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

The subject of occult spinal dysraphism or myelodysplasia is reviewed from standpoints of embryology, clinical manifestations, and treatment, and the management of 73 cases summarized. In general, these concealed lesions arise from developmental variants in the most distal part of the neural tube, a situation which may cause distortion or partial absence of neural tissues and also lead to damage from compression or traction. Lipomyelomeningocele and congenital dermal sinus are two exampled of the many types of such lesions, but some are more complicated and border-line myelomeningocele-like forms occur. Incontinence, deformity or weakness of the feet, impaired gait, and other difficulties may appear late and increase with growth. Surgical treatment is advised to reduce chances of delayed or progressive loss of function.
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PMID:Occult spinal dysraphism: a series of 73 cases. 109

A prospective study was undertaken in 1985 to better understand how the surgical manipulation of hindbrain herniation affected abnormal spinal curvature. Eleven patients under 16 years of age with Chiari malformation (not associated with myelodysplasia) and scoliosis of at least 15 degrees were studied. The mean curve angle at the time of original treatment was 29 degrees, with the convexity to the right in seven patients. The curvature was rapidly progressing in four patients. The most common presenting signs were myelopathy and weakness. Investigative procedures included spine radiographs with the patient standing and magnetic resonance (MR) imaging of the brain, spinal cord, and craniovertebral junction. Eight children had associated hydrosyringomyelia. Surgical intervention consisted of a dorsal posterior fossa decompression in all patients and a transoral ventral decompression of the cervicomedullary junction in five. All patients were followed at 3, 6, and 12 months, and at yearly intervals thereafter with clinical evaluations, spine radiographs in the standing position, and postoperative MR imaging. The mean follow-up period was 35 months. The scoliosis improved in eight patients, stabilized in one, and progressed in two. Only one child required postoperative spinal fusion and instrumentation for progression of scoliosis. Hematomyelia or hematobulbia was associated with persistent scoliosis in two patients. The presence of hydrosyringomyelia and bone erosion did not preclude curve improvement. All patients under 10 years of age had resolution of their scoliosis, despite preoperative curves of more than 40 degrees. These findings emphasize the importance of early surgical intervention, with the restoration of normal cerebrospinal fluid dynamics at the craniovertebral junction in children with symptomatic Chiari malformations.
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PMID:Scoliosis in pediatric Chiari malformations without myelodysplasia. 160 74

Paralysis seen in children with myelomeningocele has been attributed to congenital myelodysplasia. We suspected that paralysis may be due in part to a spinal cord injury caused by exposure of the neural tube to the amniotic fluid. This hypothesis was tested using a fetal rat model of surgically created dysraphism. Each pup from the experimental group of rats in which the spinal cord was intentionally exposed to the amniotic fluid was born with severe deformity and weakness of the hind limbs and tail. Control fetal rats, subjected to the same procedure without directly exposing the spinal cord to the intrauterine environment, were normal at birth. Histological studies of the exposed spinal cord revealed extensive erosion and necrosis, findings similar to those described in children with myelomeningocele. We therefore propose a "two-hit" hypothesis to explain the paralysis seen in children with myelomeningocele: congenital myelodysplasia complicated by an intrauterine spinal cord injury. Intrauterine protection of the exposed spinal cord might prevent some or all of the paralysis. The possible implications of these findings for the future treatment of myelomeningocele are discussed.
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PMID:The paralysis associated with myelomeningocele: clinical and experimental data implicating a preventable spinal cord injury. 236 76

Sixty cats with hematologic abnormalities indicative of non-lymphoid hematopoietic neoplasia were classified into two groups, myelodysplastic syndromes (MDS) and acute myelogenous leukemias (AML), using criteria developed for human patients with similar diseases. Cats with myeloblast counts in bone marrow of less than 30% were classed as MDS and cats with myeloblast counts of 30% or greater were classed as AML. The clinical, laboratory, and postmortem findings in each group were described and compared. Clinical signs of disease were similar in both groups, the most common being inappetance, lethargy, and weakness. Non-regenerative anemia, macrocytosis, neutropenia, and thrombocytopenia were frequent hemogram abnormalities in both groups. Diagnostically useful differences in physical and peripheral blood findings were a higher prevalence of splenomegaly and/or hepatomegaly, thrombocytopenia, and severe anemia in the AML group. Circulating myeloblasts were found only in cats in the AML group. Outcome of disease was similar in both groups; 85% of the cats in each group died or were euthanatized within one week of diagnosis. In cats that were necropsied, extramedullary leukemic infiltrates were found in all cats in the AML group and in none of the cats in the MDS group.
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PMID:Non-lymphoid hematopoietic neoplasia in cats: a retrospective study of 60 cases. 282 80

