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Query: UMLS:C0026986 (myelodysplastic syndrome)
 14,926 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A previously healthy 13-year-old boy without myelodysplasia who had mild scoliosis was seen with complaints of nasal congestion, noisy nighttime breathing, and difficulty sleeping. Flattening of the inspiratory loop on the flow-volume curve was found on pulmonary function testing, suggesting a variable extrathoracic obstruction due to a laryngeal lesion. Bilateral abductor vocal cord paralysis and sleep apnea developed precipitously following general anesthesia. Further workup demonstrated a type-I Chiari malformation with syringomyelia. Brainstem abnormalities such as Chiari malformation with secondary tenth cranial nerve deficits should be considered in previously healthy children and adolescents with signs and symptoms of upper airway obstruction and apnea.
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PMID:Sleep apnea and vocal cord paralysis secondary to type I Chiari malformation. 361 93

The most common factor associated with neurotrophic ulcers is either a peripheral or central somesthetic deficit, such as may occur in peripheral neuropathies, myelodysplasia, syringomyelia, sciatic nerve injury, spinal cord injury, tabes dorsalis, and most commonly, in the peripheral neuropathy of diabetes. Distribution of the ulcers indicates a mechanical factor in development. Here, three patients with an unusual type of neurotrophic ulcer, giant neurotrophic verruca vulgaris, are presented.
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PMID:Giant verruca vulgaris presenting as a neurotrophic foot ulcer. 717 44

Children with myelodysplasia frequently have abnormal hand function. Etiologies include brainstem and cervical cord compression due to the Chiari II malformation, syringomyelia, hydrocephalus, and cerebral dysmorphism. Determination of potentially correctable causes of hand dysfunction may be difficult. We describe the use of the Jaymar dynamometer for assessing grip strength as a means of detecting early changes in hand function.
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PMID:Value of dynamometry in assessing upper extremity function in children with myelomeningocele. 749 67

We attempted to characterize the signs and symptoms of patients presenting with scoliosis as a manifestation of occult syringomyelia, and to determine the effect of syrinx decompression on the deformity and neurologic deficits. This study represented 21 patients treated from 1981 through 1991 at the authors' institution. All patients with occult syringomyelia and no other central nervous system lesions underwent decompression of the syrinx, with improvement in neurologic signs and symptoms. Three of the four with scoliosis that were not treated by arthrodesis showed improvement of their deformity at an average follow-up of 35 months. Three of five patients with myelodysplasia, occult syringomyelia, and scoliosis not previously treated with spinal arthrodesis showed stabilization or improvement of their deformity at an average follow-up of 21 months. We conclude that syringomyelia is associated with a high incidence of developmental scoliosis, and that decompression of the syrinx leads to improvement in, or stabilization of, the majority of scoliotic curves, or postpones the need for fusion.
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PMID:Syringomyelia and developmental scoliosis. 796 97

Patients with Chiari's malformation without myelodysplasia often suffer from another associated spinal cord lesion--syringomyelia. A condition entirely overriding the clinical picture, affecting adversely the results of surgery as well as the prognosis of this congenital developmental defect. Proceeding from the results of their surgical treatment of 40 patients with hindbrain malformation, the authors recommend, in accordance with the literature, a different approach to and classification of patients depending on the presence of syringomyelia (A/B classification; A: syringomyelia on MR--present, B: absent). The pathological development of the syrinx is caused by obstruction to the natural CSF circulation in the subarachnoid spaces of the craniocervical junction (Williams' dissociation theory). The authors are convinced that routine (static) imaging methods (CT, MR) cannot prove the presence or behaviour of a CSF block, and that they cannot help choose reliably the optimum type of treatment. In contrast, direct measurement (and monitoring) of CSF pressure in different compartments of the CSF pathways (intracranial/intraspinal compartment, i.e., in front of and beyond the suspected block) are a method which can help ascertain with precision the presence and behaviour of a CSF circulatory block, and which also provides dynamic information on such a block during the diurnal rhythm for a period of several days. The authors present the results of a prospective study of 25 patients with hindbrain malformation and describe a technique of parallel monitoring of the two CSF compartments using an adaptation of the Williams method, as well as two different types of dissociation tests designed to prove the presence of a CSF block. CSF flow obstruction was found in 11 patients, in 14 patients it could not be proved. In 8 out of 9 patients with a fully developed syrinx (group A) the block was found as expected. It was, however, diagnosed also in 3 patients until then without any graphic proof of a syrinx (group B). The authors also report on short-term (3-year) results of surgical treatment in patients with rhombencephalic malformation, who had 2 different modes of treatment indicated on the basis of dissociation test results. The advantages and disadvantages are discussed of direct measurement and parallel monitoring of CSF in comparison with radiological methods; emphasis is laid on inevitable radicality in the recanalization of CSF passages in patients with well established CSF blocks.
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PMID:Simultaneous cerebral and spinal fluid pressure recordings in surgical indications of the Chiari malformation without myelodysplasia. 1168 23

Diabetic neuroarthropathy was observed in four patients; these are the first cases of this nature reported in the Canadian medical literature. The criteria for this diagnosis included: (1) long-standing diabetes; (2) arthropathy, most frequently involving the foot, which shows deformity, shortening and ulceration without evidence of infection or peripheral circulatory failure; (3) abolition or diminution of pain on weight-bearing; (4) diabetic peripheral neuropathy with impaired sense of position or vibration and weak or absent deep tendon reflexes. Radiographic findings were similar to those in patients with Charcot's arthropathy from any cause.Tabes dorsalis, leprosy, syringomyelia, myelodysplasia and the arthropathies of corticosteroid therapy were ruled out in these cases. In addition to conventional medical therapy the patients were treated by means of walking-casts for several months.Diabetic neuroarthropathy is probably more common than the medical literature would indicate. Diminished sensation in the lower limbs in diabetics of long standing appears to be the major factor contributing to this disorder.
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PMID:DIABETIC NEUROARTHROPATHY: REPORT OF FOUR CASES. 1419 8

Segmental hypoplasia not associated with vertebral abnormalities was found in a Japanese Black calf that was unable to stand. Constriction occurred between the third and 5th segments of the lumbar spinal cord, and was most severe in the 4th segment. Myelodysplasia, such as hydromyelia and syringomyelia, absence or interruption of the central canal, dysplasia of the gray matter, and absence or divergence of the septal connective tissue at the dorsal median septum or the ventral median fissure, were confirmed histologically. These changes indicate hypoplasia of the segments affected following neural tube closure. Therefore, this case was suspected to be a closed neural tube defect.
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PMID:Segmental hypoplasia of the spinal cord in a Japanese black calf. 1934 3

Syringomyelia is a form of myelodysplasia defined by the formation of one or more fluid-filled cavities within the spinal cord that do not communicate with the central canal. The defect may be congenital or acquired. Clinical signs correlate to the segment of spinal cord affected and include pain, paresis, proprioceptive deficits, alterations in sensation, scoliosis, and autonomic dysfunction. This report describes the clinical and pathologic changes in a case of acquired syringomyelia in a 10-year-old American Paint Horse mare. The horse had a six-week history of progressive proprioceptive deficits in all four limbs, bilateral pelvic limb ataxia, and muscle fasciculations that were unresponsive to treatment with stall rest, phenylbutazone, and dexamethasone. Syringomyelia was diagnosed postmortem within cervical, thoracic, and lumbar spinal cord segments. Acquired syringomyelia should be considered among differential diagnoses in adult horses displaying progressive neurologic deficits.
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PMID:Syringomyelia in an Adult American Paint Horse. 2962 Nov 91