Magnetic resonance imaging findings in lupus ataxia

Lancman, Marcelo E.; Pomeraniec, C.; Norscini, Jorge

doi:10.1111/j.1600-0404.1992.tb05111.x

Cited by 9 publications

(8 citation statements)

References 11 publications

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“…Previous studies suggested that cerebral infarction and vasculopathy are possible pathogenic mechanisms involved in cerebellar ataxia in SLE patients. [4][5][6] In our patients, cerebral ischemia was less likely seen in patients #1 and #2 because of the absence of typical ischemic MRI abnormalities on initial and follow-up MRI. Although patient #3 had an ischemic cerebellar lesion, her MRI also showed slight contrast enhancement, suggesting blood-brain barrier rupture secondary to an underlying vasculitis.…”

Section: Discussionmentioning

confidence: 61%

“…Focal CNS manifestations secondary to cerebellar involvement has rarely been described in SLE. [2][3][4][5][6][7][8][9][10][11][12] The sudden onset of nystagmus and ataxia suggests the presence of cerebellar dysfunctions that may occur as an isolated presentation, associated with brainstem or more widespread CNS manifestations. [7][8][9] We report three SLE patients who presented in our clinic with acute cerebellar ataxia.…”

Section: Discussionmentioning

confidence: 99%

“…[2][3][4][5][6][7][8][9][10][11][12] Cerebral ischemia, vasogenic oedema and antibody-mediated cerebral dysfunction are possible etiologies of acute cerebellar ataxia related to SLE. [2][3][4][5][6][7][8][9][10][11][12] We report the clinical and radiological features of three patients who showed signs of acute cerebellar ataxia. A review of the literature was performed by documenting cases of cerebellar dysfunction in SLE and the importance of neuroimaging in the evaluation of these patients.…”

Section: Introductionmentioning

confidence: 99%

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Cerebellar ataxia in systemic lupus erythematosus

Appenzeller

Cendes

Costallat

2008

Lupus

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Neuropsychiatric manifestations are commonly observed in systemic lupus erythematosus (SLE) patients; however, cerebellar involvement has rarely been reported. In the presence of acute cerebellar ataxia, etiologies related (focal edema and ischemia) and not related (infections, malignancy and paraneoplastic syndromes) to lupus have to be considered and they imply different treatment strategies. We report the clinical and radiological features of 3 SLE patients who presented with acute cerebellar ataxia. A review of the literature was performed by documenting cases of cerebellar ataxia in SLE and the importance of neuroimaging in the evaluation of these patients.

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Section: Discussionmentioning

confidence: 61%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Cerebellar ataxia in systemic lupus erythematosus

Appenzeller

Cendes

Costallat

2008

Lupus

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“… 136 – 138 , 141 – 145 , 148 , 149 There was a single patient who had normal imaging studies, 140 and a single patient with T2 hyperintensity affecting the cerebellum. 146 Interestingly, there was 1 patient who had additional features of a limbic encephalopathy associated with an ataxia. 139 This patient presented with confusion and MRI showing FLAIR-hyperintense signals which affected the right medial temporal lobe, and also extended into the ipsilateral limbic pathways.…”

Section: Literature Reviewmentioning

confidence: 99%

Movement and Other Neurodegenerative Syndromes in Patients with Systemic Rheumatic Diseases

