A new optical treatment for oscillopsia.

A simple optical device (spectacles plus contact lens) enabling viewing of the real world with either partial or almost-complete retinal image stabilisation has been tested in patients with oscillopsia caused by nystagmus. The device gave a useful improvement in vision in two of 14 patients. Reasons for success and failure were clear and are discussed. Obvious contraindications include severe optic atrophy, titubation and dementia. Net benefit is also unlikely if there is a good null point or area to the nystagmus, or if acuity (corrected but unstabilized) is 6/9 or better. It is not suitable for the treatment of oscillopsia caused by failure of the vestibulo-ocular reflex.     

The diagnostic usefulness of isocitrate dehydrogenase (ICDH) in the marmoset (Callithrix jacchus)

Marmosets were given either a hepatotoxin, carbon tetrachloride, orally or an i.m. injection of a mytoxin, chlorpromazine. Although muscle damage alone caused small increases in the plasma levels of lactate dehydrogenase, alanine aminotransferase, aspartate aminotransferase and isocitrate dehydrogenase (ICDH), only the isoenzyme analysis of ICDH can differentiate definitely between liver and muscle damage. Only very severe muscle damage can increase the plasma concentration of this enzyme but, in this case, the elevation of plasma creatinine kinase levels helps differentiation. It is recommended that the elevation of ICDH is the most specific indicator of hepatic damage in the marmoset.     

Occult cerebral vascular malformations: high-field MR imaging

Occult cerebral vascular malformations (OCVMs) have characteristic appearances on high-field magnetic resonance (MR) images. These consist of circumscribed regions of low intensity, most prominent on T2-weighted images and representing hemosiderin deposits. Interspersed within most of these lesions are multiple areas of various signal intensity patterns, which correspond to hematomas in different stages of evolution and to fibrous regions containing calcium as well as hemosiderin. Forty-six lesions were found in 19 patients (34 supratentorial and 12 infratentorial). The supratentorial lesions tended to be subcortical or periventricular. Computed tomography depicted 24 of the 46 lesions demonstrated by high-field MR. Comparison of images obtained with both low-field MR (0.12 T and 0.35 T) and high-field MR (1.5 T) revealed that high-field MR imaging was superior in depicting OCVMs. High-field MR appears to be both sensitive and specific for OCVMs and may obviate the need for possible biopsy of these lesions.     

Familial Nephropathic Non-neuropathic Amyloidosis: Clinical Features, Immunohistochemistry and Chemistry

Classification of familial amyloidosis by the chemical natureof the fibrillar protein has become possible. Most such amyloidogenicproteins so far recognized are variant transthyretins, but twokindreds with the same apolipoprotein AI modification have beenreported. We describe the clinical features of another suchfamily in whom petechial skin rash appeared to be a marker forthe disease, which was non-neuropathic and of the Ostertag-type.Immunohistochemistry showed the protein to be apolipoproteinAI, but allele-specific DNA amplification indicated that itwas not the Arg²⁶ variant previously identified.     

Anaphylaxis after treatment with recombinant factor VIII

BACKGROUND: Treatment of hemophilia patients with recombinant factor VIII concentrates has not previously been associated with anaphylaxis. STUDY DESIGN AND METHODS: A 5-week-old boy with severe hemophilia A developed dyspnea, cyanosis, hypotension, and a diffuse urticarial rash following treatment with a recombinant factor VIII (Recombinate). To identify the cause of anaphylaxis in this patient, the vial lot was examined for the presence of endotoxin, and a checkerboard immunoblotting technique was used to test serum and/or plasma samples from the patient and mother for the presence of antibodies (IgA, IgG, IgE, and IgM) to Recombinate-related antigens (recombinant factor VIII, von Willebrand factor, human serum albumin, Chinese hamster ovary proteins, bovine serum albumin, mouse monoclonal anti-human factor VIII, polyethylene glycol 3350), and to ethylene oxide, the agent used to sterilize the infusion equipment. RESULTS: No immune response directed against the Recombinate-related antigens or ethylene oxide that could be associated with the anaphylactic reaction was identified. Endotoxin was not present upon rabbit pyrogen testing of the therapeutic product. CONCLUSION: These studies failed to show any association between Recombinate and the onset of the allergic reaction. This seems to be the first reported case of anaphylaxis following the infusion of a recombinant form of factor VIII concentrate.     

Discrepancies in reverse ABO typing due to prozone

Three group O sera manifesting prozone in reverse ABO tests are reported. All were implicated in erroneous blood typing results. One sample failed to react with A1 red cells (RBCs) in immediate-spin (IS) tests, had anti-A and -B titers of 8192 and 2048, respectively, by indirect antiglobulin technique (IAT), and was from a diabetic patient; the parenteral administration of A substance present in porcine insulin is a possible cause of hyperimmunity in this case. The second sample was from the recipient of a single unit of group B fresh-frozen plasma; the serum anti-A and -B titers were 10,240 by IAT, but only weak reactions with A1 and B RBCs were noted in routine IS reverse typing tests; the hyperimmunity in the patient concerned was likely due to crossreacting anti-A, B stimulated by B-active glycoproteins and/or glycolipids in the transfused plasma. The third serum also had anti-A and anti-B IAT titers of 10,240 but did not react with A1 and B RBCs by IS; the hyperimmunity in this case may be related to sepsis from intestinal flora carrying A- and/or B-like antigens. These antibodies lysed A1 and/or B RBCs in tests incubated at room temperature (RT) and strongly agglutinated those RBCs by IS when diluted 10-fold with saline. The absence of the prozone phenomenon in tests with RBCs suspended in diluents containing EDTA is consistent with the previously published mechanism for anti-A prozone: namely, the steric hindrance of agglutination by the C1 component of human complement.(ABSTRACT TRUNCATED AT 250 WORDS)