syringomyelia


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Related to syringomyelia: syringobulbia

syringomyelia

 [sĭ-ring″go-mi-e´le-ah]
a slowly progressive syndrome in which cavitation occurs in the central (usually cervical) segments of the spinal cord; the lesions may extend up into the medulla oblongata (syringobulbia) or down into the thoracic region. It may be of developmental origin, arise secondary to tumor, trauma, infarction, or hemorrhage, or be without known cause. It results in neurological deficits that generally consist of segmental muscular weakness and atrophy accompanied by a dissociated sensory loss (loss of pain and temperature sensation, with preservation of the sense of touch), and thoracic scoliosis is often present. Called also cystic myelopathy and Morvan's syndrome.

sy·rin·go·my·e·li·a

(sĭ-ring'gō-mī-ē'lē-ă), [MIM*186700 and MIM*272480]
The presence in the spinal cord of longitudinal cavities lined by dense, gliogenous tissue, which is not caused by vascular insufficiency. Syringomyelia is marked clinically by pain and paresthesia, followed by muscular atrophy of the hands and analgesia with thermoanesthesia of the hands and arms, but with the tactile sense preserved; later marked by painless whitlow, spastic paralysis in the lower extremities, and scoliosis of the lumbar spine. Some cases are associated with low grade gliomas or vascular malformations of the spinal cord.
[syringo- + G. myelos, marrow]

syringomyelia

/sy·rin·go·my·elia/ (sĭ-ring″go-mi-e´le-ah) a slowly progressive syndrome of varying etiology, in which cavitation occurs in the central segments of the spinal cord, generally in the cervical region, with resulting neurologic defects; thoracic scoliosis is often present.

syringomyelia

(sə-rĭng′gō-mī-ē′lē-ə)
n.
A chronic disease of the spinal cord characterized by the presence of fluid-filled cavities and leading to spasticity and sensory disturbances.

sy·rin′go·my·el′ic (-ĕl′ĭk) adj.

syringomyelia

[-mī·ē′lyə]
Etymology: Gk, syrinx, tube, myelos, marrow
a chronic progressive disease of the spinal cord, marked by elongated central fluid-containing cavities surrounded by gliosis or a proliferation of neurological tissue. Symptoms begin early in adulthood, usually involving the cervical region, with muscular wasting in the upper limbs. The disease is more common in males.
observations Although present at birth, onset is insidious, and manifestations are often not seen until individuals are in their 20s or 30s. These symptoms are often ambiguous and mimic a host of other diseases. The cervical spine is most commonly affected and manifests as weakness, atrophy, and sensory loss in the shoulders, arms, and hands, including loss of pain and temperature sensation and sweating on the face. Upper extremity reflexes are diminished or absent, whereas weakness, altered gait, spasticity, and hyperreflexia may be noted in the lower extremities. Brainstem involvement may cause dysphagia, ptosis, miosis, or diplopia. GI symptoms include nausea, vomiting, weight loss, and abdominal spasms. Respiratory disturbances may manifest during sleep. Joint arthropathy and trophic skin changes may eventually develop. The course of the disease is variable and may result in slow, long-term incapacitation. Disease progress may slow or stop at any time. Diagnosis is made through MRI. Bony abnormalities at the base of the skull and C1-2 spine and scoliosis may be seen on x-ray.
interventions The primary intervention is a cervical decompression laminectomy at C1-2 spine with repair or removal of bony abnormalities, with possible myelotomy or shunt placement. Although surgical intervention halts disease progression, it seldom leads to significant improvement in current neurological manifestations.
nursing considerations Nursing care after surgery is aimed at careful positioning and turning to maintain proper alignment of the cervical spine and head; control of postoperative pain; surgical wound care; monitoring for CSF leakage and peripheral vital signs; assessment of motor and sensory function in the extremities and bowel and bladder function. Chronic care focuses on rehabilitation for the sequelae from neurological damage that occurred before the surgery. These are varied and may include bowel and bladder programs for management of neurogenic bowel or bladder; protection against injury and breakdown of skin related to decreased sensation; physical therapy to build strength and endurance; occupational therapy to improve or adapt functioning; respiratory therapy to increase vital capacity and tidal volume; and speech therapy if swallowing is affected. Counseling and support services are provided for the individual and family to aid in adaptation.
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Syringomyelia

syringomyelia

Neurology A disorder characterized by damage to the spinal cord, caused by formation of a fluid-filled cavity within the spinal cord, due to trauma, tumors, or congenital defects; the cavity begins in the cervical region and slowly expands, causing progressive pressure-related damage to the spinal cord. Cf Spinal cord trauma.

