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Cureus Jul 2023Arnold-Chiari malformation (ACM) is a rare neurological developmental disorder that presents at birth. No such cases have been reported in support of microbial...
Arnold-Chiari malformation (ACM) is a rare neurological developmental disorder that presents at birth. No such cases have been reported in support of microbial infections causing Chiari malformation, yet there is evidence of how microorganisms can lead to brain abscess, brain empyema, and meningoencephalitis. We present a 23-year-old young woman with progressive back and leg pain after a streptococcal throat infection, followed by a single episode of syncope. Radiographs of the spine revealed a mild reversal of cervical spine curvature and minimal levocurvature of the lumbar spine. Magnetic resonance imaging of the brain showed herniation of the cerebral tonsils into the foramen magnum, which suggested the diagnosis of ACM type I. Neurosurgery was recommended for posterior fossa decompression, but the patient was reluctant to undergo the procedure. This is a rare case of ACM symptomology that became evident only after a streptococcal throat infection in a young adult female.
PubMed: 37593297
DOI: 10.7759/cureus.42024 -
Neurological Sciences : Official... Dec 2023
Topics: Humans; Torticollis; Syringomyelia; Arnold-Chiari Malformation; Cervical Vertebrae
PubMed: 37592123
DOI: 10.1007/s10072-023-06984-6 -
Journal of Clinical Medicine Jul 2023Chiari malformations are a group of craniovertebral junction anomalies characterized by the herniation of cerebellar tonsils below the foramen magnum, often accompanied... (Review)
Review
Chiari malformations are a group of craniovertebral junction anomalies characterized by the herniation of cerebellar tonsils below the foramen magnum, often accompanied by brainstem descent. The existing classification systems for Chiari malformations have expanded from the original four categories to nine, leading to debates about the need for a more descriptive and etiopathogenic terminology. This review aims to examine the various classification approaches employed and proposes a simplified scheme to differentiate between different types of tonsillar herniations. Furthermore, it explores the most appropriate terminology for acquired herniation of cerebellar tonsils and other secondary Chiari-like malformations. Recent advances in magnetic resonance imaging (MRI) have revealed a higher prevalence and incidence of Chiari malformation Type 1 (CM1) and identified similar cerebellar herniations in individuals unrelated to the classic phenotypes described by Chiari. As we reassess the existing classifications, it becomes crucial to establish a terminology that accurately reflects the diverse presentations and underlying causes of these conditions. This paper contributes to the ongoing discussion by offering insights into the evolving understanding of Chiari malformations and proposing a simplified classification and terminology system to enhance diagnosis and management.
PubMed: 37510741
DOI: 10.3390/jcm12144626 -
Boletin Medico Del Hospital Infantil de... 2023Scoliosis is a spinal deformity that usually manifests as a structural curve determining a variable deformity of the trunk. According to some published series, 4 to 26%...
BACKGROUND
Scoliosis is a spinal deformity that usually manifests as a structural curve determining a variable deformity of the trunk. According to some published series, 4 to 26% of scoliosis initially classified as idiopathic show neurological alterations when studied with nuclear magnetic resonance, such as syringomyelia and Chiari malformation, among the most frequent. Chiari malformations are characterized by descending herniation of the cerebellar tonsils, brainstem, and IV ventricle into the spinal canal.
CASE REPORT
We present the case of a patient whose first symptomatic manifestation was early-onset scoliosis. The overlapping of some physical examination signs, such as postural lateralization and scoliosis, reinforced the active suspicion of neuroaxis alterations.
CONCLUSIONS
Early childhood-onset scoliosis should raise a high degree of suspicion for association with neuro-spinal diseases. Although the frequency of Chiari malformation is low, its early approach could reduce the progression of associated comorbidities. An early detection could change the prognosis of the disease.
Topics: Humans; Child, Preschool; Arnold-Chiari Malformation; Syringomyelia; Scoliosis; Magnetic Resonance Imaging; Decompression, Surgical
PubMed: 37490682
DOI: 10.24875/BMHIME.M23000069 -
The Journal of Craniofacial SurgeryHere, we focused on the association between minor suture fusion and Chiari malformation (CM) occurrence in nonsyndromic craniosynostosis (NSC), and evaluated how the...
