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Journal of Postgraduate Medicine 2001
Review
Topics: Arnold-Chiari Malformation; Humans; Hydrocephalus; Spinal Canal; Syringomyelia
PubMed: 11832595
DOI: No ID Found -
Scientific Reports Jun 2023Aquaporin-4 (AQP4) has been implicated in post-traumatic syringomyelia (PTS), a disease characterised by the formation of fluid-filled cysts in the spinal cord. This...
Aquaporin-4 (AQP4) has been implicated in post-traumatic syringomyelia (PTS), a disease characterised by the formation of fluid-filled cysts in the spinal cord. This study investigated the expression of AQP4 around a mature cyst (syrinx) and the effect of pharmacomodulation of AQP4 on syrinx size. PTS was induced in male Sprague-Dawley rats by computerized spinal cord impact and subarachnoid kaolin injection. Immunofluorescence of AQP4 was carried out on mature syrinx tissue 12 weeks post-surgery. Increased AQP4 expression corresponded to larger, multiloculated cysts (R = 0.94), yet no localized changes to AQP4 expression in perivascular regions or the glia limitans were present. In a separate cohort of animals, at 6 weeks post-surgery, an AQP4 agonist (AqF026), antagonist (AqB050), or vehicle was administered daily over 4 days, with MRIs performed before and after the completion of treatment. Histological analysis was performed at 12 weeks post-surgery. Syrinx volume and length were not altered with AQP4 modulation. The correlation between increased AQP4 expression with syrinx area suggests that AQP4 or the glia expressing AQP4 are recruited to regulate water movement. Given this, further investigation should examine AQP4 modulation with dose regimens at earlier time-points after PTS induction, as these may alter the course of syrinx development.
Topics: Animals; Male; Rats; Aquaporin 4; Cysts; Rats, Sprague-Dawley; Syringomyelia
PubMed: 37316571
DOI: 10.1038/s41598-023-36538-x -
The Neuroradiology Journal Jun 2023The reporting case is a 27 year-old woman. She was admitted with complaints of clumsy gait and dysesthesia over her upper limbs and spasticity in the legs. The...
The reporting case is a 27 year-old woman. She was admitted with complaints of clumsy gait and dysesthesia over her upper limbs and spasticity in the legs. The investigations revealed a long segment cervical intramedullary tumor, associated with whole cord syringomyelia. The superior part of syringomyelia image intensity matched intramedullary lipoma completely. C1-C5 intramedullary tumor was resected grossly under microscopic view, and a fat-containing liquid exited the syringomyelia cavity at its superior pole. After a transient paresis just following the surgery, neurological condition was improved. The follow-up imaging showed that the fatty syringomyelia reduced in dimensions. . According to this case and another similar case due to instability, it appears that as a rare phenomenon, syringomyelia liquid content sometimes could have fatty droplets which could mimic intramedullary lipoma with an unknown mechanism. It seems these fat droplets in syringomyelia would not change its natural history.
Topics: Humans; Female; Adult; Syringomyelia; Magnetic Resonance Imaging; Spinal Cord Neoplasms; Lipoma; Astrocytoma; Spinal Cord
PubMed: 36384332
DOI: 10.1177/19714009221140535 -
American Journal of Veterinary Research May 2011To determine the effects of syringomyelia on electromyography (EMG) findings, somatosensory-evoked potentials (SEPs), and transcranial magnetic motor-evoked potentials...
OBJECTIVE
To determine the effects of syringomyelia on electromyography (EMG) findings, somatosensory-evoked potentials (SEPs), and transcranial magnetic motor-evoked potentials (TMMEPs) in Cavalier King Charles Spaniels (CKCSs).
ANIMALS
27 client-owned CKCSs that underwent prebreeding magnetic resonance imaging screening or investigation of clinical signs consistent with syringomyelia.
PROCEDURES
In dogs with (n = 11) and without (16) magnetic resonance imaging-confirmed syringomyelia, the median nerve in each thoracic limb was stimulated and SEPs were recorded over the C1 vertebra; onset latency and latency and amplitude of the largest negative (N1) and positive (P1) peaks were measured. The TMMEPs were recorded bilaterally from the extensor carpi radialis and tibialis cranialis muscles; onset latencies in all 4 limbs were measured. Bilateral systematic needle EMG examination was performed on the cervical epaxial musculature, and the number of sites with spontaneous activity was recorded.
