and computed tomography images have been systematically evaluated for the presence of lesions in localizations that are previously associated with HH. We conducted comparative analyses between patients with and without HH to reveal the discrepancies between groups. The logistic regression analyses were also performed to reveal the predictive values of some features. Overall, the data of 124 post-stroke patients were analyzed. The mean age was 67.9 ± 12.4 years (F/M = 57/67). Six patients were determined to develop HH. The comparative analyses between patients with and without HH revealed that the mean age tended to be higher in the HH group (p = 0.08) and caudate nucleus involvement was more common in the HH group (p = 0.005). Besides cortical involvement was absent in all subjects developing HH. The logistic regression model revealed the presence of a caudate lesion and advanced age as factors associated with HH. We found that the caudate lesion was a crucial determinant of the occurrence of HH in post-stroke patients. With the significance of the other factors of increased age and cortical sparring, we observed differences in the HH group may be investigated also in future-related studies on larger groups.

Motor band sign in a Huntington disease phenocopy.

We report a 68-year-old lady who presented with Huntington phenocopy with generalized chorea and was genetically proven to have Spinocerebellar ataxia (SCA)17. MRI Brain demonstrated motor band sign, which is most commonly reported in motor neuron disease. This is the first case of motor band sign with SCA 17 and highlights the widening spectrum of radiological signs in SCA 17.

Hemiballism and chorea with acute/subacute onset: a retrospective series.

INTRODUCTION: Chorea is a hyperkinetic movement disorder with sudden, irregular, random, dance-like involuntary movements, and ballism is usually one-sided, high-amplitude movements at the proximal of the extremities. In the etiology of acute chorea/hemiballismus, it is necessary to distinguish drugs first and then focus on metabolic causes. The most important etiological causes that may provoke acute/subacute onset chorea/hemiballismus are hypo-hyperglycemia and electrolyte disorders. In this study, we aim to present 19 patients who were admitted to our clinic with movement disorder with acute/subacute onset and diagnosed with chorea/hemiballismus. METHODS: The study was completed with 19 patients. Routine biochemistry, HbA1c level, hemogram, sedimentation, CRP, hepatitis panels, detailed infective parameters, HIV, vitamin B12 level, folate levels, and thyroid function tests were studied. All patients underwent neuro-imaging. RESULTS: 16(84.2%) were female and 3(15.8%) were male. The lowest age of the patients was 48 years, the highest age was 89 years, and the mean age was 72.21 years. Thirteen (68.42%) patients had a diagnosis of diabetes mellitus in their history. The blood glucose levels of these patients at the time of admission: the lowest was 99 mg/dl and the highest was 1200 mg/dl. HbA1c values of 11(84.61%) of the 13 patients were also found elevated. Thirteen (68.4%) patients had hemiballismus, 4(21.1%) patients had bilateral choreoathetosis in the four extremities, and 2(10.2%) patients had ballism limited to one upper extremity. CONCLUSIONS: Chorea/hemiballismus is a movement disorder that is rare and can occur due to a wide range of etiologies. The most common metabolic cause is NKHHS.

[18F]FDG PET in conditions associated with hyperkinetic movement disorders and ataxia: a systematic review.

PURPOSE: To give a comprehensive literature overview of alterations in regional cerebral glucose metabolism, measured using [18F]FDG PET, in conditions associated with hyperkinetic movement disorders and ataxia. In addition, correlations between glucose metabolism and clinical variables as well as the effect of treatment on glucose metabolism are discussed. METHODS: A systematic literature search was performed according to PRISMA guidelines. Studies concerning tremors, tics, dystonia, ataxia, chorea, myoclonus, functional movement disorders, or mixed movement disorders due to autoimmune or metabolic aetiologies were eligible for inclusion. A PubMed search was performed up to November 2021. RESULTS: Of 1240 studies retrieved in the original search, 104 articles were included. Most articles concerned patients with chorea (n = 27), followed by ataxia (n = 25), dystonia (n = 20), tremor (n = 8), metabolic disease (n = 7), myoclonus (n = 6), tics (n = 6), and autoimmune disorders (n = 5). No papers on functional movement disorders were included. Altered glucose metabolism was detected in various brain regions in all movement disorders, with dystonia-related hypermetabolism of the lentiform nuclei and both hyper- and hypometabolism of the cerebellum; pronounced cerebellar hypometabolism in ataxia; and striatal hypometabolism in chorea (dominated by Huntington disease). Correlations between clinical characteristics and glucose metabolism were often described. [18F]FDG PET-showed normalization of metabolic alterations after treatment in tremors, ataxia, and chorea. CONCLUSION: In all conditions with hyperkinetic movement disorders, hypo- or hypermetabolism was found in multiple, partly overlapping brain regions, and clinical characteristics often correlated with glucose metabolism. For some movement disorders, [18F]FDG PET metabolic changes reflected the effect of treatment.

