Globoid cells, glial nodules, and peculiar fibrillary changes in the cerebro-hepato-renal syndrome of zellweger

1977 ◽  
Vol 2 (6) ◽  
pp. 473-484 ◽  
Author(s):  
Guillermo A. de León ◽  
Warren D. Grover ◽  
Dale S. Huff ◽  
Gladys Morinigo-Mestre ◽  
Hope H. Punnett ◽  
...  
Keyword(s):  
2000 ◽  
Vol 92 (4) ◽  
pp. 722-725 ◽  
Author(s):  
Robert R. Hammond ◽  
Neil Duggal ◽  
John M. J. Woulfe ◽  
John P. Girvin

✓ A 29-year-old man presented in 1984 with a recent onset of partial seizures marked by speech arrest. Electroencephalography identified a left frontotemporal dysrhythmia. Computerized tomography (CT) scanning revealed a superficial hypodense nonenhancing lesion in the midleft frontal convexity, with some remodeling of the overlying skull. The patient was transferred to the London Health Sciences Centre for subtotal resection of what was diagnosed as a “fibrillary astrocytoma (microcystic).” He received no chemotherapy or radiation therapy and remained well for 11 years.The patient presented again in late 1995 with progressive seizure activity. Both CT and magnetic resonance imaging demonstrated a recurrent enhancing partly cystic lesion. A Grade IV astrocytoma was resected, and within the malignant tumor was a superficial area reminiscent of a dysembryoplastic neuroepithelial tumor (DNT). Data on the lesion that had been resected in 1984 were reviewed, and in retrospect the lesion was identified as a DNT of the complex form. It was bordered by cortical dysplasia and contained glial nodules, in addition to the specific glioneuronal element. The glial nodules were significant for moderate pleomorphism and rare mitotic figures. The Ki67 labeling index averaged 0.3% in the glial nodules and up to 4% focally. Cells were rarely Ki67 positive within the glioneuronal component. This case is the first documented example of malignant transformation of a DNT. It serves as a warning of the potential for malignant transformation in this entity, which has been traditionally accepted as benign. This warning may be especially warranted when confronted with complex forms of DNT. The completeness of resection in the benign state is of paramount importance.


2021 ◽  
Vol 33 (6) ◽  
pp. 1137-1141
Author(s):  
Risako Yamashita ◽  
Toshinori Yoshida ◽  
Mio Kobayashi ◽  
Suzuka Uomoto ◽  
Saori Shimizu ◽  
...  

Neuroleptospirosis is a rare disease caused by pathogenic Leptospira interrogans in humans; however, it has not been fully studied in animals. A young wild raccoon dog was found convulsing in the recumbent position and died the next day. Histologic examination revealed nonsuppurative meningoencephalitis in the cerebrum, cerebellum, midbrain, and medulla oblongata. The lesions consisted of mixed infiltrates of Iba1-positive macrophages and CD3-positive T cells, with a small number of CD79α-positive B cells and myeloperoxidase-positive neutrophils. In the frontal cortex, perivascular cuffs and adjacent microglial nodules were distributed diffusely, especially in the molecular layer. Glial nodules were comprised of Iba1- and myeloperoxidase-positive activated microglia. Immunohistochemistry revealed leptospires in mononuclear cell perivascular cuffs, but not in glial nodules. Neuroleptospirosis was accompanied by Leptospira-related nonsuppurative interstitial nephritis, pulmonary edema and hemorrhage, and coronary periarteritis, as well as Toxocara tanuki in the small intestine and nonspecific foreign-body granulomas in the lungs and stomach.


2019 ◽  
Vol 17 ◽  
pp. 100213
Author(s):  
Tadanori Hamano ◽  
Tatsuro Mutoh ◽  
Hironobu Naiki ◽  
Norimichi Shirafuji ◽  
Masamichi Ikawa ◽  
...  

1993 ◽  
Vol 5 (2) ◽  
pp. 212-225 ◽  
Author(s):  
Donal O'Toole ◽  
Martin Jeffrey ◽  
Derek Challoner ◽  
Roz Maybey ◽  
Valerie Welch

Bilateral pelvic limb paresis developed in 7 of 15 10-month-old Blueface Leicester ram lambs on a mixed enterprise farm in the North of England. Clinical signs were principally mild to severe paresis of the pelvic limbs. Two mildly affected lambs recovered. Multifocal spinal cord white matter edema and necrosis, glial nodules, and mild to moderate nonsuppurative encephalomyelitis were the principal findings in 3 severely paretic lambs examined histologically. Protozoan bodies (12.7–23.0 μm) that stained immunocytochemically for Sarcocystis epitopes were in spinal cord glial cells of 2 of 3 lambs. Protozoa did not react immunocytochemically with anti-Toxoplasma gondii or Neospora canium antisera. Serology indicated there was recent exposure to Sarcocystis spp. in some surviving lambs. These cases resembled those in previous reports of paresis due to an unidentified Sarcocystis-like protozoan in sheep (ovine protozoan myeloencephalitis) in the British Isles, the USA, France, Australia, and New Zealand.


2018 ◽  
Vol 30 (5) ◽  
pp. 770-773 ◽  
Author(s):  
Arnaud J. Van Wettere ◽  
Jaqueline P. Kurz ◽  
Amanda Wilhelm ◽  
Jay Dee Ipsen

A juvenile, wild-caught prairie falcon ( Falco mexicanus) kept for falconry was presented to a veterinary hospital for intermittent opisthotonos and torticollis. Clinical examination, complete blood count, serum biochemistry panel, and fecal analysis were unremarkable. Clinical signs did not resolve, and the bird was euthanized 6 mo after the appearance of clinical signs. Autopsy revealed a mild, unilateral hydrocephalus and nematodes within the thoracic air sac. Histopathology demonstrated mild, unilateral hydrocephalus; scattered glial nodules; meningeal nematode sections; and meningeal and intraventricular embryonated eggs. Morphology and molecular characterization were consistent with the air sac nematode Serratospiculum or Serratospiculoides spp. Air sac nematode infection can be associated with air sacculitis or pneumonia in falcons. Aberrant migration of air sac filariid nematodes Serratospiculum or Serratospiculoides spp. into the nervous system resulting in clinical disease is rare, but should be included in the differential diagnosis of neurologic diseases in falcons.


1985 ◽  
Vol 20 (1) ◽  
pp. 49-54 ◽  
Author(s):  
Shigeichi Kobayashi ◽  
Morio Katayama ◽  
Elizabeth Bourque ◽  
Kinuko Suzuki ◽  
Kunihiko Suzuki

2002 ◽  
Vol 39 (4) ◽  
pp. 494-496 ◽  
Author(s):  
C. J. Sigurdson ◽  
R. J. Basaraba ◽  
E. M. Mazzaferro ◽  
D. H. Gould

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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