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Pediatric and Developmental Pathology :... 2022Spina bifida (SB) is the most common neural tube defect in humans. Here, we analyzed systematically the neuropathological findings of the brain in SB cases.
INTRODUCTION
Spina bifida (SB) is the most common neural tube defect in humans. Here, we analyzed systematically the neuropathological findings of the brain in SB cases.
METHODS
79 cases with SB aperta (SBA) and 6 cases with SB occulta (SBO) autopsied at the Charité Neuropathology from 1974 to 2000 were re-evaluated retrospectively. For this, case files and spinal cord as well as brain sections were studied.
RESULTS
While no brain malformations were detected in SBO cases, 95% of SBA cases had brain malformations. Main brain anomalies identified were hydrocephalus (71%), Chiari II malformation (36%), heterotopia (34%), other cerebellar anomalies (36%), gyrification defects (33%), and ependymal denudation (29%). Hydrocephalus was observed as early as gestational week 17 and was highly associated to Chiari II and ependymal denudation. In 55% SBA was accompanied by further anomalies not primarily affecting the CNS.
CONCLUSION
We confirm using neuropathologic methods brain malformations in most SBA but none in SBO cases. In addition to our previous radiologic study, we now demonstrate the high prevalence of cerebellar malformations and cerebral heterotopias in SBA. The early detection of hydrocephalus and Chiari II malformation in fetuses raises the question whether these arise parallel rather than in strict temporal sequence.
Topics: Arnold-Chiari Malformation; Humans; Hydrocephalus; Nervous System Malformations; Retrospective Studies; Spinal Dysraphism
PubMed: 34614376
DOI: 10.1177/10935266211040500 -
Psychiatria Danubina 2020
Topics: Adult; Arnold-Chiari Malformation; Bipolar Disorder; Female; Humans; Panic Disorder
PubMed: 32303050
DOI: No ID Found -
Anesthesia Progress 2019Type II Arnold-Chiari malformation (ACM) is an abnormality in which the cerebellum, pons, and medulla oblongata are displaced downward into the spinal cord. Type II ACM...
Type II Arnold-Chiari malformation (ACM) is an abnormality in which the cerebellum, pons, and medulla oblongata are displaced downward into the spinal cord. Type II ACM is often complicated by respiratory depression, sleep-disordered breathing, and deglutition disorder as a result of medullary dysfunction and impairment of the lower cranial nerves. Bending and stretching of the neck is restricted, and anesthetic management is problematic in patients with the disorder. We performed dental treatment twice under intravenous sedation in a patient with intellectual disability with type II ACM complicated by hypercapnic respiratory failure. Propofol was used for the first sedation procedure. Repeated bouts of respiratory depression occurred on that occasion, so the airway was managed manually by lifting the jaw. However, aspiration pneumonitis occurred postoperatively. A combination of dexmedetomidine and midazolam was used for sedation on the second occasion, and the intervention was completed uneventfully without any respiratory depression. Our experience with this patient highlights the need for selection of an agent for intravenous sedation that does not require neck extension and has minimal effect on respiration in patients with type II ACM, who are at high risk of respiratory depression and pulmonary aspiration.
Topics: Adult; Anesthesia; Arnold-Chiari Malformation; Humans; Hypercapnia; Male; Propofol; Respiratory Insufficiency
PubMed: 30883227
DOI: 10.2344/anpr-66-01-06 -
Clinics (Sao Paulo, Brazil) 2019Basilar invagination (BI) and Chiari malformation type I (CM-I) are very important anomalies that introduce instability and compression in the occipitocervical... (Review)
Review
Basilar invagination (BI) and Chiari malformation type I (CM-I) are very important anomalies that introduce instability and compression in the occipitocervical transition region and have complex clinical characteristics. These anomalies vary according to the affected structures. The present study revises current knowledge regarding the anatomy, anatomo-physiology, clinical manifestations, and radiological findings of these entities and the associated surgical treatment approaches. A bibliographic survey was performed through a search in the Medline, PubMed, SciELO, Science and LILACS databases. When associated, these craniovertebral malformations result in neurological deficits due to neural parenchyma compression; however, the presence of microtraumas due to repetitive lesions caused by the bulb and cervical marrow instability has been highlighted as a determinant dysfunction. Surgical treatment is controversial and has many technical variations. Surgery is also challenging due to the complex anatomical characteristics and biomechanics of this region. Nevertheless, advances have been achieved in our understanding of related mechanisms, and compression and atlantoaxial instability are considered key elements when selecting the surgical approach.
Topics: Arnold-Chiari Malformation; Decompression, Surgical; Humans; Joint Instability; Magnetic Resonance Imaging; Odontoid Process; Platybasia
PubMed: 30970117
DOI: 10.6061/clinics/2019/e653 -
The Pan African Medical Journal 2023
Topics: Humans; Arnold-Chiari Malformation; Hydrocephalus; Meningomyelocele
PubMed: 37275283
DOI: 10.11604/pamj.2023.44.115.36848 -
Medicine May 2016SPOndylar and NAsal changes, with STRIations of the Metaphyses (SPONASTRIME) dysplasia (SD) is a dwarfing autosomal recessive syndrome, characterized by a variety of...
