Relationship of brain parenchyma within the caudal cranial fossa and ventricle size to syringomyelia in cavalier King Charles spaniels

Driver, Colin; Rusbridge, Clare; Cross, H. R.; McGonnell, Imelda M.; Volk, Holger A.

doi:10.1111/j.1748-5827.2010.00952.x

Cited by 56 publications

(84 citation statements)

References 26 publications

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“…Morphometric studies further suggest a probable difference between human and canine conditions. CLM canines with syringomyelia display shallower caudal cranial fossa measurements, 7 overcrowding of the caudal cranial fossa by an increased cerebellar volume, 49 and greater volume of brain parenchyma situated in the caudal cranial fossa 15 than CLM canines without syringomyelia. Limited morphometric studies in humans have not made such associations with syringomyelia.…”

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confidence: 85%

Cerebellar and hindbrain motion in Chiari malformation with and without syringomyelia

Leung

Magnussen

Stoodley

et al. 2016

SPI

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OBJECTIVE The pathogenesis of syringomyelia associated with Chiari malformation type I (CM-I) is unclear. Theories of pathogenesis suggest the cerebellar tonsils may obstruct CSF flow or alter pressure gradients, or their motion might act as a piston to increase CSF pressure in the spinal subarachnoid space. This study was performed to measure cerebellar tonsillar and hindbrain motion in CM-I and assess the potential contributions to syrinx formation. METHODS Sixty-four CM-I patients and 25 controls were retrospectively selected from a clinical database, and all subjects had undergone cardiac-gated cine balanced fast-field echo MRI. There were a total of 36 preoperative CM-I scans, which consisted of 15 patients with and 21 patients without syringomyelia. Nineteen patients underwent paired pre- and postoperative imaging. Anteroposterior (AP) and superoinferior (SI) movements of the tip of the cerebellar tonsils, obex, fastigium of the fourth ventricle, pontomedullary junction, and cervicomedullary junction were measured. The distance between the fastigium and tip of the tonsils was used to calculate tonsillar tissue strain (Δi/i0). RESULTS CM-I patients had significantly greater cerebellar tonsillar motion in both the AP and SI directions than controls (AP +0.34 mm [+136%], p < 0.001; SI +0.49 mm [+163%], p < 0.001). This motion decreased after posterior fossa decompression (AP −0.20 mm [−33%], p = 0.001; SI −0.29 mm [−36%]; p < 0.001), but remained elevated above control levels (AP +56%, p = 0.021; SI +67%, p = 0.015). Similar trends were seen for all other tracked landmarks. There were no significant differences in the magnitude or timing of motion throughout the hindbrain between CM-I patients with and without syringomyelia. Increased tonsillar tissue strain correlated with Valsalva headaches (p = 0.03). CONCLUSIONS Cerebellar tonsillar motion may be a potential marker of CM-I and may have use in tailoring surgical procedures. The lack of association with syringomyelia suggests that tonsillar motion alone is not the driver of syrinx formation. Tonsillar tissue strain may play a part in the pathophysiology of Valsalva headaches.

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Cerebellar and hindbrain motion in Chiari malformation with and without syringomyelia

Leung

Magnussen

Stoodley

et al. 2016

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“…18 Syringomyelia was defined as hyperintense lesions on the transverse and/or sagittal T2-weighted images with maximal dorsoventral dimension greater than 2 ml. 11,20 All images were subsequently reviewed using image viewer software, b and syrinx size was measured by a boardcertified neurologist. Patient medical records were reviewed; the site and date of CSF collection, the age (in months), gender, and additional historical data were recorded.…”

Section: Electronic Patient Recordsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

“…3 The disease is recognized on magnetic resonance (MR) imaging as relatively reduced caudal fossa volume, with subsequent foramen magnum herniation of the cerebellum, 11,18,23 and has been associated with the development of fluid-filled cavities within the spinal cord parenchyma (syringomyelia [SM]). 11,17,21 CM/SM is a debilitating and painful disease complex likely to be multifactorial in origin, secondary to altered cerebrospinal fluid (CSF) dynamics and damage to the spinal cord parenchyma; clinical signs frequently exhibited by affected dogs include phantom scratching and vocalization, cervical scoliosis, thoracic and pelvic limb ataxia, and thoracic limb paresis. [21][22][23] Despite an abundance of literature having been published describing the clinical and imaging findings in dogs with CM and CM/SM and, additionally, the use of CSF analysis being a routine diagnostic procedure in dogs presenting with neurologic signs, there remains a paucity of information on CSF composition in CKCS with CM or CM/SM.…”

