Correlation between cerebral atrophy and total burden of cerebral small vessel disease in patients with recent small subcortical infarct / 中华老年心脑血管病杂志

Objective To investigate the relationship between cerebral atrophy and total burden of cerebral small vessel disease in patients with recent small subcortical infarct(RSSI).Methods A total of 194 elderly RSSI patients admitted to Department of Neurology of Changzhou Second People's Hospital Affiliated to Nanjing Medical University from September 2021 to November 2022 were recruited in this study.All patients completed cranial MRI examination and were divid-ed into a non-mild group 1(97 cases)and a moderate to severe group 1(97 cases)based on the to-tal burden of cerebral small vessel diseases(CSVD)imaging.Visual assessment scale was used to assess the severity of brain atrophy in the whole brain and various regions of the brain.According to the global cortical atrophy scale(GCA)score,all patients were divided into a non-mild group 2(88 cases)and a moderate to severe group 2(106 cases).Brain atrophy in various regions,medial temporal lobe atrophy(MTA)score,frontal temporal lobe atrophy(FTA)score,and posterior cortical atrophy(PA)score were evaluated.Their general clinical and imaging data were collected,multivariate logistic regression analysis was employed to analyze the relationship between GCA score and total burden of CSVD imaging in RSSI patients,and Spearman correlation analysis was further adopted to explore the correlation of GCA score and different parts of brain atrophy with total burden of CSVD imaging.Results When compared with the non-mild group 1,the moderate to severe group 1 had significantly larger proportions of having GCA score of 2-3 points,PA score of 2-3 points,MTA score of 2-4 points and FTA score of 2-4 points(P＜0.01).The ra-tio of having 2-4 points in total imaging burden score of CSVD was obviously higher in the mod-erate to severe group 2 than the non-mild group 2(P＜0.01).Multivariate logistic regression anal-ysis showed that age and total burden of CSVD imaging were independent risk factors for brain atrophy in RSSI patients(OR=1.184,95％CI:1.099-1.276,P=0.000;OR=3.537,95％CI:1.664-7.518,P=0.001).Spearman correlation analysis revealed that the total burden score of CSVD imaging was positively correlated with GCA,MTA,FTA and PA scores in RSSI patients(r=0.518,r=0.382,r=0.471,r=0.388,P=0.000).Conclusion The total burden of CSVD is an independent risk factor for GCA in elderly RSSI patients.The more serious the total burden of CSVD is,the higher the grade of GCA is.The total burden of CSVD is related to the whole brain and brain atrophy in other different regions of the brain.

The role of MRI in assessing cognitive impairment changes

Aging brings about various changes in the brain, leading to cognitive alterations that are increasingly relevant with extended life expectancy. Dementia, characterized by chronic cognitive impairment, is on the rise due to longer life expectancy, imposing a substantial burden on healthcare systems. Dementia encompasses conditions like Alzheimer's disease (AD), vascular dementia (VaD), Lewy body dementia (LBD), and frontotemporal dementia, each with its distinct symptoms and progression. Magnetic resonance imaging (MRI), especially 3T MRI, plays a crucial role in monitoring and diagnosing dementia, aiding in patient selection for emerging therapies. Study involves a comprehensive literature search without restrictions on date, language, age/publication type. Dementia can be divided into neurodegenerative and nondegenerative categories, with AD being the most prevalent. Diagnosis relies on clinical evaluation, supported by neuroimaging techniques like MRI. Various MRI findings, such as cerebral atrophy, microbleeds, white matter hyperintensities, lacunes, and strategic infarcts, offer insights into dementia-related brain changes. These findings facilitate early diagnosis, prognosis, and treatment monitoring, with standardized assessment tools and volumetric analysis enhancing diagnostic accuracy. As life expectancy continues to rise, MRI's role in assessing cognitive impairment changes becomes increasingly vital in addressing the growing challenge of dementia.