We describe a case of cauda equina syndrome secondary to disseminated zygomycosis. A 52-year-old man had myelodysplastic syndrome, progressive weakness of the lower extremities, and incontinence. Neuroradiological findings were consistent with lumbar stenosis and probable disk herniation. A lung lesion was also discovered on the chest roentgenogram. The patient's condition rapidly deteriorated after surgical decompression. Attempts at determining other etiologies were unsuccessful. At autopsy, disseminated zygomycosis was found affecting the lung and the vasculature of the cauda equina and lumbosacral nerve roots, with resultant focal demyelination of these structures. Ribbon-like hyphal elements were also present in the caudal roots. This opportunistic pathogen is discussed along with the diagnostic challenge presented by its unusual clinical presentation.
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PMID:Cauda equina syndrome secondary to disseminated zygomycosis. 319 92

Chiari malformations without myelodysplasia are rarely diagnosed in the pediatric age group. With current neurodiagnostic techniques, however, they are being seen more frequently. Unfortunately, the prognosis is not clear because publications have included a number of different entities, used a variety of surgical approaches, and lacked long term follow-up. Sixteen patients younger than 20 years were treated for Chiari malformations (without myelodysplasia) between 1975 and 1985. The average age was 11 years, and the average duration of symptoms was 20 months. The common symptoms were isolated motor weakness (56%), pain (37.5%), and sensory loss (25%). Frequently seen signs were motor deficit (81%), sensory loss (50%), scoliosis (50%), and cranial nerve palsy (50%). The surgical procedures used were foramen magnum decompression (3 transoral clivus odontoid resections and 15 posterior fossa decompressions with dural grafting), alteration of cerebrospinal fluid (CSF) pathways at the cervicomedullary junction (plugging the foramen cecum and a 4th ventricle to subarachnoid shunt with posterior fossa decompression), and ventriculoperitoneal shunting (2 cases). In follow-up, 37.5% of the patients are asymptomatic, 50% are improved, and 12.5% are stable after an average follow-up period of 43 months. The asymptomatic group was younger (9.3 years) and had a shorter symptom duration (7.2 months) than both the improved (11.9 years, 16.4 months) and the stable groups (15 years, 20 months). Optimal outcome depends on complete evaluation of the abnormal CSF pathways and bony abnormalities at the craniovertebral junction. Operation is then directed toward correction of these abnormalities as delineated radiographically. Of our patients, 87.5% have at least shown improvement, which has been long term in all cases.
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PMID:Presentation and management of pediatric Chiari malformations without myelodysplasia. 275 73

Patients with neuropathic bladders who have intractable hyperreflexia often require cystoplasty and in patients adequately motivated and counselled, continence rates in excess of 90% can be achieved. Seventy-eight patients with neurological disease have undergone enterocystoplasty and are presented. Twenty-two patients had acquired cord lesions of which 17 were traumatic. Of the 56 patients with congenital myelodysplasia, 49 were meningomyelocele, 5 had sacral agenesis, and 2 lipoma of cauda equina. The mean age was 26 (range 13-61) years with 48 male and 30 females. Pre-operative video urodynamics showed sphincter weakness in 54 patients, all patients had intermediate type bladders; hyperreflexia in 52, reduced compliance in 17, and in 7 a combination of both. Forty-seven patients had ileal, 20 had sigmoid, and 11 had ileocaecal cystoplasty. Fifty-two patients had an artificial urinary sphincter inserted, 9 had a colposuspension, and 1 had both for concurrent sphincter weakness incontinence. Acceptable continence has been achieved in 73 (93.6%) patients, 65 claiming complete continence with no pads or incontinence episodes and the 8 others needing pharmacotherapy or pads. Two of our 5 failures have been converted to an ileal loop, 2 are awaiting further surgery, and 1 is wet despite normal urodynamics. Our threshold for initiating intermittent catheterisation is low and 63 (81%) of our patients catheterise. Complications in these patients are few and the only one of note is persistent bowel problems in 30% of our patients. Cystoplasty is recommended as safe and reliable in the treatment of neurogenic detrusor over activity with a continence rate of 93% being achieved.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Enterocystoplasty in the neuropathic bladder. 774 49

