Objective: To investigate clinicopathological features of multinodular and vacuolar neurodegenerative tumor (MVNT) of the cerebrum, and to investigate its immunophenotype, molecular characteristics and prognosis. Methods: Four cases were collected at the General Hospital of Southern Theater Command, Guangzhou, China and one case was collected at the First People's Hospital of Huizhou, China from 2013 to 2021. Clinical, histological, immunohistochemical and molecular characteristics of these five cases were analyzed. Follow-up was carried out to evaluate their prognoses. Results: There were four females and one male, with an average age of 42 years (range, 17 to 51 years). Four patients presented with seizures, while one presented with discomfort on the head. Pre-operative imaging demonstrated non-enhancing, T2-hyperintense multinodular lesions in the deep cortex and superficial white matter of the frontal (n=1) or temporal lobes (n=4). Microscopically, the tumor cells were mostly arranged in discrete and coalescent nodules primarily within the deep cortical ribbon and superficial subcortical white matter. The tumors were composed of large cells with ganglionic morphology, vesicular nuclei, prominent nucleoli and amphophilic or lightly basophilic cytoplasm. They exhibited varying degrees of matrix vacuolization. Vacuolated tumor cells did not show overt cellular atypia or any mitotic activities. Immunohistochemically, tumor cells exhibited widespread nuclear staining for the HuC/HuD neuronal antigens, SOX10 and Olig2. Expression of other neuronal markers, including synaptophysin, neurofilament and MAP2, was patchy to absent. The tumor cells were negative for NeuN, GFAP, p53, H3K27M, IDH1 R132H, ATRX, BRG1, INI1 and BRAF V600E. No aberrant molecular changes were identified in case 3 and case 5 using next-generation sequencing (including 131 genes related to diagnosis and prognosis of central nervous system tumors). All patients underwent complete or substantial tumor excision without adjuvant chemoradiotherapy. Post-operative follow-up information over intervals of 6 months to 8 years was available for five patients. All patients were free of recurrence. Conclusions: MVNT is an indolent tumor, mostly affecting adults, which supports classifying MVNT as WHO grade 1. There is no tumor recurrence even in the patients treated with subtotal surgical excision. MVNTs may be considered for observation or non-surgical treatments if they are asymptomatic.
Adult ; Female ; Humans ; Male ; Brain Neoplasms/pathology* ; Cerebrum/pathology* ; Neurons/metabolism* ; Seizures ; Temporal Lobe/pathology* ; Biomarkers, Tumor/metabolism*

OBJECTIVE: To explore the clinical features and genetic variant in a child featuring megalencephalic leukoencephalopathy with subcortical cyst (MLC) type 2B. METHODS: Clinical and imaging data of the child was collected. Potential variant of hepatocyte adhesion molecule (HEPACAM) gene was detected by Sanger sequencing. The growth and development of her mother and uncle was also reviewed. RESULTS: The patient, a 1-year-and-7-month female, presented with convulsion, mental retardation and abnormally increased head circumference. Cranial MRI revealed extensive long T1 long T2 signals in the white matter of bilateral cerebral hemisphere, right anterior sac cyst, cerebral gyrus widening, and shallow sulcus. Sanger sequencing identified a c.437C>T missense variant in exon 3 of the HEPACAM gene. The same variant was detected in her mother but not father. Her mother and maternal uncle both had a history of increased head circumference when they were young. In their adulthood, the head circumference was in the normal range but still greater than the average. CONCLUSION The heterozygous variant of the HEPACAM gene probably underlies the MLC2B in this child. The variant has derived from her asymptomatic mother, which suggested incomplete penetrance of the MLC2B.
Adult ; Cell Cycle Proteins ; genetics ; Cerebrum ; diagnostic imaging ; Cysts ; diagnostic imaging ; genetics ; Female ; Genetic Variation ; Hereditary Central Nervous System Demyelinating Diseases ; diagnostic imaging ; genetics ; Humans ; Infant

cerebrum, sciatic nerve, spleen, lung, stomach, ileum, colon, liver, pancreas, kidney, testis, and skin in C57BL/6 mice, using Western blotting and immunohistochemistry. Western blot analysis showed that Ninjurin-1 was differentially expressed among organs. Ninjurin-1 was abundant in skin and ileum, weakly expressed in cerebrum, and moderately expressed in the other organs studied. Immunohistochemical analysis largely confirmed the results of the western blot analysis with often localization of Ninjurin-1 in the regions with abundant connective tissues. In addition, Ninjurin-1 was differentially expressed in various cell types in the tissues under the investigation. These findings suggest that Ninjurin-1 may play organ-specific roles in development and homeostasis of many organs.]]>
Animals ; Blotting, Western ; Cell Adhesion ; Cerebrum ; Colon ; Connective Tissue ; Homeostasis ; Ileum ; Immunohistochemistry ; Kidney ; Liver ; Lung ; Mice ; Neurons ; Pancreas ; Rats ; Schwann Cells ; Sciatic Nerve ; Skin ; Spleen ; Stomach ; Testis