Infantile tremor syndrome: a case report

Infantile tremor syndrome (ITS) is a rare but significant clinical condition characterized by a tetrad of pallor, developmental delay or regression, skin pigmentation abnormalities, and sparse brown scalp hair, often accompanied by involuntary tremors. ITS primarily affects children aged 5 months to 3 years, with a higher prevalence in males, especially in regions like the Indian subcontinent, Southeast Asia, and Africa. The exact cause of ITS remains uncertain, but a strong association with vitamin B12 deficiency has been observed in numerous studies. Malnutrition, including deficiencies of zinc, magnesium, iron, calcium, and hypoalbuminemia, is common among affected infants, who often come from lower socioeconomic backgrounds with vegetarian mothers lacking animal food in their diets. In this case report, an 8-month-old female child presented with progressive tremors, poor feeding, and developmental delay. The child had been exclusively breastfed by a vegetarian mother and lacked proper complementary feeding. Clinical examination revealed pallor, hypopigmented hair, hyperpigmented knuckles, and generalized hypotonia. Laboratory tests confirmed vitamin B12 deficiency, anemia and other nutritional deficiencies. Neuroimaging showed cerebral atrophy. Treatment involved addressing the nutritional deficiencies, primarily through vitamin B12 supplementation, iron, magnesium, calcium, and a protein-rich diet. Tremors were managed with propranolol and phenobarbitone. Within a month of treatment, the child exhibited significant improvement in tremors, developmental milestones, and overall well-being. This case emphasizes the importance of considering ITS in infants with specific clinical features and highlights the role of healthcare providers in educating parents about proper nutrition during pregnancy and infancy. Early diagnosis and management, including nutritional interventions and symptom control, can lead to substantial improvements in affected children's health and development. Further research is necessary to better understand the etiology and optimal management strategies for ITS.

La atrofia cerebral como hallazgo o factor predictor del deterioro cognitivo en el envejecimiento normal / Cerebral Atrophy as a Finding of Predictive Factor for Cognitive Impairment in Normal Aging

Introducción: En el curso del envejecimiento es conocida la existencia de un patrón complejo de cambios estructurales cerebrales, conductuales y cognitivos, en ocasiones relacionados con enfermedades neurológicas y psiquiátricas. Objetivo: Determinar la posible relación de causalidad de la atrofia cerebral en la aparición del deterioro cognitivo en el curso del envejecimiento normal. Métodos: Se desarrolló un estudio retrospectivo, transversal, descriptivo y observacional. El universo estuvo conformado por el total de los pacientes de ambos sexos con edades comprendidas entre 35-74 años de edad, con indicaciones previas de tomografía computarizada de cráneo y cuyos resultados fueron informados con signos de atrofia cerebral, cuya cifra ascendió a 733. Resultados: El grupo de edad que predomino fue el de 45-54 años (35,3 por ciento), así como las pacientes del sexo femenino (66,3 por ciento). El 27,7 por ciento tenía como nivel de escolaridad el técnico medio superior y 36,2 por ciento fueron pacientes amas de casa. El 99,7 por ciento fueron diestros. Un total de 368 voluntarios presentaron deterioro cognitivo y 365 sujetos no evidenciaron declive en las funciones exploradas. Las funciones de atención y cálculo y retención verbal a corto plazo fueron las que se vieron más afectadas, seguidas de orientación espacial y memoria verbal de fijación. Conclusiones: No se logró establecer una relación de causalidad significativa entre el diagnóstico radiológico de atrofia cerebral y la presencia de deterioro cognitivo(AU)

Introduction: In the course of aging, the existence of a complex pattern of behavioral, cognitive and cerebral structural changes is known, sometimes related to neurological and psychiatric diseases. Objective: To determine the possible causal relationship of cerebral atrophy with the onset of cognitive impairment in the course of normal aging. Methods: A retrospective, cross-sectional, descriptive and observational study was carried out. The study universe consisted of all patients of both sexes aged 35-74 years, with previous indications for cranial computed tomography and whose results were reported with signs of cerebral atrophy, which numbered 733. Results: The predominant age group was 45-54 years old (35.3percent), as well as female patients (66.3percent). The educational level of 27.7percent of the patients was technical high school and 36.2percent were housewife patients. A total of 99.7percent were right-handed. A total of 368 volunteers showed cognitive impairment and 365 subjects showed no decline in the tested functions. The functions of attention and calculation, as well as short-term verbal retention, were the most affected, followed by spatial orientation and speech retention memory. Conclusions: No significant causal relationship was established between the radiological diagnosis of cerebral atrophy and the presence of cognitive impairment(AU)

