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BMJ Case Reports Jun 2021The Chiari I malformation (CM-I) is characterised by overcrowding of the posterior fossa and descent of the cerebellar tonsils and is associated with syringomyelia. With... (Review)
Review
The Chiari I malformation (CM-I) is characterised by overcrowding of the posterior fossa and descent of the cerebellar tonsils and is associated with syringomyelia. With the increasing availability of magnetic resonance imaging, CM-I is placing a growing burden on neurosurgical services. However, its natural history remains poorly understood, and the timing and nature of surgical intervention is controversial. We present a case of a significant, symptomatic CM-I with associated syrinx which underwent complete spontaneous resolution over a 4-year period. Spontaneous regression of Chiari malformation and syringomyelia is exceedingly rare; a literature review reveals 15 other cases and only one case which underwent complete resolution. The present case and literature review suggest a more benign natural history of CM-I and support a more conservative approach to its management. Further studies are required to determine whether any factors can predict resolution for certain patient cohorts.
Topics: Arnold-Chiari Malformation; Decompression, Surgical; Humans; Magnetic Resonance Imaging; Syringomyelia; Trachea
PubMed: 34155013
DOI: 10.1136/bcr-2021-241789 -
Neurology India 2018
Review
Topics: Arachnoid; Arachnoid Cysts; Humans; Laminectomy; Magnetic Resonance Imaging; Male; Middle Aged; Syringomyelia; Treatment Outcome
PubMed: 30504600
DOI: 10.4103/0028-3886.246271 -
Neurology India 2018
Topics: Arnold-Chiari Malformation; Atlanto-Axial Joint; Decompression, Surgical; Humans; Joint Instability; Platybasia; Syringomyelia
PubMed: 30038072
DOI: 10.4103/0028-3886.236992 -
Acta Neurochirurgica Oct 2020Following spinal cord injury (SCI), the routine use of magnetic resonance imaging (MRI) resulted in an incremental diagnosis of posttraumatic syringomyelia (PTS)....
BACKGROUND
Following spinal cord injury (SCI), the routine use of magnetic resonance imaging (MRI) resulted in an incremental diagnosis of posttraumatic syringomyelia (PTS). However, facing four decades of preferred surgical treatment of PTS, no clear consensus on the recommended treatment exists. We review the literature on PTS regarding therapeutic strategies, outcomes, and complications.
METHODS
We performed a systematic bibliographic search on ("spinal cord injuries" [Mesh] AND "syringomyelia" [Mesh]). English language literature published between 1980 and 2020 was gathered, and case reports and articles examining syrinx due to other causes were excluded. The type of study, interval injury to symptoms, severity and level of injury, therapeutic procedure, duration of follow-up, complications, and outcome were recorded.
RESULTS
Forty-three observational studies including 1803 individuals met the eligibility criteria. The time interval from SCI to the diagnosis of PTS varied between 42 and 264 months. Eighty-nine percent of patients were treated surgically (n = 1605) with a complication rate of 26%. Symptoms improved in 43% of patients postoperatively and in 2% treated conservatively. Stable disease was documented in 50% of patients postoperatively and in 88% treated conservatively. The percentage of deterioration was similar (surgery 16%, 0.8% dead; conservative 10%). Detailed analysis of surgical outcome with regard to symptoms revealed that pain, motor, and sensory function could be improved in 43 to 55% of patients while motor function deteriorated in around 25%. The preferred methods of surgery were arachnoid lysis (48%) and syrinx drainage (31%).
CONCLUSION
Even diagnosing PTS early in its evolution with MRI, to date, no satisfactory standard treatment exists, and the present literature review shows similar outcomes, regardless of the treatment modality. Therefore, PTS remains a neurosurgical challenge. Additional research is required using appropriate study designs for improving treatment options.
