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Medicine May 2023Chiari malformation type I (CM-I) is a congenital anomaly of the hindbrain. The most common symptoms include suboccipital tussive headache, dizziness and neck pain....
Chiari malformation type I (CM-I) is a congenital anomaly of the hindbrain. The most common symptoms include suboccipital tussive headache, dizziness and neck pain. Recently there has been growing interest in the psychological and psychiatric aspects of functioning in patients with CM-I, which have a significant effect on treatment outcomes and quality of life (QoL). The aim of the study was to assess the severity of depressive symptoms and the QoL in patients with CM-I and to identify the main factors responsible for these phenomena. A total of 178 people participated in the study and were divided into 3 groups: patients with CM-I who had undergone surgery (n = 59); patients with CM-I who had not undergone surgery (n = 63); and healthy volunteers (n = 56). Psychological evaluation included a set of questionnaires: the Beck Depression Inventory II, the World Health Organization shortened version of the WHOQOL-100 quality of life questionnaire, the Acceptance of Illness Scale and the Beliefs about Pain Control Questionnaire. Results showed that the control group participants obtained significantly better results than both groups of CM-I patients in terms of all indicators of QoL, symptoms of depression, acceptance of illness, pain level (average and present) and perceived influence of doctors regarding coping with pain. Patients with CM-I (operated and non-operated) obtained similar results in most of the questionnaires and the QoL indices correlated significantly with the majority of the analyzed variables. Moreover, CM-I patients with higher depression scores described their pain as more severe and had a stronger belief that pain levels were not influenced by them but only by doctors, or that it could be controlled randomly; they were also less willing to accept their illness. CM-I symptoms affect the mood and QoL of patients. Psychological and psychiatric care should be the golden standard in managing this clinical group.
Topics: Humans; Depression; Quality of Life; Cross-Sectional Studies; Arnold-Chiari Malformation; Neck Pain; Surveys and Questionnaires
PubMed: 37335726
DOI: 10.1097/MD.0000000000033738 -
The Pan African Medical Journal 2023
Topics: Humans; Arnold-Chiari Malformation; Hydrocephalus; Meningomyelocele
PubMed: 37275283
DOI: 10.11604/pamj.2023.44.115.36848 -
American Journal of Veterinary Research Jul 2023To evaluate hearing loss in Cavalier King Charles Spaniels (CKCS), breed-specific brainstem auditory-evoked response (BAER) testing parameters are needed to help assess...
OBJECTIVE
To evaluate hearing loss in Cavalier King Charles Spaniels (CKCS), breed-specific brainstem auditory-evoked response (BAER) testing parameters are needed to help assess the Chiari-like malformation (CM) grade. The purpose of this study was to establish breed-specific BAER data and to determine if BAER indexes differed based on the CM grade. We hypothesized that there would be latency differences based on the CM grade.
ANIMALS
20 CKCS without apparent hearing abnormalities as assessed by the owners.
PROCEDURES
Under general anesthesia, CKCS underwent a CT scan (to assess the middle ear), BAER testing, and MRI (to assess the grade of CM).
RESULTS
No CKCS had CM0. Nine (45%) CKCS had CM1; 11 (55%) had CM2. All had at least 1 morphologic abnormality in waveforms. Absolute and interpeak latencies were reported for all CKCS and compared between CM grades. The median threshold for CKCS with CM1 was 39 and for CM2 was 46. Absolute latencies for CKCS with CM2 were consistently longer than those for CKCS with CM1 with the exception of waves II and V at 33 dB. Significant differences were found for wave V at 102 dB ( P = .04) and wave II at 74 dB (P = .008). Interpeak latency comparisons were inconsistent between CM1 and CM2.
CLINICAL RELEVANCE
Breed-specific BAER data for CKCS with CM1 and CM2 were established. The results suggest that CM impacts BAER latency results, but the influence of the malformation is not always statistically significant or predictable.
Topics: Dogs; Animals; Reaction Time; Dog Diseases; Arnold-Chiari Malformation; Tomography, X-Ray Computed; Brain Stem
PubMed: 37236609
DOI: 10.2460/ajvr.23.03.0064 -
Concurrent Presence of Thoracolumbar Scoliosis and Chiari Malformation: Is Operative Risk Magnified?Asian Spine Journal Aug 2023Retrospective review of Kids' Inpatient Database (KID).
STUDY DESIGN
Retrospective review of Kids' Inpatient Database (KID).
PURPOSE
Identify the risks and complications associated with surgery in adolescents diagnosed with Chiari and scoliosis.
