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This is a list of citations and summaries of veterinary research journal articles which are applicable to Chiari-like malformation (CM) and syringomyelia (SM) and cavalier King Charles spaniels. A study of a larger population is needed to attain more reliable information. Most reports describe patients with cervical myelopathy due to syringohydromyelia.

MRI showed no regression of syrinx size 3 months postoperatively. Given the progressive nature of the disorder, evaluation over a longer period of time is necessary to detect if progression has stopped. A disorder similar to human Chiari type 1 malformation occurs in dogs, but characteristic disease features have not been thoroughly described.

Three dogs were evaluated neurologically 24 hours, 1 month, and 3 months postoperatively and evaluations were compared with preoperative neurological examination. Results— Neurological examinations showed neither improvement nor progression of clinical signs 3 months postoperatively. Central nervous system dysfunction often develops in patients with Chiari type 1 malformation, with a variety of possible neurologic manifestations.

The relationship between occipital dysplasia and syringo/hydromyelia in these dogs remains unclear, however, similar associated abnormalities are occasionally found in humans with Chiari malformation." . Response to diuretic therapy was moderate but surgical decompression may offer better long term prognosis." . The dogs with these abnormalities had a wide variety of neurological signs, but there was no apparent correlation between the neurological signs and the severity of cerebellar herniation, syringohydromyelia or hydrocephalus." . Study Design— Four CKCS diagnosed by Magnetic resonance imaging (MRI) of SHM because of cerebellar tonsil herniation and not responsive to medical therapy underwent a suboccipital craniectomy and dorsal laminectomy of C1 (2 dogs) and of C1 and partial C2 (2 dogs) with durotomy and placement of a dural graft. Both direct bony compression and progressive meningeal hypertrophy at the level of the posterior (dorsal) cervicomedullary junction are believed to lead to abnormalities of cerebrospinal fluid flow dynamics.

While occipital dysplasia alone is not thought to cause any clinical abnormalities, the dogs of this report suggest that intramedullary central nervous system abnormalities may be present concurrently with occipital dysplasia and should be considered as a possible cause of the clinical signs. Mechanical obstruction at the craniocervical junction, altering CSF flow dynamics, may lead to syrinx formation. Abnormalities compatible with such a malformation were identified by magnetic resonance imaging in 39 cavalier King Charles spaniels with neurological signs and in one neurologically normal cavalier King Charles spaniel that was examined postmortem. Vermeersch K, Van Ham L, Caemaert J, Tshamala M, Taeymans O, Bhatti S, Polis I. This technique is used with great success in human medicine. Quote: "Chiari type 1 malformation in humans is a congenital abnormality of the caudal occipital bone, resulting in overcrowding of the caudal fossa and compression at the level of the cervicomedullary junction.

In the other dog, a presumptive syringo/hydromyelia of the cervical spinal cord was found on magnetic resonance imaging. Three dogs with hydromyelia {two Maltese poodles and one King Charles spaniel] due to differing etiologies are described." Javaheri S., Corbett W. However, the effect is independent of the K , H -ATPase activity, since Sch 28080 which is more potent than omeprazole did not significantly affect CSF production." . Advanced imaging studies revealed hydrocephalus, caudal herniation of part of the caudal lobe of the cerebellum through the foramen magnum and marked syrinx formation to the level of the caudal thoracic spine, resembling Arnold-Chiari malformation with secondary hydromyelia in humans. Quote: "In human beings a Chiari type 1 malformation is a developmental condition characterised by cerebellar herniation and syringohydromyelia. Seen the progressive nature of the disorder, follow-up over a longer period of time is necessary to see if this surgical technique may influence the progression of syrinx formation and clinical signs in Cavalier King Charles spaniels with Chiari type I malformation." . Quote: "Objective— To evaluate retrospectively the efficacy of the suboccipital craniectomy and dorsal laminectomy of C1 with durotomy and placement of a dural graft for treatment of syringohydromyelia (SHM) because of cerebellar tonsil herniation in Cavalier King Charles spaniels (CKCS).

In dog 1, a midline cervical spinal cord defect consistent with a communicating syrinx was found. Hydromyelia, and its possible etiology, may be confirmed by means of computed tomography or magnetic resonance imaging. significantly more than the respective values in the control group. We conclude that in the canine model, physiological doses of omeprazole decrease CSF production by about 26. Neurological examination suggested a myelopathy of C1 to C4 spinal cord segments. The results of this study indicate that there is no improvement on short term basis in either syrinx size or clinical symptoms after surgical intervention with a suboccipital craniectomy and cranial dorsal laminectomy with opening of the dura.

One dog presented for tetraparesis and cervical hyperesthesia, the other [a Yorkshire terrier] for historical cervical hyperesthesia and mild paraparesis. In dogs hydromyelia may be accidentally diagnosed during routine cisterna magna myelography. Quote: "We investigated the effects of omeprazole and Sch 28080, a more specific and a more potent inhibitor of K , H -ATPase than omeprazole, in canine cerebrospinal fluid (CSF) production. [T]he percent decreases in CSF production in the omeprazole treated group were ... Quote: "A 9-year-old King Charles Spaniel presented with a history of progressive forelimb weakness and paroxysmal involuntary flank scratching over a 2-year period. Control MRI of the 3 dogs at that time showed no regression of syrinx size.

Quote: "Occipital dysplasia was found in association with cervical spinal cord abnormalities in two dogs. In man this may be due to congenital malformations such as Dandy-Walker syndrome and Chiari malformations or may be acquired as result of infection, trauma or neoplasia. The dogs were evaluated neurologically 24 hours, 1 month and 3 months postoperatively. Neurologically, the 3 dogs did worse 24 hours after surgery (neck pain, neck weakness and head tilt), improved gradually and by 3 months postoperatively, achieved the same neurological state as before surgery.

Quote: "Hydromyelia is a dilation of the spinal cord central canal. This surgical technique was performed on 4 Cavalier King Charles spaniels diagnosed with Chiari type I malformation by symptoms (scratching of neck region) and by MRI. The fourth dog was euthanized within 24 hours after surgery at owners request due to progressive seizures and decreased capability of oxygen saturation.

Therefore, in human medicine, a suboccipital craniectomy and cranial dorsal laminectomy with opening of the dura mater is the procedure of choice for surgical treatment of CIM.

It is thought that syrinx formation in humans and dogs with CIM occurs secondary to partial obstruction of CSF flow at the cranio-cervical junction.

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