Longitudinal assessment of syringomyelia in Pomeranians.

Introduction: Chiari-like malformation (CM) and syringomyelia (SM) are disorders that, in dogs, affect mainly small and toy breeds, including the Pomeranian. These disorders are linked to a great number of (owner-reported) clinical signs (ORCS) suggestive of pain. Aging was associated with an increased risk of having SM in several studies. However, there are only a few longitudinal studies that assess the presence and severity of CM/SM over time in CKCS dogs and progression of SM was linked to progression of clinical signs. The aim of this study was to investigate ORCS, CM/SM classification, and quantitative syrinx parameters in relation to progression of time (age) within individual Pomeranians. Materials and methods: Pomeranians with or without ORCS and with or without diagnoses of CM/SM were included that had undergone two (or more) MRI studies of the craniocervicothoracic region between January 2020 and June 2023. Classification of CM/SM and quantitative syrinx measurements were performed. Absolute values as well as ratios for syrinx height, width, and cross-sectional area were included for analysis. Results: A total of 19 Pomeranians were included in the study, of which 11 were male (58%) and 8 were female (42%). The median age at the time of MRI1 was 26 months (range 7-44 months). The median scan interval was 26 months (range 11-49 months). Eleven dogs (58%) were presented with ORCS at the time of MRI1, whereas the other 8 dogs (42%) had no ORCS at that time. At the time of MRI2, there were 17/19 dogs (89%) with ORCS and 2/19 dogs without ORCS (11%). Dogs were significantly more likely to have ORCS at MRI2 than MRI1 (p = 0. 0411). There was no significant difference between CM/SM classification at the time of MRI1 and MRI2. Significant differences were found between MRI1 and MRI2 for syrinx height (based on transverse images) (absolute value and ratio P = 0.0059), syrinx width (absolute value P = 0.1055, ratio P = 0.0039), and syrinx cross sectional area (absolute value P = 0.0195, ratio P = 0.0217). Discussion: There are differences in the presence or absence of ORCS as well as quantitative syrinx measurements in Pomeranians at different ages. This finding supports that longitudinal changes occur in the SM status of Pomeranians.

Relationship between syringomyelia and myxomatous mitral valve disease in Cavalier King Charles spaniels.

BACKGROUND: Syringomyelia (SM) and myxomatous mitral valve disease (MMVD) are highly prevalent in Cavalier King Charles spaniels (CKCS). Cardiac status in CKCS with and without SM is currently unknown. OBJECTIVES: To investigate the association between SM and MMVD severity in CKCS and CKCS with SM with and without clinical signs of SM. ANIMALS: Fifty-five CKCS: 40 with SM (22 symptomatic and 18 asymptomatic) and 15 without SM. METHODS: A combined retrospective and prospective study. MRI and echocardiography were used to diagnose SM and MMVD, respectively. The association between SM and MMVD severity (left ventricle internal diameter in diastole normalized to bodyweight [LVIDDN] and left atrium to aortic ratio [LA/Ao]) were tested using multivariable linear regression analysis adjusting for sex and age. RESULTS: Overall, no significant difference in LVIDDN and LA/Ao was found between CKCS with or without SM. However, CKCS with symptomatic SM had significantly smaller LVIDDN (1.45 [1.30-1.50]) (median [IQR]) and LA/Ao (1.20 [1.10-1.28]) compared to CKCS with asymptomatic SM (1.60 [1.50-1.90] and 1.40 [1.20-1.75]) as well as CKCS without SM (0.24 [0.03-0.45] and 0.30 [0.05-0.56]) (all P values <.03). CONCLUSIONS AND CLINICAL IMPORTANCE: An association between MMVD and SM was not confirmed in this cohort of CKCS, indicating that MMVD and SM do not co-segregate. However, CKCS with symptomatic SM had smaller left ventricle and atrial size compared to CKCS with asymptomatic SM and CKCS without SM.

Primary Leptomeningeal Medulloblastoma in Adults: A Diagnostic Challenge-Case Report and Systematic Review.

