Clinical diagnosis-part II: what is attributed to Chiari I.

Purpose: Chiari malformation type I is identified as radiological appearance of cerebellar tonsil herniation below the foramen magnum. The wide spectrum of clinical manifestations variably associated sometimes encompasses signs and symptoms whose correlation with the malformation remains debatable. However, a correct clinical framework is relevant in tailoring the strategy of management, and in particular, establishing the appropriate surgical intervention.

Methods: An analysis of relevant articles on this issue was performed, involving both single case reports and case series. The papers were obtained from electronic databases including PubMed/MEDLINE and Google Scholar, as well as direct contact with some authors.

Results: This review focuses on those unusual clinical pictures attributed to Chiari malformation type I (CMI), investigating their actual relationship with the morphological condition. Supposed physiopathogenetic mechanisms and clinical relevance are discussed along with an analysis on the surgical indications and results.

Conclusion: There is no clear explanation on why some patients may exhibit uncommon symptoms, often in association with the most frequent ones, but even reported as single initial clinical occurrence. Their actual incidence may have been roughly underestimated in the literature so far, because of wide heterogeneity of selection and analysis among different studies. Most of the authors appear sharing the impression that CMI surgical decompression plays a significant role in controlling the majority of these unusual symptoms, stressing their actual occurrence in relation with the malformation. A routinely multidisciplinary clinical approach has become advisable to encompass the diversity of conditions potentially associated with CMI and improve the care of CMI patients.