Exercise-induced chronic fatigue impairs working memory in rats via basal ganglia output nuclei / 神经解剖学杂志

Objective:To investigate possible neuromodulatory mechanisms involved in the involvement of parvalbu-min(PV)expression in the basal ganglia output nuclei,entopeduncular nucleus(EPN)and substantia nigra pars etic-ulata(SNr),in exercise-induced chronic fatigue impairs working memory capacity.Methods:Male SD rats were divid-ed into control group and Fatigue group by random number method,and a three-stage incremental load treadmill training program was selected to establish a chronic exhaustion exercise-induced fatigue rat model.The working memory ability of rats was assessed by the Y-maze autonomous alternation experiment.Immunohistochemical staining was used to ob-serve the expression of parvalbumin(PV)positive neurons and cysteine aspartate-specific protease-3(caspase-3)in EPN and SNr of rats.Results:The accuracy of voluntary alternation in the fatigue group was obviously lower than that in control group(P＜0.05).The results of immunohistochemical staining showed that the density of PV positive neu-rons and the degree of positive fiber staining in EPN and SNr in the fatigue group were obviously lower than those in the control group(P＜0.05,P＜0.01).The number of caspase-3 positive cells per unit area of EPN and SNr in the fa-tigue group was obviously higher than that in the control group(P＜0.05,P＜0.01).Conclusion:The mechanism of impairing working memory in rats caused by exercise-induced chronic fatigue may be related to the apoptosis of PV posi-tive neurons in EPN and SNr.

Correlation between enlarged perivascular space and cerebral venous reflux in recent small subcortical infarcts within the lenticulostriate artery territory / 中华神经科杂志

Objective:To summarize the incidence of cerebral venous reflux (CVR) in patients with recent small subcortical infarct (RSSI) and explore its correlation with enlarged perivascular spaces (EPVS).Methods:Patients with RSSI in the lenticulostriate artery admitted to the Department of Neurology of the First Affiliated Hospital of Zhengzhou University from January 2019 to December 2022 were included. The baseline demographic data, medical history, and laboratory results of the patients were collected. CVR was assessed by time-of-flight magnetic resonance angiography. Patients were stratified into 2 groups based on the presence (CVR group) or absence of CVR (non-CVR group), and baseline characteristics as well as laboratory test results were compared between the 2 groups. The location and number of EPVS were evaluated using a visual grading scale, with EPVS with higher scores defined as high-grade EPVS (HEPVS). Simultaneous evaluation of cerebral white matter hyperintensities and lacunar infarctions was conducted, followed by intergroup comparisons. The relationship between EPVS and CVR was studied using multiple Logistic regression analysis.Results:A total of 571 patients with RSSI in the lentiform artery area were ultimately included, including 180 females (31.5%). Their age was (59.37±12.87) years. Among them, 73 patients (12.8%) exhibited CVR based on imaging findings, so the incidence of CVR was 12.8%. In comparison between the CVR group ( n=73) and the non-CVR group ( n=498), the proportion of females [21.9% (16/73) vs 32.9% (164/498), χ 2=3.578, P=0.059] was lower and the proportion of history of smoking [38.4% (28/73) vs 27.7% (138/498), χ 2=3.499, P=0.061] was higher in the CVR group, but without statistical significance. Additionally, the history of alcohol consumption [34.2% (25/73) vs 21.7% (108/498), χ 2=5.621, P=0.018] and the proportion of patients with concomitant HEPVS in the basal ganglia area [41.1% (30/73) vs 25.3% (126/498), χ 2=7.999, P=0.005] was higher in the CVR group with statistical significance. Multiple Logistic regression analysis showed that HEPVS in the basal ganglia region remained independently associated with CVR ( OR=1.988, 95% CI 1.190-3.320, P=0.009). Conclusion:EPVS in the basal ganglia region is significantly associated with CVR in the RSSI population, suggesting that venous dysfunction may be closely related to the formation of EPVS.

Chorea Hyperglycemia Basal Ganglia Syndrome: A case report of a rare diabetes complication

Introduction@#We present a patient with long-standing uncontrolled type 2 diabetes mellitus (T2 DM) who developed sudden onset of choreiform movement, which rapidly resolved after insulin therapy and haloperidol.@*Case Description@#A 53-year-old Filipino male, with T2DM and hypertension for more than 10 years, presented with sudden onset of hyperkinetic, involuntary, non-patterned, continuous movements of the left upper and lower extremities. Investigations revealed severe hyperglycemia without acidemia and ketonuria. Cranial computed tomography scan showed hyperdensity on the right caudate and lentiform nuclei. On cranial magnetic resonance imaging, there was T1- weighted hyperintense and T2 - weighted hypointense signal involving the right putamen, globus pallidus and caudate. Cranial magnetic resonance angiography showed stenosis on the cavernous segment of the right internal carotid artery (ICA), left ICA and middle cerebral artery (MCA) junction, the A1 segment of the left anterior communicating artery and proximal P2 segments of the bilateral posterior cerebral arteries. The patient was managed with a basal-bolus insulin regimen to control the blood glucose and haloperidol to manage the extrapyramidal symptoms. Consequently, there was complete resolution of the involuntary movements. @*Conclusion@#This case illustrates the importance of a systematic approach to movement disorders and early recognition of this rare diabetes complication known as chorea hyperglycemia basal ganglia syndrome or diabetic striatopathy.