Primary leptomeningeal medulloblastoma (PL-MB) in adults is a rare disease with a severe prognosis. A 35-year-old woman presented with headaches, diplopia, and gait ataxia, with triventricular hydrocephalus and descent of the cerebellar tonsils beyond the foramen magnum. Endoscopic third ventriculostomy was performed. Six months later, headaches recurred. Dilatation of the supratentorial ventricular system and massive cerebellar swelling without contrast-enhancing nodularities were reported. Occipitocervical decompression with duraplasty was performed. A bioptic diagnosis of PL-MB was made. Craniospinal irradiation and chemotherapy were administered. After 18 months, no recurrence was observed. Few cases of PL-MB have been reported: patients die before treatment or within a few days after surgery. Our long-term survival could be ascribable to a slow clinical presentation and an early diagnosis that allowed surgical treatment and the administration of a combined chemoradiotherapy protocol. Cerebellar swelling, even without associated enhancing lesions, with or without hydrocephalus, should be a neuroradiological alarm sign, and PL-MB should be considered.

Biopsy and histologic findings of the dura mater at the level of the foramen magnum in 121 CKCS with Chiari-like malformation.

To describe histopathologic features found in dural biopsies of Cavalier King Charles Spaniels (CKCS) with Chiari-like malformation (CM) and identify any associations between age, duration of clinical signs, syrinx location or syringomyelia (SM, and quality of life (QOL). The medical records of 121 consecutive client owned CKCS with CM and SM, confirmed by whole body magnetic resonance imaging (MRI), that underwent foramen magnum decompression (FMD) with cranioplasty and durectomy with biopsy from 2006 to 2016 were retrospectively reviewed. Dural biopsies were submitted to a board-certified veterinary pathologist for histopathologic interpretation. The chi-square test was used to analyze associations between histologic findings and categorical variables. For continuous measures, the Kruskal-Wallis non-parametric test was used to compare distributions across pathology categories. A result was considered statistically significant at the p < 0.05 level of significance. The mean age, duration of pre-surgical clinical signs, and pre-operative QOL (1-5 scale) were 44.27 months, 44.78 weeks, and 2.72, respectively. Syringomyelia was found in the cervical region only in 39 of 121 (32.23%) of dogs, in the cervical and thoracic region only in 17 of 121 (14.05%) of dogs, and in the cervical, thoracic, and lumbar region combined in 65 of 121 (53.72%) of dogs. Sixty-six of one hundred twenty-one (54.55%) dural biopsy specimens had histopathology changes; fifty-five (45.45%) did not. Forty-three of one hundred twenty-one (35.54%) dural biopsy specimens had osseous metaplasia, 16 of 121 (13.22%) had evidence of fibrosis, 4 of 121 (3.31%) had arachnoid hyperplasia, and 3 of 121 (2.48%) had evidence of mineralization. Most dogs with CM were found to have histopathologic changes in the dura at the time of FMD cranioplasty was performed. These dural changes can be observed in dogs experiencing clinical signs for a time period as short as 4 weeks prior to presentation. The histopathologic changes were not associated with age, breed, duration of clinical signs, the location of syringomyelia or QOL. The influence of histopathologic changes on long-term prognosis in dogs without dural decompression is unknown since all dogs in this study had dural resection.

Utility of a flexed neck sagittal magentic resonance imaging sequence for the assessment of cerebellomedullary cistern in dogs.

Cerebrospinal fluid (CSF) collection from the cerebellomedullary cistern (CM) of dogs with congenital or acquired cerebellar herniation could lead to serious complications. It is anecdotally more challenging in large brachycephalic breeds possibly due to the increased distance between the skin and CM. The first objective of this study was to assess whether flexed-neck sagittal magnetic resonance imaging (MRI) sequences would assist in the decision-making process of collecting CSF from the CM. The second objective was to examine the dimensions of the CM measured in extended and flexed views, and whether cranial index (CI), skull height and body weight correlated with the distance of the CM from the skin surface. Forty-one dogs of various breeds were included in the study. Measurements were performed on T2-weighted sagittal sequences acquired in extended and flexed-neck positions, and transverse sequences acquired in an extended-neck position. Mild cerebellar herniation was detected in 23/41 (56%) of the flexed-neck views versus none in the extended views. The CM area was significantly larger in flexed-neck views than in extended views (p < 0.05). In 29% of the cases (12/41), the trajectory of the needle intersected the cerebellar vermis. There was a positive correlation between the distance of the CM from the skin and body weight (p < 0.05) and skull height (p < 0.05), but not with the CI (p = 0.23). These findings suggest that a flexed-neck sagittal MRI sequence helps with assessment of the size of the CM and degree of cerebellar herniation, and that skull height and body weight, but not cranial index, affect the distance of the CM from the skin surface.