sy·rin·go·my·e·li·a

(si-ring'gō-mī-ē'lē-ă)
The presence in the spinal cord of longitudinal cavities lined by dense, gliogenous tissue, which are not caused by vascular insufficiency. Syringomyelia is marked clinically by pain and paresthesia, followed by muscular atrophy of the hands and analgesia with thermoanesthesia of the hands and arms, but with the tactile sense preserved; it is later marked by painless whitlows, spastic paralysis in the lower extremities, and scoliosis of the lumbar spine. Some cases are associated with low-grade astrocytomas or vascular malformations of the spinal cord.
Synonym(s): hydrosyringomyelia, Morvan disease.
[syringo- + G. myelos, marrow]

syringomyelia

A rare brainstem or upper spinal cord defect in which the central canal progressively expands and damages adjacent nerve tracts. There is loss of pain and temperature sensation in the neck, shoulders and upper limbs followed by muscle wasting and loss of the sense of touch. Later, there is paralysis and SPASTICITY and progressive disablement.

Morvan,

Augustin Marie, French physician, 1819-1897.
Morvan chorea - continuous involuntary quivering of muscles at rest. Synonym(s): myokymia
Morvan disease - the presence of longitudinal cavities in the spinal cord. Synonym(s): Morvan syndrome; syringomyelia
Morvan syndrome - Synonym(s): Morvan disease

syringomyelia

sensory dysfunction (pain and paraesthesia, thermoanaesthesia but preservation of tactile sensation), motor effects (muscular atrophy, especially of hands, spastic paralysis of legs) and lumbar scoliosis associated with spinal cord syrinxes

syringomyelia

the presence of fluid-filled cavities in the substance of the spinal cord, with destruction of nervous tissue. Clinical signs are posterior paralysis, or if the animal is able to stand the posture is a wide spacing of the feet and overextension of the legs when walking. An inherited trait in Weimaraner dogs. See also spinal dysraphism.
References in periodicals archive ?
In conclusion, this is a case of DM1 with syringomyelia reported in China.
The items showed appropriate internal consistency; therefore, the CSP in its final form consists of 57 self-reported questions that cover the range of patient-reported symptoms related to CMI and/or syringomyelia.
Mechanistic insights into posttraumatic syringomyelia based on a novel in vivo animal model.
Other chapters address mechanisms of neuropathic pain, therapeutics, carpal tunnel syndrome, cervical radiculopathy, meralgia paresthetica, Lyme radiculopathy, neuralgic amyotrophy, herpes zoster infection, polyneuropathies, inherited amyloidosis, sarcoidosis, Fabry disease, proximal myotonic myopathy, complex regional pain syndrome, polymyalgia rheumatica, phantom pain, Brown-Sequard syndrome, syringomyelia, and headache disorders, and give information on the patient's history, clinical findings, investigations, diagnosis, and treatment.
Comparison of CSF flow patterns between symptomatic and asymptomatic patients, those with or without syringomyelia, and those with syringomyelia but with or without tonsillar herniation, is possible.
Pathogenesis of syringomyelia associated with Chiari type 1 malformation: Review of evidences and proposal of a new hypothesis.
Differential diagnoses for these lesions observed under magnetic resonance include infections (tabes dorsalis, human immunodeficiency virus, and herpes Zoster), neoplasms (lymphoma, paraneoplastic myelopathy), vascular (arterial or venous ischemia, arteriovenous malformation of spinal cord), post-radiation myelitis, multiple sclerosis, acute transverse myelitis, and syringomyelia (8).
Like much research and discovery on spinal-cord injury and disease (SCID), possible treatment options for syringomyelia take time to develop.
3) This disease usually has a wide range of presentations ranging from subtle skeletal abnormalities to severe cord involvement leading to syringomyelia.
Given her age and, in particular, her breed, we were concerned Ruby was suffering from syringomyelia, an inherited condition where the brain is too big for the skull.
Syringomyelia refers to an eccentric CSF-filled cavity that may reflect a prior insult to the cord parenchyma.