Why Is This Auntminnie a Diagnostic Conundrum?: A Knowledge-Based Approach to Balo's Concentric Sclerosis From Reports of 3 Cases and Pooled Data From 68 Other Patients in the Literature. Curr Probl Diagn Radiol 2019;48(4):415-422
Date
02/13/2018Pubmed ID
29428181DOI
10.1067/j.cpradiol.2017.12.008Scopus ID
2-s2.0-85044503739 (requires institutional sign-in at Scopus site) 4 CitationsAbstract
INTRODUCTION: We came across 3 cases of Balo's concentric sclerosis (BCS). The first of these patients presented to an outside hospital and was transferred to our institution due to complications resulting from a biopsy. The other 2 patients, despite having a characteristic imaging appearance and despite insistence on our part on the diagnosis of BCS, underwent a surgical procedure, which could have been prevented. This led us to review the available literature on BCS.
MATERIAL AND METHODS: A total of 68 patients diagnosed with BCS between 1995 and 2015 were studied and the data collected for the clinical presentation and course, imaging, spinal fluid analysis, treatment, and clinical and imaging outcome.
CONCLUSIONS: A 25% surgery rate (biopsy or resection) was found in the study. We concluded that this relatively high surgery rate in this auntminnie nonsurgical disease is multifactorial; and includes factors like nonfamiliarity with the disease, anxiety on the part of patients and physicians, due to a sometimes rapidly deteriorating clinical picture; and resemblance of the disease with other entities such as tumor and infection. However, characteristic imaging appearance combined with acute or subacute presentation and dramatic improvement in clinical status after high-dose steroid chemotherapy; are highly suggestive of the disease, and can prevent unnecessary surgery.
Author List
Agarwal M, Ulmer JL, Klein AP, Mark LPAuthors
Mohit Agarwal MD Associate Professor in the Radiology department at Medical College of WisconsinAndrew P. Klein MD Chief, Associate Professor in the Radiology department at Medical College of Wisconsin
MESH terms used to index this publication - Major topics in bold
AdolescentAdrenal Cortex Hormones
Adult
Biopsy, Needle
Child
Diffuse Cerebral Sclerosis of Schilder
Female
Hemiplegia
Humans
Immunohistochemistry
Magnetic Resonance Imaging
Male
Middle Aged
Neurosurgical Procedures
Prognosis
Rare Diseases
Risk Assessment
Severity of Illness Index
Survival Rate
Treatment Outcome
Young Adult