Persistent fontanelles in Chihuahuas. Part I. Distribution and clinical relevance.

BACKGROUND: The Chihuahua dog breed is known for frequent occurrence of a bregmatic fontanelle on the dorsal skull. A common conception is that this skull defect is a clinically irrelevant finding. No studies, however, describe its prevalence or whether it is accompanied by other persistent fontanelles (PFs). Although Chihuahuas are predisposed to Chiari-like malformation (CM) and syringomyelia (SM), it is unknown whether PFs occur more commonly in dogs with clinical signs that are caused by CM or SM. HYPOTHESIS/OBJECTIVES: To describe the number and location of PFs at cranial sutures (CSs) and to compare the occurrence of these PFs in dogs with and without CM/SM-related clinical signs. We hypothesized that PFs also occur commonly at lateral and caudal cranial surfaces, affect a higher number of CSs, and are larger in dogs with CM/SM-related clinical signs. ANIMALS: Fifty client-owned Chihuahuas with or without CM/SM-related clinical signs. RESULTS: Of the 50 dogs evaluated, 46 (92%) had either 1 or several PFs. The mean ± SD number of PFs was 2.8 ± 3.0 (range, 0-13). A total of 138 PFs occupied 118 CSs with 57 (48%) located dorsally, 44 (37%) caudally, and 17 (14%) laterally. The number of CSs affected by PFs was significantly higher (P ≤ .001) and total PF area was significantly larger (P = .003) in dogs with CM/SM-related clinical signs. CONCLUSIONS AND CLINICAL IMPORTANCE: Persistent fontanelles are very common in this group of Chihuahuas and appear at dorsal, lateral, and caudal cranial surfaces. They are more numerous and larger in Chihuahuas with CM/SM-related clinical signs.

Persistent fontanelles in Chihuahuas. Part II: Association with craniocervical junction abnormalities, syringomyelia, and ventricular volume.

BACKGROUND: Persistent fontanelles (PFs) are, in Chihuahuas, almost ubiquitous. Furthermore, Chihuahuas are predisposed to other craniomorphological abnormalities, including syringomyelia (SM), ventriculomegaly, and craniocervical junction (CCJ) overcrowding resulting in neural tissue deviation. It is, however, undetermined if PFs are more common in dogs with these structural abnormalities, and their etiology is unknown. HYPOTHESIS/OBJECTIVES: Persistent fontanelles are more numerous and larger in Chihuahuas with low body weight, older age, SM, dilated fourth ventricle, ventriculomegaly, and CCJ overcrowding. ANIMALS: Fifty client-owned Chihuahuas. METHODS: Cross-sectional study evaluating the association of both the number of cranial sutures affected by PFs (NAS) and total fontanelle area (TFA), based on computed tomography with SM, fourth ventricle dilatation, lateral ventricle volume, and extent of neural tissue compression at the CCJ based on magnetic resonance images. RESULTS: The NASs was higher and TFA larger in dogs with low body weight (NAS: P = .007; 95% confidence interval [CI] = 0.384-0.861; TFA: P = .002; 95% CI = -1.91 to -0.478), larger lateral ventricles (NAS: P ≤ .001; 95% CI = 1.04-1.15; TFA: P ≤ .001; 95% CI = 0.099-0.363), and more severe neural tissue compression at the CCJ (NAS: P ≤ .001; 95% CI = 1.26-2.06; TFA: P = .03; 95% CI = 0.066-1.13). Similarly, dogs with SM (NAS: P = .004; 95% CI = 1.26-3.32; TFA: mean ± SD, 130 ± 217 mm2 ; P = .05) had higher NAS and larger TFA than did dogs without SM (43.7 ± 61.0 mm2 ). Age was not associated with NAS (P = .81; 95% CI = 0.989-1.01) or TFA (P = .33; 95% CI = -0.269 to 0.092). CONCLUSIONS AND CLINICAL IMPORTANCE: Persistent fontanelles are associated with small size, SM, ventriculomegaly, and CCJ overcrowding.