Enfermedad de Fahr. A propósito de un caso

El síndrome de Fahr, o ferrocalcinosis cerebrovascular, o calcinosis de los núcleos del cerebro, se caracteriza por un depósito anómalo del calcio sin anomalía en el metabolismo del calcio. Se acumula, principalmente, en los ganglios basales, núcleos dentados cerebelosos y sustancia blanca. Es un trastorno neurológico degenerativo asociado a síntomas tales como trastornos del movimiento y síntomas neuropsiquiátricos. Se presenta el caso de un paciente masculino de 65 años de edad, de procedencia rural, viudo, con antecedentes personales de hipertensión arterial, diabetes mellitus tipo 1 y adenoma de próstata, para lo cual lleva tratamiento con Cardicor, Lasix, Plaunac, DuoPlavin y Adenuric. Es llevado a emergencias por la ambulancia presentando convulsiones tónico-clónicas generalizadas sin relajación de esfínter, que respondieron a la administración de diazepam 10 mg endovenoso. Se diagnosticó enfermedad de Fahr. El paciente evolucionó satisfactoriamente y egresó a los cinco días de su ingreso.

Fahr's syndrome, or cerebrovascular ferro-calcinosis, or calcinosis of the nuclei of the brain, is characterized by abnormal calcium deposition without abnormality in calcium metabolism. It accumulates mainly in the basal ganglia, cerebellar dentate nuclei and white matter. It is a degenerative neurological disorder associated with symptoms such as movement disorders and neuropsychiatric symptoms. The case of a 65-year-old male patient, from rural origin, widower, with a personal history of high blood pressure, type 1 diabetes mellitus, and prostate adenoma is presented. For this, he is treated with Cardicor, Lasix, Plaunac, DuoPlavin and Adenuric. He was taken to the emergency room by ambulance presenting generalized tonic-clonic seizures without sphincter relaxation, which responded to the administration of intravenous diazepam 10 mg. Fahr's syndrome was diagnosed, the patient progressed satisfactorily and was discharged 5 days after admission.

Hyperglycemia-induced hemichorea-hemiballismus syndrome - a systematic review

ABSTRACT Nonketotic hyperglycemia may occur as a cause of chorea in patients with chronic decompensated diabetes. Because it is rare and consequently poorly studied, diagnosis and treatment can be delayed. Therefore, our objective was to summarize clinical and radiological features, as well as treatments performed, from previously reported cases to facilitate adequate management in clinical practice. We searched MEDLINE/PubMed, EMBASE, Cochrane, CINAHL, Web of Science, Scopus, and LILACS databases for studies published before April 23, 2021. We included case reports and case series of adults (aged ≥ 18 years) that described hyperglycemic chorea with measurement of glycated hemoglobin (HbA1c) and cranial magnetic resonance imaging (MRI). Studies were excluded if participants were pregnant women, aged < 18 years, and had no description of chorea and/or physical examination. We found 121 studies that met the inclusion criteria, for a total of 214 cases. The majority of the included studies were published in Asia (67.3%). Most patients were women (65.3%) aged > 65 years (67.3%). Almost all patients had decompensated diabetes upon arrival at the emergency department (97.2%). The most common MRI finding was abnormalities of the basal ganglia (89.2%). There was no difference in patient recovery between treatment with insulin alone and in combination with other medications. Although rare, hyperglycemic chorea is a reversible cause of this syndrome; therefore, hyperglycemia should always be considered in these cases.

Impacto de la superación profesional en la meteoro labilidad de la enfermedad cerebrovascular / Climate change and cardiovascular disease: impact of professional development

Introducción: La enfermedad cerebrovascular (ECV) es una urgencia neurológica que en ocasiones se encuentra influenciada por los cambios del tiempo. La ECV isquémica es una emergencia médica con una ventana estrecha para su diagnóstico y tratamiento. El daño generado por esta enfermedad se estima mediante de la mortalidad, discapacidad e impacto social. Objetivo: Proponer un programa de capacitación basado en los efectos fisiopatológicos que provocan en los pacientes, cambios de tiempo y ritmo circadiano en el contexto de la adaptación al cambio climático. Método: Se desarrolló un estudio multietápico prospectivo en el Hospital Universitario Mártires del 9 de Abril del municipio Sagua la Grande entre los años 1993 a 2017. En la primera etapa se seleccionó un universo de 52 profesionales de la salud que atendieron a estos pacientes; en la segunda, 48 y en la tercera, 61. Resultados: El nivel de conocimiento del personal de salud en una primera etapa era inadecuado (78,85 %); en la tercera, este porcentaje disminuyó (14,75 %). Como resultado de la capacitación mejoraron los tiempos de atención a los pacientes con ECV a su llegada al hospital, y en la tercera, más del 50 % de los enfermos fueron atendidos antes de las seis horas de inicio de los síntomas. Conclusiones: La capacitación sobre ECV basada en los efectos fisiopatológicos que provocan en los pacientes el cambio climático y el ritmo circadiano disminuyó el tiempo de espera, y se asoció con una reducción de la morbilidad y la mortalidad, después de aplicar este programa.

