A case of recurrent Miller Fisher syndrome mimicking botulism

Abstract Miller Fisher syndrome (MFS) is a rare and usually monophasic polyradiculoneuropathy characterised by ophthalmoplegia, decreased or absent tendon reflexes, and ataxia. The objective of this study was to report a case of recurrent MFS with a clinical presentation virtually indistinguishable from botulism. The patient was a young man with two episodes of increasing external ophthalmoplegia, ptosis, and ataxia with a long asymptomatic interval in between. The second episode occurred after consumption of rotten fish and was accompanied by gastrointestinal symptoms and an anticholinergic syndrome. Very rarely, MFS can present with a recurrent course. The importance of this case of recurrent MFS lies not only in its long asymptomatic period and identical clinical presentation, but also in its instructiveness regarding the differential diagnosis of MFS, particularly life-threatening botulism.     

MRI findings of optic pathway involvement in Miller Fisher syndrome in 3 pediatric patients and a review of the literature

Background: Miller Fisher syndrome (MFS) is a rare demyelinating condition which may have involvement of cranial nerves. There are a few case reports of optic pathway involvement in children. We describe 3 patients with optic pathway enhancement in pediatric patients with MFS. Case series: We retrospectively reviewed brain imaging findings in 17 pediatric patients with of Guillain–Barré syndrome (GBS) meeting Brighton criteria who had brain MRIs performed during their acute illness. Cranial nerve enhancement was seen in 6/17 patients and optic nerve/chiasm enhancement was seen in 3 patients. Conclusion: Cranial nerve enhancement and optic pathway in particular, can be seen in patients with MFS. Imaging findings do not always correlate with clinical manifestations of cranial nerve involvement.     

The Montana Adaptive Equipment Project:

A statewide service delivery project which provides specialized adaptive equipment to multi-handicapped persons living in rural communities is described. In addition to client consultation regarding motor programs, the adaptive equipment project provided specialized equipment such as adaptive chairs, standing and walking aids, feeding utensils, bathing aids, etc. to handicapped client of all ages. Services were delivered via an itinerant approach and relied on parents and others in the community as primary intervention agents. The project is viewed as an important activity in maintaining recently deinstitutionalized clients in community settings.     

Comparison of five elastin histochemical stains to identify pulmonary small vasculature*

Optimization of an elastic tissue histochemical stain to enable clear, crisp visualization and quantification of pulmonary small vasculature is central to the histomorphologic quantitation of pulmonary vasculature wall thickness. To accomplish elastic tissue histochemical stain optimization, five histochemical elastin stains were compared to identify the internal and external elastic laminae of small arteries (50–100 μm in external diameter) to very small intra-acinar vessels (10–50 μm in external diameter) in rat lung tissue sections. The five elastin stains included: a modified Verhoeff’s elastin stain, Miller’s elastic van Gieson, and three modifications of the Miller’s stain. The Miller elastin stain is a progressive procedure that does not require a differentiation step, thus enabling consistency and reliability of staining from slide to slide. A modified Miller’s histochemical staining methodology successfully highlighted the pulmonary small caliber vasculature wall thickness. The modified method was technically easier and less time consuming to perform than regressive methods. To improve elastin-to-background contrast, modifications to the Miller’s stain included bypassing the nuclear staining and using a neutral red counterstain in place of the van Gieson counterstain, both of which greatly facilitated observer-assisted pulmonary vascular structure identification for histomorphometric quantitation.     

Pump dysfunction after myocardial infarction: Importance of location,extent and pattern of abnormal left ventricular segmental contraction

To delineate the relative effects on left ventricular function of the site, extent and nature of abnormal left ventricular segmental contraction (dyssynergy) and thereby determine the mechanism by which anterior myocardial infarction results in greater depression of left ventricular performance than does inferior infarction, 43 patients with remote myocardial infarction of similar extent (average 38 percent of left ventricular systolic perimeter) and associated hypokinesia or dyskinesia confined to either the anterior or inferior wall were compared; 10 additional patients were evaluated who exhibited generalized dyssynergy (72 percent of left ventricular perimeter). When the pattern of dyssynergy and extent of infarction were similar, the location alone of dyssynergy did not influence variables of left ventricular function. However, paradoxical outward systolic movement (dyskinesia) of the anterior or inferior wall resulted in greater depression (P < 0.05) of measures of left ventricular performance than did diminished inward systolic motion (hypokinesia) associated with infarction of similar extent and location. All measures of left ventricular performance were considerably more depressed (P < 0.05) in the 10 patients with generalized dyssynergy than in the 43 patients with localized dyssynergy. Thus, the location of infarction is not a unique determinant of left ventricular performance. Instead, the size of infarction is the principal characteristic of dyssynergy that impairs left ventricular function; the severity of the pattern of dyssynergy is significant but of lesser importance. It is therefore concluded that the greater reduction of left ventricular function in anterior than in inferior myocardial infarction is largely the result of the more extensive area of necrosis rather than of the location of the infarction.     

Macromolecular dispersion of human plasma low-density lipoproteins in hyperlipoproteinemia.

Low-density lipoprotein (LDL) has previously been reported to exist in either a polydisperse or a monodisperse state. Using the techniques of analytical velocity sedimentation and/or equilibrium density-gradient ultracentrifugation, the macromolecular dispersion of LDL has been investigated in 139 subjects classified by their lipoprotein phenotypes as follows: 63 normal, 25 type IIA, 6 type IIB, and 45 type IV. LDL polydispersion was found in 78% of subjects with hypertriglyceridemia (type IIB or IV phenotypes), while only 9% of normotriglyceridemic subjects had polydisperse LDL. A study of LDL dispersion in two families, one with hyperpreβ-lipoproteinemia and one with combined hyperlipoproteinemia, also demonstrated the frequent association of LDL polydispersity with increased plasma very low density lipoprotein (VLDL) concentrations. LDL polydispersion results from the presence of higher molecular weight, lipid-enriched lipoproteins of the LDL class. Among hyperlipoproteinemic subjects with a type IV phenotype and with polydisperse LDL, the concentrations of these higher molecular weight subspecies of LDL appear to increase with severity of the hyperlipemia. In 6 subjects, reduction of VLDL concentration resulted in a decrease in the concentration of the higher molecular weight LDL; however, LDL remained polydisperse. By contrast, approximately one-third of subjects with hypertriglyceridemia were observed to have monodisperse LDL, even in the presence of high VLDL concentrations. This observation raises the possibility of two separate populations of subjects with hypertriglyceridemia arising from increased VLDL concentration.