Periodic acid-Schiff-positive cells in cerebrospinal fluid of dogs with globoid cell leukodystrophy

Globoid cell leukodystrophy (GLD; Krabbe disease), is a rare heritable metabolic disorder in humans, dogs, mutant twitcher mice, and rhesus monkeys that is caused by a deficiency in the lysosomal enzyme galactocerebrosidase (GALC). GALC deficiency results in the accumulation of psychosine, which is toxic to oligodendrocytes and Schwann cells of the central and peripheral nervous systems. Clinical signs include hypotonia, mental regression, and death by 2 years of age in most human patients. Here we describe a domestic longhaired kitten with rapidly progressive neurologic disease and brain and spinal cord lesions characteristic of GLD. Pathologic hallmarks of the disease reflect the loss of oligodendrocytes and include myelin loss, gliosis, and the perivascular accumulation of large mononuclear cells with fine cytoplasmic vacuoles (globoid cells) in the peripheral and central nervous systems. Globoid cells were CD68 and ferritin positive, confirming their monocytic origin, and cytoplasmic contents were nonmetachromatic and periodic acid-Schiff positive.

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Swine Cerebrospinal Angiopathy with Demyelination and Malacia

Veterinary Pathology ◽

10.1177/030098588201900205 ◽

1982 ◽

Vol 19 (2) ◽

pp. 140-149 ◽

Cited By ~ 3

Author(s):

K. Nakamura ◽

M. Kubo ◽

S. Shoya ◽

M. Kashiwazaki ◽

S. Koizumi ◽

...

Keyword(s):

Escherichia Coli ◽

Cerebrospinal Fluid ◽

Small Intestine ◽

Medulla Oblongata ◽

Pure Culture ◽

Periodic Acid ◽

Histologic Examination ◽

Vascular Lesions ◽

Periodic Acid Schiff ◽

E Coli

In 15 pigs affected with cerebrospinal angiopathy accompanied by demyelination and malacia, the main symptoms were diarrhea and subsequent circling, spasms, sudden forward movements, ataxia, and inability to hold the head straight. Escherichia coli was isolated in a pure culture from the small intestine of pigs with diarrhea. The only gross change was a slight increase in cerebrospinal fluid. Histologic examination showed vascular lesions, demyelination, and malacia, most commonly located in the medulla oblongata, pons, and midbrain. The vascular lesions were degenerative and there were necrotic changes of the vessel walls and formation of periodic acid-Schiff-positive perivascular eosinophilic droplets. Ultrastructurally, the swollen astrocytes around the vessels had many osmiophilic bodies in their cytoplasm with no limiting membrane. Demyelination and malacia, as well as vascular lesions, were considered to be the characteristic changes of cerebrospinal angiopathy. Our study suggests that E. coli may be a cause of cerebrospinal angiopathy.

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Globoid Cell Leucodystrophy in Polled Dorset Sheep

Veterinary Pathology ◽

10.1177/030098588001700402 ◽

1980 ◽

Vol 17 (4) ◽

pp. 399-405 ◽

Cited By ~ 33

Author(s):

D. H. Pritchard ◽

D. V. Napthine ◽

A. J. Sinclair

Keyword(s):

White Matter ◽

Hind Limb ◽

Periodic Acid ◽

Clinical Signs ◽

Periodic Acid Schiff ◽

Histologic Changes ◽

The Brain ◽

Globoid Cell Leucodystrophy ◽

Globoid Cell ◽

Globoid Cells

Globoid cell leucodystrophy (Krabbe's disease) was diagnosed in two Polled Dorset sheep from a stud farm. Clinical signs were hind limb incoordination progressing to tetraplegia. Histologic changes in white matter of the brain were myelin destruction, loss of oligodendroglia, astrogliosis and accumulation of distinctive periodic acid-Schiff (PAS)-positive globoid cells. The activities of galactocerebroside β-galactosidase, the lysosomal enzyme specifically deficient in globoid leucodystrophy, and of three other glycosidase enzymes were compared in brain tissue of one affected and six unaffected sheep. Activities of the three nonspecific glycosidases were similar in all seven brains. Galactocerebrosidase activity was similar in the six control sheep, but in the affected brain was less than 6% of the control mean.