Association between filum terminale internum length and pain in Cavalier King Charles spaniels with and without syringomyelia.

BACKGROUND: Lumbar syringomyelia (SM), lumbosacral pain, and more caudal spinal cord termination are reported in Cavalier King Charles spaniels (CKCS). Data are lacking on the clinical relevance of alterations in their spinal cord terminal structures. OBJECTIVES: To compare spinal cord termination level and filum terminale internum length (FTIL) with presence of lumbar SM and clinical signs in CKCS. ANIMALS: Forty-eight CKCS. METHODS: In this prospective study, pain was quantified using owner and clinician assessments. Vertebral level of spinal cord and dural sac termination, presence of SM, and FTIL were determined from sagittal magnetic resonance imaging (MRI) sequences. Kappa and intraclass correlation (ICC) analyses determined interobserver reliability. The MRI findings were compared to owner and clinician-reported pain quantification. RESULTS: Interobserver reliability was good for spinal cord and dural sac termination (kappa = 0.61 and 0.64, respectively) and excellent for FTIL (ICC: 92% agreement). The spinal cord terminated at 6th lumbar vertebra in 1, 7th lumbar vertebra in 31, and the sacrum in 15 dogs, and termination level was associated with lumbar SM (P = .002) but not clinical signs. Mean FTIL was 2.9 ± 1.08 mm; it was associated with owner-reported pain (P = .033) and spinal palpation scores (P = .023). Painful CKCS without SM had shorter FTIL compared to normal CKCS and painful CKCS with SM (P = .02). CONCLUSIONS: Painful CKCS without SM have decreased distance between the termination of the spinal cord and dural sac, suggesting a shorter FTIL. More caudal spinal cord termination is associated with development of lumbar SM.

Clinical Presentation of Chiari-like Malformation in 2 Persian Cats.

Two Persian breed cats, 10 and 5 years of age, were presented separately for difficulty prehending food as well as behavioral abnormalities including interanimal aggression and restlessness, pacing, or compulsive overgrooming. Both cats would regularly rest their head and neck in an extended position. Neurologic examination demonstrated calvarial and craniocervical junction pain in both and an L4-S3 myelopathy in one. Brain MRI of both cases, and CT and necropsy in 1 case, demonstrated ventriculomegaly and caudal fossa crowding, cerebellar indentation, and foramen magnum herniation consistent with Chiari-like malformation. No syringomyelia was present in either cat. The 2 cats were treated with anti-inflammatory doses of prednisolone with little to no clinical response, but experienced improvement with pregabalin and omeprazole. The 2 cats' clinical signs were consequently attributed to neuropathic and posture-related pain secondary to Chiari-like malformation. Persian breed cats may have a predisposition to Chiari-like malformation, which may not be solely a morphometric variant, and symptomatic cats may present with manifestations of neuropathic pain different from the classic signs reported in dogs.

Medical infrared thermal imaging of syringomyelia in the Cavalier King Charles Spaniel.

BACKGROUND: Medical infrared thermal imaging (MITI) is a non-invasive imaging modality gaining popularity in the veterinary field. An infrared camera captures emission of heat and creates a color map in the form of a thermogram. Topical heat emission is influenced by localized disease processes as a result of autonomic nervous system imbalance. The purpose of this study was to determine the utility of using thermography to identify changes in thermographic patterns associated with syringomyelia (SM) presence or absence in Cavalier King Charles Spaniels (CKCS) with Chiari-like Malformation (CLM). RESULTS: In CKCS with CLM, MITI was most accurate at a texture distance of 6. Optimizing imaging feature sets produced a highest accuracy of 69.9% (95% CI: 59.5-79.0%), with 81.3% sensitivity and 57.8% specificity for identifying the presence of syringomyelia. CONCLUSION: Thermographic image analysis is a successful non-invasive, diagnostic test that can be used to screen for syringomyelia presence in a CKCS with CLM.

