Acute presentation of Chiari 1 malformation in children.

PURPOSE: To review the literature about the acute presentation of Chiari 1 malformation in children, with a focus on acute cervical cord syndromes with impairment of the cortico-spinal tract. To analyze the possible precipitating factors and the pathophysiology of the acute onset. METHOD: Illustrative case presentation with literature review. RESULTS: The literature includes just a few dozens of children with acute presentation of Chiari 1 malformation. The more frequent presentations consist of oropharyngeal dysfunction, cranial nerve impairment, high intracranial pressure, peripheral motor deficit. Acute impairment of cervical long tracts is very rare and we could find only 16 previously reported cases of cervical cord impairment with quadriparesis or hemiparesis. CONCLUSIONS: Nowadays, a lot of asymptomatic Chiari 1 malformations are frequently observed owing to the wide diffusion of magnetic resonance imaging. This raised the question about the management of these patients. Despite severe and even lethal manifestations being reported in previously asymptomatic patients, the absolute rarity of acute deterioration does not justify prophylactic surgery. The diagnosis of Chiari malformation may be initially difficult in patients with isolated, acute, and unusual presentations but physicians should bear in mind its possibility, because prompt cranio-cervical decompression may be decisive.

Effect of a systemic intervention combined with a psychological intervention in stroke patients with oropharyngeal dysfunction.

BACKGROUND: Stroke frequently results in oropharyngeal dysfunction (OD), leading to difficulties in swallowing and eating, as well as triggering negative emotions, malnutrition, and aspiration pneumonia, which can be detrimental to patients. However, routine nursing interventions often fail to address these issues adequately. Systemic and psychological interventions can improve dysphagia symptoms, relieve negative emotions, and improve quality of life. However, there are few clinical reports of systemic interventions combined with psychological interventions for stroke patients with OD. AIM: To explore the effects of combining systemic and psychological interventions in stroke patients with OD. METHODS: This retrospective study included 90 stroke patients with OD, admitted to the Second Affiliated Hospital of Qiqihar Medical College (January 2022-December 2023), who were divided into two groups: regular and coalition. Swallowing function grading (using a water swallow test), swallowing function [using the standardized swallowing assessment (SSA)], negative emotions [using the self-rating anxiety scale (SAS) and self-rating depression scale (SDS)], and quality of life (SWAL-QOL) were compared between groups before and after the intervention; aspiration pneumonia incidence was recorded. RESULTS: Post-intervention, the coalition group had a greater number of patients with grade 1 swallowing function compared to the regular group, while the number of patients with grade 5 swallowing function was lower than that in the regular group (P < 0.05). Post-intervention, the SSA, SAS, and SDS scores of both groups decreased, with a more significant decrease observed in the coalition group (P < 0.05). Additionally, the total SWAL-QOL score in both groups increased, with a more significant increase observed in the coalition group (P < 0.05). During the intervention period, the total incidence of aspiration and aspiration pneumonia in the coalition group was lower than that in the control group (4.44% vs 20.00%; P < 0.05). CONCLUSION: Systemic intervention combined with psychological intervention can improve dysphagia symptoms, alleviate negative emotions, enhance quality of life, and reduce the incidence of aspiration pneumonia in patients with OD.

Clinical Manifestations of Chiari I Malformation.

Chiari 1 malformation (CM1) includes a spectrum of clinical manifestations. These signs and symptoms result from compression at the cervicomedullary junction and alteration in cerebrospinal fluid dynamics thus affecting several structures above, at, and below the cervicomedullary junction. Differences in presentation exist among different age groups and high clinical suspicion should be present in younger children. Additionally, CM1 can be associated with other diagnoses and can have unusual acute presentations that should be recognized to ensure excellent outcomes..

Evaluation of Feeding Disorders Including Gastro-Esophageal Reflux and Oropharyngeal Dysfunction in Children With Cerebral Palsy.

Objective: This cross sectional study aims to survey developing feeding disorders and nutritional deficiencies disorders in children with neurodevelopmental disorders such as cerebral palsy. Materials and methods: A total of 50 children (28 boys and 22 girls) with cerebral palsy and symptoms suggesting gastrointestinal problems such as choking, recurrent pneumonia and poor weight gain, who referred to the Pediatric department of Vali-asr Hospital, Imam Khomeini hospital complex between 1 October 2012 and 30 October 2013, were checked. Motor function classification system was used to classify patient's functional gross motor severity. All patients were examined and underwent deglutition videofluroscopy (modified barium swallow) and upper GI endoscopy with esophageal biopsies. Outcome of this study was the prevalence of oropharyngeal incoordination and GERD. Its relationship with some variables like motor and cognitive developmental delay were analyzed and p value < 0.05 was considered significant. Medical therapy and/or oral physiotherapy and nutritional rehabilitation were started. They were examined after 6 months of treatment. Decrease in choking and episodes of respiratory infections that needed hospitalization and weight gain after 6 months treatment were considered as secondary outcomes (response to treatment). Results: Prevalence of GERD was 66% and oropharyngeal dysphagia was estimated 82%. According to results of video-fluroscopy and endoscopic biopsies, 52% of patients were affected by both GERD and oropharyngeal dysfunction. The gross motor function disability was the only variable that significantly related to the prevalence of feeding disorders (p = 0.015). Despite nutritional rehabilitation only 46% of children have weight gain. Conclusion: Feeding disorders such as GERD and oropharyngeal dysfunction are more prevalent in children with cerebral palsy especially in children with severe gross motor disabilities. Since, clinical manifestations of these disorders can be similar accurate diagnostic methods should be selected for all children with cerebral palsy and gastrointestinal symptoms. Treatment should start early to reduce the complications and improve outcomes.

The Correlation between Peripheral Facial Neuropathy and Oropharyngeal Dysfunction

OBJECTIVE: To know the correlation between the grading of facial neuropathy by facial nerve conduction study (FNCS) and dysphagia severity by videofluoroscopic swallowing study (VFSS) in patients with acute peripheral facial palsy (PFP). METHOD: Twenty patients with acute PFP were recruited for this study. The causes of acute PFP were limited to idiopathic Bell's palsy and Ramsay-Hunt syndrome. The time interval from the onset of PFP to study of FNCS and VFSS was 10 to 14 days. The severity of PFP was graded according to House-Brackmann facial nerve grade (H-B FNG). Percent degeneration grade (PDG) was determined by FNCS. Baseline-to-peak amplitude of compound muscle action potentials in orbicularis oris muscle was used as an evaluation parameter. Oral transit time (OTT), pharyngeal transit time (PTT), pharyngeal delay time (PDT) and dysphagia limit were obtained by VFSS. RESULTS: There was a significant correlation between PDG and H-B FNG. The severity of oropharyngeal dysfunction was increased as the severity of the PDG increases. Delayed OTT, delayed PDT, and/or reduced dysphagia limit were revealed as a presentation of oropharyngeal dysfunction in acute PFP. CONCLUSION: The severity of PFP and oropharyngeal dysfunction were significantly correlated. And so we think that precise evaluation and adequate management of oropharyngeal dysfunction will be needed in acute PFP patients.