Sporadic Parkinson disease and Amyotrophic Lateral Sclerosis complex (Brait–Fahn–Schwartz Disease)

Manno, Concetta; Lipari, Alessio; Bono, Valeria; Taiello, Alfonsa Claudia; Bella, Vincenzo La

doi:10.1016/j.jns.2013.01.009

Cited by 24 publications

(17 citation statements)

References 21 publications

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“…77 The parkinsonism in these patients is usually typical for PD, including the response to levodopa. 78 Onset of motor neuron dysfunction usually follows a few years later, but simultaneous onset has been reported. 77,79 Progression is similar to that of classic ALS.…”

Section: Als With Extrapyramidal Involvementmentioning

confidence: 99%

“…77,79 Progression is similar to that of classic ALS. 78 These rare patients are thought to have two separate diseases, although mutations in PARK7 (also known as DJ1) have been reported to cause a PD-ALS-FTD syndrome, providing a possible genetic link. 80 ALS-parkinsonism is much more common than ALS-PD complex.…”

Section: Als With Extrapyramidal Involvementmentioning

confidence: 99%

“…Mild parkinsonism is present in 5-15% of patients with ALS, and is typically characterized by postural instability and backward falls. 78,81 In such patients, abnormalities in the nigro striatal system can be seen on dopamine transporter imaging or at autopsy. 78,[81][82][83] In our experience, distinguishing the spastic, extrapyramidal and frontal motor findings in such patients is not trivial, which could explain why different studies have reported variable frequencies of ALS-parkinsonism.…”

Section: Als With Extrapyramidal Involvementmentioning

confidence: 99%

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The phenotypic variability of amyotrophic lateral sclerosis

2014

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| Classic textbook neurology teaches that amyotrophic lateral sclerosis (ALS) is a degenerative disease that selectively affects upper and lower motor neurons and is fatal 3-5 years after onset-a description which suggests that the clinical presentation of ALS is very homogenous. However, clinical and postmortem observations, as well as genetic studies, demonstrate that there is considerable variability in the phenotypic expression of ALS. Here, we review the phenotypic variability of ALS and how it is reflected in familial and sporadic ALS, in the degree of upper and lower motor neuron involvement, in motor and extramotor involvement, and in the spectrum of ALS and frontotemporal dementia. Furthermore, we discuss some unusual clinical characteristics regarding presentation, age at onset and disease progression. Finally, we address the importance of this variability for understanding the pathogenesis of ALS and for the development of therapeutic strategies.

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Section: Als With Extrapyramidal Involvementmentioning

confidence: 99%

Section: Als With Extrapyramidal Involvementmentioning

confidence: 99%

Section: Als With Extrapyramidal Involvementmentioning

confidence: 99%

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The phenotypic variability of amyotrophic lateral sclerosis

2014

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“…The daily intake of aluminium hydrochloride is estimated to be approximately 10-20 mg of cooking utensils, food additives, and medicine such as antacid or deodorants. Accumulation of aluminium hydrochloride in brain induces pathophysiology of neurodegenerative including Alzheimer's, dementia, amylotrophic, lateral sclerosis, GuamParkinson's dementia etc [3]. Since a variety of biomolecules are bound to aluminium hydrochloride so it can displace the other biological cations (calcium and magnesium) from their binding sites in every metabolic pathway and shows the adverse effect of aluminium hydrochloride of the potential target site.…”

Section: Introductionmentioning

confidence: 99%

Neuroprotective Activity of Fractional Flower Extracts of Mirabilis Jalapa Against Aluminium Hydrochloride Induced Neurotoxicity in Male Wister Rats

Babu

Swain

Renuka

2017

Int J Pharm Pharm Sci

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Objective: The major objective of this present study was to evaluate the neuroprotective effect of fractional flower extracts (acetone, petroleum ether, methanol and aqueous) of Mirabilis jalapa (MJ) against aluminium hydrochloride-induced neurotoxicity in male wister rats. Methods:From the different fractional flower extracts of Mirabilis jalapa (MJ), two doses (250 and 500 mg/kg body weight) of each extract was initially selected and administered per orally 30 min prior to aluminium hydrochloride administration to the different animal groups once a day for a period of 45 d. Rat serum was collected from different animal groups on 1 st , 15 th , 30 th and 45 th Results: On the 9 days for estimation of marker enzymes, where a reduction in marker was observed. Animal was sacrificed by decapitation and the whole brain of rats was analyzed to estimate the levels of nitrite, thiobarbituric acid reactive substances (TBARS), superoxide dismutase (DOS), catalase, reduced glutathione and acetylcholinesterase (AchE).th Conclusion: This result is indicating evidence for Mirabilis jalapa had a significant neuroprotective effect on aluminium hydrochloride-induced neurotoxicity and also supports by histopathological studies.day the Wister rats were sacrificed and cerebral cortex was removed. One-half of the cerebral cortex samples from different groups of Aluminium hydrochloride treated rats were stored in FAM mixture (40% formaldehyde, acetic acid and methanol in the ratio of 1:1:8) for histological analysis. From the study confirmed that dose of 250 and 500 mg/kg bwt of methanolic extract of MJ significantly (p˂0.001) increases the reduced glutathione, superoxide dismutase and catalase level, whereas petroleum ether, acetone and aqueous fractional flower extracts of MJ significantly (p˂0.01) decreases nitrite, TBARS and AchE levels of aluminium hydrochloride treated groups.

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“…Гораздо реже выявля-ется сочетание БАС с болезнью Паркинсона, отвечающей на терапию леводопой. Нельзя исклю-чить, что в этих случаях речь идет о случайном сочета-нии 2 нейродегенеративных болезней [49]. В литера-туре описаны случаи БАС с развитием хореи или гемибаллизма на поздних стадиях болезни [50], а так-же случаи сочетания БАС с генетически подтвержденной болезнью Гентингтона [51].…”

Section: том 7 Volunclassified