Disorders of the Cerebellum, Including the Degenerative Ataxias

Subramony, S.H.; Xia, Guangbin

doi:10.1016/b978-1-4377-0434-1.00097-9

Cited by 4 publications

(4 citation statements)

References 168 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…28 Reduced concentration of dopaminergic transporter in the nigrostriatal pathway has also been reported in SCA2 29 and SCA3. 3,4 Therefore, it is conceivable that the REM sleep reduction in Parkinson disease, SCA2 and SCA3 may have similar pathomechanism.…”

Section: Discussionmentioning

confidence: 99%

Sleep disruption in spinocerebellar ataxia type 3: A genetic and polysomnographic study

Chi¹,

Shiao²,

Ku³

et al. 2013

Journal of the Chinese Medical Association

View full text Add to dashboard Cite

show abstract

Section: Discussionmentioning

confidence: 99%

Sleep disruption in spinocerebellar ataxia type 3: A genetic and polysomnographic study

Chi¹,

Shiao²,

Ku³

et al. 2013

Journal of the Chinese Medical Association

View full text Add to dashboard Cite

show abstract

“…Cerebellum controls the maintenance of equilibrium (balance), influences posture and muscle tone as well as coordinating movement [8]. Cerebellar ataxia may be acquired from exposure to many toxic materials [39]. Myelin is an electrical insulator that increases conduction velocity of nerve fibres and is the physical basis for rapid saltatory conduction (in which impulses jump from one node of Ranvier to another) [29].…”

Section: Introductionmentioning

confidence: 99%

Acrylamide-induced adverse cerebellar changes in rats: possible oligodendrogenic effect of omega 3 and green tea

Imam

Gadallah

2019

Folia Morphol

View full text Add to dashboard Cite

Background: Humans are widely exposed to acrylamide (ACR) and its neurotoxicity is a significant public health issue attracting wide attention. The aim of the study was to investigate ACR-induced adverse cerebellar changes in rats and study the possible oligodendrogenic effect of omega 3 and green tea. Materials and methods: Twenty-four adult albino rats weighing 150-200 g were randomly divided into four equal groups (6 rats each): control group (Group I), the rats that received ACR 45 mg/kg/day (Group II), the rats that received ACR concomitant with omega 3 at a dosage of 200 mg/kg/day (Group III), the rats that received ACR concomitant with green tea dissolved in drinking water at a dosage of 5 g/L (Group IV). The rats were euthanized after 8 weeks of the experiment. Malondialdehyde (MDA) and glutathione (GSH) were measured in cerebellar homogenates. Sections of 5 µm thickness from specimens from the cerebellum were stained with haematoxylin and eosin, silver stain and immunohistochemical stains: platelet-derived growth factor alpha (PDGFα; for oligodendrocytes), glial fibrillary acidic protein (GFAP; for astrocytes) and BCL2 (antiapoptotic). Results: Omega 3 and green tea had improved MDA and GSH as compared to the ACR group. Histologically, the ACR group showed variable degrees of cellular degeneration. Omega 3 had induced oligodendrogenesis in Group III. The optical density of silver stain was significantly (p < 0.05) increased in Groups III and IV as compared to the ACR group. Area per cent of positive PDGFα was significantly increased in the ACR + omega 3 group as compared to the ACR group. Area per cent of positive GFAP was significantly decreased in Groups III and IV as compared to the ACR group. Area per cent of positive BCL2 was significantly increased in the omega 3-trated group as compared to the ACR group. Conclusions: Concomitant administration of omega 3 or green tea with ACR might mitigate the adverse cerebellar changes caused by ACR thanks to an oligodendrogenic effect of omega 3. (Folia Morphol 2019; 78, 3: 564-574)

show abstract

“…[1423] In community-based studies also, the prevalence rate of ataxia-1 was reported to be 1-2 per 1,00,000 population. [6] To our knowledge, no report has yet been published on the incidence of SCA from central Indian region. The present study is, thus,first in this respect.…”

Section: Discussionmentioning

confidence: 99%

“…At the pathological level, the most frequent and severe alterations seen in SCA1 patients are the loss of Purkinje cells in the cerebellar cortex and the degeneration of neurons in the inferior olivary nuclei, cerebellar dentate nuclei and red nuclei. [268–11] Numerous observations have established that the polyglutamine repeat, by itself, has a central role in the pathogenesis of polyglutamine diseases, although its effects are strongly modulated by the protein context within which it resides. [12] Nine distinct polyglutamine or triplet repeat disorders and the corresponding genes have, thus far, been identified.…”

Section: Introductionmentioning

confidence: 99%

Analysis of autosomal dominant spinocerebellar ataxia type 1 in an extended family of central India

et al. 2012

View full text Add to dashboard Cite

BACKGROUND:Spinocerebeller ataxia type 1 (SCA1) is a specific type of ataxia among a group of inherited diseases of the central nervous system. In SCA1, genetic defects lead to impairment of specific nerve fibers carrying messages to and from the brain, resulting in the degeneration of the cerebellum, the coordination center of the brain. We investigated 24 members of an extended family in Gwalior city, India, some of which were earlier clinically diagnosed to be suffering from yet unconfirmed type of SCA neurodegenerative disorder.MATERIALS AND METHODS:All the family members from each age group were screened clinically and the characteristics of those resembling with ataxia were recorded for diagnosis by MRI. The confirmed patients of the family were genetically tested by PCR based molecular testing to identify the type of SCA (i.e., SCA 1, 2, 3, 4, 6 or 7). Family tree of the disease inheritance was constructed by pedigree based method.RESULT AND CONCLUSION:We found the clinical (symptoms and MRI) and genetic (Pedigree and PCR) results to be correlated. The PCR result revealed the disease to be of SCA 1 type being inherited in the family.

show abstract

Disorders of the Cerebellum, Including the Degenerative Ataxias

Cited by 4 publications

References 168 publications

Sleep disruption in spinocerebellar ataxia type 3: A genetic and polysomnographic study

Sleep disruption in spinocerebellar ataxia type 3: A genetic and polysomnographic study

Acrylamide-induced adverse cerebellar changes in rats: possible oligodendrogenic effect of omega 3 and green tea

Analysis of autosomal dominant spinocerebellar ataxia type 1 in an extended family of central India

Contact Info

Product

Resources

About