Here, we focused on the association between minor suture fusion and Chiari malformation (CM) occurrence in nonsyndromic craniosynostosis (NSC), and evaluated how the minor suture affects the posterior cranial fossa by measuring the posterior fossa deflection angle (PFA). In this retrospective study, the clinical records of 137 patients who underwent surgery for NSC at Aichi Children's Health and Medical Center between April 2010 and May 2022 were analyzed. Clinical data from Aichi Developmental Disability Center Central Hospital was collected for 23 patients as the external validation set. Among the 137 patients, 123 were diagnosed with NSC and the remaining 14 with syndromic craniosynostosis. Of the 123 NSC patients, 23 patients presented with CM. Multivariate analysis showed that occipito-mastoid fusion was the only significant risk factor for CM ( P =0.0218). Within the NSC group, CM patients had a significantly increased PFA (6.33±8.10 deg) compared with those without CM (2.76±3.29 deg, P =0.0487). Nonsyndromic craniosynostosis patients with occipito-mastoid suture fusion had a significantly increased PFA (6.50±7.60 deg) compared with those without occipito-mastoid fusion (2.60±3.23 deg, P =0.0164). In the validation cohort, occipito-mastoid suture fusion was validated as an independent risk factor for CM in univariate analysis. Minor suture fusion may cause CM associated with NSC. Chiari malformation could develop due to an increased PFA due to minor suture fusion, which causes growth disturbance in the affected side and compensatory dilation in the contralateral side within the posterior cranial fossa.
Topics: Child; Humans; Retrospective Studies; Craniosynostoses; Neurosurgical Procedures; Decompression, Surgical; Arnold-Chiari Malformation; Sutures
PubMed: 37485951
DOI: 10.1097/SCS.0000000000009552 -
Cureus Jun 2023Chiari malformation 1 (CM1) is defined as a herniation of encephalon matter through the base of the skull. The amount of herniation is cited as greater than 3 mm or 5...
Chiari malformation 1 (CM1) is defined as a herniation of encephalon matter through the base of the skull. The amount of herniation is cited as greater than 3 mm or 5 mm, depending on the source of literature. We report a rare case of a 55-year-old male initially presenting with bilateral papilledema and monocular right lower quadrantanopia, found to have CM1. An MRI confirmed 4.87 mm herniation of the cerebellar tonsils at the foramen magnum, and he was diagnosed with CM1. He was later found to have a normal opening pressure on lumbar puncture at 10 cm HO. This poses an interesting clinical question as papilledema is defined by elevated intracranial pressure. The ophthalmic defects of this patient with normal intracranial pressure and CM1 are explored in this report.
PubMed: 37476137
DOI: 10.7759/cureus.40652 -
Anatomy & Cell Biology Sep 2023This study was conducted to investigate the clinical and anatomical importance of the relevant region from the perspective of surgical approaches by determining the...
This study was conducted to investigate the clinical and anatomical importance of the relevant region from the perspective of surgical approaches by determining the morphometric analysis of the craniocervical junction and foramen magnum (FM) region and determining their distances from important anatomical points. This research was carried out with 59 skulls found at the Anatomy Laboratories of Erciyes and Ankara Medipol University. Metric measurements of FM and condyle, FM shape, condyle-fossa relationship, and pharyngeal tubercle (PT) were made in mm-based dry bone samples of unknown age and sex. The distance between the anterior notches and the FM was 87.01±4.35, the distance between the anterior notches and the PT was 77.70±4.24, the distance between the PT-sphenooccipital junction was 13.23±2.42, and the FM index was 81.86±7.47. The anteroposterior and transverse lengths of FM were determined as 33.80±2.99 and 27.72±2.30, respectively. The morphometric and morphological data available regarding the craniocervical junction showed significant differences between populations. Comprehensive knowledge of this topic will provide a better approach to treat Arnold Chiari Malformation, FM meningiomas, and other posterior cranial fossa lesions. Therefore, we believe that FM and craniocervical junction morphology will be a guide not only for anatomists, but also for radiologists, neurosurgeons, ENT surgeons, and orthopedists.
PubMed: 37460407
DOI: 10.5115/acb.23.006 -
In Vivo (Athens, Greece) 2023
Letter to the Editor: RE: Reyes-Ruiz : Encephalitis Associated With SARS-CoV-2 Infection in a Child With Chiari Malformation Type I. In Vivo : 933-939, 2023 - SARS-CoV-2-related Encephalitis Does Not Adequately Explain Oral Ulcers and Odynophagia.
Topics: Humans; Child; Arnold-Chiari Malformation; COVID-19; SARS-CoV-2; Oral Ulcer; Encephalitis; Pain
PubMed: 37369503
DOI: 10.21873/invivo.13287 -
Journal of Neurosurgery. Spine Oct 2023The authors analyzed the National Spina Bifida Patient Registry (NSBPR), a national registry that tracks the outcomes for people with various forms of spinal dysraphism,...