Introduction: cerebrovascular disease (CVD) is a neurological emergency that is sometimes influenced by climate changes. Ischemic CVD is a medical emergency with a narrow window for diagnosis and treatment. The damage generated by this disease is estimated through mortality, disability and social impact. Objective: to propose a training program based on the pathophysiological effects in patients caused by changes in time and circadian rhythm in the context of adaptation to climate change. Methods: a multistage prospective study was conducted at "Mártires del 9 de Abril" University Hospital in Sagua la Grande municipality between 1993 and 2017. A universe of health professionals who cared for these patients was selected as follows: 52 in the first stage; 48 in the second stage, and 61 in the third ones. Results: the level of knowledge of health personnel in the first stage was inadequate (78.85%); this percentage decreased in the third stage (14.75%). Care times for CVD patients improved upon arrival at the hospital as a result of the training, as well as in the third ones, more than 50% of the patients were seen within six hours of the onset of symptoms. Conclusions: CVD training based on the pathophysiological effects of climate change and circadian rhythm on patients decreased waiting time, and was associated with reduced morbidity and mortality, after applying this program.

Choreiform movements and basal ganglia calcification as a presentation of Fahr's disease

Basal ganglia calcifications may be a radiological finding in approximately 20% of the general population. When they are associated with neuropsychiatric and motor symptoms in an idiopathic form, they are known as Fahr's disease. They are termed "Fahr's syndrome" when they are secondary to an identifiable and potentially treatable cause. In this report, we present the clinical case of a 69-year-old woman with the onset of subacute chorea, with no other associated symptoms, in whom extensive basal ganglia calcifications were found on neuroimaging, due to which metabolic disorders were subsequently ruled out. The objective is to contribute to the characterization of the potential motor manifestations which would give rise to clinical suspicion. Due to its low incidence and the little information on this condition in the region, we want to encourage documentation of other cases and the process for ruling out other differential diagnoses, in order to obtain more information on its actual epidemiology and signs and symptoms in Colombia. (Acta Med Colomb 2022; 48. DOI:https://doi.org/10.36104/amc.2023.2635).

Lesiones metabólicas bilaterales en núcleos basales: Serie de casos y Revisión de literatura

Introducción: Los núcleos basales son sustancias de materia gris, involucradas en la regulación de distintas funciones metabólicas y son vulnerables a situaciones de hipoxia y distintas situaciones patológicas. Los hallazgos imagenológicos no son específicos en la mayoría de los casos. Reporte de caso: Se reportan los casos de diez pacientes con lesiones metabólicas bilaterales en núcleos basales atendidos en un hospital de referencia nacional de Perú y se realiza una revisión de la literatura: 3 casos de Hipoparatiroidismo postquirúrgico, 2 de encefalopatía de Wernicke, 1 con degeneración hepatolenticular, 1 con mielinólisis extrapontina, 1 Intoxicación por metanol y 2 con encefalopatía Hipóxico-isquémica. Conclusión: Las lesiones bilaterales de núcleos basales producto de lesiones metabólicas son en gran parte inespecíficas, por lo que el contexto clínico es de vital importancia, así como las particularidades de los hallazgos imagenológicos, para el adecuado reconocimiento de las entidades etiológicas y su manejo oportuno.

Introduction: Basal nuclei are gray matter substances, involved in the regulation of different metabolic functions and are vulnerable to situations of hypoxia and different pathological situations. Imaging findings are not specific in most cases. Case of report: The cases of ten patients with bilateral metabolic lesions in the basal nuclei treated at a national reference hospital in Peru are reported, and a review of the literature is carried out: 3 cases of postoperative hypoparathyroidism, 2 of Wernicke's encephalopathy, 1 with hepatolenticular degeneration, 1 with extrapontine myelinolysis, 1 methanol intoxication and 2 with hypoxic-ischemic encephalopathy. Conclusion: Bilateral lesions of the basal nuclei as a result of metabolic lesions are nonspecific, so the clinical context is of vital importance, as well as the particularities of the imaging findings, for the adequate recognition of the etiological entities and their timely management.

Research progress of traditional Chinese medicine treatment of ischemic stroke / 心血管康复医学杂志

Ischemic stroke(IS)is a common cerebrovascular disease.Due to its characteristics of high disability rate,high recurrence rate and high mortality,life health and quality of life of patients are seriously damaged.In recent years,Chinese medicine treatment has been widely used in clinical treatment of IS,and has achieved good result.s The present article makes a review on research progress of traditional Chinese medicine treatment of IS in recent years.

