scholarly journals The neuropathology of “typical” Friedreich's ataxia in Quebec

1984 ◽  
Vol 11 (S4) ◽  
pp. 592-600 ◽  
Author(s):  
J.B. Lamarche ◽  
B. Lemieux ◽  
H.B. Lieu
Keyword(s):  
Spinal Cord ◽  
Central Nervous System ◽  
Nervous System ◽  
Peripheral Nervous System ◽  
Diagnostic Criteria ◽  
Clinical Findings ◽  
Spinal Ganglia ◽  
Cooperative Study ◽  
Segmental Demyelination ◽  
Dying Back

AbstractWe present the pathological data from the autopsies performed on 6 Friedreich's disease patients since the start of the Quebec Cooperative Study. All patients met the strict diagnostic criteria of the QCSFA. The anatomical lesions found in the peripheral and central nervous system were similar in all 6 cases and do not differ from those described in the literature. The clinical findings correlate closely with the histological lesions found in the peripheral nervous system and spinal cord. The evidence of segmental demyelination and remyelination in the spinal ganglia and posterior roots further supports the dying-back axonopathy hypothesis.

scholarly journals Microtubule-associated protein 2 within axons of spinal motor neurons: associations with microtubules and neurofilaments in normal and beta,beta'-iminodipropionitrile-treated axons.

1985 ◽  
Vol 100 (1) ◽  
pp. 74-85 ◽  
Author(s):  
S C Papasozomenos ◽  
L I Binder ◽  
P K Bender ◽  
M R Payne
Keyword(s):  
Spinal Cord ◽  
Central Nervous System ◽  
Nervous System ◽  
Peripheral Nervous System ◽  
Dorsal Root Ganglia ◽  
Motor Neurons ◽  
Dorsal Root ◽  
Spinal Motor Neurons ◽  
Spinal Motor ◽  
The Central Nervous System

We have examined the distribution of microtubule-associated protein 2 (MAP2) in the lumbar segment of spinal cord, ventral and dorsal roots, and dorsal root ganglia of control and beta,beta'-iminodipropionitrile-treated rats. The peroxidase-antiperoxidase technique was used for light and electron microscopic immunohistochemical studies with two monoclonal antibodies directed against different epitopes of Chinese hamster brain MAP2, designated AP9 and AP13. MAP2 immunoreactivity was present in axons of spinal motor neurons, but was not detected in axons of white matter tracts of spinal cord and in the majority of axons of the dorsal root. A gradient of staining intensity among dendrites, cell bodies, and axons of spinal motor neurons was present, with dendrites staining most intensely and axons the least. While dendrites and cell bodies of all neurons in the spinal cord were intensely positive, neurons of the dorsal root ganglia were variably stained. The axons of labeled dorsal root ganglion cells were intensely labeled up to their bifurcation; beyond this point, while only occasional central processes in dorsal roots were weakly stained, the majority of peripheral processes in spinal nerves were positive. beta,beta'-Iminodipropionitrile produced segregation of microtubules and membranous organelles from neurofilaments in the peripheral nervous system portion and accumulation of neurofilaments in the central nervous system portion of spinal motor axons. While both anti-MAP2 hybridoma antibodies co-localized with microtubules in the central nervous system portion, only one co-localized with microtubules in the peripheral nervous system portion of spinal motor axons, while the other antibody co-localized with neurofilaments and did not stain the central region of the axon which contained microtubules. These findings suggest that (a) MAP2 is present in axons of spinal motor neurons, albeit in a lower concentration or in a different form than is present in dendrites, and (b) the MAP2 in axons interacts with both microtubules and neurofilaments.

Observations on the Golgi Material of the Neurones of the Central Nervous System of Fowl affected with Neurolymphomatosis gallinarum.