Integrated Treatment Approach in Hemiplegic Stroke Patient: A Case Study

Hemiplegic stroke is one of the major prevalent mortality in many countries including India. In hemiplegic stroke spasticity, and muscle atrophy results in the inability to move certain muscles. Most post-stroke complications like inability and shoulder pain are a major concern for therapeutic interventions. Recent advances have been made in the interdisciplinary approach of rehabilitation, artificial intelligence, brain-computer interface, and much more mainly targeting post-stroke complications. In the present case report study 55-year-old male addicted to tobacco, altered HBA1c, elevated neutrophils, erythrocyte sedimentation rate, and C-reactive protein and CT scan report of mild cerebral atrophy suggestive of hemiplegic stroke. The patient was admitted on a stretcher with no sensation in his left hand and Vaksanga (impaired speech). After receiving the patient and family member's consent the integrated treatment was initiated. After 15 days of integrated treatment including T-AYU-HM Premium, significant improvement in the patient’s condition was noticed. The patient recovered from the impaired mobility of the left hand and remarkable improvement in slurred speech. This confirms that the intervention of Ayurvedic medicine in post-stroke management and prevention of stroke might play a crucial role. More such kinds of interventional case studies or trials should be warranted to justify the same.

Aspectos neuroquirúrgicos de las mucopolisacaridosis / Neurosurgical aspects of mucopolysaccharidoses

Mucopolisacaridosis es una rara enfermedad que afecta al metabolismo de los mucopolisacaridos debida a la ausencia o deficiencia de las enzimas encargadas de su síntesis lo que produce depósitos de aminoglucósidos en casi todos los tejidos del organismo. De acuerdo a la enzima faltante se clasifican los distintos tipos de la enfermedad, siendo más frecuente el Tipo I con sus tres variantes: Hurler, Hurler-Sheie y Sheie de distinta gravedad y tratamiento. Al nacimiento el niño no presenta síntomas, éstos van apareciendo a partir del año de vida: retardo físico y mental, múltiples deformidades esqueléticas, hepatoesplenomegalia, sordera, opacidades corneanas, entre otras. La intervención neuroquirúrgica en esta entidad es en dos patologías: la hidrocefalia, que al no presentar los signos clásicos de hipertensión endocraneana puede ser confundida con atrofia y la compresión medular cervical por los depósitos de mucopolisacáridos en vértebras, ligamentos y leptomeninges.

Mucopolysaccharidosis is a rare illness that involves the metabolism of mucopolysaccharides, that due to the absence or deficiency of corresponding enzymes, accumulate in almost all the tissues of the body. According to which enzyme is missing, different types of the disease have been identified; the most frequent being Type I with its three variants: Hurler, Hurler-Sheie, and Sheie. Symptoms of this disorder progress and range from mental and physical retardation, multiple skeletal deformities, hepatosplenomegaly, deafness, and corneal opacities among others. Children affected usually appear normal at birth and the slowness in their development may be the first evidence of the disorder whose progression is downhill. Neurosurgical intervention occurs in two pathologies: hydrocephalus that does not show signs of intracranial hypertension and can be confused with atrophy, and cervical cord compression due to storage of mucopolysaccharides in vertebrae, ligaments, and leptomeninges.

