Clinical diagnosis—part I: what is really caused by Chiari I

Ciaramitaro, P.; Ferraris, Marilena; Massaro, Fulvio; Garbossa, Diego

doi:10.1007/s00381-019-04206-z

Cited by 24 publications

(11 citation statements)

References 30 publications

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“…In our study, 42.1% of the adults, 69.2% of the adolescents, and 26.3% of the children had preoperative syringomyelia. The prevalence of syringomyelia was in accordance with previous studies, which showed the presence of syringomyelia in 20–70% of cases [ 8 , 18 , 33 , 36 ]. Interestingly, syringomyelia predominated in female patients, being present in 52% of females and only 28% of males.…”

Section: Discussionsupporting

confidence: 91%

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Preoperative measurements on MRI in Chiari 1 patients fail to predict outcome after decompressive surgery

et al. 2021

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Background The purpose of our study was to research the parameters of magnetic resonance imaging (MRI) that would predict the outcome of surgery in patients with Chiari 1 malformation (CM1) and to evaluate changes in MRI parameters after surgery. Methods Fifty-one patients (19 children, 13 adolescents, and 19 adults) operated on due to CM1 in Oulu University Hospital between 2004 and 2018 were evaluated. Seventeen parameters were measured from the preoperative MRI and 11 from the postoperative MRI. The correlations between the MRI parameters and the clinical variables before and after surgery were analyzed. Results The majority (88.2%) of the patients had favorable surgical outcomes. Postoperatively, subjective symptoms improved in 88.6% of the patients and syringomyelia in 81.8%. The location of the cerebellar tonsils, when measured in relation to the C2 synchondrosis or the end plate, postoperatively moved cranially in 51.0% (n = 26), did not change in 27.4% (n = 14), and moved caudally in 21.6% (n = 11) of the patients. However, neither the location of the tonsils nor any other parameters measured from pre- or postoperative MRI correlated with the patients’ symptoms or surgical outcomes. Conclusions No specific parameters on preoperative MRI evaluation were predictive of the outcome of surgery, emphasizing clinical examination in surgical decision-making. Furthermore, the postoperative MRI parameters did not correlate with the surgical outcomes. Thus, routine postoperative imaging is suggested only for patients with preoperatively diagnosed syringomyelia or worsening of symptoms.

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Section: Discussionsupporting

confidence: 91%

“…The prevalence of scoliosis is reported to vary between 13 and 36% in patients with CM1 [8,30,34]. In the present study, 10.5% of the children and 30.8% of the adolescents had scoliosis, while scoliosis was not mentioned in any medical records of the adults.…”

Section: Discussioncontrasting

confidence: 45%

Preoperative measurements on MRI in Chiari 1 patients fail to predict outcome after decompressive surgery

et al. 2021

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“…Firstly, it is important for the differential diagnosis between CM1 headache and migraine or headaches resulting from other conditions. Inadequate and inaccurate assessment of headache will result in a wrong indication for surgery [ 37 , 38 ]. Several studies have attempted to address this topic and proposed criteria to correctly diagnose the Chiari headache (cough headache, Valsalva headache, occipital or suboccipital, lasting a few minutes) [ 39 , 40 ].…”

Section: Resultsmentioning

confidence: 99%

Diagnosis and treatment of Chiari malformation type 1 in children: the International Consensus Document

et al. 2021

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Background Chiari malformation type 1 (CM1) is a rare condition where agreed classification and treatment are still missing. The goal of this study is to achieve a consensus on the diagnosis and treatment of CM1 in children. Methods A multidisciplinary panel formulated 57 provisional statements based on a review of the literature. Thirty-four international experts (IE) participated in a Delphi study by independently rating each statement on a 4-point Likert scale (“strongly disagree,” “disagree,” “agree,” “strongly agree”). Statements that were endorsed (“agree” or “strongly agree”) by < 75% of raters were re-formulated, or new statements were added, and another Delphi round followed (up to a maximum of three). Results Thirty-five IE were contacted and 34 agreed to participate. A consensus was reached on 30/57 statements (52.6%) after round 1. Three statements were added, and one removed. After round 2, agreement was reached on 56/59 statements (94.9%). Finally, after round 3, which took place during the 2019 Chiari Consensus Conference (Milan, Italy), agreement was reached on 58/59 statements (98.3%) about four main sections (Definition and Classification, Planning, Surgery, Isolated Syringomyelia). Only one statement did not gain a consensus, which is the “definition of radiological failure 24 month post-surgery.” Conclusions The consensus document consists of 58 statements (24 on diagnosis, 34 on treatment), serving clinicians and researchers following children with CM1. There is a clear need for establishing an international network and registry and to promote collaborative studies to increase the evidence base and optimize the long-term care of this patient population.