OVERVIEW OF LITERATURE
Scoliosis is frequently associated with Chiari malformation (CM). More specifically, reports have been made about this association with CM type I in the absence of syrinx status.
METHODS
The KID was used to identify all pediatric inpatients with CM and scoliosis. The patients were stratified into three groups: those with concomitant CM and scoliosis (CMS group), those with only CM (CM group), and those with only scoliosis (Sc group). Multivariate logistic regressions were used to assess association between surgical characteristics and diagnosis with complication rate.
RESULTS
A total of 90,707 spine patients were identified (61.8% Sc, 37% CM, 1.2% CMS). Sc patients were older, had a higher invasiveness score, and higher Charlson comorbidity index (all p<0.001). CMS patients had significantly higher rates of surgical decompression (36.7%). Sc patients had significantly higher rates of fusions (35.3%) and osteotomies (1.2%, all p<0.001). Controlling for age and invasiveness, postoperative complications were significantly associated with spine fusion surgery for Sc patients (odds ratio [OR], 1.8; p<0.05). Specifically, posterior spinal fusion in the thoracolumbar region had a greater risk of complications (OR, 4.9) than an anterior approach (OR, 3.6; all p<0.001). CM patients had a significant risk of complications when an osteotomy was performed as part of their surgery (OR, 2.9) and if a spinal fusion was concurrently performed (OR, 1.8; all p<0.05). Patients in the CMS cohort were significantly likely to develop postoperative complications if they underwent a spinal fusion from both anterior (OR, 2.5) and posterior approach (OR, 2.7; all p<0.001).
CONCLUSIONS
Having concurrent scoliosis and CM increases operative risk for fusion surgeries despite approach. Being independently inflicted with scoliosis or Chiari leads to increased complication rate when paired with thoracolumbar fusion and osteotomies; respectively.
PubMed: 37226444
DOI: 10.31616/asj.2022.0077 -
Children (Basel, Switzerland) Mar 2023Gabriele-de Vries syndrome is a rare autosomal dominant genetic disease caused by de novo pathogenic variants in the ( gene. Individuals with this syndrome present with...
Gabriele-de Vries syndrome is a rare autosomal dominant genetic disease caused by de novo pathogenic variants in the ( gene. Individuals with this syndrome present with multiple congenital anomalies, as well as a delay in development and intellectual disability. Herein, we report the case of a newborn male patient with a novel de novo pathogenic variant in the Guanine Nucleotide-Binding Protein, Alpha Stimulating () gene, which was identified by whole-exome sequencing. Our patient suffered from a large open spinal dysraphism which was treated surgically immediately after birth. During the follow-up, facial dysmorphism, bladder and bowel incontinence, and mildly delayed motor and speech development were observed. Congenital central nervous system disorders were also confirmed radiologically. In this case report, we present our diagnostic and treatment approaches to this patient. To our knowledge, this is the first reported case of Gabriele-de Vries syndrome presenting with spinal dysraphism. Extensive genetic evaluation is the cornerstone in treatment of patients with suspected Gabriele-de Vries syndrome. However, in cases with potentially life-threatening conditions, surgery should be strongly considered.
PubMed: 37189872
DOI: 10.3390/children10040623 -
CMAJ : Canadian Medical Association... May 2023
Topics: Humans; Hiccup; Arnold-Chiari Malformation; Magnetic Resonance Imaging
PubMed: 37156552
DOI: 10.1503/cmaj.221870 -
Neurology India 2023
Topics: Humans; Elbow Joint; Syringomyelia; Arthropathy, Neurogenic; Arnold-Chiari Malformation
PubMed: 37148079
DOI: 10.4103/0028-3886.375402 -
Graefe's Archive For Clinical and... Dec 2023Acute acquired comitant esotropia (AACE) is an uncommon subtype of esotropia characterized by sudden and usually late onset of a relatively large angle of comitant... (Review)
Review
BACKGROUND
Acute acquired comitant esotropia (AACE) is an uncommon subtype of esotropia characterized by sudden and usually late onset of a relatively large angle of comitant esotropia with diplopia in older children and adults.
METHODS
A literature survey regarding neurological pathologies in AACE was conducted using databases (PubMed, MEDLINE, EMBASE, BioMed Central, the Cochrane Library, and Web of Science) in order to collect data for a narrative review of published reports and available literature.
