1/57. MRI in vitamin B12 deficiency myelopathy.BACKGROUND: Little is known about vitamin B12 deficiency myelopathy's magnetic resonance imaging (MRI) manifestations and their relationship to the onset, evolution, and resolution of neurologic signs and symptoms. methods: We present a case and review eleven additional reported cases of subacute combined degeneration of the spinal cord detected by MRI. RESULTS: Our patient had increased T2-weighted signal and gadolinium contrast enhancement of the posterior columns in the cervical and thoracic regions and enhancement of the lateral columns in the high cervical region. This is a case with imaging evidence for lateral column lesions. Two prior reports have shown posterior column enhancement. T1-weighted images may show decreased signal in the posterior columns and sometimes demonstrate reversible spinal cord swelling. MRI abnormalities typically improve after vitamin replacement therapy. However, clinical signs may persist despite resolution of imaging abnormalities, and these abnormalities do not always resolve completely. In addition, symptoms may precede the imaging abnormality. CONCLUSIONS: Vitamin B12 deficiency may produce an increased T2-weighted signal, decreased T1-weighted signal, and contrast enhancement of the posterior and lateral columns of the spinal cord, mainly of the cervical and upper thoracic segments. Because the symptoms may precede any imaging abnormality, it is clear that spinal cord MRI may not be a highly sensitive, early test for subacute combined degeneration.- - - - - - - - - - ranking = 1keywords = deficiency (Clic here for more details about this article) |
2/57. Primary antiphospholipid syndrome associated with pernicious anaemia.AIM: To determine whether there is an association between pernicious anaemia and antiphospholipid antibody syndrome. methods: Fifteen patients with pernicious anaemia and 11 patients with iron deficiency anaemia (controls) were evaluated for clinical parameters of thrombosis and/or the presence of antiphospholipid antibodies, antinuclear antibodies or lupus anticoagulant. RESULTS: One asymptomatic patient with pernicious anaemia had laboratory features suggestive of the antiphospholipid syndrome. An additional three patients in each group had slightly elevated antiphospholipid antibody concentrations, within one standard deviation of the normal range. CONCLUSION: The association between pernicious anaemia and antiphospholipid antibody syndrome is rare and evaluation should be guided by clinical indications.- - - - - - - - - - ranking = 0.16666666666667keywords = deficiency (Clic here for more details about this article) |
3/57. nitrous oxide anesthesia-associated myelopathy.BACKGROUND: The role of nitrous oxide exposure in neurologic complications of subclinical cobalamin deficiency has been reported, but few cases are well documented. observation: Two weeks after surgery for prosthetic adenoma, a 69-year-old man developed ascending paresthesia of the limbs, severe ataxia of gait, tactile sensory loss on the 4 limbs and trunk, and absent tendon reflexes. After a second surgical intervention, the patient became confused. Four months after onset, the patient had paraplegia, severe weakness of the upper limbs, cutaneous anesthesia sparing the head, and confusion. Moderate macrocytosis, low serum B12 levels, and a positive schilling test result led to the diagnosis of pernicious anemia. Results of electrophysiologic examinations showed a diffuse demyelinating neuropathy. magnetic resonance imaging of the spinal cord disclosed hyperintensities of the dorsal columns on T2-weighted images. CONCLUSIONS: Pernicious anemia can result in severe neurologic symptoms with only mild hematologic changes. The role of nitrous oxide anesthesia in revealing subclinical B12 deficiency must be emphazised. magnetic resonance imaging of the spinal cord might be helpful in making the diagnosis.- - - - - - - - - - ranking = 0.33333333333333keywords = deficiency (Clic here for more details about this article) |
4/57. common variable immunodeficiency with mosaic trisomy 8: report of one case.This case study reported a 17-year-old female of common variable immunodeficiency (CVID) associated with bronchiectasis, pernicious anemia and mosaic trisomy 8. Clinically this patient presented with recurrent sinopulmonary infections, intractable diarrhea, macrocytic anemia, and primary amenorrhea. Immunological tests showed pan-hypogammaglobulinemia and a decrease of peripheral blood B cells (4%) and CD4 cells (25%). Lymphoproliferative responses to mitogen (PHA) and specific antigen (BCG) were profoundly impaired in the patient in comparison to those in control. Production of interleukin 4 (IL-4) and gamma interferon (IFN-gamma) in the in vitro lymphoproliferation was also profoundly depressed. Pernicious anemia demonstrated by larger MCV (112.9 fl) and hyper-segmental granulocytes on peripheral blood smear responded to parental administration of vitamin B12. Interestingly, she had a mosaic trisomy 8 in peripheral blood mononuclear cells but normal 46XX karyotype in the bone marrow cells. To our knowledge, this is the first case of CVID associated with mosaic trisomy 8 reported in the literature. As the case exemplifies, CVID should be considered when the physicians evaluate the patient presenting with recurrent sinopulmonary infections, diarrhea, malnutrition, and pernicious anemia. It requires further study to explore whether the genes in the chromosome 8 are linked to CVID.- - - - - - - - - - ranking = 0.83333333333333keywords = deficiency (Clic here for more details about this article) |
5/57. Development of rheumatoid arthritis in a patient with pernicious anemia: case report.Very few cases of rheumatoid arthritis combined with pernicious anemia have been reported in the world literature and none in the Chinese literature. A 62-year-old female initially presented with anemia. Pernicious anemia was diagnosed by characteristic blood and bone marrow morphology. Laboratory data showed a deficiency of vitamin B12 and positive anti-gastric parietal cell antibodies. Her anemia improved after vitamin B12 therapy. Painful swelling of multiple joints developed 6 years later. The clinical presentation supported a diagnosis of rheumatoid arthritis. We report herein a rare case of rheumatoid arthritis and pernicious anemia in the same ethnic Chinese patient. We also review the literature and discuss a possible association between a non-organ-specific autoimmune disease, rheumatoid arthritis, and an organ-specific autoimmune disease, pernicious anemia.- - - - - - - - - - ranking = 0.16666666666667keywords = deficiency (Clic here for more details about this article) |
