Background: This study aims to elucidate the effect of isolated cerebellar lesions sparing the brainstem on the auditory brainstem responses in children.
Methods: We enrolled 10 children (aged 1-16 years) with cerebellar lesions on neuroimaging but lacking clinical brainstem involvement signs and with normal brainstem volumes on magnetic resonance imaging.
Results: The interpeak latency of waves I and V was normal in 9 patients and was marginally prolonged in 1 patient. While amplitudes of waves I and III were normal, we noted a decreased amplitude of wave V and/or an increased I/V amplitude ratio in 6 patients; these included 5 of 8 patients with cerebellar hypoplasia/atrophy and 1 patient with acute cerebellar ataxia.
Conclusion: Our results support the hypothesis of an inhibitory input from the cerebellar fastigial nucleus on the inferior colliculus, which might be disinhibited because of Purkinje cells dysfunction due to cerebellar cortex lesions, especially within the cerebellar vermis.
Download full-text PDF |
Source |
---|---|
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6308102 | PMC |
http://dx.doi.org/10.33160/yam.2018.12.005 | DOI Listing |
Neurol Sci
January 2025
Department of Neurology, Guangdong Provincial Key Laboratory of Diagnosis and Treatment of Major Neurological Diseases, National Key Clinical Department and Key Discipline of Neurology, The First Affiliated Hospital, Sun Yat-Sen University, Guangzhou, 510080, China.
Background And Objectives: Vanishing white matter disease (VWMD) is an autosomal recessive leukoencephalopathy caused by mutations in the EIF2B1-5 genes, typically rare in adulthood. We present a case of adult-onset VWMD with a novel EIF2B2 mutation.
Methods: We collected the patient's clinical data, cerebrospinal fluid (CSF) results, laboratory tests, imaging features, genetic analysis, and follow-up data over a 4-year period.
Zhonghua Bing Li Xue Za Zhi
January 2025
Department of Pathology, the First Affiliated Hospital of Zhengzhou University, Zhengzhou450052, China.
To investigate the clinicopathological and molecular genetic characteristics of intracranial mesenchymal tumors with FET::CREB fusion transcript. The clinical and imaging data of 6 cases of intracranial mesenchymal tumors with FET::CREB fusion from December 2018 to December 2023 were collected at the First Affiliated Hospital of Zhengzhou University. Their histological features, immunophenotype and molecular characteristics were analyzed.
View Article and Find Full Text PDFFront Oncol
December 2024
Head and Neck Oncology Ward, West China Hospital of Sichuan University, Chengdu, China.
Background: Histiocytic sarcoma originates in various tissues, including the skin, lymph nodes, gastrointestinal tract, lungs, bone marrow, and central nervous system. Primary central nervous system histiocytic sarcoma (PCNSHS) is exceptionally rare, known for its aggressive behavior and poor prognosis. This report describes a case of PCNSHS in the cerebellum treated with surgery and radiotherapy.
View Article and Find Full Text PDFRadiol Case Rep
March 2025
Department of Radiology, Medical School, University of Ioannina, Stavros Niarchos Avenue, Ioannina 45500, Greece.
Mitochondrial neurogastrointestinal encephalopathy (MNGIE) is a rare autosomal recessive disorder, manifesting with gastrointestinal dysmotility, cachexia, ptosis and peripheral neuropathy. Diffuse leukoencephalopathy in brain MRI is a hallmark of MNGIE. We report a case of a 21-year-old female with MNGIE, presenting with cachexia and chronic diarrhea.
View Article and Find Full Text PDFPediatr Radiol
January 2025
Department of Radiology, Korea University Ansan Hospital, Korea University College of Medicine, Ansan, Republic of Korea.
Background: Cerebellar hemorrhage in neonates is increasingly being identified but is still underdiagnosed. While magnetic resonance imaging (MRI) is the optimal imaging modality for cerebellar hemorrhage evaluation, ultrasonography (US) is commonly used for screening. Characterizing the patterns and distribution of cerebellar hemorrhage lesions can help facilitate its detection by aiding to focus on prevailing type of cerebellar hemorrhage.
View Article and Find Full Text PDFEnter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!