SPOndylar and NAsal changes, with STRIations of the Metaphyses (SPONASTRIME) dysplasia (SD) is a dwarfing autosomal recessive syndrome, characterized by a variety of clinical and radiographic features, which form the basis for diagnosis. We describe the presentation of an Arnold Chiari malformation in a patient with a clinical diagnosis of SD. The malformation was successfully treated by decompression of the foramen magnum and elevation of the cerebellum, with complete resolution of pain.We report a rare case of Arnold Chiari malformation in a patient presenting with clinical and radiographic features strongly suggestive of SD and be successfully treated.
Topics: Adult; Arnold-Chiari Malformation; Cervical Vertebrae; Decompression, Surgical; Foramen Magnum; Humans; Magnetic Resonance Imaging; Male; Osteochondrodysplasias; Radiography; Spinal Cord; Syringomyelia; Treatment Outcome
PubMed: 27149441
DOI: 10.1097/MD.0000000000003155 -
Tidsskrift For Den Norske Laegeforening... Jun 2019Chiari malformation denotes a pathological caudal ectopy of the cerebellar tonsils below the level of the foramen magnum. Several types of the condition exist, of which... (Review)
Review
Chiari malformation denotes a pathological caudal ectopy of the cerebellar tonsils below the level of the foramen magnum. Several types of the condition exist, of which Type 1 is the most common. It often results in few if any symptoms, and in many cases is detected as an incidental finding when an MRI is performed. Symptoms such as headache, dizziness or nausea may be related to narrowing of the foramen magnum due to blocked circulation of spinal fluid. Surgical treatment must be considered in symptomatic cases.
Topics: Arnold-Chiari Malformation; Humans; Magnetic Resonance Imaging
PubMed: 31238671
DOI: 10.4045/tidsskr.18.0455 -
AJNR. American Journal of Neuroradiology 1987Four normal controls and 26 cases of Arnold-Chiari I malformations and/or syringomyelia were reviewed. The pathologic cases included five isolated Arnold-Chiari I... (Comparative Study)
Comparative Study
Four normal controls and 26 cases of Arnold-Chiari I malformations and/or syringomyelia were reviewed. The pathologic cases included five isolated Arnold-Chiari I malformations, nine communicating syringomyelia, five idiopathic syringomyelia, four posttraumatic syringomyelia, one syringomyelia with hemangioblastoma, and two postshunt syringomyelia. The objectives of this study were to compare the accuracy of conventional metrizamide myelography with CT metrizamide myelography and to study indirectly the hydrodynamics of CSF flow in syringomyelia by comparing the sequential enhancement patterns of the spinal cords and cord cavities in the different groups of patients. Twenty-five patients underwent conventional metrizamide myelography immediately before CT metrizamide myelography, and one patient underwent CT metrizamide myelography only. Scans were obtained 1-2 hr, 4-8 hr, and 12-24 hr after injection of metrizamide, but not all patients were scanned during all three intervals. CT metrizamide myelography was found to be more sensitive than conventional metrizamide myelography in the diagnosis of both Arnold-Chiari I malformation and syringomyelia. Performing just an immediate and a delayed scan was found to be more cost-effective than doing all three scans. Contrary to previous reports, it was found that delayed (12-24 hr) scans demonstrated more syrinx cavities than intermediate ones. In studying the sequential enhancement patterns of the spinal cords and cord cavities, some interesting trends were observed that tend to support the theories of Aboulker and of Ball and Dayan of transneural passage of CSF into cord cavities in syringomyelia.
Topics: Adult; Aged; Arnold-Chiari Malformation; Female; Humans; Male; Metrizamide; Middle Aged; Myelography; Radiographic Image Enhancement; Spinal Cord; Syringomyelia; Time Factors; Tomography, X-Ray Computed
PubMed: 3101451
DOI: No ID Found -
Neurology India 2019
Topics: Adult; Arnold-Chiari Malformation; Decompression, Surgical; Foramen Magnum; Humans
PubMed: 31347529
DOI: 10.4103/0028-3886.263222 -
Neurologia (Barcelona, Spain) Jun 2014Chiari type I malformation (CM-I) is characterised by caudal ectopia of the cerebellar tonsils through the foramen magnum. This is associated with brain stem, high... (Review)
Review
INTRODUCTION
Chiari type I malformation (CM-I) is characterised by caudal ectopia of the cerebellar tonsils through the foramen magnum. This is associated with brain stem, high spinal cord, and cranial nerve compression phenomena. The most frequent symptoms are occipital headaches and dizziness. Less well-known symptoms are sleep disorders and nocturnal respiratory abnormalities.
SOURCES
MEDLINE and information from patients evaluated at the Neurosurgery and Clinical Neurophysiology Departments at Hospital Universitario Vall d'Hebron.
DEVELOPMENT
Review article based on data obtained from MEDLINE articles since 1966, using combinations of the following keywords: «Chiari malformation» or «Arnold-Chiari malformation» and «sleep apnea» or «sleep disorders».
CONCLUSIONS
CM-I patients show a higher prevalence of sleep disorders than that observed in the general population. Some studies report a 50% prevalence of sleep apnea-hypopnea syndrome (SAHS), probably associated with sudden death in some cases. These results support analysing sleep respiratory parameters in theses patients. Identifying SAHS symptoms may help optimise treatment, thereby improving quality of life and prognosis.
Topics: Arnold-Chiari Malformation; Brain Stem; Humans; Polysomnography; Quality of Life; Sleep Apnea Syndromes; Sleep Wake Disorders
PubMed: 21420201
DOI: 10.1016/j.nrl.2011.01.008