ArticleQuantitative imaging in medicine and surgery2016
Isolated macrocerebellum: description of six cases and literature review.
Article in Quantitative imaging in medicine and surgery, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 4 papers.
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4 citing papers in PubMed, 6 citations in OpenAlex.
- Genetic Insights in Hindbrain Abnormalities Through Network Analysis Expose Key Biological Pathways in Hindbrain Development.Cerebellum (London, England) · 2025Article
- "Growing cerebellum" requiring operative decompression following perinatal ventriculoperitoneal shunting.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2021Review
- Macrocerebellum in Achondroplasia: A Further CNS Manifestation ofAJNR. American journal of neuroradiology · 2020Article
- Neuroradiological findings in three cases of pontocerebellar hypoplasia type 9 due toQuantitative imaging in medicine and surgery · 2019Article
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Authors and funding
7 authors at 3 institutions in 2 countries.
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No grant is acknowledged in the PubMed record.
Abstract
backgroundMacrocerebellum is a rare entity described as an isolated and abnormal increase of the cerebellum (CB) size without morphological or signal abnormalities. There have been only eleven patients with macrocerebellum reported in the literature so far.
methodsFrom December 2011 to March 2014, among 950 paediatric patients that underwent a magnetic resonance scan of the brain in our department, in six subjects an abnormal increase of the cerebellar volume was suspected. A volumetric analysis was performed in all patients on T1- weighted 3D imaging to confirm the diagnosis of macrocerebellum. The ratios between (I) volume of the CB and volume of the supratentorial structures (STB) and (II) volume of the CB and the sum of CB and STB (WB) were calculated in order to normalize the absolute values obtained and compared with the normal values present in literature. RESULTS AND DISCUSSION: Quantitative analysis confirmed an increased cerebellar volume relatively to the STB volume ("t": 6.9518; P<0.001) and to the WB ("t": 7.1415; P<0.001) volume in comparison to the normal controls available in literature. Clinical characteristics and other neuroradiological findings of the patients are described. We also describe the differential features between isolated macrocerebellum and other pathological conditions that are characterized by cerebellar enlargement such as Lhermitte-Duclos, Sotos syndrome, Costello syndrome, Williams syndrome, Alexander disease and fucosidosis. Furthermore a detailed literature review is provided. Macrocerebellum is always associated with an abnormal mental and motor development.
conclusionMacrocerebellum is a neuroradiological entity that can be identified qualitatively and confirmed quantitatively through volumetric analysis. This is the largest cohort of patients with macrocerebellum described so far. The data available in literature on this entity show that macrocerebellum is not a specific disease but an epiphenomenon found in heterogeneous brain disorders.
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