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Clinical and MRI findings in two unrelated cats with globoid cell leucodystrophy

Veterinary Record Case Reports ◽

10.1136/vetreccr-2020-001129 ◽

2020 ◽

Vol 8 (2) ◽

pp. e001129

Author(s):

Alessia Silvia Colverde ◽

Teresa Gagliardo ◽

Cristian Falzone ◽

Gualtiero Gandini

Keyword(s):

Periodic Acid ◽

Mri Findings ◽

Periodic Acid Schiff ◽

Flexor Reflex ◽

Neurological Signs ◽

Brain And Spinal Cord ◽

The Brain ◽

Unrelated Male ◽

Globoid Cell Leucodystrophy ◽

Globoid Cell

Two unrelated male cats, both five months old, were referred for progressive neurological signs characterised by intentional tremors, paraplegia with absence of nociception in the pelvic limbs, weakness, dysmetria with reduced flexor reflex in the thoracic limbs, and bilaterally reduced menace response. MRI, performed by a 0.25 T Esaote Vet Grande, showed diffuse and irregular intramedullary T2 weighted hyperintensity between T3 and L3; these lesions were isointense on T1 weighted images without contrast uptake. In one patient MRI of the brain was obtained and mild cerebellar volume reduction was found. Histological examination of the brain and spinal cord demonstrated myelin loss and perivascular accumulation of periodic acid-Schiff-positive big macrophages. These findings were consistent with globoid cell leucodystrophy, as previously described in cats. Although not specific, in young cats with progressive spinal neurological signs, especially when associated with cerebellar signs, and irregular but diffuse T2 intramedullary hyperintensity and T1 isointensity without contrast uptake, globoid cell leucodystrophy should be suspected.

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Cerebrospinal fluid and serum glycosphingolipid biomarkers in canine globoid cell leukodystrophy (Krabbe Disease)

Molecular and Cellular Neuroscience ◽

10.1016/j.mcn.2019.103451 ◽

2020 ◽

Vol 102 ◽

pp. 103451 ◽

Cited By ~ 1

Author(s):

Carley R. Corado ◽

Jason Pinkstaff ◽

Xuntian Jiang ◽

Evelyn M. Galban ◽

Samantha J. Fisher ◽

...

Keyword(s):

Cerebrospinal Fluid ◽

Krabbe Disease ◽

Globoid Cell Leukodystrophy ◽

Globoid Cell

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Millipore Analysis of Valvular Fluid in Sterile Valve Malfunctions

Neurosurgery ◽

10.1227/00006123-199106000-00010 ◽

1991 ◽

Vol 28 (6) ◽

pp. 848-852 ◽

Cited By ~ 2

Author(s):

Vincent C. Traynelis ◽

Crystl D. Willison ◽

Kenneth A. Follett ◽

Janet Chambers ◽

Sydney S. Schochet ◽

...

Keyword(s):

Cerebrospinal Fluid ◽

Mononuclear Cells ◽

Periodic Acid ◽

Inflammatory Cells ◽

Allergic Reactions ◽

Millipore Filter ◽

Periodic Acid Schiff ◽

Hematoxylin And Eosin ◽

Valve Malfunction ◽

Millipore Filters

Abstract Malfunctions of sterile shunts may result from valvular dysfunction. The cerebrospinal fluid shunt valves of 14 patients were excised during surgery for sterile shunt malfunctions. In 6 patients, the malfunction was due specifically to a valve malfunction. Cerebrospinal fluid from each valve was passed through a millipore filter, which was then stained using either hematoxylin and eosin or periodic acid-Schiff. The stained millipore filters were examined by a neuropathologist who was unaware of the cause of the shunt malfunction. Although inflammatory cells were detected in all cases, the patients with valve malfunctions were found to have numerous macrophages and giant multinucleated reactive cells within their valves, while cerebrospinal fluid from valves that had been removed during shunt revisions for reasons other than a malfunctioning valve contained only rare mononuclear cells or macrophages. No valve contained erythrocytes, fibrinous matter, neural or glial tissue, or choroid plexus. The possible causes of valve malfunction, including infection and allergic reactions, are discussed. All patients did well after simple replacement of the valve.