RESULTS
In dogs with syringomyelia, amplitudes of N1 and P1 and the amplitude difference between P1 and N1 were significantly smaller than those recorded for dogs without syringomyelia (approx 2-fold difference). No difference in SEP latencies, TMMEP latencies, or the proportion of dogs with > 2 sites of spontaneous activity detected during EMG examination was detected between groups.
CONCLUSIONS AND CLINICAL RELEVANCE
Results indicated that SEP amplitude at the C1 vertebra was a more sensitive measure of spinal cord function in CKCSs with syringomyelia, compared with results of EMG or TMMEP assessment. Measurement of SEP amplitude may have use as an objective assessment of the evolution and treatment of this disease.
Topics: Animals; Cervical Vertebrae; Dog Diseases; Dogs; Electromyography; Evoked Potentials, Motor; Evoked Potentials, Somatosensory; Female; Forelimb; Magnetic Resonance Imaging; Male; Median Nerve; Pedigree; Prospective Studies; Spinal Cord; Syringomyelia; Transcranial Magnetic Stimulation
PubMed: 21529209
DOI: 10.2460/ajvr.72.5.595 -
Neurology India 2023
Topics: Humans; Elbow Joint; Syringomyelia; Arthropathy, Neurogenic; Arnold-Chiari Malformation
PubMed: 37148079
DOI: 10.4103/0028-3886.375402 -
Journal of Veterinary Internal Medicine Mar 2019There is an association between Chiari malformations, syringomyelia (CMSM) and tethered cord syndrome (TCS) in people, suggesting Cavalier King Charles Spaniels (CKCS)...
BACKGROUND
There is an association between Chiari malformations, syringomyelia (CMSM) and tethered cord syndrome (TCS) in people, suggesting Cavalier King Charles Spaniels (CKCS) with CMSM could also have TCS. Currently there are no data on the position of the caudal spinal cord structures in CKCS.
OBJECTIVE
To describe and compare location of spinal cord termination in CKCS with weight-matched controls and to examine the relationship between SM and spinal cord termination.
ANIMALS
Thirty-nine CKCS and 33 controls with thoracolumbar MRIs; 34 of 39 CKCS also had cervical MRIs.
METHODS
Blinded retrospective study. Spinal cord and dural sac termination were determined from T2-weighted sagittal and transverse images and half-Fourier acquisition single-shot turbo spin echo sequences. Intra-observer reliability was determined using kappa analysis. Presence of SM was compared with location of spinal cord and dural sac termination.
RESULTS
Intra-observer reliability was moderate for identifying spinal cord termination (Kappa = 0.6) and good for dural sac termination (Kappa = 0.8). The spinal cord terminated at lumbar vertebra 6 (L6) in 1, 7 (L7) in 22, and sacrum in 16 CKCS versus 9 at L6, 23 at L7, 1 at sacrum in controls. Spinal cord (P < .001) and dural sac (P = .002) termination were significantly more caudal in CKCS compared to controls. The presence of thoracolumbar SM was associated with more caudal dural sac termination in CKCS (P = .03).
CONCLUSIONS AND CLINICAL IMPORTANCE
The relationship between TL SM and possible spinal cord tethering because of a more caudal dural sac termination should be investigated.
Topics: Animals; Dog Diseases; Dogs; Dura Mater; Female; Magnetic Resonance Imaging; Male; Neural Tube Defects; Reproducibility of Results; Retrospective Studies; Spinal Cord; Syringomyelia
PubMed: 30758868
DOI: 10.1111/jvim.15437 -
Journal of Veterinary Internal Medicine Jan 2018Chiari-like malformation (CM) and syringomyelia (SM) cause a pain syndrome in Cavalier King Charles spaniels (CKCS). Clinical signs are not consistently apparent on...