OBJECTIVE: To determine whether post-stroke patient’s perceived exertion correlates with effort intensity score as measured by a wearable sensor and to assess whether estimates of perceived exertion are correlated to the cerebral hemisphere involved in the stroke. METHODS: We evaluated the effort intensity score during physiotherapy sessions using a wearable sensor and subjects assessed their perceived exertion using the modified Borg CR10 Scale. RESULTS: Fifty-seven subacute stroke patients participated in the study. The correlation between perceived exertion rating and measured effort intensity was insignificant—mean (r=−0.04, p=0.78) and peak (r=−0.05, p=0.70). However, there was a significant difference (p<0.02) in the perceived exertion ratings depending on the cerebral hemisphere where the stroke occurred. Patients with left-hand side lesions rated their perceived exertion as 4.5 (min–max, 0.5–8), whereas patients with right-hand side lesions rated their perceived exertion as 5.0 (2–8). CONCLUSION: While there was an insignificant correlation between perceived exertion and effort intensity measured by a wearable sensor, a consistent variations in perceived exertion estimates according to the side of the cerebral lesion was identified and established.
Accelerometry ; Cerebrum ; Humans ; Motor Activity ; Rehabilitation ; Stroke

We present the case of a 71-year-old man who was diagnosed with amoebic encephalitis caused by Balamuthia mandrillaris. He had rheumatic arthritis for 30 years and had undergone continuous treatment with immunosuppressants. First, he complained of partial spasm from the left thigh to the left upper limb. Magnetic resonance imaging revealed multifocal enhancing nodules in the cortical and subcortical area of both cerebral hemispheres, which were suggestive of brain metastases. However, the patient developed fever with stuporous mentality and an open biopsy was performed immediately. Microscopically, numerous amoebic trophozoites, measuring 20 to 25 µm in size, with nuclei containing one to four nucleoli and some scattered cysts having a double-layered wall were noted in the background of hemorrhagic necrosis. Based on the microscopic findings, amoebic encephalitis caused by Balamuthia mandrillaris was diagnosed. The patient died on the 10th day after being admitted at the hospital. The diagnosis of amoebic encephalitis in the early stage is difficult for clinicians. Moreover, most cases undergo rapid deterioration, resulting in fatal consequences. In this report, we present the first case of B. mandrillaris amoebic encephalitis with fatal progression in a Korean patient.
Aged ; Balamuthia mandrillaris ; Biopsy ; Brain ; Cerebrum ; Diagnosis ; Encephalitis ; Fever ; Humans ; Immunosuppressive Agents ; Magnetic Resonance Imaging ; Necrosis ; Neoplasm Metastasis ; Rheumatic Fever ; Spasm ; Stupor ; Thigh ; Trophozoites ; Upper Extremity

Objective To explore the relationship between the electrical conductivity （EC） and biochemical indicators of rat cerebrum tissues and postmortem intervals （PMIs） and discuss the mechanism of applying EC to infer PMI. Methods Forty healthy Sprague-Dawley rats were sacrificed and stored in an environment of about 25 ℃. The whole cerebrum tissues of rats were removed respectively at different PMIs of 0, 1, 2, 3, 4, 5, 6, and 7 d, and then made into homogenized impregnation solution. The EC and related biochemical indicators （potassium, sodium, chloride, calcium, inorganic phosphorus, magnesium, uric acid, urea nitrogen and creatinine） in cerebrum tissue impregnation solution were determined, and the relationships among EC in impregnation solution, related biochemical indicators and PMI were analyzed. Results The EC in cerebrum tissues increased gradually with the extension of PMI, and the content of uric acid, urea nitrogen and inorganic phosphorus in its impregnation solution also increased gradually with the extension of PMI. The correlation of EC, uric acid, urea nitrogen, and inorganic phosphorus with PMI was relatively good （R² was 0.95-0.99）, and there was a linear correlation between the content change of uric acid, urea nitrogen, inorganic phosphorus and EC （R² was 0.97-0.99）. The changes of the other 6 kinds of biochemical indicators with the extension of PMI within 7 d after the rats' death were non-significant （P>0.05）. Conclusion The correlation between EC in cerebrum tissues, uric acid, urea nitrogen, inorganic phosphorus and PMI were relatively good, and combining various indicators can also improve the accuracy of PMI estimation.
Animals ; Cerebrum/pathology* ; Electric Conductivity ; Forensic Pathology ; Postmortem Changes ; Rats ; Rats, Sprague-Dawley ; Time Factors

