Diagnostic and Prognostic Value of Human Prion Detection in Cerebrospinal Fluid
A report appearing in the current issue ofAnnals of Neurology (2017;81:79-92) titled " Diagnostic and Prognostic Value of Human Prion Detection in Cerebrospinal Fluid " investigates the second-generation real-time quaking-induced conversion (RT-QuIC) prion test in a broad spectrum of prion diseases. The study concludes as follows: " The diagnostic performance of the improved CSF RT-QuIC is superior to surrogate marker tests for prion diseases such as 14-3-3 and tau proteins, and together with PRNP gene sequencing the test allows the major prion subtypes to be differentiated in vivo. This differentiation facilitates predict...
Source: neuropathology blog - January 25, 2017 Category: Radiology Source Type: blogs
FDA approves first drug for spinal muscular atrophy
The U.S. Food and Drug Administrationrecently approved Spinraza (nusinersen), the first drug approved to treat children and adults with spinal muscular atrophy (SMA). The FDA granted this application fast track designation and priority review. The drug also received orphan drug designation, which provides incentives to assist and encourage the development of drugs for rare diseases.Thanks to Dr. Nick Willard for alerting me to this development. (Source: neuropathology blog)
Source: neuropathology blog - January 23, 2017 Category: Radiology Tags: muscle Source Type: blogs
Corneal ulceration secondary to Candidal keratitis
GMS stain highlights fungal forms in the corneal stroma (Source: neuropathology blog)
Source: neuropathology blog - January 20, 2017 Category: Radiology Tags: ophthalmic pathology Source Type: blogs
Nice Example of STAT6 nuclear positivity in a WHO grade III Solitary Fibrous Tumor/Hemangiopericytoma
STAT6 immunostaining shows strong nuclear positivity among tumor cells, while the cytoplasm is uniformly negative. STAT6 nuclear immunoreactivity has been reported as a surrogate marker for the NAB2-STAT6 gene fusion, which is the defining driver mutation of solitary fibrous tumor/hemangiopericytoma. (Source: neuropathology blog)
Source: neuropathology blog - January 18, 2017 Category: Radiology Tags: neoplasms Source Type: blogs
Bo Jackson says he won't let his kids play football
Bo Jackson in 2004The implications of the description ofChronic Traumatic Encephalopathy continue to reverberate as yet another former professional football player states that he will not let his children play football. The Kansas City Star reported yesterday that former Heisman Trophy winner and Oakland Raider star running backBo Jackson said he would not play football if he were growing up today and will not let his children play the sport.“If I knew back then what I know now. I would have never played football. Never. I wish I had known about all of those head injuries, but no one knew that. And the people that did kn...
Source: neuropathology blog - January 13, 2017 Category: Radiology Tags: trauma Source Type: blogs
Neuroscience Online, the Open-Access Neuroscience Electronic Textbook
Thisonline, interactive coursewarefor the study of neuroscience is worth bookmarking. It ' s provided byMcGovern Medical School at UTHealth in Houston. The project is under the direction of Neurobiology and Anatomy ChairJohn H. Byrne, PhD.John H. Byrne, PhD. (Source: neuropathology blog)
Source: neuropathology blog - January 4, 2017 Category: Radiology Tags: websites Source Type: blogs
Neuropathology History: D ésiré Magloire Bourneville (1840-1909)
D ésiré Magloire Bourneville (1840-1909)Born in Garenci éres, France. From 1905 until his death, Bourneville headed the Foundation Vallée for the Study of Feebleminded Children. " Was recognized as the leading continental authority on all aspects of mental abnormality of children. Most of his neuropathologic work was onidiocy. His description of tuberous sclerosis, since known as Bourneville ' s disease, appeared in 1880. "Source: Haymaker, Webb (Army Institute of Pathology). Guide to the exhibit on the history of neuropathology. Presented at the annual meetings of the American Psychiatric Association (Washington...
Source: neuropathology blog - December 7, 2016 Category: Radiology Tags: history Source Type: blogs
Neuropathology History: Sir Edward Farquhar Buzzard (1871-1945)
" Born in London... Became Regius Professor of Medicine at Oxford University, and consulting physician at St. Thomas ' s Hospital (1928). In the field of neuropathology, he is well known for his textbook in collaboration with Greenfield (1921)... Important also were his studies on myasthenia gravis (to which he contributed the term ' lymphorrhages ' ) (1905), chronic progressive cerebral softening (1906),... delayed traumatic apoplexy (1909), and epidemic encephalitis (1919). "Sir Edward Farquhar Buzzard (1871-1945)Source: Haymaker, Webb (Army Institute of Pathology). Guide to the exhibit on the history of neuropathology. ...