BACKGROUND
Chiari-like malformation (CM) and syringomyelia (SM) cause a pain syndrome in Cavalier King Charles spaniels (CKCS). Clinical signs are not consistently apparent on neurologic examination, and owner reporting of signs provides vital clinical history. However, owner questionnaires for this disease are not well developed.
OBJECTIVES
To develop a tool to capture owner-reported clinical signs for use in clinical trials and to compare owner-reported signs with the presence of pain on neurologic examination and SM on magnetic resonance imaging (MRI).
ANIMALS
Fifty client-owned CKCS.
METHODS
Owners completed a questionnaire and pain/scratch map. Each dog underwent a neurologic examination and craniocervical magnetic resonance imaging (MRI). Questionnaire responses were developed into scores, area of shading for pain/scratch maps was measured, and consistency of responses between these tools was assessed. Owner-reported findings were compared with neurologic examination findings and presence and severity of SM on MRI.
RESULTS
Thirty-three dogs were symptomatic and 17 asymptomatic; 30 had SM. The most common sign of pain was crying out when lifted (n = 11). Extent of shaded areas on maps positively correlated with questionnaire scores for pain (r = 0.213, P = 0.006) and scratch (r = 0.104, P = 0.089). Owner-reported findings were not significantly associated with presence or severity of SM or neurologic examination findings. Owner-reported lateralization of signs was significantly associated with SM lateralization (P < 0.0001).
CONCLUSIONS
The questionnaire and maps may be useful for clinical trials. Lack of association of owner-reported signs with SM highlights our lack of understanding of the pathophysiology of pain in this disease.
Topics: Animals; Arnold-Chiari Malformation; Dog Diseases; Dogs; Female; Magnetic Resonance Imaging; Male; Neuroimaging; Pain; Pruritus; Surveys and Questionnaires; Syringomyelia
PubMed: 29105875
DOI: 10.1111/jvim.14856 -
Neurological Sciences : Official... Dec 2011Piemonte and Valle d'Aosta Interregional Network for Rare Diseases (RD) is a model of "diffuse" network; it involves all the health system specialists devoted to the...
Piemonte and Valle d'Aosta Interregional Network for Rare Diseases (RD) is a model of "diffuse" network; it involves all the health system specialists devoted to the diagnosis, the therapy and the follow-up of a RD. The Consortium is a multidisciplinary team operating throughout the Region composed of volunteer Physicians and Biologists that promotes periodical meetings to develop shared protocols. In 2008 the Specialist-Technical Committee for the Interregional RD Network approved the set up of the "Chiari and Syringomyelia Consortium" (CSC) with two specific objectives: to identify the shared diagnostic criteria and to develop the interdisciplinary diagnostic-therapeutic-assistance path (DTAP) to be used interregionally. Other objectives are: to define the prevalence of the pathologies (both symptomatic and asymptomatic, both in adults and children) and to improve collaboration between the patient Associations and the Physicians.
Topics: Arnold-Chiari Malformation; Cooperative Behavior; Female; Humans; Interdisciplinary Studies; Male; Rare Diseases; Retrospective Studies; Syringomyelia
PubMed: 21922315
DOI: 10.1007/s10072-011-0725-y -
Fluids and Barriers of the CNS Mar 2019Fluid homeostasis in the central nervous system (CNS) is essential for normal neurological function. Cerebrospinal fluid (CSF) in the subarachnoid space and interstitial...
BACKGROUND
Fluid homeostasis in the central nervous system (CNS) is essential for normal neurological function. Cerebrospinal fluid (CSF) in the subarachnoid space and interstitial fluid circulation in the CNS parenchyma clears metabolites and neurotransmitters and removes pathogens and excess proteins. A thorough understanding of the normal physiology is required in order to understand CNS fluid disorders, including post-traumatic syringomyelia. The aim of this project was to compare fluid transport, using quantitative imaging of tracers, in the spinal cord from animals with normal and obstructed spinal subarachnoid spaces.