Research progress of traditional Chinese medicine in treatment of vascular cognitive impairment / 心血管康复医学杂志

With aging of Chinese population,prevalence of vascular cognitive impairment(VCI)is increasing year by year.At present,drugs for VCI treatment at home and abroad still lack large samples of evidence-based medicine evidence.Compared with western medicine,Chinese medicine has characteristics of multi-component and multi-target.The present article makes a review on research progress of traditional Chinese medicine in treatment of VCI,aiming at providing new thinking for VCI treatment.

Effect of electroacupuncture at the acupoints for Tiaozang Xingshen on cerebral metabolism in the patients with type 2 diabetes mellitus-associated cognitive dysfunction / 中国针灸

OBJECTIVES@#To investigate the cerebral metabolism in the patients with type 2 diabetes mellitus-associated cognitive dysfunction (T2DACD) and explore the mechanism of electroacupuncture (EA) at the acupoints for Tiaozang Xingshen (adjusting zangfu function and rescuing the spirit) in treatment of T2DACD, using magnetic resonance spectroscopy.@*METHODS@#Fifteen patients with T2DACD (observation group) and 22 healthy subjects (control group) were enrolled. In the observation group, the patients were treated with EA for Tiaozang Xingshen at Baihui (GV 20) and Shenting (GV 24), and bilateral Feishu (BL 13), Pishu (BL 20), Shenshu (BL 23), Zusanli (ST 36), Sanyinjiao (SP 6), Hegu (LI 4) and Taichong (LR 3). EA was operated with disperse-dense wave, 2 Hz/100 Hz in frequency and 0.1 mA to 1.0 mA in current intensity; 30 min each time, once daily. One course of EA consisted of 5 treatments, at the interval of 2 days and the intervention lasted 8 courses. Before treatment in the control group, before and after treatment in the observation group, the score of Montreal cognitive assessment scale (MoCA), the score of clinical dementia rating (CDR), Flanker paradigm, Stroop paradigm, Nback paradigm, the score of self-rating anxiety scale (SAS), the score of self-rating depression scale (SDS), and the score of Hamilton depression rating scale (HAMD) were evaluated separately; the glycolipid metabolic indexes (fasting plasma glucose [FPG], glycosylated hemoglobin type A1c [HbA1c], total cholesterol [TC], triacylglycerol [TG], high-density lipoprotein cholesterol [HDL-C] and low-density lipoprotein cholesterol [LDL-C]) were determined；with the magnetic resonance spectroscopy technique adopted, the metabolites in the basal ganglia area were detected. The correlation analysis was performed for the metabolite values with MoCA score, CDR score , Flanker paradigm, Stroop paradigm, and Nback paradigm.@*RESULTS@#Before treatment, compared with the control group, in the observation group, MoCA score was lower (P<0.001), CDR score and the levels of FPG and HbA1c were higher (P<0.001); the reaction times of Flanker non-conflict, Flanker conflict, Stroop neutrality, Stroop congruence, Stroop conflict, and 1-back were prolonged (P<0.05, P<0.001), and the accuracy of Flanker conflict, Stroop conflict, and 1-back decreased (P<0.05, P<0.01); the ratio of N-acetyl aspartate (NAA) to creatine (Cr) in the left basal ganglia area was dropped (P<0.001), and that of myo-inositol (MI) to Cr in the right side increased (P<0.05). In the observation group after treatment, compared with the levels before treatment, MoCA score was higher (P<0.001), the scores of CDR, SAS and HAMD were reduced (P<0.01, P<0.05), the reaction times of Flanker conflict and Stroop conflict shortened (P<0.001, P<0.05), and the accuracy of Flanker conflict and 1-back increased (P<0.001, P<0.05); the ratio of NAA to Cr in the left basal ganglia area and that of the gamma-aminobutyric acid (GABA) to Cr in the right increased (P<0.05), that of MI to Cr in the right decreased (P<0.05). Before treatment, in the observation group, the ratio of MI to Cr in the right basal ganglia area was positively correlated with the reaction time of Stroop congruence (r=0.671, P=0.012) and this ratio was positively correlated with the reaction time of Stroop conflict (r=0.576, P=0.039).@*CONCLUSIONS@#Electroacupuncture for "adjusting zangfu function and rescuing the mind" improves the cognitive function of T2DACD patients, which may be related to the regulation of NAA, MI and GABA levels in the basal ganglia.