1950 ◽  
Vol 64 (2) ◽  
pp. 172-181
Author(s):  
K. S. Chodnik
Keyword(s):  
Spinal Cord ◽  
Central Nervous System ◽  
Nervous System ◽  
Cerebral Cortex ◽  
Morphological Changes ◽  
Pyramidal Cells ◽  
Small Cells ◽  
Spinal Ganglia ◽  
The Central Nervous System ◽  
The Subject

SynopisThe Golgi material of the neurones of the central nervous system of normal fowl and of birds affected with spontaneous cases of Neurolymphomatosis gallinarum (fowl paralysis) is described. Material was prepared according to the methods of Kolatchev and of Aoyama. The Golgi material of normal pyramidal cells of the cerebral cortex, the Purkinje cells of the cerebellum, the multipolar cells of the spinal cord, and the cells of the spinal ganglia, is present as rods, irregular bodies and filaments. Compact net-like Golgi material is present in the small cells of the spinal ganglia.In the neurones of fowl affected with Neurolymphomatosis gallinarum the Golgi material undergoes hypertrophy, followed by clustering of the Golgi elements and fragmentation. The intensity of the morphological changes and the number of neurones affected in a particular region of the central nervous system varies considerably, except in the spinal ganglia where all the cells examined exhibited marked changes. It was not possible to determine whether the changes were primary or secondary in nature. The literature of the subject is discussed.

scholarly journals Multiple Immediate-Early Gene-Deficient Herpes Simplex Virus Vectors Allowing Efficient Gene Delivery to Neurons in Culture and Widespread Gene Delivery to the Central Nervous System In Vivo

2001 ◽  
Vol 75 (9) ◽  
pp. 4343-4356 ◽  
Author(s):  
Caroline E. Lilley ◽  
Filitsa Groutsi ◽  
ZiQun Han ◽  
James A. Palmer ◽  
Patrick N. Anderson ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Central Nervous System ◽  
Nervous System ◽  
Gene Delivery ◽  
Transgene Expression ◽  
Retrograde Transport ◽  
Spinal Ganglia ◽  
The Central Nervous System ◽  
Simplex Virus

ABSTRACT Herpes simplex virus (HSV) has several potential advantages as a vector for delivering genes to the nervous system. The virus naturally infects and remains latent in neurons and has evolved the ability of highly efficient retrograde transport from the site of infection at the periphery to the site of latency in the spinal ganglia. HSV is a large virus, potentially allowing the insertion of multiple or very large transgenes. Furthermore, HSV does not integrate into the host chromosome, removing any potential for insertional activation or inactivation of cellular genes. However, the development of HSV vectors for the central nervous system that exploit these properties has been problematical. This has mainly been due to either vector toxicity or an inability to maintain transgene expression. Here we report the development of highly disabled versions of HSV-1 deleted for ICP27, ICP4, and ICP34.5/open reading frame P and with an inactivating mutation in VP16. These viruses express only minimal levels of any of the immediate-early genes in noncomplementing cells. Transgene expression is maintained for extended periods with promoter systems containing elements from the HSV latency-associated transcript promoter (J. A. Palmer et al., J. Virol. 74:5604–5618, 2000). Unlike less-disabled viruses, these vectors allow highly effective gene delivery both to neurons in culture and to the central nervous system in vivo. Gene delivery in vivo is further enhanced by the retrograde transport capabilities of HSV. Here the vector is efficiently transported from the site of inoculation to connected sites within the nervous system. This is demonstrated by gene delivery to both the striatum and substantia nigra following striatal inoculation; to the spinal cord, spinal ganglia, and brainstem following injection into the spinal cord; and to retinal ganglion neurons following injection into the superior colliculus and thalamus.

Polyarteritis Nodosa

2013 ◽  
Author(s):  
Aaron E. Miller ◽  
Teresa M. DeAngelis
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Gastrointestinal Tract ◽  
Peripheral Nervous System ◽  
Diagnostic Criteria ◽  
Polyarteritis Nodosa ◽  
Treatment Recommendations ◽  
General Treatment ◽  
Neurological Manifestations

Polyarteritis nodosa (PAN) is a systemic illness that most often involves the peripheral nervous system, skin, kidneys, and gastrointestinal tract. In this chapter, we review the diagnostic criteria for PAN, its most common neurological manifestations affecting the peripheral nervous system as well as central nervous system presentations, and discuss general treatment recommendations.

