Cleidocranial Dysplasia with Neonatal Death Due to Central Nervous System Injury in Utero: Case Report and Literature Review

1998 ◽  
Vol 1 (4) ◽  
pp. 314-318 ◽  
Author(s):  
Calvin E. Oyer ◽  
Nina G. Tatevosyants ◽  
Selina C. Cortez ◽  
Abby Hornstein ◽  
Michael Wallach
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
In Utero ◽  
Cleidocranial Dysplasia ◽  
Related Condition ◽  
Wormian Bones ◽  
Bilateral Absence ◽  
The Central Nervous System ◽  
The Right ◽  
A Minor

Cleidocranial dysplasia (CCD), an uncommon disorder involving membranous bones, is rarely lethal in early life. The calvaria is defective and wormian bones are present. Abnormalities of the clavicles vary in severity from a minor unilateral defect to bilateral absence. This report concerns pre- and postmortem anatomical and radiological findings in a 15-day-old female neonate with CCD. Her postnatal course was characterized by seizures and recognition of hydrocephalus during the first day of life. The calvaria was hypoplastic with numerous wormian bones. A pseudofracture of the right clavicle was present. Hydrocephalus was present in the brachycephalic brain which had a severely thinned cerebral cortex. Hemosiderin in the ventricular lining and marked subependymal gliosis were interpreted as evidence of old intraventricular hemorrhage that had occurred in utero. A CCD-related condition, Yunis-Varon syndrome (YVS), is noted for early lethality and for developmental and secondary abnormalities of the central nervous system. The present case only partially matches the phenotype of YVS and might represent a part of a spectrum of phenotypic variants ranging from viable CCD to lethal YVS.

scholarly journals Malignant Solitary Fibrous Tumor of the Right Cerebellum: A Case Report

2021 ◽  
Vol 13 (1) ◽  
pp. 259-266
Author(s):  
Ye-Tao Zhu ◽  
Yang Liu ◽  
Li-Gang Chen ◽  
Da-Ping Song
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Solitary Fibrous Tumor ◽  
Immunohistochemical Staining ◽  
Biological Behavior ◽  
Resonance Imaging ◽  
Solitary Fibrous Tumors ◽  
The Central Nervous System ◽  
Fibrous Tumor ◽  
The Right

Solitary fibrous tumor is a very rare mesenchymal tumor that occurs mostly in the pleura, and there are few reported cases of a presence in the central nervous system, particularly in the cerebellum. In 2016, the WHO classified solitary fibrous tumors into grade I. In this article, we present a case of malignant solitary fibrous tumor recurring 8 years after surgery in a 63-year-old male. Magnetic resonance imaging showed low to intermediate mixed signal intensity on T1W1. Immunohistochemical staining positivity for Vimentin, CD99, CD34 and Bcl-2, it is consistent with the immunohistochemical characteristics of solitary fibrous tumor. We resected the patient’s tumor, and the patient was followed up for 3 months with no signs of recurrence. Solitary fibrous tumors are very rare in the central nervous system. Immunohistochemical staining positivity for CD34 and Bcl-2 is strongly expressed in most solitary fibrous tumor. Surgical resection is the preferred treatment. Due to the small number of cases, the biological behavior and prognosis of this tumor need to be further explored.

Blood Pressure and Pulse Rate in the Foetal Sheep

1945 ◽  
Vol 22 (1-2) ◽  
pp. 63-74
Author(s):  
JOSEPH BARCROFT ◽  
D. H. BARRON
Keyword(s):  
Blood Pressure ◽  
Central Nervous System ◽  
Nervous System ◽  
Pulse Rate ◽  
Umbilical Artery ◽  
The Central Nervous System ◽  
The Right ◽  
First Moment ◽  
Left Vagus ◽  
Stimulation Of

1. A method (the needle method) is described for the measurement of the pressure in the stream going through a vessel. 2. In the foetal sheep the needle method applied to the umbilical artery gives substantially the same results as the mercurial manometer applied to the carotid, until about half-way through the gestation period. 3. As gestation proceeds the needle method applied at the first moment at which it can be applied to the umbilical artery (or a branch) gives readings substantially lower, and increasingly lower as gestation proceeds, than does the mercurial manometer read at the first moment at which it can be read. 4. The discrepancy is due to the sum of a number of causes which are discussed, but of these the most important is an actual rise of pressure between the time of delivery and the completion of the dissections contingent on the use of the mercurial manometer. 5. The cause of this is not at present demonstrated, but either or both of two factors may be concerned: (a) a dulling of the central nervous system which weakens the depressor reflex; (b) the establishment of a greater degree of vasomotor tone consequent on the bombardment of the central nervous system with sensory stimuli. 6. The pulse rates in utero and just after delivery of the foetus into a saline bath at 39-40°C. (the umbilical circulation being unimpaired) are not significantly different. 7. The pulse rate quickens up to the 70th-80th day, after which it becomes slower as gestation proceeds. 8. If both vagi be severed, the pulse rate te to quicken throughout gestation. The pulse, therefore, comes increasingly under vagus inhibition from the 80th-90th day onwards. 9. Even after the vagi have been cut after the 120th day (it has not been tried before) adrenalin in sufficient quantity will cause a further quickening of the pulse. 10. The earliest date at which stimulation of the peripheral end of the right vagus was observed to slow the heart was the 77th day. On the 85th day peripheral stimulation of the left vagus also failed, but succeeded on the 101st day. 11. Central stimulation of the left vagus, with the right vagus intact, produced slowing on the 77th day. 12. Slowing of the heart synchronous with rise of arterial pressure has been observed on the 111th day. 13. Slowing of the heart which bears evidence of being reflex has been obtained by raising the blood pressure (clamping the cord) on the 121st day and by injection of adrenalin on the 118th day. 14. Approaching term both the carotid sinus and cardiac depressor mechanisms are functional. 15. Lowering of the blood pressure as the result of stimulation of the central end of the vagus and with both vagi severed can be demonstrated late in gestation.