Mechanical quantitative sensory testing in cavalier King Charles spaniels with and without syringomyelia.

BACKGROUND: Syringomyelia (SM) is a debilitating condition in the cavalier King Charles spaniel (CKCS) that results in neuropathic pain and diminished quality of life. Von Frey aesthesiometry (VFA) is a method of mechanical quantitative sensory testing that provides an objective sensory threshold (ST) value and can be used to quantify neuropathic pain (NP) and monitor response to therapy. The utility of VFA has been previously established in client-owned dogs with acute spinal cord injury but the technique has not been evaluated in dogs with SM. The goal of this study was to evaluate ST, as determined by VFA, in dogs with and without SM, to assess the utility of VFA in quantifying NP in SM-affected dogs. We hypothesized the SM-affected CKCS would have lower ST values, consistent with hyperesthesia, when compared to control CKCS. Additionally, we hypothesized that ST values in SM-affected dogs would be inversely correlated with syrinx size on MRI and with owner-derived clinical sign scores. RESULTS: ST values for the thoracic and pelvic limbs differed significantly between the SM-affected and control CKCS (p = 0.027; p = 0.0396 respectively). Median ST value (range) for the thoracic limbs was 184.1 g (120.9-552) for control dogs, and 139.9 g (52.6-250.9) for SM-affected dogs. The median ST value (range) for the pelvic limbs was 164.9 g (100.8-260.3) in control dogs and 129.8 g (57.95-168.4) in SM-affected dogs. The ST values in SM-affected dogs did not correlate with syrinx height on MRI (r = 0.314; p = 0.137). Owner-reported clinical sign scores showed an inverse correlation with pelvic limb ST values, where dogs with lower ST values (hyperesthesia) were reported by their owners to display more frequent and severe clinical signs (r = - 0.657; p = 0.022). CONCLUSION: ST values were lower in SM-affected CKCS compared to control dogs, suggesting the presence of neuropathic pain. Dogs with lower ST pelvic limb values were perceived by their owners to have more severe clinical signs classically associated with SM. Our results suggest that VFA might offer quantitative assessment of neuropathic pain in SM-affected dogs and could be useful for monitoring response to therapy in future clinical studies.

Pregabalin alleviates clinical signs of syringomyelia-related central neuropathic pain in Cavalier King Charles Spaniel dogs: a randomized controlled trial.

OBJECTIVE: We aimed to assess the efficacy and benefit-risk profile of pregabalin (PGN) to reduce the clinical signs of central neuropathic pain (CNeP) as reflected by scratching episodes in dogs with symptomatic syringomyelia (SM). STUDY DESIGN: Randomized, double-blind, placebo-controlled crossover study. ANIMALS: A total of 12 client-owned Cavalier King Charles Spaniels (age, 1.1-7.4 years, bodyweight, 8.2-10.8 kg) with magnetic resonance imaging-confirmed SM and clinical signs of CNeP. METHODS: Dogs were randomized to either PGN 150 mg or placebo for 25 days, followed by 48 hour washout period before crossover to the alternate phase of 25 days. The primary outcome was defined as number of scratching events during 10 minutes of video-recorded physical activity. Treatment effect was estimated using a generalized estimation equation model. Benefit-risk and quality of life assessments were obtained through owner interviews focusing on potential adverse events. RESULTS: The treatment effect estimate was an 84% (95% confidence interval = 75-89%) reduction in mean number of scratching events relative to baseline compared with placebo (p < 0.0001). Owner-assessed satisfactory quality of life was status quo and rated as 'good' or 'could not be better' in six/11 dogs and improved in four/11 dogs. The most prevalent adverse events were increased appetite in nine/12 dogs and transient ataxia in nine/12 dogs. There was one dog withdrawn by the owner 7 days after crossover to PGN owing to persistent ataxia. No dogs needed rescue analgesia during the trial. CONCLUSIONS AND CLINICAL RELEVANCE: PGN is superior to placebo in the reduction of clinical signs of SM-related CNeP in dogs. At a dose range of 13-19 mg kg-1 orally twice daily, the encountered adverse events were acceptable to all but one owner.