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“…One hundred patients (75 females; average age: 51 ± 13.08 SD; range: 18–76) affected by Chiari syndrome and/or syringomyelia without dyspnea were selected for the study. The diagnosis was based on the patient’s history, clinical examination and magnetic resonance imaging, according to the standardized diagnostic criteria [ 3 , 4 ]. All the patients presented typical symptoms, i.e., “cough”, headache and neck pain, associated with signs of brainstem compression, cerebellar syndrome and myelopathy.…”

Section: Methodsmentioning

confidence: 99%

“…The presence of at least two of the clinical criteria is enough to judge symptomatic Chiari [ 3 ]. Brainstem syndromes have frequently been reported in Chiari syndrome and in syringobulbia; central sleep apnea syndromes (CSASs) were evidenced in 5–8% of a cohort of 600 CM1 adults by polysomnography [ 4 ], and the same frequency was reported in a large pediatric study [ 5 ]. It is therefore possible that CM1 and/or Syr patients present clinical or subclinical respiratory dysfunctions.…”

Section: Introductionmentioning

confidence: 98%

Neurophysiological Correlates in Patients with Syringomyelia and Chiari Malformation: The Cortico-Diaphragmatic Involvement

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Garbossa

et al. 2022

JCM

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Purpose. Brainstem syndromes have frequently been reported in Chiari syndrome and in syringobulbia; previous studies have shown that determining the central motor conduction time (CMCT) along the circuit of the phrenic nerve makes the assessment of the voluntary control of the respiratory pathway possible. In our study, we evaluated the transcranial magnetic stimulation (TMS) of the phrenic nerve in patients affected by Chiari syndrome and/or syringomyelia (Syr) with the aim of identifying subclinical neurophysiological alterations. Methods. One hundred patients (75 females; average age: 51 ± 13.08 SD; range: 18–76) affected by Chiari syndrome and/or Syr without dyspnea were selected. The magnetic stimulation of the second motor neuron correlating with the phrenic nerve was performed using cervical magnetic stimulation (C5-MEP); the cortical MEP after magnetic stimulation (Cz-MEP) was recorded by magnetic stimulation of the motor cortex (areas corresponding to the diaphragm). The CMCT was calculated. The differences between the patients and controls were calculated (Student’s t test). Results. In 13% of the patients, the Cz-MEP were absent bilaterally, and the CMCT was not evaluable. In all these cases, bulbar/cervical Syr was present at MRI; in 10 of them, this was associated with CM1. A bilateral response was obtained in all the other patients (87%), and the CMCTs were normal. All the patients with alterations/absence of Cz-MEP presented bulbar/cervical Syr at MRI. The C5-MEP latency was prolonged or absent in 48%; of these, 84% presented bulbar/cervical Syr associated with CM1 at MRI. The C5-MEP latency values were significantly higher in the group of patients. Conclusions. Neurophysiological alterations of the diaphragmatic pathway were recorded in a group of Chiari syndrome and, particularly, in bulbar/cervical Syr. Future studies with larger cohorts of patients are needed to further assess the specific role of the TMS of the phrenic nerve in CM/Syr patients.

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Clinical diagnosis—part I: what is really caused by Chiari I

Cited by 24 publications

References 30 publications

Preoperative measurements on MRI in Chiari 1 patients fail to predict outcome after decompressive surgery

Preoperative measurements on MRI in Chiari 1 patients fail to predict outcome after decompressive surgery

Diagnosis and treatment of Chiari malformation type 1 in children: the International Consensus Document

Neurophysiological Correlates in Patients with Syringomyelia and Chiari Malformation: The Cortico-Diaphragmatic Involvement

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