Isolated pyramidal tract impairment in the central nervous system of adult-onset Krabbe disease with novel mutations in the GALC gene

2013 ◽  
Vol 35 (6) ◽  
pp. 579-581 ◽  
Author(s):  
Shin-ichi Tokushige ◽  
Tomohiro Sonoo ◽  
Risa Maekawa ◽  
Yuichiro Shirota ◽  
Ritsuko Hanajima ◽  
...  
2014 ◽  
Vol 4 (1) ◽  
pp. 52-55
Author(s):  
R Habib ◽  
SB Mizan ◽  
A Rahman ◽  
NB Bhowmik ◽  
A Haque

Most clinicians consider a peripheral nerve lesion in patients with drop foot. However, causes stemming from the central nervous system represent rare, important, and underappreciated differential etiologies. Central causative lesions usually occur at locations where pyramidal tract connections are condensed and specific and the function is somatotopically organized. Here we report case presenting as central foot drop or spastic foot-drop and other myriad clinical features which after investigations was found due to bilateral parasagittal meningiomas. DOI: http://dx.doi.org/10.3329/birdem.v4i1.18559 Birdem Med J 2014; 4(1): 52-55


Author(s):  
Ulrike Schrifl ◽  
SakkuBai Naidu ◽  
Ali Fatemi

The term “leukodystrophies” refers to a group of genetic diseases characterized by degeneration of white matter in the central nervous system. Depending on the type of leukodystrophy, the phenotype can range from early infantile-onset, rapid, progressive forms to adult-onset slowly progressive variants. The understanding, definition, and classification have been enhanced greatly by the combined use of neuroimaging, especially MRI, and genetic techniques. The window for targeted therapeutic interventions remains brief and management is often limited to symptomatic, supportive, and palliative care, and new approaches for treatment remain a great task for future research.


In a previous paper in these ‘Proceeding’ (E. C. Hoff, 1932), a report was given of investigation concerning the boutons terminaux which constitute the terminals of nerve fibres around dendrites and perikarya in the central nervous system. It was shown that 24 to 48 hours after selection of the afferent roots of the spinal cord of the cat, the boutons undergo degenerative swelling and granulation. This degenerative process reaches a climax in 3 days and ends in the complete destruction of the terminals after 4 to 6 days.* In the present studies, the normal synapses of the monkey have been investigated; further, the phenomenon of degeneration of boutons following section of nerve fibres has been applied as a method for determining the endings of the pyramidal tracts in the cat and the monkey.


Author(s):  
Gladys Harrison

With the advent of the space age and the need to determine the requirements for a space cabin atmosphere, oxygen effects came into increased importance, even though these effects have been the subject of continuous research for many years. In fact, Priestly initiated oxygen research when in 1775 he published his results of isolating oxygen and described the effects of breathing it on himself and two mice, the only creatures to have had the “privilege” of breathing this “pure air”.Early studies had demonstrated the central nervous system effects at pressures above one atmosphere. Light microscopy revealed extensive damage to the lungs at one atmosphere. These changes which included perivascular and peribronchial edema, focal hemorrhage, rupture of the alveolar septa, and widespread edema, resulted in death of the animal in less than one week. The severity of the symptoms differed between species and was age dependent, with young animals being more resistant.


Author(s):  
John L.Beggs ◽  
John D. Waggener ◽  
Wanda Miller ◽  
Jane Watkins

Studies using mesenteric and ear chamber preparations have shown that interendothelial junctions provide the route for neutrophil emigration during inflammation. The term emigration refers to the passage of white blood cells across the endothelium from the vascular lumen. Although the precise pathway of transendo- thelial emigration in the central nervous system (CNS) has not been resolved, the presence of different physiological and morphological (tight junctions) properties of CNS endothelium may dictate alternate emigration pathways.To study neutrophil emigration in the CNS, we induced meningitis in guinea pigs by intracisternal injection of E. coli bacteria.In this model, leptomeningeal inflammation is well developed by 3 hr. After 3 1/2 hr, animals were sacrificed by arterial perfusion with 3% phosphate buffered glutaraldehyde. Tissues from brain and spinal cord were post-fixed in 1% osmium tetroxide, dehydrated in alcohols and propylene oxide, and embedded in Epon. Thin serial sections were cut with diamond knives and examined in a Philips 300 electron microscope.


Author(s):  
Ezzatollah Keyhani

Acetylcholinesterase (EC 3.1.1.7) (ACHE) has been localized at cholinergic junctions both in the central nervous system and at the periphery and it functions in neurotransmission. ACHE was also found in other tissues without involvement in neurotransmission, but exhibiting the common property of transporting water and ions. This communication describes intracellular ACHE in mammalian bone marrow and its secretion into the extracellular medium.


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