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Ultrastructure of the Parotid Gland of the Nine-Banded Armadillo

Proceedings, annual meeting, Electron Microscopy Society of America ◽

10.1017/s0424820100080626 ◽

1977 ◽

Vol 35 ◽

pp. 640-641

Author(s):

J. R. Ruby

Keyword(s):

Endoplasmic Reticulum ◽

Parotid Gland ◽

Periodic Acid ◽

Acinar Cells ◽

Duct System ◽

Parotid Glands ◽

Dasypus Novemcinctus ◽

Anterior Portion ◽

Periodic Acid Schiff ◽

Thin Sections

Parotid glands were obtained from five adult (four male and one female) armadillos (Dasypus novemcinctus) which were perfusion-fixed. The glands were located in a position similar to that of most mammals. They extended interiorly to the anterior portion of the submandibular gland.In the light microscope, it was noted that the acini were relatively small and stained strongly positive with the periodic acid-Schiff (PAS) and alcian blue techniques, confirming the earlier results of Shackleford (1). Based on these qualities and other structural criteria, these cells have been classified as seromucous (2). The duct system was well developed. There were numerous intercalated ducts and intralobular striated ducts. The striated duct cells contained large amounts of PAS-positive substance.Thin sections revealed that the acinar cells were pyramidal in shape and contained a basally placed, slightly flattened nucleus (Fig. 1). The rough endoplasmic reticulum was also at the base of the cell.

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Detection of Carriers in Glanzmann's Thrombasthenia

Thrombosis and Haemostasis ◽

10.1055/s-0038-1657357 ◽

1983 ◽

Vol 49 (03) ◽

pp. 182-186

Author(s):

G T E Zonneveld ◽

E F van Leeuwen ◽

A Sturk ◽

J W ten Cate

Keyword(s):

Bleeding Time ◽

Periodic Acid ◽

Type I ◽

Clot Retraction ◽

Periodic Acid Schiff ◽

Glanzmann’S Thrombasthenia ◽

Aggregation Response ◽

Glanzmann's Thrombasthenia ◽

Sds Page ◽

Reduced State

SummaryQuantitative glycoprotein (GP) analysis of whole platelets or platelet membranes was performed by SDS-polyacrylamide gelelectrophoresis (SDS-PAGE) and periodic acid Schiff staining in the families of two unrelated Glanzmann’s thrombasthenia (GT) patients. Each family consisted of two symptom free parents, a symptom free daughter and a GT daughter. All symptom free members had a normal bleeding time, clot retraction and platelet aggregation response to adenosine 5’-diphosphate (ADP), collagen and adrenalin. Platelet Zw* antigen was normally expressed in these subjects. GT patiens, classified as a type I and II subject, showed reduced amounts of GP lib and of GP nia. Analysis of isolated membranes in the non-reduced state, however, showed that the amount of GP Ilia was also reduced in three of the four parents, whereas one parent (of the GT type I patient) and the two unaffected daughters had normal amounts of GP Ilia. Quantitative SDS-PAGE may therefore provide a method for the detection of asymptomatic carriers in GT type I and II.