6/57. Concurrent pernicious anemia and myelodysplastic syndrome.Megaloblastic anemia (MA) due to vitamin B12 deficiency is a reversible form of ineffective hematopoiesis. Myelodysplastic syndrome (MDS) is an acquired, irreversible disorder of ineffective hematopoiesis, characterized by stem cell dysfunction as a consequence of dna damage manifested in part by karyotype anomalies. Importantly, MA and MDS are generally considered mutually exclusive diagnoses. We report the case of a 73-year-old woman with a profound macrocytic anemia, monocytosis and neurologic symptoms. Low cobalamin levels and the presence of anti-intrinsic-factor antibodies definitively established a diagnosis of pernicious anemia. Replacement therapy resulted in resolution of neurologic findings and macrocytosis; however, the anemia and monocytosis persisted. Bone marrow biopsy revealed trilineage myelodysplasia, which together with the peripheral monocytosis suggested a diagnosis of chronic myelomonocytic leukemia. karyotype analysis revealed a clone with 45, XX, der(1;7)(q10;p10)-7 [20]. Eighteen months after documented vitamin B12 replenishment her MDS transformed to terminal acute myeloid leukemia with the same clonal abnormality. Reversible cytogenetic abnormalities have been observed with MA, occasionally including karyotypes typically associated with MDS or myeloid leukemias. These abnormalities, like the anemia, resolve with vitamin replacement. This case suggests that MA and MDS can occur simultaneously; clinicians should be aware that this phenomenon occurs. Whether acquired karyotype abnormalities from the MA were related to the MDS and subsequent myeloid leukemia in this woman is a speculative but intriguing consideration that is discussed.- - - - - - - - - - ranking = 0.16666666666667keywords = deficiency (Clic here for more details about this article) |
7/57. Normal vitamin B12 turnover in subacute combined degeneration of the spinal cord.Vitamin B12 turnover studies were carried out in 2 patients with pernicious anemia and subacute combined degeneration of the spinal cord. The retention of a tracer dose (0.5 mug) of intravenously administered 58Co-labeled vitamin B12 in these 2 patients was measured by whole-body monitoring over periods of 94 and 134 days. Vitamin B12 turnover (per cent per day) was calculated and the results compared with normal subjects and with pernicious anemia patients having no neurologic dysfunction. The loss of radioactive-B12 from the body was described by a single exponential model, confirming that the loss of vitamin B12 takes place as though from a single pool. There was no difference in vitamin B12 turnover between the patients with subacute combined degeneration of the spinal cord and normal subjects or other pernicious anemia patients without neurologic involvement. These findings contradict the suggestion that the development of neuropathy in vitamin B12 deficiency might be related to an increased requirement for vitamin B12.- - - - - - - - - - ranking = 0.16666666666667keywords = deficiency (Clic here for more details about this article) |
8/57. subacute combined degeneration of the spinal cord and air encephalography.subacute combined degeneration of the spinal cord is a rare complication of vitamin B12 deficiency and is seldom encountered today. A case of Addisonian pernicious anaemia is reported in which the classical signs of subacute combined degeneration developed suddenly after air encephalography had been performed. The patient made a complete recovery.- - - - - - - - - - ranking = 0.16666666666667keywords = deficiency (Clic here for more details about this article) |
9/57. Autoimmune cholangiopathy associated with systemic lupus erythematosus.We report a 39-year-old female who presented over 11 years with autoimmune cholangiopathy associated with kaleidoscopic manifestations of systemic lupus erythematosus (SLE), including, arthritis, skin changes, pleuritis, diffuse proliferative glomerulonephritis, lymphadenopathy, splenomegaly, hyperglobulinemia, and major depression. While antimitochondrial antibodies (AMA) were absent, antinuclear (ANA) and anti-DNA antibodies were detected in high titres associated with hypocomplementemia. The patient also had vitamin B12 deficiency and antiphospholipid antibodies. The patient required steroids and repeated courses of cyclophosphamide for the management of lupus nephritis, and ursodeoxycholic acid (ursolite) administration resulted in amelioration of cholestatic laboratory abnormalities. This unusual case report and review of literature illustrate that immune liver disease may be an important clinical manifestation of SLE, especially autoimmune cholangiopathy.- - - - - - - - - - ranking = 0.16666666666667keywords = deficiency (Clic here for more details about this article) |
10/57. Long-term neurologic consequences of nutritional vitamin B12 deficiency in infants.A review of the clinical findings in six infants with nutritional vitamin B12 deficiency seen during the last 10 years was undertaken and an attempt made to obtain long-term neurologic follow-up. There was a consistent clinical pattern in vitamin B12-deficient infants; irritability, anorexia, and failure to thrive were associated with marked developmental regression and poor brain growth. Two of the four patients who qualified for long-term review had a poor intellectual outcome. Although early response to treatment is satisfying, the long-term consequences of nutritional vitamin B12 deficiency in infants emphasize the need for prevention or early recognition of this syndrome.- - - - - - - - - - ranking = 1keywords = deficiency (Clic here for more details about this article) |
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