scholarly journals Cerebrospinal fluid ceftazidime kinetics in patients with external ventriculostomies.

1996 ◽  
Vol 40 (3) ◽  
pp. 763-766 ◽  
Author(s):  
R Nau ◽  
H W Prange ◽  
M Kinzig ◽  
A Frank ◽  
A Dressel ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Cerebrospinal Fluid ◽  
Nervous System ◽  
High Performance ◽  
Elimination Half ◽  
Daily Dose ◽  
Order Of Magnitude ◽  
The Central Nervous System ◽  
Occlusive Hydrocephalus ◽  
A Minor

Ceftazidime has proven to be effective for the treatment of bacterial meningitis caused by multiresistant gram-negative bacteria. Since nosocomial central nervous system infections are often accompanied by only a minor dysfunction of the blood-cerebrospinal fluid (CSF) barrier, patients with noninflammatory occlusive hydrocephalus who had undergone external ventriculostomy were studied (n = 8). Serum and CSF were drawn repeatedly after the administration of the first dose of ceftazidime (3 g over 30 min intravenously), and concentrations were determined by high-performance liquid chromatography by using UV detection. The concentrations of ceftazidime in CSF were maximal at 1 to 13 h (median, 5.5 h) after the end of the infusion and ranged from 0.73 to 2.80 mg/liter (median, 1.56 mg/liter). The elimination half-lives were 3.13 to 18.1 h (median, 10.7 h) in CSF compared with 2.02 to 5.24 h (median, 3.74 h) in serum. The ratios of the areas under the concentration-time curves in CSF and serum (AUCCSF/AUCS) ranged from 0.027 to 0.123 (median, 0.054). After the administration of a single dose of 3 g, the maximum concentrations of ceftazidime in CSF were approximately four times higher than those after the administration of 2-g intravenous doses of cefotaxime (median, 0.44 mg/liter) and ceftriaxone (median, 0.43 mg/liter) (R. Nau, H. W. Prange, P. Muth, G. Mahr, S. Menck, H. Kolenda, and F. Sörgel, Antimicrob. Agents Chemother. 37:1518-1524, 1993). The median AUCCSF/AUCS ratio of ceftazidime was slightly below that of cefotaxime (0.12), but it was 1 order of magnitude above the median AUCCSF/AUCS of ceftriaxone (0.007) (Nau et al., Antimicrob. Agents Chemother. 37:1518-1524, 1993). The concentrations of ceftazidime observed in CSF were above the MICs for most Pseudomonas aeruginosa strains. However, they are probably not high enough to be rapidly bactericidal. For this reason, the daily dose should be increased to 12 g in cases of P. aeruginosa infections of the central nervous system when the blood-CSF barrier is minimally impaired.

Extracranial Metastases of Oligodendroglioma

Neurosurgery ◽  
1981 ◽  
Vol 8 (3) ◽  
pp. 391-396 ◽  
Author(s):  
Nelson G. Ordóñez ◽  
Alberto G. Ayala ◽  
Milam E. Leavens
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Lymph Nodes ◽  
Masseter Muscle ◽  
Lumbar Vertebrae ◽  
Cervical Lymph Nodes ◽  
Extraneural Metastases ◽  
The Central Nervous System ◽  
The Right ◽  
Extracranial Metastases

Abstract The case of a 33-year-old woman with oligodendroglioma with extraneural metastases involving the masseter muscle, the cervical lymph nodes, the lumbar vertebrae, and the right iliac bone is described. Oligodendroglioma with metastases outside the central nervous system is extremely rare, and only a very few cases have been reported.