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Swim bladder mycosis in farmed rainbow trout Oncorhynchus mykiss caused by Phoma herbarum and experimental verification of pathogenicity

Diseases of Aquatic Organisms ◽

10.3354/dao03464 ◽

2020 ◽

Vol 138 ◽

pp. 237-246 ◽

Cited By ~ 1

Author(s):

J Řehulka ◽

A Kubátová ◽

V Hubka

Keyword(s):

Rainbow Trout ◽

Histopathological Examination ◽

Periodic Acid ◽

Bladder Wall ◽

Swim Bladder ◽

Cell Reactivity ◽

Periodic Acid Schiff ◽

Posterior Portion ◽

Hepatic Congestion ◽

Phoma Herbarum

In this study, spontaneous swim bladder mycosis was documented in a farmed fingerling rainbow trout from a raceway culture system. At necropsy, the gross lesions included a thickened swim bladder wall, and the posterior portion of the swim bladder was enlarged due to massive hyperplasia of muscle. A microscopic wet mount examination of the swim bladder contents revealed abundant septate hyphae, and histopathological examination showed periodic acid-Schiff-positive mycelia in the lumen and wall of the swim bladder. Histopathological examination of the thickened posterior swim bladder revealed muscle hyperplasia with expansion by inflammatory cells. The causative agent was identified as Phoma herbarum through morphological analysis and DNA sequencing. The disease was reproduced in rainbow trout fingerlings using intraperitoneal injection of a spore suspension. Necropsy in dead and moribund fish revealed extensive congestion and haemorrhages in the serosa of visceral organs and in liver and abdominal serosanguinous fluid. Histopathological examination showed severe hepatic congestion, sinusoidal dilatation, Kupffer cell reactivity, leukostasis and degenerative changes. Fungi were disseminated to the liver, pyloric caeca, kidney, spleen and heart. Although infections caused by Phoma spp. have been repeatedly reported in fish, species identification has been hampered by extensive taxonomic changes. The results of this study confirmed the pathogenicity of P. herbarum in salmonids by using a reliably identified strain during experimental fish infection and provides new knowledge regarding the course of infection.

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Erdheim-Chester Disease Presenting with Histiocytic Colitis and Cytokine Storm

Journal of Gastrointestinal and Liver Diseases ◽

10.15403/jgld.2014.1121.262.erd ◽

2017 ◽

Vol 26 (2) ◽

pp. 183-187

Author(s):

George P. Christophi ◽

Yeshika Sharma ◽

Quader Farhan ◽

Umang Jain ◽

Ted Walker ◽

...

Keyword(s):

Periodic Acid ◽

Poor Response ◽

Rare Type ◽

Periodic Acid Schiff ◽

Hematopoietic Stem ◽

Hemodynamic Compromise ◽

Erdheim Chester Disease ◽

Erdheim Chester ◽

Chester Disease ◽

Segmental Colitis

Background: Non-Langerhans histiocytosis is a group of inflammatory lymphoproliferative disorders originating from non-clonal expansion of hematopoietic stem cells into cytokine-secreting dendritic cells or macrophages. Erdheim-Chester Disease (ECD) is a rare type of non-Langerhans cell histiocytosis characterized by tissue inflammation and injury caused by macrophage infiltration and histologic findings of foamy histiocytes. Often ECD involves the skeleton, retroperitoneum and the orbits. This is the first report documenting ECD manifesting as segmental colitis and causing cytokine-release syndrome.Case presentation: A 68-year old woman presented with persistent fever without infectious etiology and hematochezia. Endoscopy showed segmental colitis and pathology revealed infiltration of large foamy histiocytes CD3-/CD20-/CD68+/CD163+/S100- consistent with ECD. The patient was empirically treated with steroids but continued to have fever and developed progressive distributive shock.Conclusion: This case report describes the differential diagnosis of infectious and immune-mediated inflammatory and rheumatologic segmental colitis. Non-Langerhans histiocytosis and ECD are rare causes of gastrointestinal inflammation. Prompt diagnosis is imperative for the appropriate treatment to prevent hemodynamic compromise due to distributive shock or gastrointestinal bleeding. Importantly, gastrointestinal ECD might exhibit poor response to steroid treatment and other potential treatments including chemotherapy, and biologic treatments targeting IL-1 and TNF-alpha signaling should be considered.Abbreviations: AFB: acid-fast bacilli; ECD: Erdheim-Chester Disease; IBD: inflammatory bowel disease; PASD: periodic acid-Schiff with diastase; TB: tuberculosis

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