Source: neuropathology blog - December 6, 2016 Category: Radiology Tags: history Source Type: blogs
Neuropathology History: Carl Wernicke (1848-1905)
" Born in Tarnowitz, Poland... A highly original thinker, he can be said to have been a pupil only of Meynert, though he was greatly influenced by the works of Hitzig and Munk. "Carl Wernicke (1848-1905)Source: Haymaker, Webb (Army Institute of Pathology). Guide to the exhibit on the history of neuropathology. Presented at the annual meetings of the American Psychiatric Association (Washington, DC, May 17-20, 1948) and the American Neurological Association (Atlantic City, June 14-17, 1948). (Source: neuropathology blog)
Source: neuropathology blog - December 5, 2016 Category: Radiology Tags: history Source Type: blogs
Guest Post: Fibrous Bodies Nicely Demonstrated in a Smear from a Somatotroph Pituitary Adenoma
Christian Davidson, MDDr. Christian Davidson, director of neuropathology at theRobert Wood Johnson University Hospital in New Jersey, provides today ' s blog post:A 30-year-old man presented with bitemporal hemianopsia and a 3.0 cm pituitary mass was discovered upon MRI. His IGF-1 was elevated to 900, but he had no signs of acromegaly. A smear of tissue sent for frozen section evaluation (see above below) revealed that most cells had round, eosinophilic, perinuclear inclusions suggestive of fibrous bodies (some examples are circled). Dot-like CAM5.2 immunostain (not shown) confirmed my smear-based diagnostic suspicion...
Source: neuropathology blog - December 2, 2016 Category: Radiology Tags: neoplasms pituitary Source Type: blogs
Pineal Parenchymal Tumor of Intermediate Differentiation, WHO grade III
" A tumor of the pineal gland that is intermediate in malignancy between pineocytoma and pineoblastoma and is composed of diffuse sheets or large lobules of monomorphic round cells that appear more differentiated than those observed in pineoblastoma. " -- WHO Book (2016)The particular example depicted above recurred with leptomeningeal spread. (Source: neuropathology blog)
Source: neuropathology blog - December 1, 2016 Category: Radiology Tags: neoplasms Source Type: blogs
Vestige of a choroidal melanoma
Only melanin and melanophages remain in an enucleation specimen from a patient successfully treated with brachytherapy for choroidal melanoma. The eye was enucleated not because of the tumor, but because it was blind and intractably painful in the aftermath of treatment. (Source: neuropathology blog)
Source: neuropathology blog - November 29, 2016 Category: Radiology Tags: ophthalmic pathology Source Type: blogs
Best Post of October 2016: Brain Cancer Surpasses Leukemia as #1 Pediatric Cancer Killer
The next in our " Best of the Month " series comes from October 18, 2016:The following post appeared on the Johns Hopkins Neuropathology Blog last month. The author is Andrew Black:New data from the CDC shows the mortality rates for pediatric cancers is in decline. A study published by the CDC found that during 1999 –2014, the cancer death rate for patients aged 1–19 years in the United States dropped 20%. What is also changing are the type of patients dying. In 1999, leukemia was the leading killer of childhood cancer. That has been replaced by brain cancer. Numerous other trends were also observed in the st...
Source: neuropathology blog - November 28, 2016 Category: Radiology Tags: Best of the Month series facts and figures Source Type: blogs
Germline and somatic BAP1 mutations in high-grade rhabdoid meningiomas
Ganesh Shankar of Brigham and Women ' s Hospital and colleagues recently published an article in Neuro-Oncology entitled Germline and somatic BAP1 mutations in high-grade rhabdoid meningiomas. Rhabdoid meningiomas are designated in the World Health Organization Classification of Tumours as high grade, despite the fact that only a subset follow an aggressive clinical course. To define genomic aberrations of rhabdoid meningiomas, the authors performed sequencing of cancer-related genes in 27 meningiomas from 18 patients with rhabdoid features and evaluated breast cancer [BRCA]1 –associated protein 1 (BAP1) expres...
Source: neuropathology blog - November 23, 2016 Category: Radiology Tags: neoplasms Source Type: blogs
MOC Exam Topic: Acute Hemorrhagic Leukoencephalopathy
First recognized as a discrete entity by Weston Hurst in 1941, acute hemorrhagic leukoencephalopathy (AHL) is a usually fatal disease characterized clinically by an abrupt onset of fever, neck stiffness, and neurological deficits, often progressing rapidly to seizures and coma. The presenting clinical picture is similar to that of acute disseminated encephalomyelitis (ADEM) but with a more fulminant course. At autopsy, the brain is swollen with multiple petechial hemorrhages centered in the white matter. Large foci of necrosis with cavitation may be present. The cerebral cortex and basal ganglia usually appear intact. Hist...
Source: neuropathology blog - November 22, 2016 Category: Radiology Tags: autoimmune MOC Source Type: blogs