A study of clinical profile of intractable epilepsy in children

Background: This study was undertaken to find out the prevalence and clinical features of intractable epilepsy (IE) in a tertiary referral center.Methods: Study was conducted in a tertiary care hospital on 60 children with intractable epilepsy. Cases includes' intractable epilepsy is when seizures continue to occur despite maximally tolerated doses of more than two antiepileptics, occurrence of an average of one seizure per month for 18 months with no more than a 3 month seizure free period during these 18 months. Controls: epileptic children who had good control of seizures for the previous 18 months.Results: The prevalence of intractable seizures was 10% with maximum number of children 25 (41.6%) belonged to the 5-12 years. 15 (50%) children had daily seizures. Myoclonic seizures proved to be an important predictor of intractability. 4 children among the cases had history of family seizures, 6 children in cases had history febrile seizure, whereas, 6 children among the controls had history of family seizures, 8 children in controls had history febrile seizure.' 23.3% of children presented with Status epilepticus in the cases and 16.6% of the children in the controls. Remote symptomatic etiology 12(40%) is the commonest cause of seizure. 13 (43.3%) children in cases and 3 (10%) children among the controls had a history suggestive of birth asphyxia. EEG was abnormal in 17 (56.6%) cases when compared to 11 (36.6%) children in the controls. CT scan was abnormal in 14 (46.6%) cases and 10 (33.3%) controls. MRI was abnormal in 16 (53.3%) children of the cases and 8 (26.6%) children of the controls.Conclusions: The commonest cause of intractable epilepsy was perinatal asphyxia. Perinatal asphyxia can be prevented by good nutrition during pregnancy, regular antenatal check ups with detection of high risk pregnancy, promoting hospital deliveries and prompt resuscitation of newborn when required.

El raro caso de una joven con colitis ulcerativa crónica inespecífica grave y atrofia cerebral / The rare case of a young girl with ulcerative colitis and cerebral atrophy

Resumen La enfermedad inflamatoria intestinal es un grupo de enfermedades caracterizadas por inflamación crónica gastrointestinal y en ocasiones con repercusión extraintestinal. Las manifestaciones neurológicas y psiquiátricas corresponden a menos de 3%. Se comunica el caso de una mujer joven con colitis ulcerativa y atrofia cerebral como inicio.

Abstract Inflammatory bowel disease is a group of diseases characterized by chronic gastrointestinal inflammation and occasionally with extraintestinal repercussion. The neurological and psychiatric manifestations correspond to less than 3%. This paper reports the case of a young woman with ulcerative colitis and cerebral atrophy as debut.

Classical oral manifestations of Dyke-Davidoff-Masson syndrome: a case report with review of the literature

Dyke-Davidoff-Masson syndrome is a non-inherited rare condition that presents during childhood and is characterized by seizures, hemiplegia, mental retardation, cerebral hemiatrophy, calvarial thickening, and hyperpneumatization of the frontal sinuses. The present article highlights a case of a 12-year-old male child with additional clinical findings of café-au-late pigmentation and ocular lipodermoid. This is the first case report of Dyke-Davidoff-Masson syndrome to describe oral manifestations, such as unilateral delayed eruption of teeth, hypoplasia, and taurodontism, which could be unique and characteristic of this condition. Oral health care providers and physicians should be aware of these oral observations as dental referrals could warrant early dental prophylactic care and can be useful in diagnosing the possible time of injury and type of Dyke-Davidoff-Masson syndrome.

Cerebral Atrophy in a Vitamin B12-deficient Infant of a Vegetarian Mother.

In developed countries, vitamin B12 (cobalamin) deficiency usually occurs in children, exclusively breastfed ones whose mothers are vegetarian, causing low body stores of vitamin B12. The haematologic manifestation of vitamin B12 deficiency is pernicious anaemia. It is a megaloblastic anaemia with high mean corpuscular volume and typical morphological features, such as hyperlobulation of the nuclei of the granulocytes. In advanced cases, neutropaenia and thrombocytopaenia can occur, simulating aplastic anaemia or leukaemia. In addition to haematological symptoms, infants may experience weakness, fatigue, failure to thrive, and irritability. Other common findings include pallor, glossitis, vomiting, diarrhoea, and icterus. Neurological symptoms may affect the central nervous system and, in severe cases, rarely cause brain atrophy. Here, we report an interesting case, a 12-month old infant, who was admitted with neurological symptoms and diagnosed with vitamin B12 deficiency.

Alive with almost no Brain: Severe Post-Hemorrhagic Internal Hydrocephalus

Objectives: Severe post-hemorrhaghic internal hydrocephalus with almost complete atrophy of the cerebral parenchyma, as in the following case, is rare. Case report: A 19yo Caucasian female with a history of premature birth, perinatal intraventricular bleeding, developmental delay, mental retardation, and epilepsy, was admitted for recurrent generalized tonic-clonic seizures. She was able to produce some noises but was unable to communicate with understandable speech. There was severe mental retardation, motor deficits, and tetraspasticity. She was able to sit and eat but was otherwise dependent on the parents’ support. Monotherapy with primidon since age 15y was increased to 500mg/d. A CT scan of the cerebrum showed a massive internal hydrocephalus with atrophy of the basal ganglia, the white matter, the cerebellum, but also the cortex. Neurosurgeons decided against a shunt. Conclusions: Despite severe atrophy of the cerebral parenchyma, severe post-hemorrhagic internal hydrocephalus, manifesting as psychomotor retardation, epilepsy, and tetraspasticity, is compatible with life.