Pregabalin for the treatment of syringomyelia-associated neuropathic pain in dogs: A randomised, placebo-controlled, double-masked clinical trial.

Pregabalin is the first-line treatment for neuropathic pain (NeP) in humans. Dogs with Chiari-like malformation and syringomyelia (CM/SM) associated with NeP could benefit from pregabalin. The aim of this study was to evaluate the efficacy of pregabalin for NeP in dogs with CM/SM. Eight dogs with symptomatic CM/SM were included in a double-masked, randomised, crossover placebo-controlled clinical trial. All dogs received anti-inflammatory drugs as base-line treatment during placebo or pregabalin phase of 14±4 days each. Analgesic efficacy was assessed with a daily numerical rating scale (NRS) recorded by dog owners (0-10, 10=worst pain) and quantitative sensory testing at baseline, placebo and pregabalin phases. Blood samples were collected to report pregabalin exposure and to assess renal function. Daily NRS scores recorded by dog owners in the pregabalin group were lower than in the placebo group (P=0.006). Mechanical thresholds were higher with pregabalin compared to baseline or placebo (P=0.037, P<0.001). Cold latency at 15°C was prolonged on the neck and humeri with pregabalin compared to baseline (P<0.001 for both) or placebo (P=0.02, P=0.0001). Cold latency at 0°C was longer on pregabalin compared to baseline and placebo (P=0.001, P=0.004). There was no pregabalin accumulation between first and last dose. This study demonstrates the efficacy of pregabalin for the treatment of NeP due to CM/SM on daily pain scores recorded by dog owners. Pregabalin significantly reduced mechanical hyperalgesia, cold hyperalgesia (0°C) and allodynia (15°C) compared to placebo. Pregabalin was non-cumulative and well tolerated with occasional mild sedation.

Mechanical sensory threshold in Cavalier King Charles spaniels with syringomyelia-associated scratching and control dogs.

It is assumed that Cavalier King Charles spaniels with Chiari-like malformation and syringomyelia experience central neuropathic pain. An association between spinal cord parenchymal lesions and specific clinical signs (e.g. spontaneous and evoked scratching, withdrawal, and paroxysmal pain manifestations with vocalisation) has been suggested. This led to the hypothesis that mechanical sensory threshold is altered in clinical cases. The aim of this study was to quantify the cervical mechanical sensory threshold using Semmes-Weinstein monofilaments in nine Cavalier King Charles spaniels with Chiari-like malformation and assumed syringomyelia-associated central neuropathic pain compared to eight control dogs. Clinical and neurological examination including magnetic resonance imaging was undertaken. Mean mechanical sensory threshold was not significantly different between case and control dogs (t-test on log10 transformed data; P=0.25). Substantial variation within and between dogs was seen, with individual thresholds ranging from 0.04 to 26g in case dogs and from 0.02 to 10g in control dogs. Based on these results, it is unlikely that Cavalier King Charles spaniels with Chiari-like malformation and syringomyelia have increased mechanical sensation characterised by lower mechanical sensory threshold when quantified with Semmes-Weinstein monofilaments. Whether clinical cases experience central neuropathic pain remains unknown. The assessment of sensory function in dogs with assumed central neuropathic pain should be multimodal and include not only mechanical but also tactile and thermal threshold quantification. The use of threshold quantification in a clinical setting is challenging due to an insufficient signal relative to the biological background noise within and between dogs.

Signs consistent with syringobulbia may be detected in dogs undergoing MRI.