OBJECTIVE
The authors analyzed the National Spina Bifida Patient Registry (NSBPR), a national registry that tracks the outcomes for people with various forms of spinal dysraphism, to determine the ongoing longitudinal risk for tethered cord release (TCR) among adults with myelomeningocele who had not previously undergone TCR during childhood. The authors also sought to identify the impact of lesion level, ambulation status, and prior treatments for hydrocephalus or Chiari malformations on TCR rates.
METHODS
Adults in the registry who had not previously undergone TCR during childhood were studied. This group was compared with the remaining adults in the registry. The frequency of first-time TCR and time to TCR (using Kaplan-Meier analysis) were determined independently for males and females. Cox proportional hazards analysis identified correlations between sex, best lesion level and ambulation status prior to TCR, and previous treatments for hydrocephalus and Chiari decompression.
RESULTS
Among 967 adults in the NSBPR (422 [43.6%] males and 545 [56.4%] females) who had not undergone TCR during childhood, the authors identified 47 people (4.9%) who underwent their first TCR during adulthood. This study cohort had significantly better mean functional motor levels and ambulation compared with the remaining adult cohort (both p < 0.001). The study group included 35 females (74.5%) and 12 males (25.5%); this sex distribution was significantly different in comparison with the remaining adult cohort (p = 0.016). The Kaplan-Meier curves for first TCR for females and males were significantly different (p = 0.01, log-rank test). TCR rates were correlated with sex (males had decreased risk; OR 0.31, 95% CI 0.16-0.62, p < 0.001), prior treatment for hydrocephalus (those who underwent prior treatment had decreased risk; OR 0.21, 95% CI 0.20-0.42, p < 0.001), and prior treatment for Chiari malformation (those who underwent prior treatment had greater risk; OR 3.84, 95% CI 1.50-9.88, p = 0.005).
CONCLUSIONS
Adults with myelomeningocele who escape childhood without undergoing TCR have an ongoing, albeit decreased, risk for spinal cord tethering requiring TCR. This risk is obviously not due to spinal column growth and therefore must reflect other factors such as dynamic changes in spinal cord health over time. Among people with MMC who underwent their first TCR as adults, females seemed to be overrepresented. Similar to the authors' prior childhood study, people who underwent previous Chiari decompression seemed to be overrepresented, whereas those who underwent previous treatment for hydrocephalus seem to be underrepresented. These novel findings deserve further study.
Topics: Male; Female; Adult; Humans; Meningomyelocele; Spinal Dysraphism; Neural Tube Defects; Arnold-Chiari Malformation; Hydrocephalus; Registries; Receptors, Antigen, T-Cell
PubMed: 37347622
DOI: 10.3171/2023.5.SPINE2328 -
Turkish Journal of Ophthalmology Jun 2023Eight syndrome is defined as the combination of a unilateral conjugate gaze palsy and ipsilateral seventh cranial nerve palsy. It may occur as a result of demyelinating,...
Eight syndrome is defined as the combination of a unilateral conjugate gaze palsy and ipsilateral seventh cranial nerve palsy. It may occur as a result of demyelinating, vascular, infectious, or compressive lesions of the brainstem localized to the caudal pontine tegmentum. A 43-year-old woman was admitted to our clinic with complaints of headache, inability to look to the left, and weakness on the left side of her face. The complaints had begun abruptly about a month before her admission. Suboccipital decompression surgery for type I Chiari malformation had been performed 10 years earlier. Neuro-ophthalmological examination revealed left-sided horizontal gaze palsy and anisocoria. Cranial and cervical magnetic resonance images revealed cerebellar tonsillar herniation and syringomyelia, the latter of which was considered to be the cause of eight syndrome. No interventions were performed, and periodic follow-up was advised on neurosurgical consultation. Left gaze palsy and facial palsy recovered almost completely in three months, while the anisocoria persisted. Syringomyelia should be considered among the causes of horizontal gaze palsy plus ipsilateral seventh nerve palsy, termed as eight syndrome. Clinical suspicion and appropriate radiological examination can aid in the diagnosis.
Topics: Female; Humans; Adult; Syringomyelia; Anisocoria; Arnold-Chiari Malformation; Magnetic Resonance Imaging; Strabismus; Paralysis
PubMed: 37345329
DOI: 10.4274/tjo.galenos.2023.19054