Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0026986 (myelodysplastic syndrome)
 14,926 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report instances of fatal leukoencephalopathy associated with the intravenous administration of the antifungal agent amphotericin B (AmB) to a 16-year-old girl treated for acute lymphoblastic leukemia and a 22-year-old man who underwent bone marrow transplantation for a myelodysplastic syndrome. Both received AmB in association with cranial irradiation and developed a subacutely evolving neurologic disorder characterized by personality change and confusion rapidly progressing to akinetic mutism. Neuroimaging studies disclosed diffuse nonenhancing abnormalities of the cerebral, particularly frontal, white matter that included hypodensity on CT and increased signal on T2-weighted MRI. Postmortem examinations demonstrated a diffuse, noninflammatory leukoencephalopathy with florid astrogliosis, demyelination, and infiltration of the hemispheric white matter by foamy macrophages. In neither case was there evidence of opportunistic infection or neoplastic infiltration of the nervous system. We review the evidence that polyene macrolide antibiotics such as AmB are potential leukotoxins.
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PMID:Amphotericin B-associated leukoencephalopathy. 140 84

A 41-year-old female patient with mental retardation and generalized epileptic seizure had a nonmosaic idic (X) (pter-q21.32::q21.32-pter) chromosome in peripheral lymphocytes and bone marrow cells. Primary amenorrhea, myelodysplastic syndrome, pigmented nevi and characteristic facial appearance were also observed. A few cases with the nonmosaic idic (X) (q::q) with various breakpoints reported previously commonly showed ovarian failure with dysfunction of relevant hormone. CNS abnormalities of the present case were demonstrated by CT, MRI and SPECT using 123I-IMP. CNS abnormalities were considered to be possibly due to karyotype with a nonmosaic idic (X) (q21.32).
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PMID:Neuropsychiatric disturbances in a patient with a nonmosaic isodicentric (X) (q21.32) chromosome. 212 33

In 114 patients with postrepair myelomeningocele MRI of the spine was performed. Tethered cord (89%) and associated malformations (syrinx, lipoma etc.) (33%) were the most important findings. Additional MRI scans of the head (44 patients) revealed numerous further anomalies. Arnold Chiari malformation was found in 76% of the patients (ACM I:32%, ACM II:44%). In the ACM II group compression of lower cranial nerves, brain stem, and cerebellum can lead to considerable neurologic symptoms. Therefore in patients with progressive neurologic dysfunction a complete investigation of the whole spine and brain is necessary. MRI proves to be the diagnostic procedure of choice in patients with dysraphic myelodysplasia.
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PMID:Cerebral and spinal MR-findings in patients with postrepair myelomeningocele. 233 86

A comprehensive approach to diagnosis of patients with lissencephaly using clinical, CT and MRI scan, and sometimes other laboratory data will allow a specific diagnosis to be made in a large majority of patients. The most common diagnoses in order of decreasing frequency will probably prove to be WWS, MDS, and ILS. The remainder constitute a heterogeneous group. Both diagnosis and counseling have been modified by several recent and important advances. Diagnostic criteria for WWS have been revised. Several molecular probes have been located within the MDS critical region in chromosome band 17p13.3. Prenatal diagnosis should prove to be reliable in both WWS and MDS.
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PMID:The neurogenetics of lissencephaly. 264 23

Late deterioration is common in patients with a myelodysplasia. Most, possibly all, of this deterioration is preventable or correctable. It is not simply the natural history of the disease. Only through close follow-up by trained observers can these problems be anticipated and discovered early. Regularly scheduled evaluations of intellectual, musculoskeletal, and urinary systems are essential. When the clinician is familiar with the signs and symptoms of the various causes of deterioration and armed with ultrasound, myelography, CT, and MRI, the most likely cause can be identified and a treatment plan outlined.
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PMID:Spina bifida today: problems adults face. 270 May 6

An extremely rare case of chondromyxoid fibroma of the skull base extending from middle fossa to posterior fossa is reported. The patient is a forty-seven year old woman who became paranoiac four months before admission and her gait was unsteady for one month. On April 27th, 1985, she fainted and was admitted to Kanto Rosai Hospital on April 30th. She was drowsy and disoriented. Neurologic examination revealed left pyramidal tract signs and impaired functions of right Vth (first and second branches), VIth and VIIIth cranial nerves. She was anemic and bleeding tendency due to thrombocytopenia was also recognized. Hematologist's diagnoses were myelodysplastic syndrome plus iron deficiency anemia. Neuroradiologic studies including CT scan, cerebral angiographies and MRI revealed a huge extra-axial skull base tumor extending from middle fossa to posterior fossa completely destroying the skull base. Stenosis of cavernous portion of internal carotid artery due to tumoral involvement was also noted. Surgical excision of the tumor was performed three weeks after the admission with the use of platelet transfusion. Transpetrosal transtentorial approach was performed removing the intracranial part of the tumor which was a semi-translucent jelly-like soft mass. The patient had a good post-operative course and improvement in the neurologic condition was recognized. Microscopically, the tumor showed marked lobular formation with small vessels and fibroblasts in the periphery of the lobule. The fibroblasts took stellate figures in the myxoid matrix at the center of the lobule. Chondroid pattern was also observed. Mitosis and nuclear atypism were not observed. Histological diagnosis was chondromyxoid fibroma.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Chondromyxoid fibroma of the skull base extending from the middle fossa to the posterior fossa--case report]. 343 32