Syringobulbia is a pathologic condition characterized by one or more fluid-filled cavities within the brainstem. This retrospective case series describes observations in eight dogs with syringobulbia diagnosed during MRI. All dogs were adult, small-breed dogs with concurrent syringomyelia and neurologic deficits localized to sites rostral to the spinal cord, which cannot be explained by syringomyelia (eg, six dogs had vestibular signs). On MRI, the fluid-filled cavities had signal intensity characteristics like cerebrospinal fluid, were in the medulla oblongata, and were solitary in each dog. Initially, the shape of the cavity was a slit in five dogs and bulbous in two dogs. Magnetic resonance imaging was repeated in five dogs (6-55 months of age). One dog had progression of syringobulbia from slit-like to bulbous, and four dogs had unchanged slit-like syringobulbia. One dog developed slit-like syringobulbia after cranioplasty. A variety of medical and surgical treatments were performed with improvement of some but not all clinical signs. One dog died following surgery due to cardiopulmonary failure and the other seven dogs were alive at least 1 year after the initial diagnosis, which was the least time of follow-up. One surviving dog developed a unilateral hypoglossal nerve deficit 2 months after the initial diagnosis and megaesophagus 14 months later. In conclusion, detecting a fluid-filled cavity in the medulla oblongata consistent with syringobulbia is possible in dogs undergoing MRI. The cavity is likely acquired, slit-like or bulbous, progressive, or static, and might be associated with breed size and neurologic signs localized to the medulla oblongata.

Pilot study of head conformation changes over time in the Cavalier King Charles spaniel breed.

Modern interpretation of head conformation in the Cavalier King Charles spaniel (CKCS) has favoured a smaller, more exaggerated, brachycephalic type than originally described in the 1929 breed standard. Recent research studies identified brachycephaly and reduced hind cranium as two conformational (dysmorphic) features that increase risk for symptomatic Chiari-like malformation and secondary syringomyelia (SM). A prospective pilot study investigated the hypothesis that dysmorphic head features could be assessed visually and correlated with risk of SM. Thirteen CKCS, selected from anonymised photographic evidence, were physically appraised by authorised Kennel Club judges using a head shape checklist. These subjective evaluations were then matched with objective measurements of the cranium (cephalic index and rostrocaudal doming) and their subsequent MRI. A positive correlation (P=0.039) between the judges' checklist score and rostrocaudal doming (hindskull ratio) and a positive correlation between the cephalic index and hindskull ratio (P=0.042) were identified. Five CKCS had no SM and their status tallied with 62 per cent of the judges' evaluation. Although the ability of adjudicators to identify differences in head conformation varied, there was sufficient association between the dysmorphic parameters and the risk of SM to cause concern and propose a larger study in CKCS breed.

Secondary sacral tethered cord syndrome after treatment of secondary cervical syringomyelia.

A female patient operated at the age of 11 on a pilocytic astrocytoma in the posterior fossa, was re-operated for a recurrence 9 years later. A Torkildsen drain was placed in the 4th ventricle and she remained asymptomatic for 15 years before presenting again with acute hydrocephalus, tonsillar herniation, and a massive cervicothoracic syrinx. The symptoms retreated following craniocervical untethering and decompression. Two weeks later, however, she suffered from debilitating radiculopathy provoked by tethering of the cauda equina. Again, symptoms retreated completely after microsurgical intervention. This combination of a secondary Chiari-like malformation with cervical syringomyelia and tethering of the cauda equina in a single patient as a delayed complication of posterior fossa surgery has not been reported before. Diagnosis and treatment of lower tethered cord syndrome may be obscured and delayed in complex clinical situations. In particular, awareness to symptoms that are not related to syringomyelia is important in patients with a known syrinx and a history of posterior fossa surgery.

Understanding and Treating Chiari-like Malformation and Syringomyelia in Dogs.

