An aggressive approach to limb dystonia: A case report

Moberg-Wolff, Elizabeth

doi:10.1016/s0003-9993(98)90079-x

Cited by 5 publications

(4 citation statements)

References 15 publications

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“…It is most uncommon for patients with disabling neurological diseases affecting the limbs in a focal or multifocal fashion, such as Parkinson's disease, stroke, multiple sclerosis, or neuropathy, to resort to amputation. In fact, there are only 3 case reports of this occurring in the medical literature, 1 due to a paralyzed limb after stroke,17 1 due to painful contractured legs after spinal cord injury,18 and 1 in a child with secondary dystonia,19 However, we acknowledge that other cases may exist and have simply not been reported (and were alerted by an anonymous reviewer of this article that he/she had experience of 2 cases of amputation for osteomyelitis associated with abnormal postures due to corticobasal degeneration and Rassmussen's‐like encephalopathy).…”

Section: Discussionmentioning

confidence: 99%

Hepatocyte NAD(P)H oxidases as an endogenous source of reactive oxygen species during hepatitis C virus infection

et al. 2010

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Oxidative stress has been identified as a key mechanism of hepatitis C virus (HCV)-induced pathogenesis. Studies suggest that HCV increases the generation of hydroxyl radical and peroxynitrite close to the cell nucleus, inflicting DNA damage, but the source of reactive oxygen species (ROS) remains incompletely characterized. We hypothesized that HCV increased the generation of superoxide and H2O2 close to the hepatocyte nucleus and that this source of ROS was Nox4. Huh7 human hepatoma cells and telomerase-reconstituted primary human hepatocytes, transfected or infected with virus-producing HCV strains of genotype 2a and 1b, were examined for mRNA, protein, and subcellular localization of Nox proteins, along with human liver. We found that genotype 2a HCV induced persistent elevation of Nox1 and Nox4 mRNA and proteins in Huh7 cells. Genotype 1b HCV likewise elevated the levels of Nox1 and Nox4 in telomerase-reconstituted primary human hepatocytes. Furthermore, Nox1 and Nox4 proteins were increased in HCV-infected human liver compared to uninfected liver. Unlike Nox1, Nox4 was prominent in the nuclear compartment of these cells as well as human liver, particularly in the presence of HCV. HCV-induced ROS and nuclear nitrotyrosine could be decreased with siRNAs to Nox1 and Nox4. Finally, HCV increased the level of transforming growth factor beta-1 (TGFβ1). TGFβ1 could elevate Nox4 expression in the presence of infectious HCV, and HCV increased Nox4 at least in part through TGFβ1. Conclusion HCV induced a persistent elevation of Nox1 and Nox4 and increased nuclear localization of Nox4 in hepatocytes in vitro and in human liver. Hepatocyte Nox proteins are likely to act as a persistent, endogenous source of ROS during HCV-induced pathogenesis.

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Section: Discussionmentioning

confidence: 99%

Hepatocyte NAD(P)H oxidases as an endogenous source of reactive oxygen species during hepatitis C virus infection

et al. 2010

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“…39,44 The severity of symptoms and uncertainty regarding the diagnosis often lead to invasive procedures, including limb amputation, however with poor outcome. 44 Of note, seeking of limb amputation is only encountered extremely rarely in other dystonic conditions, 67,68 but has been reported in CRPS-I. [69][70][71] Thus, the possibility has been raised that patients with fixed dystonia and CRPS-I who seek limb amputation might have deficits in their body schema perception as part of the body identity integrity disorder spectrum.…”

Section: Functional Fixed Dystoniamentioning

confidence: 99%

The Phenomenology of Functional (Psychogenic) Dystonia

Ganos

Edwards

Bhatia

2014

Mov Disord Clin Pract

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From the very first descriptions of dystonia, there has been a lack of agreement on the differentiation of organic from functional (psychogenic) dystonia. This lack of agreement has had a significant effect on patients over the years, most particularly in the lack of access to appropriate management, whether for those with organic dystonia diagnosed as having a functional cause or vice versa. However, clinico‐genetic advances have led to greater certainty about the phenomenology of organic dystonia and therefore recognition of atypical forms. The diagnosis of functional dystonia rests on recognition of its phenomenology and should not be, as far as possible, a diagnosis of exclusion. Here, we present an overview of the phenomenology of functional dystonia, concentrating on the three main phenotypic presentations: functional cranial dystonia; functional fixed dystonia; and functional paroxysmal dystonia. We hope that this review of phenomenology will aid in the positive diagnosis of functional dystonia and, through this, will lead to more rapid access to appropriate management.

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“…The decision for amputation in a patient with this clinical history has been previously described. 10 This was based on many factors, including failure of all other treatments, disabling pain, and localized incapacitating posturing in an otherwise healthy, well-adjusted individual. This decision led to a rapid treatment, recovery, and resumption of all desired activities.…”

Section: Discussionmentioning

confidence: 99%

“…1,2 Trauma or closed head injury may result in dystonia. 10 Dystonia is the movement disorder most commonly associated with reflex sympathetic dystrophy. It also may occur with other neurologic diseases.…”

Section: Introductionmentioning

confidence: 99%

Adult-Onset Lower Extremity Focal Dystonia: Case Reports

Steel

2007

Foot Ankle Int.

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An aggressive approach to limb dystonia: A case report

Cited by 5 publications

References 15 publications

Hepatocyte NAD(P)H oxidases as an endogenous source of reactive oxygen species during hepatitis C virus infection

Hepatocyte NAD(P)H oxidases as an endogenous source of reactive oxygen species during hepatitis C virus infection

The Phenomenology of Functional (Psychogenic) Dystonia

Adult-Onset Lower Extremity Focal Dystonia: Case Reports

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