Further Observations on the Influence of Toxins on the Central Nervous System

1914 ◽  
Vol 60 (249) ◽  
pp. 184-191
Author(s):  
D. Orr ◽  
R. G. Rows
Keyword(s):  
Spinal Cord ◽  
Central Nervous System ◽  
Nervous System ◽  
Peripheral Nervous System ◽  
Mode Of Action ◽  
Peripheral Nerves ◽  
The Central Nervous System

For some years, we have been engaged in an investigation into the mode of action of toxins upon the central nervous system, and up to the present time have devoted our attention exclusively to the question of the upward passage of bacterial poisons along the sheaths of peripheral nerves to the spinal cord and brain. Experiment has shown us that toxins readily travel upwards in the perineural lymphatics, in which they induce an inflammation whose phenomena vary with the intensity of the irritant; and that this is continued without interruption to the central nervous system, granted that the toxins gain that level. Continuity of extension is, therefore, an important feature of lymphogenous inflammation, and is as constant in the central as in the peripheral nervous system.

scholarly journals Central Nervous System Involvement by Leukemia in Children. I. Relationship to Systemic Leukemia and Description of Clinical and Laboratory Manifestations

Blood ◽  
1965 ◽  
Vol 25 (1) ◽  
pp. 1-12 ◽  
Author(s):  
CAROL B. HYMAN ◽  
JAMES M. BOGLE ◽  
CHARLES A. BRUBAKER ◽  
KENNETH WILLIAMS ◽  
DENMAN HAMMOND
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Survival Time ◽  
Diagnostic Criteria ◽  
Systemic Disease ◽  
Central Nervous System Involvement ◽  
Clinical Findings ◽  
Cns Involvement ◽  
Leukemia In Children ◽  
Csf Findings

Abstract Observations on the course of 59 children who experienced 109 distinct episodes of CNS involvement by leukemia showed that: 1. This complication may be associated with all types of acute and subacute leukemia. 2. There is no single or combination of diagnostic criteria. Manifestations of increased CSF pressure, such as vomiting, headache, and papilledema are the most frequent clinical findings. However, it should be emphasized that CNS involvement may be associated with normal CSF findings. 3. CNS involvement may be present at the onset of leukemia or can occur at any time during the course. 4. Approximately 26 per cent of children with leukemia develop CNS involvement. 5. CNS involvement may occur when the disease is under apparently good therapeutic control as well as during relapse. 6. There is no relationship between the agents which had been previously used to treat the systemic disease and the later development of CNS involvement. However, the onset of CNS symptoms was less frequent when the systemic disease was under treatment with steroids. 7. The development of CNS involvement does not appear to shorten the survival time of patients with leukemia when treatment for CNS involvement is given.

Glucuronyl 3-sulfate occurs exclusively on distal unmyelinated terminals of selected sensory neurons in the peripheral nervous system

1995 ◽  
Vol 53 ◽  
pp. 958-959
Author(s):  
S.S. Spicer ◽  
B.A. Schulte
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Cerebral Cortex ◽  
Peripheral Nervous System ◽  
Sensory Neurons ◽  
Sensory Nerves ◽  
Tissue Antigens ◽  
The Central Nervous System ◽  
The Brain ◽  
Leu 7