Analysis of changes in connectivity of resting brain functional network before and after acupuncture combined with language rehabilitation training in patients with basal ganglia aphasia after stroke / 中国医师杂志

Objective:To use the resting state functional network connectivity (FNC) method based on independent component analysis (ICA) to analyze the characteristics of FNC changes in patients with basal ganglia aphasia (BGA) after stroke, and to explore its occurrence and recovery mechanism under the intervention of acupuncture combined with language rehabilitation training.Methods:Using a prospective observational research method, 16 right-handed BGA patients who were treated at Beijing Hospital of Traditional Chinese Medicine, Capital Medical University from July 2021 to December 2022, as well as 14 healthy subjects matched in age, gender, education level, and handedness, were included. The resting state functional magnetic resonance imaging, demographic information, and Western Aphasia Examination data of healthy subjects and BGA patients before and after intervention were collected. The GIFT toolbox based on MATLAB platform was applied for ICA and resting state brain network FNC analysis. The FNC differences between BGA patients and healthy subjects were compared horizontally, and the FNC changes in BGA patients before and after intervention were compared vertically.Results:Compared with healthy subjects, post-stroke BGA patients showed decreased connectivity between the basal ganglia network, default network, and visual network before intervention, while increased connectivity between the auditory network, right frontoparietal network, and anterior cuneiform network; After the intervention of acupuncture combined with language rehabilitation training, the connectivity between the basal ganglia network, visual network, and anterior cuneiform network decreased, while the connectivity between the anterior convex network and bilateral frontoparietal network decreased, while the connectivity between the default network, auditory network, right frontoparietal network, and visual network increased. The BGA patient group showed enhanced connectivity between the basal ganglia network and the left frontoparietal network before and after intervention.Conclusions:The FNC changes between the basal ganglia network and other brain networks are key to reflecting the mechanism of BGA occurrence and language function recovery. Acupuncture combined with language rehabilitation training may improve language function by enhancing the connectivity between the basal ganglia network and the left frontoparietal network, and the redistribution of attention resources may also be one of the reasons for promoting language function recovery in BGA patients.

Basal Ganglia Traumatic Hematoma: Case Series and Literature Review / Hematoma traumático em gânglios da base: Série de casos e revisão da literatura

Introduction Basal ganglia traumatic hematoma (BGTH) is rare, occurring in 3% of closed traumatic brain injuries, and it is associated with a poor prognosis. In the present paper, the authors present a series of 16 BGTH cases, describing their causes, treatment, and results. Patients and Methods Thisis a retrospective study of 16 patients diagnosed with BGTH, associated with a literature review in the PubMed, ScienceDirect, and Google Scholar databases, using the terms craniocerebral trauma AND basal ganglia and basal ganglia AND hematoma AND trauma. Articles published in the period from 1986 to 2019 were selected, resulting in a total of 19 articles that met the inclusion criteria taking into account their citations and their respective impacts. Results Sixteen patients were studied. They were all male, with an average age of 21 years and 5 months. The main cause of BGTH was traffic accident (12). The mean score in the Glasgow coma scale at admission was 8. All patients underwent a computed tomography (CT) scan of the skull. The putamen was the most affected structure (5). Thirteen patients underwent conservative treatment, and three drained the associated intracranial hematoma. Nine patients died, and seven, and four had neurological sequel. Conclusions The neurosurgeon's knowledge of GBTH, including diagnosis and clinical surgical management, is extremely important, as this type of lesion is associated with a poor prognosis.

Introdução O hematoma traumático dos gânglios da base (HTGB) é raro, ocorrendo em 3% dos traumatismos cranioencefálicos fechados, estando associado a mau prognóstico. Neste artigo, os autores apresentam uma série de 16 casos de HTGB, descrevendo suas causas, tratamento e resultados. Pacientes e métodos Estudo retrospectivo de 16 pacientes com diagnóstico de HTGB, associado a uma revisão de literatura nas bases de dados PubMed, ScienceDirect e Google Scholar, utilizando os termos: "Craniocerebral Trauma" AND "Basal Ganglia" and "Basal Ganglia"AND "Hematoma"AND "Trauma." Foram selecionados artigos com período de 1986 a 2019, resultando em um total de 19 artigos que atenderam aos critérios de inclusão levando em consideração suas citações e seus respectivos impactos. Resultados Dos 16 pacientes foram estudados, todos do sexo masculino e com idade média de 21 anos e 5 meses. A principal causa de HTGB foi acidente de trânsito (12). A pontuação média na escala de coma de Glasgow na admissão foi de 8. Todos foram submetidos a uma tomografia computadorizada do crânio. Putamen foi o mais afetado (5). Treze pacientes foram submetidos a tratamento conservador e três apresentaram o hematoma intracraniano associado. Nove pacientes morreram e sete, e quatro tiveram sequelas neurológicas. Conclusões O conhecimento dos neurocirurgiões sobre HTGB, diagnóstico e manejo clínico cirúrgico é de extrema importância, pois está associado a um mau prognóstico.

