The Small Posterior Cranial Fossa Syndrome and Chiari Malformation Type 0
Patients showing typical Chiari malformation type 1 (CM1) signs and symptoms frequently undergo cranial and cervical MRI. In some patients, MRI documents >5 mm of cerebellar tonsillar herniation (TH) and the diagnosis of CM1. Patients with 3–5 mm TH have “borderline” CM1. Patients with less than...
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| Format: | Article |
| Language: | English |
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MDPI AG
2022-09-01
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| Series: | Journal of Clinical Medicine |
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| Online Access: | https://www.mdpi.com/2077-0383/11/18/5472 |
| _version_ | 1797486569591930880 |
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| author | Enver I. Bogdanov Aisylu T. Faizutdinova John D. Heiss |
| author_facet | Enver I. Bogdanov Aisylu T. Faizutdinova John D. Heiss |
| author_sort | Enver I. Bogdanov |
| collection | DOAJ |
| description | Patients showing typical Chiari malformation type 1 (CM1) signs and symptoms frequently undergo cranial and cervical MRI. In some patients, MRI documents >5 mm of cerebellar tonsillar herniation (TH) and the diagnosis of CM1. Patients with 3–5 mm TH have “borderline” CM1. Patients with less than 3 mm of TH and an associated cervical syrinx are diagnosed with Chiari “zero” malformation (CM0). However, patients reporting CM1 symptoms are usually not diagnosed with CM if MRI shows less than 3–5 mm of TH and no syrinx. Recent MRI morphometric analysis of the posterior fossa and upper cervical spine detected anatomical abnormalities in and around the foramen magnum (FM) that explain these patients’ symptoms. The abnormalities include a reduced size of the posterior fossa, FM, and upper cervical spinal canal and extension of the cerebellar tonsils around the medulla rather than inferior to the foramen magnum, as in CM1. These morphometric findings lead some neurologists and neurosurgeons to diagnose CM0 in patients with typical CM1 signs and symptoms, with or without cervical syringes. This article reviews recent findings and controversies about CM0 diagnosis and updates current thinking about the clinical and radiological relationship between CM0, borderline CM1, and CM1. |
| first_indexed | 2024-03-09T23:36:01Z |
| format | Article |
| id | doaj.art-63120cb1b8e84c08897279dc98e1f9d9 |
| institution | Directory Open Access Journal |
| issn | 2077-0383 |
| language | English |
| last_indexed | 2024-03-09T23:36:01Z |
| publishDate | 2022-09-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Journal of Clinical Medicine |
| spelling | doaj.art-63120cb1b8e84c08897279dc98e1f9d92023-11-23T17:01:21ZengMDPI AGJournal of Clinical Medicine2077-03832022-09-011118547210.3390/jcm11185472The Small Posterior Cranial Fossa Syndrome and Chiari Malformation Type 0Enver I. Bogdanov0Aisylu T. Faizutdinova1John D. Heiss2Department of Neurology and Rehabilitation, Kazan State Medical University, 420012 Kazan, RussiaDepartment of Neurology and Rehabilitation, Kazan State Medical University, 420012 Kazan, RussiaSurgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892, USAPatients showing typical Chiari malformation type 1 (CM1) signs and symptoms frequently undergo cranial and cervical MRI. In some patients, MRI documents >5 mm of cerebellar tonsillar herniation (TH) and the diagnosis of CM1. Patients with 3–5 mm TH have “borderline” CM1. Patients with less than 3 mm of TH and an associated cervical syrinx are diagnosed with Chiari “zero” malformation (CM0). However, patients reporting CM1 symptoms are usually not diagnosed with CM if MRI shows less than 3–5 mm of TH and no syrinx. Recent MRI morphometric analysis of the posterior fossa and upper cervical spine detected anatomical abnormalities in and around the foramen magnum (FM) that explain these patients’ symptoms. The abnormalities include a reduced size of the posterior fossa, FM, and upper cervical spinal canal and extension of the cerebellar tonsils around the medulla rather than inferior to the foramen magnum, as in CM1. These morphometric findings lead some neurologists and neurosurgeons to diagnose CM0 in patients with typical CM1 signs and symptoms, with or without cervical syringes. This article reviews recent findings and controversies about CM0 diagnosis and updates current thinking about the clinical and radiological relationship between CM0, borderline CM1, and CM1.https://www.mdpi.com/2077-0383/11/18/5472small posterior cranial fossaChiari malformation type 0Chiari malformation type 1cerebellar tonsil herniationmorphometric analysissyringomyelia |
| spellingShingle | Enver I. Bogdanov Aisylu T. Faizutdinova John D. Heiss The Small Posterior Cranial Fossa Syndrome and Chiari Malformation Type 0 Journal of Clinical Medicine small posterior cranial fossa Chiari malformation type 0 Chiari malformation type 1 cerebellar tonsil herniation morphometric analysis syringomyelia |
| title | The Small Posterior Cranial Fossa Syndrome and Chiari Malformation Type 0 |
| title_full | The Small Posterior Cranial Fossa Syndrome and Chiari Malformation Type 0 |
| title_fullStr | The Small Posterior Cranial Fossa Syndrome and Chiari Malformation Type 0 |
| title_full_unstemmed | The Small Posterior Cranial Fossa Syndrome and Chiari Malformation Type 0 |
| title_short | The Small Posterior Cranial Fossa Syndrome and Chiari Malformation Type 0 |
| title_sort | small posterior cranial fossa syndrome and chiari malformation type 0 |
| topic | small posterior cranial fossa Chiari malformation type 0 Chiari malformation type 1 cerebellar tonsil herniation morphometric analysis syringomyelia |
| url | https://www.mdpi.com/2077-0383/11/18/5472 |
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