Is there a relationship between the extent of tonsillar ectopia and the severity of the clinical Chiari syndrome?
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ORIGINAL ARTICLE - NEUROSURGERY GENERAL
Is there a relationship between the extent of tonsillar ectopia and the severity of the clinical Chiari syndrome? Dan S. Heffez 1,2 & John Broderick 2,3 & Michael Connor 2,3 & Michael Mitchell 2,3 & JoAnna Galezowska 2,3 & Ramin Golchini 4 & Jugal Ghorai 5 Received: 13 February 2019 / Accepted: 10 December 2019 # Springer-Verlag GmbH Austria, part of Springer Nature 2019
Abstract Background Chiari 1 malformation is diagnosed if the cerebellar tonsils extend at least 5 mm below the opisthion-basion line. Objective To examine the correlation of the extent of tonsillar ectopia with the prevalence and severity of the symptoms associated with the Chiari malformation. Methods Patients (N = 428) were grouped according to the extent of tonsillar ectopia on the mid-sagittal MRI image (group 1, 0–< 3 mm; group 2, 3–5 mm; group 3, > 5 mm). Groups were compared regarding demographics, symptoms, neurological signs, pain score, and response to HADS and sf-36 questionnaires. Results were analyzed using one-way ANOVA, chi-square, and two sample Z test, and Student’s t test for pairwise comparison, (statistical significance p < 0.05). A logistic regression analysis was performed to determine the relationship between tonsillar ectopia and the probability of a patient reporting any particular symptom. Results There were 97,148 and 183 patients in groups 1, 2, and 3 respectively. Groups did not differ with regard to antecedent trauma or female preponderance. Patients in group 1 were more symptomatic than those in groups 2 and 3 with regard to some symptoms, (p = 0.04–p = 0.000). Regression analysis confirmed an inverse relationship between the extent of tonsillar ectopia and the likelihood of many symptoms. The pain score was greatest in group 1, (p = 0.006). Prevalence of objective signs of myelopathy did not differ between groups except for Hoffmann sign which was more prevalent in group 1, (p = 0.034). HADS and sf-36 scores did not differ between groups. Conclusion The severity of the symptoms associated with the Chiari malformation does not correlate directly with the extent of tonsillar ectopia. The extent of tonsillar ectopia should be re-evaluated as the threshold for diagnosis of Chiari 1 malformation. Keywords Chiari malformation . Diagnosis . Tonsillar ectopia . Brain stem . Pathophysiology
Introduction The Chiari type 1 malformation has become a controversial diagnosis. This reflects the fact that the initial description was
based on pathological specimens and the only obvious anatomical anomaly related to the position of the cerebellar tonsil (Chiari, H: Concerning alterations in the cerebellum resulting from cerebral hydrocephalus, 1891) [4, 23]. Until the avail-
This article is part of the Topical Collection on Neurosurgery general Accepted for poster presentation Congress of Neurological Surgeons, Boston 2014 Presented in part at the meeting of the American Syringomyelia and Chiari Alliance Project, Long Island, NY, 2017. * Dan S. Heffez [email protected] 1
2
Milwaukee Neur
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