Hypoparathyroidism and Fahr's syndrome: case series / Hipoparatireoidismo e síndrome de Fahr: série de casos

Abstract Hypoparathyroidism (HP) is a rare metabolic disorder and causes hypocalcemia because parathyroid hormone secretion is inadequate to mobilize calcium from bone and reabsorb calcium from kidney and gut. Anterior neck surgery is the most common cause of acquired HP and autoimmune HP is the next most common form in adults. The duration, severity, and rate of development of hypocalcemia determine the clinical presentation. A variety of organs can be affected by calcification, more frequently kidneys, but also joints, eyes, skin, vasculature, and other organ systems and, although rarely seen, intracerebral calcifications. We report four cases of bilateral basal ganglia calcifications (BGC) also known as Fahr's syndrome related to hypoparathyroidism. Fahr's syndrome is characterized by bilateral symmetrical calcification of areas of the brain that control movements including basal ganglia, thalamus, and others; it is a rare inherited or sporadic neurological disorder with a prevalence of less than 1/1.000.000. Main symptoms related to bilateral BGC include extra-pyramidal and cerebellar disorders, cognitive impairment, epileptic seizures, and psychiatric changes. BGC has been established as a possible outcome of HP. Its prevalence, demonstrated in the HP cohorts, varied significantly from 12 up to 74%. Currently, computed tomography (CT) is the most valuable method for diagnosis. The treatment include symptomatic support and identification of causes, but there is no specific treatment limiting the progression of calcification in the basal ganglia. Especially in HP, an early treatment can prevent calcification and neurophysiological disorders.

Resumo O hipoparatireoidismo (HP) é um distúrbio metabólico raro e causa hipocalcemia, pois a secreção do paratormônio é inadequada para mobilizar o cálcio dos ossos e reabsorver o cálcio dos rins e intestino. A cirurgia cervical anterior é a causa mais comum de HP adquirido. O HP autoimune é a segunda causa mais comum em adultos. A duração, gravidade e taxa de desenvolvimento da hipocalcemia determinam a apresentação clínica. Diversos órgãos podem ser afetados pela calcificação, mais frequentemente os rins, mas também articulações, olhos, pele, vasculatura e outros órgãos e, embora raramente vista, calcificações intracerebrais. Relatamos quatro casos de calcificações dos gânglios da base (CGB) bilaterais, também conhecidas como síndrome de Fahr relacionadas ao hipoparatireoidismo. A síndrome de Fahr é caracterizada pela calcificação bilateral simétrica de áreas do cérebro que controlam os movimentos incluindo os gânglios da base, tálamo e outros; é um distúrbio neurológico raro, hereditário ou esporádico, com uma prevalência 1/1.000.000. Os principais sintomas relacionados à CGB bilateral incluem distúrbios extrapiramidais e cerebelares, comprometimento cognitivo, convulsões e alterações psiquiátricas. A CGB foi estabelecida como uma possível consequência do HP. Sua prevalência, demonstrada nas coortes de HP, variou significativamente de 12 a 74%. Atualmente, a tomografia computadorizada (TC) é o método mais valioso para o diagnóstico. O tratamento inclui suporte sintomático e identificação de causas, mas não há tratamento específico limitando a progressão da calcificação nos gânglios da base. Especialmente no HP, um tratamento precoce pode prevenir calcificações e distúrbios neurofisiológicos.

Unusual Involvement of Basal Ganglia and Dentate Nucleus in Children with Acute Encephalopathy with COVID-19

Children account for 1% to 5% of diagnosed COVID-19 infection with relatively mild presentation compared to adults. The frequency of neurological involvement in acute COVID-19 infection in children is unclear. COVID-19 is also considered to be a neurotropic virus, but so far, in the pediatric age group, very few cases with involvement of basal ganglia and no case of dentate nucleus involvement have been reported in the literature. The present paper reports two cases of acute encephalopathy with COVID-19, the frst case with basal ganglia involvement and the second with dentate nucleus involvement. Both cases required aggressive management and had complete neurological recovery on follow-up. Hence, these cases are reported to make everyone aware of the neurological presentation with atypical neuroimaging fnding of acute COVID-19 infection in the pediatric age group; timely management improves the outcome.

Comparación del rendimiento de pruebas cognitivas en pacientes con Enfermedad de Parkinson con y sin estimulación cerebral profunda / Comparison of the performance of cognitive tests in Parkinson Disease patients with and without Deep Brain Stimulation

Resumen Los pacientes diagnosticados con Enfermedad de Parkinson (EP) presentan alteraciones motoras concomitantes a otras alteraciones de tipo cognitivo, conductual o emocional. Una de las alternativas al tratamiento farmacológico es la estimulación cerebral profunda (ECP). Existen reportes de alteraciones en el rendimiento de tareas cognitivas tras procedimiento de ECP, lo que podría sugerir que el procedimiento es responsable de estos cambios cognitivos. El objetivo del estudio fue comparar los resultados del rendimiento en pruebas cognitivas de pacientes con EP de dos grupos (con ECP y sin ECP). Se recolectaron los resultados de 47 pacientes (n = 16 ECP; n = 31 sin ECP) durante los años de 2011 hasta 2015. Dentro de las funciones y variables evaluadas se encuentran: categorización visual, flexibilidad cognitiva, solución de problemas, atención selectiva, velocidad de procesamiento, inhibición conductual y calidad de vida. En general, no se encontraron diferencias significativas entre los grupos en las funciones evaluadas. En el grupo de participantes con ECP, se encontró mayor cantidad de correlaciones entre las pruebas de semejanzas y las de dígitos inversos, listas de palabras, búsqueda de símbolos y las subpruebas del Wisconsin Card Sorting Test. Así mismo, en los pacientes con ECP se presentó una percepción menor de calidad de vida asociada al tiempo de la enfermedad en comparación con el grupo sin ECP. En conclusión, estos resultados son congruentes con estudios similares de evaluación neuropsicológica y se discute el papel del tratamiento de estimulación en los pacientes, los cuales no siempre tienen un impacto positivo sobre la calidad de vida percibida.