Topics: Adult; Decompression, Surgical; Drainage; Female; Humans; Male; Middle Aged; Postoperative Complications; Reoperation; Sensation; Spinal Cord Injuries; Syringomyelia
PubMed: 32820376
DOI: 10.1007/s00701-020-04529-w -
Journal of Neurology, Neurosurgery, and... Jul 1999The features or mechanisms of dyshidrosis have not been sufficiently clarified. Neither has the difference between hyperhidrosis and hypohidrosis. To clarify the...
The features or mechanisms of dyshidrosis have not been sufficiently clarified. Neither has the difference between hyperhidrosis and hypohidrosis. To clarify the features and mechanisms of dyshidrosis (hyperhidrosis and hypohidrosis) in syringomyelia, the clinical features focusing on hidrosis of 30 patients with syringomyelia and Chiari malformation located from a syringomyelia database were prospectively analysed. The patients were classified into three groups: eight patients (26.7%) had segmental hypohidrosis, 10 (33. 3%) had segmental hyperhidrosis, and 12 (40.0%) had normohidrosis. We found that the Karnofsky functional status for the hyperhydrosis and normohidrosis groups were significantly higher than for the hypohidrosis group (p=0.0012), with no significant differences between the hyperhidrosis and normohidrosis groups. The duration from the onset of syringomyelia to the current dyshidrosis was significantly longer in the hypohidrosis group than in the hyperhidrosis group (p=0.0027). A significant correlation was identified between the duration from the onset of syringomyelia to the time at study and the performance score (r=-0.599, p=0.0003). The results substantiate previous hypotheses that in its early stage syringomyelia causes segmental hyperactivity of the sympathetic preganglionic neurons, and hyperactivity of these gradually subsides as tissue damage progresses. Focal hyperhidrosis may be regarded as a hallmark of a relatively intact spinal cord, as well as normohidrosis.
Topics: Adolescent; Adult; Aged; Analysis of Variance; Body Temperature; Humans; Hyperhidrosis; Hypohidrosis; Middle Aged; Syringomyelia
PubMed: 10369833
DOI: 10.1136/jnnp.67.1.106 -
Neurosciences (Riyadh, Saudi Arabia) Aug 2020To assess the correlation between craniovertebral junction (CVJ) abnormalities and syringomyelia in patients with Chiari malformation type-1 (CM1).
OBJECTIVE
To assess the correlation between craniovertebral junction (CVJ) abnormalities and syringomyelia in patients with Chiari malformation type-1 (CM1).
METHODS
This was a retrospective study including patients with CM1. Identification of cases was done by searching a radiology database at a university hospital from 2012 to 2017. Patients were divided into 2 groups based on whether CVJ abnormalities were present (CVJ+) or absent (CVJ-). The patients` demographic and clinical data were reviewed. All magnetic resonance imaging studies were examined by a certified neuroradiologist.
RESULTS
Sixty-four consecutive patients with CM1 were included. The mean age was 24+/-17 years; 59% were females. The CVJ+ group had more female patients (p=0.012). The most frequent CVJ abnormality was platybasia (71%), followed by short clivus (44%) and cervical kyphosis (33%). The CVJ abnormalities were more in Syringomyelia cases (p=0.045). However, the results were not significant when hydrocephalus cases were excluded.
CONCLUSION
Among CM1 patients, CVJ abnormalities were found more in patients with syringomyelia. Future studies with larger sample size are required to further study the correlation between CVJ abnormalities and both syringomyelia and hydrocephalus in CM1 patients.
Topics: Adult; Arnold-Chiari Malformation; Atlanto-Occipital Joint; Female; Humans; Male; Retrospective Studies; Syringomyelia; Young Adult
PubMed: 33130812
DOI: 10.17712/nsj.2020.4.20200008 -
The Journal of Veterinary Medical... Sep 2022The objective of this study was to calculate lateral ventricles dimension in Cavalier King Charles Spaniel dogs with Chiari-like malformation and investigate the...