PURPOSE: This study investigated patterns of ischemic injury observed in brain images from patients with neonatal group B Streptococcal (GBS) meningitis. METHODS: Clinical findings and brain images from eight term or near-term newborn infants with GBS meningitis were reviewed. RESULTS: GBS meningitis was confirmed in all 8 infants via cerebrospinal fluid (CSF) analysis, and patients tested positive for GBS in both blood and CSF cultures. Six infants (75.0%) showed early onset manifestation of the disease (<7 days); the remaining 2 (25.0%) showed late onset manifestation. In 6 infants (75%), cranial ultrasonography showed focal or diffuse echogenicity, suggesting hypoxic-ischemic injury in the basal ganglia, cerebral hemispheres, and periventricular or subcortical white matter; these findings are compatible with meningitis. Findings from magnetic resonance imaging (MRI) were compatible with bacterial meningitis, showing prominent leptomeningeal enhancement, a widening echogenic interhemisphere, and ventricular wall thickening in all infants. Restrictive ischemic lesions observed through diffusion-weighted imaging were evident in all eight infants. Patterns of ischemic injury as detected through MRI were subdivided into 3 groups: 3 infants (37.5%) predominantly showed multiple punctuate lesions in the basal ganglia, 2 infants (25.0%) showed focal or diffuse cerebral infarcts, and 3 infants (37.5%) predominantly showed focal subcortical or periventricular white matter lesions. Four infants (50%) showed significant developmental delay or cerebral palsy. CONCLUSION: Certain patterns of ischemic injury are commonly recognized in brain images from patients with neonatal GBS meningitis, and this ischemic complication may modify disease processes and contribute to poor neurologic outcomes.
Basal Ganglia ; Brain Ischemia ; Brain* ; Cerebral Palsy ; Cerebrospinal Fluid ; Cerebrum ; Humans ; Infant ; Infant, Newborn ; Magnetic Resonance Imaging ; Meningitis* ; Meningitis, Bacterial ; Streptococcus agalactiae ; Ultrasonography ; White Matter

Neurocysticercosis (NCC) by Taenia solium is the most common parasitic infection of the central nervous system involving the cerebrum. However, spinal involvement of NCC is rare. Spinal NCC can cause radiculopathy, myelopathy, cauda equina syndrome, and even paraparesis, depending on its location and size. Spinal NCC may require surgical treatment as a first-line treatment because medical therapy can further aggravate the inflammation due to dead cysts, resulting in clinical deterioration. The current standard therapy for spinal NCC is surgical decompression followed by medical therapy. We experienced a case of widespread thoracolumbar intradural extramedullary cysticercosis involving the spinal canal with cerebral cysticercosis. We report this rare case with literature review.
Central Nervous System ; Cerebrum ; Cysticercosis* ; Decompression, Surgical ; Humans ; Inflammation ; Neurocysticercosis ; Paraparesis ; Polyradiculopathy ; Radiculopathy ; Spinal Canal* ; Spinal Cord Diseases ; Spine ; Taenia solium

Although the etiology of moyamoya disease (MMD) remains unknown, autoimmunity is one of the proposed pathogeneses. Unlike other autoimmune disorders that are associated with cerebral arteritis, concurrence of MMD and diabetes mellitus (DM) is rare. However, we encountered a patient with concurrent diabetic ketoacidosis (DKA) and acute ischemic stroke due to MMD. Our patient was diagnosed with glutamic acid decarboxylase antibody-positive type 2 DM (T2DM) based on laboratory and physical examination findings. Brain magnetic resonance images revealed an acute ischemic stroke in the left cerebral hemisphere and bilateral diffuse stenosis/occlusion in the middle cerebral artery and multiple collaterals. Thus, here, we report a patient with both T2DM and MMD who developed an acute ischemic stroke that was complicated by DKA.
Arteritis ; Autoimmunity ; Brain ; Cerebrum ; Diabetes Mellitus ; Diabetes Mellitus, Type 2 ; Diabetic Ketoacidosis* ; Glutamate Decarboxylase ; Humans ; Middle Cerebral Artery ; Moyamoya Disease* ; Physical Examination ; Stroke*

An 8-year-old female Eurasian otter (Lutra lutra) reared in a wetland center, died 2 h after sudden onset of astasia and dyspnea despite medical treatment. Gross examination of internal organs revealed 10 adult filarioid nematodes in the right ventricle of the heart and three between the left and right cerebral hemispheres. All nematodes were identified as Dirofilaria immitis by direct microscopy and polymerase chain reaction assay. Histopathological observation revealed multifocal hemorrhage in the cerebral subarachnoid space and focal necrosis with hemorrhagein the cerebellar parenchyma. Although rare, veterinarians should consider cerebral dirofilariasis as a differential diagnosis in unexplained neurological cases.
Adult ; Animals ; Brain ; Cerebrum ; Child ; Diagnosis, Differential ; Dirofilaria immitis ; Dirofilaria ; Dirofilariasis ; Dyspnea ; Female ; Heart ; Heart Ventricles ; Hemorrhage ; Humans ; Korea ; Microscopy ; Necrosis ; Otters ; Polymerase Chain Reaction ; Subarachnoid Space ; Veterinarians ; Wetlands