Generation of monoclonal antibodies (MAbs) against tissue antigens has yielded several (VC1.1, HNK- 1, L2, 4F4 and anti-leu 7) which recognize the unique sugar epitope, glucuronyl 3-sulfate (Glc A3- SO4). In the central nervous system, these MAbs have demonstrated Glc A3-SO4 at the surface of neurons in the cerebral cortex, the cerebellum, the retina and other widespread regions of the brain.Here we describe the distribution of Glc A3-SO4 in the peripheral nervous system as determined by immunostaining with a MAb (VC 1.1) developed against antigen in the cat visual cortex. Outside the central nervous system, immunoreactivity was observed only in peripheral terminals of selected sensory nerves conducting transduction signals for touch, hearing, balance and taste. On the glassy membrane of the sinus hair in murine nasal skin, just deep to the ringwurt, VC 1.1 delineated an intensely stained, plaque-like area (Fig. 1). This previously unrecognized structure of the nasal vibrissae presumably serves as a tactile end organ and to our knowledge is not demonstrable by means other than its selective immunopositivity with VC1.1 and its appearance as a densely fibrillar area in H&E stained sections.

Central Nerve System Impairment, AMA Guides, Sixth Edition: An Overview

2018 ◽  
Vol 23 (1) ◽  
pp. 10-13
Author(s):  
James B. Talmage ◽  
Jay Blaisdell
Keyword(s):  
Spinal Cord ◽  
Central Nervous System ◽  
Nervous System ◽  
Neurological Impairment ◽  
Sixth Edition ◽  
Central Nerve System ◽  
The Central Nervous System ◽  
The One ◽  
Nerve System ◽  
The Brain

Abstract Injuries that affect the central nervous system (CNS) can be catastrophic because they involve the brain or spinal cord, and determining the underlying clinical cause of impairment is essential in using the AMA Guides to the Evaluation of Permanent Impairment (AMA Guides), in part because the AMA Guides addresses neurological impairment in several chapters. Unlike the musculoskeletal chapters, Chapter 13, The Central and Peripheral Nervous System, does not use grades, grade modifiers, and a net adjustment formula; rather the chapter uses an approach that is similar to that in prior editions of the AMA Guides. The following steps can be used to perform a CNS rating: 1) evaluate all four major categories of cerebral impairment, and choose the one that is most severe; 2) rate the single most severe cerebral impairment of the four major categories; 3) rate all other impairments that are due to neurogenic problems; and 4) combine the rating of the single most severe category of cerebral impairment with the ratings of all other impairments. Because some neurological dysfunctions are rated elsewhere in the AMA Guides, Sixth Edition, the evaluator may consult Table 13-1 to verify the appropriate chapter to use.

METABOLIC STUDIES WITH 131I-LABELED THYROXINE. II.

1963 ◽  
Vol 44 (3) ◽  
pp. 475-480 ◽  
Author(s):  
R. Grinberg
Keyword(s):  
Spinal Cord ◽  
Central Nervous System ◽  
Nervous System ◽  
Optic Nerve ◽  
Pituitary Gland ◽  
Time Interval ◽  
Time Intervals ◽  
Pituitary Glands ◽  
The Central Nervous System ◽  
Tentative Explanation

ABSTRACT Radiologically thyroidectomized female Swiss mice were injected intraperitoneally with 131I-labeled thyroxine (T4*), and were studied at time intervals of 30 minutes and 4, 28, 48 and 72 hours after injection, 10 mice for each time interval. The organs of the central nervous system and the pituitary glands were chromatographed, and likewise serum from the same animal. The chromatographic studies revealed a compound with the same mobility as 131I-labeled triiodothyronine in the organs of the CNS and in the pituitary gland, but this compound was not present in the serum. In most of the chromatographic studies, the peaks for I, T4 and T3 coincided with those for the standards. In several instances, however, such an exact coincidence was lacking. A tentative explanation for the presence of T3* in the pituitary gland following the injection of T4* is a deiodinating system in the pituitary gland or else the capacity of the pituitary gland to concentrate T3* formed in other organs. The presence of T3* is apparently a characteristic of most of the CNS (brain, midbrain, medulla and spinal cord); but in the case of the optic nerve, the compound is not present under the conditions of this study.

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