Section: Introductionmentioning

confidence: 99%

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Evaluation of Cerebrospinal Fluid in Cavalier King Charles Spaniel Dogs Diagnosed with Chiari-Like Malformation with or without Concurrent Syringomyelia

et al. 2011

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Abstract. Syringomyelia is a common clinical problem in the Cavalier King Charles Spaniel dog population. The underlying pathophysiology of the development and progression of syringes is currently unknown. The primary aim of the current study was to determine whether development of syringomyelia is accompanied by alterations in cerebrospinal fluid composition. Patient records of Cavalier King Charles Spaniels with Chiari-like malformations, with or without concurrent syringomyelia, that had magnetic resonance imaging and cerebrospinal fluid analysis between December 2004 and December 2009, were retrospectively reviewed. Total nucleated cell count per microliter, with differential count, red blood cell count per microliter, and protein concentration (g/l), were reviewed. Data were analyzed with the Mann-Whitney Utest. Spearman rank test was used to test association between cerebrospinal fluid composition and syrinx size, and the Fisher exact test was used to compare the presence of macrophages, reactive lymphocytes, and myelin. A P value of #0.05 was considered significant for statistical tests. Dogs with syringomyelia had a higher total nucleated cell count (syringomyelia: 4/ml [range, 0-15/ml] vs. without syringomyelia: 2/ml [0-8/ml]; P 5 0.0047), higher protein concentration (syringomyelia: 0.26 g/l [0.07-0.42 g/l] vs. without syringomyelia: 0.2 g/l [0.12-0.39 g/l]; P 5 0.039), and an increased neutrophil percentage (syringomyelia: 0% [0-37%] vs. without syringomyelia: 0% [0-21%]; P 5 0.0203) than those with the Chiari-like malformation alone. There was a positive correlation between total nucleated cell count and syrinx size (r 5 0.51; P 5 0.0068). Development of syringomyelia is accompanied by alterations in cerebrospinal fluid composition. The pathogenesis of syringomyelia has not been fully elucidated; therefore, the exact origin of cerebrospinal fluid changes remains unclear.

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“…Specifically, Chiari‐like malformation and syringomyelia (CMSM) is a disease complex that causes a neuropathic pain syndrome in Cavalier King Charles Spaniels (CKCS). This condition arises from a congenital malformation that results in a relatively small caudal fossa with respect to the brain causing crowding of the cerebellum and brainstem 1, 2, 3, 4, 5, 6, 7. Many CKCS with CM also develop syrinxes within the spinal cord (syringomyelia, SM) because of disruption of cerebrospinal fluid (CSF) flow.…”

Section: Introductionmentioning

confidence: 99%

Investigation of sensory thresholds in Cavalier King Charles Spaniels with and without Chiari‐like malformations and syringomyelia

Sparks

Gorney

Williams

et al. 2018

Veterinary Internal Medicne

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BackgroundCavalier King Charles Spaniels (CKCS) suffer pain associated with Chiari‐like malformation and syringomyelia (CMSM). People suffer from a similar condition and describe numerous sensory abnormalities. Sensory changes have not been quantified in affected CKCS.ObjectivesTo use quantitative sensory testing (QST) to quantify thermal and mechanical thresholds in CKCS and to compare QST in dogs with and without syringomyelia (SM).AnimalsForty‐four CKCS.MethodsProspective study. Dogs underwent neurological examinations and craniocervical magnetic resonance imaging (MRI). Thermal testing was performed over the humerus and thorax (n = 32); mechanical testing was performed on the paw and neck (n = 44). Latencies, thresholds, and response rates were compared with presence and severity of SM on MRI, presence of pain reported by the owner and pain identified on examination.ResultsThirty dogs had SM, 30 were painful on examination, 29 were owner‐reported symptomatic. Thermal and mechanical variables were not significantly different based on presence or severity of SM. Dogs with pain on examination had decreased mechanical thresholds on the paw (0.38 kg, SD = 0.18) and neck (2.05 kg, SD = 0.74) compared to thresholds of dogs without pain on examination on the paw (0.60 kg, SD = 0.30) and neck (2.72 kg, SD = 0.57; P = .021).Conclusions and Clinical ImportanceMechanical and thermal sensitivity does not appear to be related to the presence of SM, but mechanical sensitivity appears to be related to the presence of pain and clinical signs. Mechanical testing may be useful for assessing sensory abnormalities during clinical trials.

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Relationship of brain parenchyma within the caudal cranial fossa and ventricle size to syringomyelia in cavalier King Charles spaniels

Abstract: A more marked overcrowding of the CCF is associated with SM, which may explain the high incidence of SM in CKCS with CM. The association between ventricle and syrinx dimensions supports the theory that SM development is the result of altered cerebrospinal fluid dynamics.

Cited by 56 publications

References 26 publications

Cerebellar and hindbrain motion in Chiari malformation with and without syringomyelia

Cerebellar and hindbrain motion in Chiari malformation with and without syringomyelia

Evaluation of Cerebrospinal Fluid in Cavalier King Charles Spaniel Dogs Diagnosed with Chiari-Like Malformation with or without Concurrent Syringomyelia

Investigation of sensory thresholds in Cavalier King Charles Spaniels with and without Chiari‐like malformations and syringomyelia

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