The objective of this study was to calculate lateral ventricles dimension in Cavalier King Charles Spaniel dogs with Chiari-like malformation and investigate the association between ventriculomegaly and signalment, clinical signs, ventricular asymmetry, grade of Chiari-like malformation, syringomyelia and index of medullary kinking. Retrospectively, 43 client-owned Cavalier King Charles Spaniels, older than 1 year of age, with magnetic resonance imaging diagnosis of Chiari-like malformation were enrolled. Initial and follow-up (up to 36 months) clinical status was graded. Images were reviewed to quantify the enlargement of lateral ventricles, evaluate ventricular symmetry, grade of Chiari-like malformation, grade of syringomyelia and medullary kinking index. Cases presenting epileptic seizures during the evaluation period were also recorded. The most common initial clinical signs were scratching and neck pain. Ventriculomegaly was identified in 70% of dogs, Chiari-like malformation grade 2 was observed in 77% of cases, ventricular asymmetry and syringomyelia were identified in 54% and 80% of dogs, respectively; the median medullary kinking index was 37.77%. Moreover, 28% of dogs presented epileptic seizures. No significant association was identified between dimension of lateral ventricles and signalment, clinical signs, and imaging findings; no significant association was identified between ventriculomegaly and epilepsy (P≥0.05). In conclusion, the prevalence of ventriculomegaly in Cavalier King Charles Spaniels is high but this finding does not seem related to the severity of clinical signs, presence of Chiari-like malformation, syringomyelia and craniocervical junction abnormalities such as medullary kinking.
Topics: Animals; Dog Diseases; Dogs; Hydrocephalus; Magnetic Resonance Imaging; Retrospective Studies; Seizures; Syringomyelia
PubMed: 35831130
DOI: 10.1292/jvms.22-0134 -
Acta Neurochirurgica 1995Based on an own material of 19 patients with syringomyelia and on the related literature a survey is given on the diagnosis, differential diagnosis, postoperative... (Review)
Review
Based on an own material of 19 patients with syringomyelia and on the related literature a survey is given on the diagnosis, differential diagnosis, postoperative evaluation and the dynamics of CSF and cyst fluids, using magnetic resonance imaging (MRI). The following conclusions can be drawn: 1. MRI is the preferred method of investigation for diagnosis and differential diagnosis of syringomyelia. 2. Using MRI, it is possible to study fluid flow in the vertebral canal and the movements of the syrinx fluid. The data are as yet limited, but in the future will form an important contribution to our understanding of the pathogenesis of syringomyelia and to the choice of a treatment method. 3. MRI is important for the postoperative follow-up of patients with syringomyelia, including tracking the cyst and detecting complications.
Topics: Arnold-Chiari Malformation; Atrophy; Diagnosis, Differential; Humans; Magnetic Resonance Imaging; Postoperative Complications; Spinal Cord; Syringomyelia
PubMed: 7668137
DOI: 10.1007/BF01428512 -
Journal of Veterinary Internal Medicine 2024Syringomyelia (SM) and myxomatous mitral valve disease (MMVD) are highly prevalent in Cavalier King Charles spaniels (CKCS). Cardiac status in CKCS with and without SM...
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.
Topics: Humans; Dogs; Animals; Mitral Valve; Retrospective Studies; Prospective Studies; Syringomyelia; Dog Diseases; Heart Valve Diseases
PubMed: 38391152
DOI: 10.1111/jvim.17018 -
Veterinary Journal (London, England :... Apr 2019It is assumed that Cavalier King Charles spaniels with Chiari-like malformation and syringomyelia experience central neuropathic pain. An association between spinal cord...
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.
Topics: Animals; Behavior, Animal; Case-Control Studies; Dog Diseases; Dogs; Female; Magnetic Resonance Imaging; Male; Mechanotransduction, Cellular; Pain; Prospective Studies; Sensory Thresholds; Spinal Cord; Syringomyelia
PubMed: 30902196
DOI: 10.1016/j.tvjl.2019.01.011