The study about impairment of episodic memory encoding in patients with cerebral infarction / 中华行为医学与脑科学杂志

Objective To investigate the impairment and the effect factors of encoding of episodic memory in patients with cerebral infarction. Methods 112 cases cerebral infarction patients and 115 healthy elders as controls were tested for episodic memory encoding with episodic pictures accomplished in computer, and compare the differences of encoding of episodic memory between the two groups. Results The remember indexes ( REM )of encoding memory test in patient group was significantly lower than that in control group( (70.81 ± 6.08 )vs (84.67 ± 4.49), P ＜ 0.01 ). The REM in patients with different impaired areas was significantly different ( (65.88 ± 5.73 ), (68.92 ± 4.65 ), (73.39 ± 6.20), ( 73.53 ± 3.44), P ＜ 0. 01 ). The REM in frontal lobe infarction group was significantly lower than that in temporal lobe infarction group (P ＜ 0.05 ), and in temporal lobe infarction group was significantly lower than that in basal ganglia infarction group and corona radiate infarction group (P＜0.05, P＜0. 01). The REM in cortex infarction group was significantly lower than that in under cortex group ( ( 67.37 ± 5.40 ), ( 73.46 ± 4.99 ), P ＜ 0.01 ). The REM in small cerebral infarction group was significantly higher than that in large cerebral infarction group( (72.67 ±4.47 ), (67.56 ± 6.18 ), P＜0.01 ). The size of cerebral infarction diameter was related with the REM( r= -0.39, P＜0. 01 ). The REM among control group,infarction with atrophy group, and infarction without atrophy group were significantly different( (67.03 ± 6. 17 ),( 72.84 ± 5. 00 ), ( 84.67 ± 4.49 ), P ＜ 0. 01 ). The REM in infarction with atrophy group was significantly lower than that in infarction without atrophy group and control group( both P＜0.01 ) ,The REM in infarction without atrophy group was significantly lower than that in control group (P ＜ 0.01). Conclusion The encoding of episodic memory was impaired in cerebral infarction patients. The infarction parts,size of infarction area and atrophy was related with the impairment of encoding of episodic memory.

Study of MRI features in the lession and brain atroplny of cerebral multiple sclerosis and its correlated factors / 临床神经病学杂志

Objective To study MRI feature in the lession and brain atrophy of cerebral multiple sclerosis (MS), and to analyze the relationship and the its correlated factors between cerebral MS and brain atrophy. Methods The MRI data from 80 patients with cerebral MS were collected and these patients were divided into two groups according to age. Each patient received T1-weighted and T2-weighted scanning. The number of lesion, characteristics of lesion and brain atrophy were evaluated and compared with control group. The correlated factors of brain atrophy were analyzed. Results (1)The most focal demyelinating lesions of cerebral MS were orbicular-ovate or similar round like with distinct boundary. Typical lesions presented with equal or long T1 and long T2 signals. The macroaxis of lesion was vertical to tangent line of lateral cerebral ventricle. (2)Compared with control group, the cerebroventricular anfractuosity was longer and lateral fissure was wider on MRI in cerebral MS group. The diameter of brain parenchyma was shorter. Statistic differences were found between two groups. (3)Among correlated factors, EDSS was the main predictive factor for cerebral atrophy. Conclusions The most lesions of cerebral MS are mainly located around lateral cerebral ventricles, orbicular-ovate or similar round like with distinct boundary, equal or slight long T1 and T2 signals on MRI.Brain atrophy is generally in cerebral MS and progress gradually, it is related to the course of disease, the number of lesion, the diameter of lesion and EDSS score. Measurement of brain atrophy may regard as an index about progression of MS.