Stroke-like presentation of autoimmune chorea with positive anti-Yo and anti-MOG antibodies: a case report.

With the in-depth study of autoimmune encephalitis, more and more antibody combinations and clinical manifestations appear in our sights, enriching the spectrum of autoimmune encephalitis. Here, we report a case of a 58-year-old male patient with sudden involuntary movement of the left limb. The brain MRI was normal. CSF analysis showed slightly elevated protein (548.38 mg/L) and normal cell count(1.00 10^6/L). No tumors were detected by the whole-body PET-CT. Positive anti-Yo and anti-MOG antibodies were found in the blood. So we considered the diagnosis of autoimmune chorea with positive anti-Yo and anti-MOG antibodies, after immunoglobulin shock and methylprednisolone shock therapy were used, the patient's involuntary movement gradually disappeared. This is the first case of autoimmune encephalitis with both anti-Yo and anti-MOG antibodies, and stroke-like chorea is also rare. This case enriches the clinical presentation of double antibody-associated encephalitis.

Clinical Reasoning: A 37-Year-Old Man Presenting With Intermittent Dizziness and Brief Choreiform Movements.

In this report, we describe the case of a 37-year-old man presenting with intermittent episodes of dizziness and choreiform movements. MRI of the head showed an acute infarction in the left cerebellar hemisphere and multiple chronic infarctions in the cerebellum and pons. Vessel imaging also showed dynamic stenoses in the posterior circulation. This case provides a framework for evaluating young patients with intermittent dizziness and recurrent strokes and highlights the need to combine anatomical angiographies or hemodynamic studies with head rotational behavior.

Antiphospholipid-related chorea.

Chorea can be associated with autoimmune diseases such as antiphospholipid syndrome and has been associated with the isolated presence of antiphospholipid antibodies (aPL). Chorea is a rare neurological manifestation of antiphospholipid syndrome. The pathophysiological mechanisms underlying aPL-related chorea are still debated. One postulated mechanism is aPL or other autoantibody binding to brain-blood vessel endothelium, resulting in endothelial dysfunction secondary to a proinflammatory cascade, with sequalae of inflammation and local microthrombosis. Another postulated mechanism considers immune-mediated attack (aPL or antibasal ganglia antibodies) against specific basal ganglia epitopes. Here, we report a patient with isolated aPL-related chorea that followed a relapsing-remitting course. We highlight the role of brain metabolic imaging with fluorodeoxy glucose positron-emission tomography in the diagnostic workup of chorea and the challenges in the practical management of aPL-related chorea with symptomatic treatments.

Clinical and imaging associations for non-ketotic hyperglycemic chorea: a case-control study.

Background: The non-ketotic hyperglycemic chorea (NKHC) was a rare complication for patients with diabetes mellitus, but not been well studied. In the present research, we aimed to investigate the clinical and imaging characteristics of NKHC and explore the potential association. Methods: We performed a case-control study with patients diagnosed as NKHC. The patients with group of NKHC were retrospectively recruited, while the matched group were set to screened patients with diabetes mellitus but no NKHC at a 1:3 ratio. The clinical and imaging data were collected for all the participants of the two groups. Firstly, Correlation analysis was conducted to test the difference of all the variables between the NKHC group and matched group. Then, the putative associated factors for NKHC were further identified. Results: Eleven men and 9 women with NKHC and 60 matched participants were analyzed. The mean age of the NKHC group was 68.5 ± 14.9 years. Participants with NKHC were more likely to have a higher glycosylated hemoglobin (HbA1c) level (13 ± 2.82 vs. 10.57 ± 2.71, P<0.001), and a higher frequency of renal dysfunction (estimated glomerular filtration rates <60 ml/min/1.73m2) (55% vs. 20%, P=0.005). Logistic regression analyses showed that both higher HbA1c and renal dysfunction were significantly correlated with NKHC. Conclusion: A higher value of HbA1c and renal dysfunction may be associated with the occurrence of NKHC.

Treatment-resistant diabetic chorea manifesting with psychiatric symptoms: a case report.

We report a case of a 69-year-old man with treatment-resistant diabetic chorea presenting psychiatric symptoms. The right chorea lasted for 3 months and was refractory to control of diabetes mellitus or administration of haloperidol and benzodiazepines. Only administration of tiapride was efficacious. Magnetic resonance spectrometry and dopamine transporter-single photon emission computed tomography suggested that sustained ischemia at the striatum may lead to impaired expression of dopamine transporters, thereby resulting in deterioration in the indirect pathway. Tiapride inhibited dopamine D2 receptors, thereby restoring the function of the indirect pathway and resulting in improvement of diabetic chorea.