METHODS
A modified extradural constriction model was used to obstruct CSF flow in the subarachnoid space at the cervicothoracic junction (C7-T1) in Sprague-Dawley rats. Alexa-Fluor 647 Ovalbumin conjugate was injected into the cisterna magna at either 1 or 6 weeks post-surgery. Macroscopic and microscopic fluorescent imaging were performed in animals sacrificed at 10 or 20 min post-injection. Tracer fluorescence intensity was compared at cervical and thoracic spinal cord levels between control and constriction animals at each post-surgery and post-injection time point. The distribution of tracer around arterioles, venules and capillaries was also compared.
RESULTS
Macroscopically, the fluorescence intensity of CSF tracer was significantly greater in spinal cords from animals with a constricted subarachnoid space compared to controls, except at 1 week post-surgery and 10 min post-injection. CSF tracer fluorescence intensity from microscopic images was significantly higher in the white matter of constriction animals 1 week post surgery and 10 min post-injection. At 6 weeks post-constriction surgery, fluorescence intensity in both gray and white matter was significantly increased in animals sacrificed 10 min post-injection. At 20 min post-injection this difference was significant only in the white matter and was less prominent. CSF tracer was found predominantly in the perivascular spaces of arterioles and venules, as well as the basement membrane of capillaries, highlighting the importance of perivascular pathways in the transport of fluid and solutes in the spinal cord.
CONCLUSIONS
The presence of a subarachnoid space obstruction may lead to an increase in fluid flow within the spinal cord tissue, presenting as increased flow in the perivascular spaces of arterioles and venules, and the basement membranes of capillaries. Increased fluid retention in the spinal cord in the presence of an obstructed subarachnoid space may be a critical step in the development of post-traumatic syringomyelia.
Topics: Animals; Cerebrospinal Fluid; Constriction, Pathologic; Disease Models, Animal; Fluorescent Dyes; Hydrodynamics; Male; Microscopy, Fluorescence; Optical Imaging; Rats, Sprague-Dawley; Spinal Cord; Subarachnoid Space; Syringomyelia
PubMed: 30909935
DOI: 10.1186/s12987-019-0127-8 -
Acta Otorrinolaringologica Espanola 2015Chiari malformation is an alteration of the base of the skull with herniation through the foramen magnum of the brain stem and cerebellum. Although the most common... (Review)
Review
INTRODUCTION
Chiari malformation is an alteration of the base of the skull with herniation through the foramen magnum of the brain stem and cerebellum. Although the most common presentation is occipital headache, the association of audio-vestibular symptoms is not rare. The aim of our study was to describe audio-vestibular signs and symptoms in Chiari malformation type i (CM-I).
MATERIALS AND METHODS
We performed a retrospective observational study of patients referred to our unit during the last 5 years. We also carried out a literature review of audio-vestibular signs and symptoms in this disease.
RESULTS
There were 9 patients (2 males and 7 females), with an average age of 42.8 years. Five patients presented a Ménière-like syndrome; 2 cases, a recurrent vertigo with peripheral features; one patient showed a sudden hearing loss; and one case suffered a sensorineural hearing loss with early childhood onset. The most common audio-vestibular symptom indicated in the literature in patients with CM-I is unsteadiness (49%), followed by dizziness (18%), nystagmus (15%) and hearing loss (15%). Nystagmus is frequently horizontal (74%) or down-beating (18%). Other audio-vestibular signs and symptoms are tinnitus (11%), aural fullness (10%) and hyperacusis (1%). Occipital headache that increases with Valsalva manoeuvres and hand paresthesias are very suggestive symptoms.
CONCLUSIONS
The appearance of audio-vestibular manifestations in CM-I makes it common to refer these patients to neurotologists. Unsteadiness, vertiginous syndromes and sensorineural hearing loss are frequent. Nystagmus, especially horizontal and down-beating, is not rare. It is important for neurotologists to familiarise themselves with CM-I symptoms to be able to consider it in differential diagnosis.
Topics: Adult; Aged; Arnold-Chiari Malformation; Child, Preschool; Female; Headache; Hearing Loss, Sensorineural; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Neuroimaging; Nystagmus, Pathologic; Symptom Assessment; Syringomyelia; Tinnitus; Vertigo
PubMed: 25195076
DOI: 10.1016/j.otorri.2014.05.002