Abstract Patients diagnosed with Parkinson's disease show motor alterations together to cognitive, behavioral, and emotional disturbances. An alternative treatment to the exclusive pharmacological medication is the Deep Brain Stimulation procedure (DBS). Some studies have shown altered behavioral patterns after DBS device implantation, suggesting a relationship between a particular performance in cognitive tests derived from the DBS procedure. Our study aimed to compare the performance of cognitive tests in Parkinson's disease patients with and without DBS. Results were analyzed from 47 patients (n = 16 DBS; n = 31 without DBS) in a range since 2011 to 2015. Functions tested were visual categorization, cognitive flexibility, problem solutions, selective attention, cognitive processing speed, behavioral inhibition, and quality of life. In general, there are non-significative differences between groups in functions tested. However, correlations were found depending on the group (DBS or without DBS patients), with more positive correlations inside the DBS group between the similarity test and inversed digits, list of words, symbol search and the sub-test of the Wisconsin Card Sorting Test. In addition, the DBS group showed a low perception of the quality of life associated with the disorder´s time compared to the without DBS group. In conclusion, these results are congruent with similar studies of neuropsychological evaluation, and the role of treatment is discussed below the perception of the quality of life.

Necrosis en espejo de ganglios basales en la enfermedad de la orina con olor a jarabe de arce. Presentación de un caso / Mirror necrosis of basal ganglia in maple syrup urine disease. A case report

RESUMEN La enfermedad de la orina con olor a jarabe de arce es una enfermedad genética autosómica recesiva, cerebral degenerativa. Es causada por un déficit en la actividad de la deshidrogenasa de los cetoácidos de cadena ramificada, que provoca inadecuado almacenamiento de los tres aminoácidos esenciales de dicha cadena. Esto genera una neurotoxicidad severa que puede llevar a la muerte. Se manifiesta clínicamente por deterioro neurológico, retraso psicomotor, problemas de alimentación, orina con olor característico. Sus consecuencias cerebrales pueden ser definidas mediante tomografía axial computarizada. Este artículo tiene como objetivo presentar un caso de enfermedad de la orina con olor a jarabe de arce con hipodensidad bilateral de los ganglios basales por necrosis en espejo, detectado mediante estudio tomográfico. Se trata de una paciente femenina, de 9 años de edad con cuadro anterior de cetoacidosis no diabética. Posterior a la realización de apendicectomía, comenzó con cuadro comatoso que requirió estudio tomográfico mediante el cual se constató edema cerebral. Evolucionó tórpidamente, por lo que requirió nuevo estudio tomográfico que demostró empeoramiento de las condiciones neurológicas al reflejarse en la imagen hipodensidad bilateral a nivel de los núcleos basales por necrosis. La enfermedad de la orina con olor a jarabe de arce es una afección rara, con diversas formas clínicas. Requiere de estudios de laboratorio que la confirmen e imágenes como tomografía computarizada que, como en este caso, ayuden a evidenciar el daño neurológico. Fue muy característica la hipodensidad de ganglios basales asociada a edema cerebral.

ABSTRACT Maple syrup urine disease is an autosomal recessive genetic degenerative brain disease. It is caused by a deficit in branched-chain ketoacid dehydrogenase activity, which causes inadequate storage of the three essential amino acids of said chain. This generates severe neurotoxicity that can lead to death. It is clinically manifested by neurological deterioration, psychomotor retardation, feeding problems, urine with a characteristic odor. Its cerebral consequences can be defined by computerized axial tomography. This article aims to present a case of maple syrup urine disease with bilateral hypodensity of the basal ganglia due to mirror necrosis, detected by tomographic study. This is a 9-years-old female patient with a history of non-diabetic ketoacidosis. After the appendectomy, she began with a coma that required a tomographic study, which revealed cerebral edema. She evolved torpidly, requiring a new tomographic study that showed worsening of the neurological conditions as bilateral hypodensity at the level of the basal nuclei due to necrosis was reflected in the image. Maple syrup urine disease is a rare condition with various clinical forms. It requires laboratory studies to confirm it and images such as computed tomography that, as in this case, help to show the neurological damage. The hypodensity of the basal ganglia associated with cerebral edema was very characteristic.