Therapy-related leukemia and myelodysplastic syndrome (TRL/MDS) in Japan were analysed in a multi-institution study to assess clinical, cytogenetic aspects, and prognostic factor. From 1985 to 1994, 405 cases of adult TRL/MDS were diagnosed and overall percentage of TRL/MDS in leukemia and MDS was 1.9%. Median age was 61 years old. The median latency from primary malignancies was 53.4 months, which latency was significantly shorter in the patients treated with chemotherapy. Primary malignancies were hematologic in 39%. Common symptoms were fatigue/ weakness and anemia. Chromosome 7,5, and 11 were frequently involved. MLL gene rearrangement were detected in 12 of 64 analysed cases. Overall median survival was 10.0 months. Body weight loss, neurologic abnormality, hypoproteinemia, hypofibrinogenemia, proteinuria, lack of Auer rods, and 5q-were prognostic factors in TRL/MDS. This large population study documented some datas useful for the prevention of TRL/MDS.
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PMID:[Therapy-related leukemia and myelodysplastic syndrome: a multi-institution study in Japan]. 946 95

We report a 60-year-old man with myelodysplastic syndrome (MDS) who developed polymyositis and cutaneous vasculitis. He noticed difficulty in climbing up stairs 4 months before admission. On admission, he showed brownish skin pigmentation in the distal positions of the four extremities. Neurological examination revealed muscle weakness of the neck flexor and proximal muscles of four extremities. Serum myogenic enzymes including creatine kinase increased, and electromyography showed denervation potentials and MUPs were myogenic, which was compatible with polymyositis. Muscle biopsy indicated perivascular mononuclear cell infiltration and muscle fiber necrosis with cytoplasmic bodies and rimmed vacuoles in the muscle fibers. Complete blood cell counts revealed macrocytic normochromic anemia and bone marrow puncture disclosed marked hyperplasia of megakaryocytes, erythroblasts and myeloblasts, suggesting a refractory anemia type of MDS. In addition, skin biopsy showed chronic cutaneous vasculitis. Serum protein electrophoresis and immunoelectrophoresis revealed the presence of IgM gamma type monoclonal gammopathy. With immunosuppressive therapy, serum creatine kinase level was decreased. Since immunological abnormalities and the presence of autoimmune diseases have been reported in patients with MDS, both polymyositis and chronic cutaneous vasculitis in the present patient may be caused by the similar autoimmune mechanisms related to MDS.
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PMID:[Polymyositis and cutaneous vasculitis in a patient with myelodysplastic syndrome]. 1050 89

We reviewed a total of 1,432 patients diagnosed with cutaneous herpes zoster at Hyogo College of Medicine Hospital between 1989 and 1997 for epidemiologic assessment and outcome in patients with zoster paralysis referred for rehabilitation. Of the 1,432 herpes zoster patients (624 males and 808 females, mean age 54.3 years), 12 were referred to our department of rehabilitation medicine for muscle weakness: one had myelitis, and eleven others had lower motor neuron damage. Except for one 43-year-old man with myelodysplastic syndrome, all the lower motor neuron deficit patients were over 60 years of age. Involved myotomes were more widespread than involved dermatomes in eight patients. Electromyography in four patients demonstrated denervation of involved muscles. Five patients experienced complete or near complete recovery from their muscle weakness. The muscle weakness related to herpes zoster was occasionally diagnosed by electromyography as motor neuron damage. Manifestations of motor neuron complications were not noticed but might in fact be more common than was the clinical muscle weakness.
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PMID:Herpes zoster infection complicated by motor paralysis. 1082 89


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