RESULTS
The results of the literature survey were analyzed to provide an overview of the current knowledge of neurological pathologies in AACE. The results revealed that AACE with unclear etiologies can occur in many cases in both children and adults. Functional etiological factors for AACE were found to be due to many reasons, such as functional accommodative spasm, the excessive near work use of mobile phones/smartphones, and other digital screens. In addition, AACE was found to be associated with neurological disorders, such as astrocytoma of the corpus callosum, medulloblastoma, tumors of the brain stem or cerebellum, Arnold-Chiari malformation, cerebellar astrocytoma, Chiari 1 malformation, idiopathic intracranial hypertension, pontine glioma, cerebellar ataxia, thalamic lesions, myasthenia gravis, certain types of seizures, and hydrocephalus.
CONCLUSIONS
Previously reported cases of AACE with unknown etiologies have been reported in both children and adults. However, AACE can be associated with neurological disorders that require neuroimaging probes. The author recommends that clinicians should perform comprehensive neurological assessments to rule out neurological pathologies in AACE, especially in the presence of nystagmus or abnormal ocular and neurological indications (e.g., headache, cerebellar imbalance, weakness, nystagmus, papilloedema, clumsiness, and poor motor coordination).
Topics: Child; Adult; Humans; Esotropia; Arnold-Chiari Malformation; Astrocytoma; Headache; Eye; Acute Disease; Retrospective Studies
PubMed: 37145335
DOI: 10.1007/s00417-023-06092-3 -
World Neurosurgery Jul 2023Patients with Chiari malformation (CM) associated with atlantoaxial dislocation (AAD) and basilar invagination (BI) may present with a small posterior cranial fossa, but...
OBJECTIVE
Patients with Chiari malformation (CM) associated with atlantoaxial dislocation (AAD) and basilar invagination (BI) may present with a small posterior cranial fossa, but data on the volumetric analysis are lacking. Additionally, whether additional foramen magnum decompression (FMD) is needed together with atlantoaxial fusion remains controversial. This study evaluated the volumetric alterations of the posterior cranial fossa in these patients and analyzed the radiological and clinical outcomes after posterior C1-C2 reduction and fixation plus C1 posterior arch resection.
METHODS
Thirty-two adult CM patients with AAD and BI (CM-AAD/BI group) and 21 AAD and BI patients without CM (AAD/BI-only group) who received posterior atlantoaxial fusion plus C1 posterior arch resection were retrospectively studied. The clinical and radiological outcomes and volumetric measurements of the posterior cranial fossa were evaluated.
RESULTS
The majority of CM-AAD/BI patients (94%) improved clinically and radiologically at 12 mo postoperatively, and none required additional FMD. Morphological analysis revealed a significant reduction in the bony posterior cranial fossa volumes of the CM-AAD/BI group (P < 0.01) and the AAD/BI-only group (P < 0.01) relative to those of the CM group. No significant differences were observed between the CM-AAD/BI and AAD/BI groups.
CONCLUSIONS
Compared with patients with simple CM, patients with AAD/BI with or without CM demonstrated a considerably and equally reduced bony posterior cranial fossa volume. No additional FMD is needed in the treatment of CM-AAD/BI patients after posterior reduction and fusion plus C1 posterior arch resection.
Topics: Adult; Humans; Retrospective Studies; Arnold-Chiari Malformation; Platybasia; Joint Dislocations; Atlanto-Axial Joint; Decompression, Surgical; Neck Injuries; Spinal Fusion
PubMed: 37087038
DOI: 10.1016/j.wneu.2023.04.064 -
The Journal of Veterinary Medical... May 2023A 2-day-old male black calf presented with neurological symptoms, including opisthotonus. It was unable to stand due to hindquarter paresis. At 5 days old, the calf was...
A 2-day-old male black calf presented with neurological symptoms, including opisthotonus. It was unable to stand due to hindquarter paresis. At 5 days old, the calf was able to stand, but exhibited a crossed forelimb gait. Computed tomography and magnetic resonance imaging revealed the expansion of the sutures between the squamous-lateral part of the occipital bone and between the occipital-temporal bone, cerebellar tonsillar herniation, posterior displacement of the brainstem, and cervical syringomyelia at 12 days old. This is the first case report of a live calf diagnosed with Arnold Chiari malformation classified as Chiari type 1.5 malformation in humans.
Topics: Animals; Cattle; Humans; Male; Arnold-Chiari Malformation; Cattle Diseases; Magnetic Resonance Imaging; Syringomyelia; Temporal Bone; Tomography, X-Ray Computed
PubMed: 37019664
DOI: 10.1292/jvms.22-0463