Surgical treatment of severe spontaneous intracerebral hemorrhage in the basal ganglia in young adults: a comparison between transsylvian-transinsular and transcortical-transtemporal approach / 国际脑血管病杂志

Objective:To investigate the difference in efficacy between transsylvian-transinsular approach and transcortical-transtemporal approach for hematoma evacuation in the treatment of severe basal ganglia intracerebral hemorrhage in young adults.Methods:Young adult patients with severe intracerebral hemorrhage in the basal ganglia region underwent craniotomy hematoma removal in Ankang Central Hospital from February 2012 to February 2021 were retrospectively enrolled. The Glasgow Outcome Scale score was used to evaluate the outcome at 6 months after onset. 4-5 were defined as good outcome and 1-3 were defined as poor outcome. Multivariate logistic regression analysis was used to determine the independent influencing factors of the poor outcomes. Results:A total of 51 patients were enrolled. Their median age was 41 (interquartile range 39-43) years, and 29 were men (56.8%). The median Glasgow Coma Scale score at admission was 6.0 (interquartile range 5.5-7.0), and the median baseline hematoma volume was 38.0 ml (34.5-47.5 ml). Twenty-one patients (41.2%) were in the transsylvian-transinsular approach group and 30 (58.8%) were in the transcortical-transtemporal approach group. There were no significant differences in demographics, vascular risk factors and baseline clinical data between the transsylvian-transinsular approach group and the transcortical-transtemporal approach group. Compared with the transcortical-transtemporal approach group, the amount of intraoperative bleeding and hematoma residue in the transsylvian-transinsular approach group were less, the proportion of patients requiring decompressive craniectomy was lower (33.3% vs. 63.3%; χ2=4.449, P=0.035), and the duration of dehydration medication and hospital stay were shorter (all P<0.05). However, there was no significant difference in the good outcome rate between the two groups (66.7% vs. 56.7%; χ2=0.518, P=0.472). Multivariate logistic regression analysis showed that lower scores of Glasgow Coma Scale at admission (odds ratio 0.128, 95% confidence interval 0.017-0.977; P=0.047) and longer hospital stay (odds ratio 1.402, 95% confidence interval 1.065-1.844; P=0.016) were independently associated with the poor outcomes. Conclusion:For young adult patients with severe basal ganglia intracerebral hemorrhage who underwent hematoma removal, although there was no significant difference between the outcomes of patients with transsylvian-transinsular approach and transcortical-transtemporal approach, the former had more advantages.

Clinical and imaging features of basal ganglia germinoma in children and adolescents / 中国医师杂志

Objective:To investigate the clinical and imaging features of basal ganglia germinoma in children and adolescents, so as to improve the accuracy of early diagnosis.Methods:The clinical symptoms and imaging findings of 10 cases of basal ganglia germinoma diagnosed by surgical examination or diagnostic radiotherapy in Xinhua Hospital Affiliated to Medical College of Shanghai Jiaotong University from January 2010 to December 2019 were analyzed retrospectively.Results:There were 9 males and 1 female, aged from 5 to 15 years, with an average age of 9.7 years.There were 10 cases of limb weakness or mild hemiplegia, 1 case of headache and vomiting, and 1 case of extrapyramidal tract sign. There were 9 cases of computed tomography (CT) examination, 5 cases of high density, 4 cases of mixed density, and 3 cases of intrafocal calcification. 10 cases of routine magnetic resonance imaging (MRI) examination, MR T1-weighted imaging (T1WI): 5 cases of low signal, 3 cases of slightly low signal, 1 case of equal low signal, 1 case of equal slightly low signal; MR T2-weighted imaging (T2WI): 3 cases of high signal, 5 cases of slightly high signal, 2 cases of high and low mixed signal; T2 fluid attenuated inversion recovery (T2FLAIR): 1 case of low signal, 2 cases of slightly high signal, 1 case of iso-high signal, 5 cases of high signal, 1 case of high and low mixed signal; Diffusion weighted imaging (DWI): 2 cases of low signal, 1 case of equal signal, 1 case of equal slightly high signal, 4 cases of slightly high signal, 2 cases of high signal; 1 case of functional imaging susceptibility-weighted imaging (SWI): 1 case of low signal; 10 cases of MRI enhancement: 2 cases of no enhancement, 5 cases of mild enhancement, 3 cases of moderate/obvious enhancement, and the enhancement methods can be seen in spot, patch, mass and ring enhancement; Functional imaging magnetic resonance angiography (MRA) in 2 cases: 1 case had slender middle cerebral artery, fewer branch arteries than the opposite side, and 1 case showed no obvious abnormality; 2 cases of positron emission (PET)-CT: the metabolism of methionine (MET) on the affected side was significantly increased in 2 cases.Conclusions:Germinoma in the basal ganglia of children and adolescents are more common in men, with weakness or mild hemiplegia as the main symptom. The imaging findings have certain characteristics. Imaging findings combined with relevant clinical data can improve the accuracy of early diagnosis of germinoma in the basal ganglia.