Chiari-like malformation (CM) and syringomyelia (SM) are common and debilitating conditions in toy and small breed dogs. CM, considered ubiquitous in the cavalier King Charles spaniel (CKCS) population, results in abnormal cerebrospinal fluid dynamics which can lead to the development of SM. The clinical signs associated with CM/SM are frequently confused with other otologic and dermatologic conditions, which may delay appropriate treatment. A definitive diagnosis of CM/SM requires advanced imaging; however, due to expense associated with this, many cases are managed presumptively and symptomatically for the condition. The primary goal of treatment is to manage neuropathic pain and neurologic deficits through pharmaceutical or surgical approaches. Current literature suggests that most CM/SM-affected dogs have progression of their clinical signs in spite of medical or surgical management; however, most maintain a good quality of life based on owner assessments. Lack of correlation between structural markers of disease and clinician and owner-derived measures of neuropathic pain highlight the need for more robust, quantitative measures of pain in this common veterinary disease.

MRI characteristics for "phantom" scratching in canine syringomyelia.

BACKGROUND: A classic sign of canine syringomyelia (SM) is scratching towards one shoulder. Using magnetic resonance imaging (MRI) we investigate the spinal cord lesion relating to this phenomenon which has characteristics similar to fictive scratch secondary to spinal cord transection. Medical records were searched for Cavalier King Charles spaniels with a clinical and MRI diagnosis of symptomatic SM associated with Chiari-like malformation (CM). The cohort was divided into SM with phantom scratching (19 dogs) and SM but no phantom scratching (18 dogs). MRI files were anonymised, randomised and viewed in EFILM ™. For each transverse image, the maximum perpendicular dimensions of the syrinx in the dorsal spinal cord quadrants were determined. Visual assessment was made as to whether the syrinx extended to the superficial dorsal horn (SDH). RESULTS: We showed that phantom scratching appears associated with a large dorsolateral syrinx that extends to the SDH in the C3-C6 spinal cord segments (corresponding to C2-C5 vertebrae). Estimated dorsal quadrant syrinx sizes based on the perpendicular diameters were between 2.5 and 9.5 times larger in dogs with phantom scratching, with the largest mean difference p-value being 0.009. CONCLUSION: SM associated phantom scratching appears associated with MRI findings of a large syrinx extending into the mid cervical SDH. We hypothesise that damage in this region might influence the lumbosacral scratching central pattern generator (CPG). If a scratching SM affected dog does not have a large dorsolateral cervical syrinx with SDH involvement then alternative explanations for scratching should be investigated.

Syringomyelia and Craniocervical Junction Abnormalities in Chihuahuas.

BACKGROUND: Chiari-like malformation (CM) and syringomyelia (SM) are widely reported in Cavalier King Charles Spaniels and Griffon Bruxellois dogs. Increasing evidence indicates that CM and SM also occur in other small and toy breed dogs, such as Chihuahuas. OBJECTIVES: To describe the presence of SM and craniocervical junction (CCJ) abnormalities in Chihuahuas and to evaluate the possible association of CCJ abnormalities with SM. To describe CM/SM-related clinical signs and neurologic deficits and to investigate the association of CM/SM-related clinical signs with signalment, SM, or CCJ abnormalities. ANIMALS: Fifty-three client-owned Chihuahuas. METHODS: Prospective study. Questionnaire analyses and physical and neurologic examinations were obtained before magnetic resonance and computed tomography imaging. Images were evaluated for the presence of SM, CM, and atlantooccipital overlapping. Additionally, medullary kinking, dorsal spinal cord compression, and their sum indices were calculated. RESULTS: Scratching was the most common CM/SM-related clinical sign and decreased postural reaction the most common neurologic deficit in 73 and 87% of dogs, respectively. Chiari-like malformation and SM were present in 100 and 38% of dogs, respectively. Syringomyelia was associated with the presence of CM/SM-related clinical signs (P = 0.034), and medullary kinking and sum indices were higher in dogs with clinical signs (P = 0.016 and P = 0.007, respectively). CONCLUSIONS AND CLINICAL IMPORTANCE: Syringomyelia and CCJ abnormalities are prevalent in Chihuahuas. Syringomyelia was an important factor for the presence of CM/SM-related clinical signs, but many dogs suffered from similar clinical signs without being affected by SM, highlighting the clinical importance of CCJ abnormalities in Chihuahuas.