Levodopa Responsive Parkinsonism in Patients with Hemochromatosis: Case Presentation and Literature Review

Girotra, Tarun; Mahajan, Abhimanyu; Sidiropoulos, Christos

doi:10.1155/2017/5146723

Cited by 4 publications

(7 citation statements)

References 18 publications

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“…Hereditary haemochromatosis (HH) was also frequently identified in this systematic review. Four papers reported typical Parkinsonism [ 31 , 32 , 33 , 34 ] while atypical features were described in the remaining three papers. All papers were related to unresponsiveness to levodopa [ 35 , 36 , 37 ].…”

Section: Discussionmentioning

confidence: 99%

Parkinson’s Disease and Metal Storage Disorders: A Systematic Review

2018

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Metal storage disorders (MSDs) are a set of rare inherited conditions with variable clinical pictures including neurological dysfunction. The objective of this study was, through a systematic review, to identify the prevalence of Parkinsonism in patients with MSDs in order to uncover novel pathways implemented in Parkinson’s disease. Human studies describing patients of any age with an MSD diagnosis were analysed. Foreign language publications as well as animal and cellular studies were excluded. Searches were conducted through PubMed and Ovid between April and September 2018. A total of 53 publications were identified including 43 case reports, nine cross-sectional studies, and one cohort study. The publication year ranged from 1981 to 2018. The most frequently identified MSDs were Pantothenate kinase-associated neurodegeneration (PKAN) with 11 papers describing Parkinsonism, Hereditary hemochromatosis (HH) (7 papers), and Wilson’s disease (6 papers). The mean ages of onset of Parkinsonism for these MSDs were 33, 53, and 48 years old, respectively. The Parkinsonian features described in the PKAN and HH patients were invariably atypical while the majority (4/6) of the Wilson’s disease papers had a typical picture. This paper has highlighted a relationship between MSDs and Parkinsonism. However, due to the low-level evidence identified, further research is required to better define what the relationship is.

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Section: Discussionmentioning

confidence: 99%

Parkinson’s Disease and Metal Storage Disorders: A Systematic Review

2018

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“…Rare reports of parkinsonian syndrome in hereditary hemochromatosis describes characteristic symptoms of resting tremor, bradykinesia, rigidity, and an asymmetric onset. Mixed resting and action tremor were also reported in one patient case ( 27 – 29 ).…”

Section: Inherited Metabolic Disordersmentioning

confidence: 66%

“…As previously discussed, the dysregulation of iron metabolism has been suggested to contribute to the risk of PD development. This is further evidenced through the observation of altered iron levels in the SNpc of PD patients through MRI findings and postmortem samples ( 27 , 123 – 125 ). Increased intracellular iron promotes the degeneration of dopaminergic neurons via several suggested pathways, in addition to the enhancement of oxidative stress as previously described ( 53 ).…”

Section: Inherited Metabolic Disordersmentioning

confidence: 94%

“…Furthermore, recent data has demonstrated the neuroprotective role of iron chelators against dopamine neuronal degeneration and suggests its therapeutic potential for PD treatment ( 126 , 127 ). Although the theoretical risk of increased parkinsonism exists with disorders of iron regulation, the association between hemochromatosis and its genetic mutations, levels of iron, and PD has conflicting results, necessitating further extensive population and genetic studies ( 27 , 128 , 129 ).…”

Section: Inherited Metabolic Disordersmentioning

confidence: 99%

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Parkinsonism in Inherited Metabolic Disorders: Key Considerations and Major Features

et al. 2018

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Parkinson's Disease (PD) is a common neurodegenerative disorder manifesting as reduced facilitation of voluntary movements. Extensive research over recent decades has expanded our insights into the pathogenesis of the disease, where PD is indicated to result from multifactorial etiological factors involving environmental contributions in genetically predisposed individuals. There has been considerable interest in the association between neurological manifestations in PD and in inherited metabolic disorders (IMDs), which are genetic disorders characterized by a deficient activity in the pathways of intermediary metabolism leading to multiple-system manifestations. In addition to the parallel in various clinical features, there is increasing evidence for the notion that genetic mutations underlying IMDs may increase the risk of PD development. This review highlights the recent advances in parkinsonism in patients with IMDs, with the primary objective to improve the understanding of the overlapping pathogenic pathways and clinical presentations in both disorders. We discuss the genetic convergence and disruptions in biochemical mechanisms which may point to clues surrounding pathogenesis-targeted treatment and other promising therapeutic strategies in the future.

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“…However, toxic effects of paraquat are attenuated by the conjugation of free radical metabolites with glutathione-S-transferases [61]. Studies on the patients with PD revealed high iron level in substantia nigra, where iron accumulation is associated with C282Y mutations responsible for hemochromatosis, a hereditary iron overload disorder than the controls, suggesting C282Y mutation increases the risk of PD [62]. Further, redox activity in neuromelanin-aggregates is significantly higher in the PD patients manifested by severe neuronal loss, may be due to severe oxidative stress [63].…”

Section: Toxicants and Oxidative Stress In Pdmentioning

confidence: 99%

Toxic Exposure and Life Style Factors on Ageing Brain Neurodegenerative Disease, Alzheimer’s and Parkinson’s: Role of Natural Antioxidants to Ameliorate the Condition

Naha¹,

Das²,

Banerjee³

2018

J Alcohol Drug Depend

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Toxic exposure is a major risk factor for many neurodegenerative diseases in ageing brain such as, Alzheimer's (AD) and Parkinson's (PD), where decline of biological activities in cellular microenvironment render the organism more susceptible to either endogenous or exogenous stressors especially free radical damage, leading to pathological conditions. PCBs, MPTP, organochlorines and organophosphates, paraquat, fipronil, pyrithroid, quinines, and metals like, lead, cadmium, chromium, cobalt, manganese, arsenic etc. are play the crucial role in age-related diseases, AD and PD. Further, the degree of changes in the ageing brain in these cases not only depends on toxic exposures and genetic susceptibility but also on food habit, life style (use of alcohol, smoking, caffeine), environment etc. Certain antioxidants through diet can improve scenario of ageing brain and modulate specific molecular signalling. Therefore, this review briefly discusses the current status of the toxic exposure and life styles on ageing brain neurodegenerative diseases especially, AD and PD, and throws a light on the disease management through natural antioxidant supplements. For this purpose, data were searched through PubMed, MedLine, ResearchGate and Google using several combinations of terms pertaining to different aspects of oxidative modification in ageing brain, exposure of several toxicants and life style factors, AD and PD as well as natural antioxidants.

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Levodopa Responsive Parkinsonism in Patients with Hemochromatosis: Case Presentation and Literature Review

Cited by 4 publications

References 18 publications

Parkinson’s Disease and Metal Storage Disorders: A Systematic Review

Parkinson’s Disease and Metal Storage Disorders: A Systematic Review

Parkinsonism in Inherited Metabolic Disorders: Key Considerations and Major Features

Toxic Exposure and Life Style Factors on Ageing Brain Neurodegenerative Disease, Alzheimer’s and Parkinson’s: Role of Natural Antioxidants to Ameliorate the Condition

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