et al. 2015

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Patients with rheumatic diseases can present with movement and other neurodegenerative disorders. It may be underappreciated that movement and other neurodegenerative disorders can encompass a wide variety of disease entities. Such disorders are strikingly heterogeneous and lead to a wider spectrum of clinical injury than seen in Parkinson's disease. Therefore, we sought to stringently phenotype movement and other neurodegenerative disorders presenting in a case series of rheumatic disease patients. We integrated our findings with a review of the literature to understand mechanisms which may account for such a ubiquitous pattern of clinical injury.Seven rheumatic disease patients (5 Sjögren's syndrome patients, 2 undifferentiated connective tissue disease patients) were referred and could be misdiagnosed as having Parkinson's disease. However, all of these patients were ultimately diagnosed as having other movement or neurodegenerative disorders. Findings inconsistent with and more expansive than Parkinson's disease included cerebellar degeneration, dystonia with an alien-limb phenomenon, and nonfluent aphasias.A notable finding was that individual patients could be affected by cooccurring movement and other neurodegenerative disorders, each of which could be exceptionally rare (ie, prevalence of ∼1:1000), and therefore with the collective probability that such disorders were merely coincidental and causally unrelated being as low as ∼1-per-billion. Whereas our review of the literature revealed that ubiquitous patterns of clinical injury were frequently associated with magnetic resonance imaging (MRI) findings suggestive of a widespread vasculopathy, our patients did not have such neuroimaging findings. Instead, our patients could have syndromes which phenotypically resembled paraneoplastic and other inflammatory disorders which are known to be associated with antineuronal antibodies. We similarly identified immune-mediated and inflammatory markers of injury in a psoriatic arthritis patient who developed an amyotrophic lateral sclerosis (ALS)-plus syndrome after tumor necrosis factor (TNF)-inhibitor therapy.We have described a diverse spectrum of movement and other neurodegenerative disorders in our rheumatic disease patients. The widespread pattern of clinical injury, the propensity of our patients to present with co-occurring movement disorders, and the lack of MRI neuroimaging findings suggestive of a vasculopathy collectively suggest unique patterns of immune-mediated injury.

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“…Manto et al (1996) reported a pancerebellar syndrome of subacute progression in an SLE patient, and al‐Arfaj and Naddaf (1995) described a 40‐year‐old SLE female who presented with truncal ataxia and intention tremor. Lancman et al (1992) found bilateral cerebellar ischemic lesions on magnetic resonance imaging in a lupus patient with lateralization of gait and ataxia, and Tuchman et al (1983) reported cerebellar atrophy on neuroimaging in a patient that exhibited lupus ataxia. The actual incidence of cerebellar involvement in SLE is unknown.…”

Section: Introductionmentioning

confidence: 99%

Cerebellar dysfunction is associated with overexpression of proinflammatory cytokine genes in lupus

Tomita

Holman

Williams

et al. 2001

J of Neuroscience Research

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Systemic lupus erythematosus (SLE) is an autoimmune disease of unknown etiology accompanied by central nervous system involvement in up to 60% of patients. The current study chronicles the expression of cerebellar dysfunction in SLE using MRL-lpr/lpr mice as the experimental model. These mice spontaneously develop an illness that has immunological and clinical features of human lupus. We found that MRL-lpr/lpr mice manifest severe and progressive behavioral disturbances indicative of cerebellar dysfunction beginning at 11 weeks of age. Although the lpr gene is known to induce autoimmune features, immunologically normal mice rendered congenic for lpr failed to exhibit disturbances in cerebellar function. Because lupus is a cytokine-driven disease and overexpression of certain proinflammatory cytokines has been associated with neurodegeneration, the relationship between cerebellar dysfunction and cytokine gene expression was examined. Relative to immunologically normal CBA/J mice, the cerebellum of young (11-15 weeks of age) MRL-lpr/lpr mice contained high levels of interleukin (IL)-6 and interferon-gamma (IFNgamma) mRNA, which became even more pronounced in old (22-30 weeks of age) autoimmune mice. mRNA levels for the cytokines IL-1beta and IL-10 were elevated in the cerebellum of old, but not young, MRL-lpr/lpr mice relative to CBA/J. In contrast, the levels of cerebellar transcripts for IL-3 and tumor necrosis factor-alpha were comparable in autoimmune and normal mice, indicating that enhanced gene expression of IL-6, IFNgamma, IL-1beta, and IL-10 was selective. These results suggest a potential role for certain proinflammatory cytokines in the pathogenesis of cerebellar disturbances in SLE.

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Magnetic resonance imaging findings in lupus ataxia

Cited by 9 publications

References 11 publications

Cerebellar ataxia in systemic lupus erythematosus

Cerebellar ataxia in systemic lupus erythematosus

Movement and Other Neurodegenerative Syndromes in Patients with Systemic Rheumatic Diseases

Cerebellar dysfunction is associated with overexpression of proinflammatory cytokine genes in lupus

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