Lethal phenotype of mice carrying a Sept11 null mutation

2011 ◽  
Vol 392 (8-9) ◽  
pp. 779-781 ◽  
Author(s):  
Sabrina Röseler ◽  
Kirstin Sandrock ◽  
Ingrid Bartsch ◽  
Anja Busse ◽  
Heymut Omran ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Binding Proteins ◽  
Vesicle Trafficking ◽  
In Utero ◽  
High Expression ◽  
Synaptic Connectivity ◽  
Null Mutation ◽  
Gtp Binding ◽  
The Central Nervous System

Abstract Septins are cytoskeletal GTP-binding proteins involved in processes characterized by active membrane movement, such as cytokinesis, vesicle trafficking and exocytosis. Most septins are expressed ubiquitously, however, some septins accumulate in particular tissues. The ubiquitous SEPT11 also shows high expression levels in the central nervous system and in platelets. Here, SEPT11 is involved in vesicle trafficking and may play a role in synaptic connectivity. Interestingly, mice that harbor a homozygous Sept11 null mutation, die in utero. From day 11.5 post coitum onwards, development of homozygous embryos seems to be retarded and the embryos from day 13.5 onwards were dead.

scholarly journals Accuracy of Force Repeatability in Relation to its Value and the Subjects’ Sex

2017 ◽  
Vol 18 (2) ◽  
Author(s):  
Ryszard Błacha ◽  
Agnieszka D. Jastrzębska
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Sex Differences ◽  
Muscle Force ◽  
Force Generation ◽  
Male Students ◽  
Minor Influence ◽  
The Central Nervous System ◽  
A Minor ◽  
Voluntary Contractions

AbstractThe purpose of the study was to determine the influence of force value and sex on force generation repeatability.The total of 17 female and 24 male students performed 3 maximal voluntary contractions for maximal force (FThe force generation repeatability rose with the increase of triggered force in both sexes; between force target 49 N vs. 98 N and 147 N (The influence of force value and a minor influence of sex on accuracy in generated forces might suggest that the control of muscle force by the central nervous system is similar in both sexes and the sex differences in muscle force generations are rather of muscle mass and structure.

Kidney and Ureteral Disease during HIV Infection

1992 ◽  
Vol 59 (2) ◽  
pp. 46-49
Author(s):  
R. Musci ◽  
T. Meroni ◽  
M. Andres ◽  
O. De Cobelli ◽  
P. Larcher ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Hiv Infection ◽  
Acquired Immunodeficiency Syndrome ◽  
Urogenital System ◽  
Hiv Virus ◽  
The Central Nervous System ◽  
Acquired Immunodeficiency ◽  
A Minor ◽  
Immunodeficiency Syndrome

Infection by Hiv virus affects the urogenital system in a minor percentage of cases in comparison to other organs such as the lungs, the central nervous system and the haemolymphopoietic system. In recent years however, with the continued spread of the disease, also urologists find themselves dealing with various urogenital pathologies that are presented in seropositive or fully-blown Aids patients. The Authors present their experience and describe the problems correlated to dealing with acquired immunodeficiency syndrome patients that are affected with urological pathologies.

KINKY HAIR DISEASE

PEDIATRICS ◽  
1972 ◽  
Vol 50 (2) ◽  
pp. 181-183
Author(s):  
John H. Menkes
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
In Utero ◽  
Degenerative Disease ◽  
Degenerative Diseases ◽  
Kinky Hair Disease ◽  
The Central Nervous System ◽  
Almost All ◽  
Enzymatic Defect ◽  
Kinky Hair

Despite many recent advances in our understanding of progressive degenerative diseases of the nervous system which have permitted us in some instances to define the underlying enzymatic defect and to detect the disease in utero, treatment for affected children has been nonexistent in almost all instances. The paper by Danks et al.1 in this issue of Pediatrics is, therefore, of considerable importance. It not only demonstrates the underlying cause for one of these disorders, Kinky Hair disease, but also suggests a relatively simple course of treatment. Ten years ago a group of Residents from the Departments of Neurology, Pediatric Neurology, Neuropathology, and Dermatology described in this journal2 what appeared to be a new degenerative disease of the central nervous system.

scholarly journals Ataxia as the main manifestation of tumor-like primary angiitis of the central nervous system: a case report and literature review

2019 ◽  
Vol 19 (1) ◽  
Author(s):  
Yu-Jing Huang ◽  
Li Zhang ◽  
Ying Mao ◽  
Guang-Xian Nan
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Glucocorticoid Therapy ◽  
Cerebellar Hemisphere ◽  
Case Presentation ◽  
System A ◽  
Intravenous Drip ◽  
Primary Angiitis ◽  
The Central Nervous System ◽  
The Right

Abstract Background Primary angiitis of the central nervous system (PACNS) is a rare disease, and tumor-like primary angiitis of the central nervous system is even rarer. Histopathology is the gold standard for tumor-mimicking PACNS. However, pathological diagnosis is relatively limited due to fewer biopsy opportunities. Case presentation A 68-year-old male presented with ataxia, and was diagnosed with tumor-like primary angiitis of the central nervous system. The patient underwent Intravenous drip glucocorticoid therapy (10 mg of dexamethasone, daily). After 10 days, the symptoms of the patient were completely relieved. Radiology revealed that the low density lesion in the right cerebellar hemisphere obviously narrowed. Cyclophosphamide therapy was not initiated. Conclusion It is crucial for clinicians to be aware of changes in radiology that indicate PACNS, since the diagnosis of tumor-like PACNS remains quite challenging. Glucocorticoid therapy is an effective therapy in this condition, and the prognosis can be favorable.

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