The bone marrow of 84 patients with hematological disorders was investigated using short inversion time inversion recovery sequence (STIR) on an 1.5 Tesla superconducting MRI system. Double echo times of 20 and 100msec were applied to research the signal characteristics of the lesion and carry out quantitative analysis of the receiver operating characteristic curve (ROC). The hematological diseases included 19 cases of myelodysplastic syndrome (MDS), 18 of multiple myeloma (MM), 18 of chronic myelocytic leukemia (CML), 9 of aplastic anemia (AA), 8 of acute myelocytic leukemia (AML), 3 of chronic lymphocytic leukemia (CLL), 3 of myelofibrosis, and 3 others. Using STIR with double echo times, bone marrow showed high signal intensity (SI) on short TE and low SI on long TE in MDS and CML; high SI on short and long TE in myelofibrosis and CLL; high SI on short TE and high to moderately high SI on long TE in MM; and low SI on short and long TE in AA. Quantitative analysis of 33 patients showed high sensitivity and specificity in AA (81% and 94%, respectively) and moderate sensitivity and high specificity in MM (61%, 88%). CML and MDS were similar with low sensitivities (40%, 41%) and high specificities (80%, 78%). Differential diagnosis between CML and MDS was difficult using STIR with the double echo time method.
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PMID:[Object analysis of bone marrow MR imaging using double echo STIR sequence in hematological diseases]. 763 52

A 72-year-old male suffering with MDS was admitted to our hospital because of slowly progressive dementia, convulsions and consciousness disturbance. A CT scan of the brain showed a low density area in the right temporal to parietal lobe and T2-weighted MRI of the brain revealed a high intensity signal in the same area. Herpes simplex virus DNA was detected in CSF by PCR method. He was diagnosed as having herpes simplex encephalitis but his clinical course was prolonged and considered atypical as herpetic infection in CNS. However, after administration of aciclovir, progression of his symptoms stopped, and a CT scan still revealed abnormal findings with the same area after more than four months. We thought his chronic course of herpes encephalitis was caused by incomplete immune function suppressed by MDS.
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PMID:[A case of chronic herpes encephalitis with myelodysplastic syndrome (MDS)]. 820 Jan 42

MR imaging of the femoral marrow was performed in 30 patients with myelodysplastic syndrome (MDS), 11 cases of which evolved to acute myeloid leukemia (AML). The MRI appearance was classified into five patterns: 1) fatty marrow; 2) faint signal; 3) nodular pattern; 4) heterogeneous infiltration; and 5) diffuse infiltration. For each type of MDS, MRI patterns of the femoral marrow were evaluated and compared with those in normal subjects as well as in patients with aplastic anemia. Signal intensity alteration, a low signal on T1-weighted SE image and a high signal on STIR image, began in the proximal femoral marrow almost symmetrically in patients with MDS. The area of abnormal signal intensity tended to gradually extend towards the distal portion of the femur as the disease progressed. MRI patterns of the femoral marrow correlated with marrow cellularity, and diffuse marrow infiltration was noted in patients with a more advanced type of MDS or with severe anemia. There were limitations to making an accurate diagnosis of the MDS type on the basis of the MRI pattern. Progression of the MRI appearance in the course of MDS was thought to be a sign suggesting evolution to AML. It was difficult to differentiate hypoplastic MDS from aplastic anemia, although the nodular pattern was commonly seen in the latter disease.
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PMID:[MR imaging findings of the femoral marrow in myelodysplastic syndrome]. 853 1

A 54-year-old woman with a myelodysplastic syndrome treated with high-dose chemotherapy and an allogenic bone marrow transplant developed acute cortical blindness while receiving tacrolimus (FK506). MRI showed white matter abnormalities. After discontinuation of FK506, the patient's vision returned within 8 days. FK506 neurotoxicity is similar to cyclosporine neurotoxicity and can occur in allogenic bone marrow transplant patients treated with FK506.
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PMID:Cortical blindness and seizures in a patient receiving FK506 after bone marrow transplantation. 1033 54


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