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A novel exaggerated "spino-bulbo-spinal like" reflex of lower brainstem origin.

ABSTRACT: BACKGROUND:Many different oligosynaptic reflexes are known to originate in the lower brainstem which share phenomenological and neurophysiological similarities. OBJECTIVE:To evaluate and discuss the differences and aberrancies among these reflexes, which are hard to discern clinically using neurophysiological investigations with the help of a case report. METHODS:We describe the clinical and neurophysiological assessment of a young man who had a childhood history of opsoclonus-myoclonus syndrome with residual mild ataxia and myoclonic jerks in the distal extremities presenting with subacute onset total body jerks sensitive to sound and touch (in a limited dermatomal distribution), refractory to medications. RESULTS:Based on clinical characteristics and insights gained from neurophysiological testing we could identify a novel reflex of caudal brainstem origin. CONCLUSIONS:The reflex described is likely an exaggerated normal reflex, likely triggered by a dolichoectatic vertebral arterial compression and shares characteristics of different reflexes known to originate in caudal brainstem, which subserve distinctive roles in human postural control.

SUBMITTER: Merchant SH

PROVIDER: S-EPMC6451678 | biostudies-literature | 2019 Apr

REPOSITORIES: biostudies-literature

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A novel exaggerated "spino-bulbo-spinal like" reflex of lower brainstem origin.

Merchant Shabbir Hussain SH Vial Felipe F Leodori Giorgio G Fahn Stanley S Pullman Seth L SL Hallett Mark M

Parkinsonism & related disorders 20181006

<h4>Background</h4>Many different oligosynaptic reflexes are known to originate in the lower brainstem which share phenomenological and neurophysiological similarities.<h4>Objective</h4>To evaluate and discuss the differences and aberrancies among these reflexes, which are hard to discern clinically using neurophysiological investigations with the help of a case report.<h4>Methods</h4>We describe the clinical and neurophysiological assessment of a young man who had a childhood history of opsoclo ...[more]

PMID: 30316728

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Project description:Image-based subject-specific models and simulations are recently being introduced to complement current state-of-the-art mostly static insights of the adult spinal deformity (ASD) pathology and improve the often poor surgical outcomes. Although the accuracy of a recently developed subject-specific modeling and simulation framework has already been quantified, its reliability to perform marker-driven kinematic analyses has not yet been investigated. The aim of this work was to evaluate the reliability of this subject-specific framework to measure spine kinematics in ASD patients, in terms of 1) the overall test-retest repeatability; 2) the inter-operator agreement of spine kinematic estimates; and, 3) the uncertainty of those spine kinematics to operator-dependent parameters of the framework. To evaluate the overall repeatability 1], four ASD subjects and one control subject participated in a test-retest study with a 2-week interval. At both time instances, subject-specific spino-pelvic models were created by one operator to simulate a recorded forward trunk flexion motion. Next, to evaluate inter-operator agreement 2], three trained operators each created a model for three ASD subjects to simulate the same forward trunk flexion motion. Intraclass correlation coefficients (ICC's) of the range of motion (ROM) of conventional spino-pelvic parameters [lumbar lordosis (LL), sagittal vertical axis (SVA), thoracic kyphosis (TK), pelvic tilt (PT), T1-and T9-spino-pelvic inclination (T1/T9-SPI)] were used to evaluate kinematic reliability 1] and inter-operator agreement 2]. Lastly, a Monte-Carlo probabilistic simulation was used to evaluate the uncertainty of the intervertebral joint kinematics to operator variability in the framework, for three ASD subjects 3]. LL, SVA, and T1/T9-SPI had an excellent test-retest reliability for the ROM, while TK and PT did not. Inter-operator agreement was excellent, with ICC values higher than test-retest reliability. These results indicate that operator-induced uncertainty has a limited impact on kinematic simulations of spine flexion, while test-retest reliability has a much higher variability. The definition of the intervertebral joints in the framework was identified as the most sensitive operator-dependent parameter. Nevertheless, intervertebral joint estimations had small mean 90% confidence intervals (1.04°-1.75°). This work will contribute to understanding the limitations of kinematic simulations in ASD patients, thus leading to a better evaluation of future hypotheses.

Dataset Information

A novel exaggerated "spino-bulbo-spinal like" reflex of lower brainstem origin.

Publications

A novel exaggerated "spino-bulbo-spinal like" reflex of lower brainstem origin.

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