Progressive Neurodegeneration in Patients with Primary Immunodeficiency Disease on IVIG Treatment

Ziegner, Ulrike; Kobayashi, Roger H.; Cunningham‐Rundles, Charlotte; Español, Teresa; Fasth, Anders; Huttenlocher, Anna; Krogstad, Paul; Marthinsen, Lars; Notarangelo, Luigi D.; Pašić, Srdjan; Rieger, Christian; Rudge, Peter; Sankar, Raman; Shigeoka, Ann O.; Stiehm, E. Richard; Sullivan, Kathleen E.; Webster, Alison; Ochs, Hans D.

doi:10.1006/clim.2001.5140

Cited by 62 publications

(54 citation statements)

References 9 publications

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“…Atrophy assessment for PID disease Accepting the fact that the small sample size requires cautious interpretation, the proposed ISS detected both presence and degree of regional GM, WM, and CSF changes in patients with PID. We detected a loss of brain parenchyma in patients affected by PID, which was expressed by a statistically significant reduction of fractional GM volumes (51.9±2.3 vs 55.2±2.3, p=0.008) paralleled by a significant increase in CSF volumes (CSF 0.24±0.09 vs 0.18±0.04 p=0.006), without changes in TIV, thus confirming the observations of Ziegner et al in their pilot study [9].…”

Section: Results On Brain Quantification and Atrophysupporting

confidence: 90%

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Accurate and fast 3D interactive segmentation system applied to MR brain quantification

Signoroni¹,

Carletti²,

Grazioli³

et al. 2008

4th IET International Conference on Advances in Medical, Signal and Information Processing (MEDSIP 2008)

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This work presents an efficient interactive segmentation system for volumetric data-sets based on advanced 3D morphological analyses and an interaction paradigm that allows a good match with user intentions. This system has been designed to produce accurate results under the complete control of the user, to minimize the interaction time and to address a generality of 3D segmentation tasks. The system has been tested and compared with other softwares on normal MR brain structure quantification and on a challenging clinical setting pointed to the detection of the presence of subtle brain atrophy associated to primitive immunodeficiency (PID).

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Section: Results On Brain Quantification and Atrophysupporting

confidence: 90%

“…A recent study reported progressive neuro-degeneration in patients affected by primary immunodeficiency disease (PID) [9]. The aetiology of this syndrome is unknown.…”

Section: Results On Brain Quantification and Atrophymentioning

confidence: 99%

Accurate and fast 3D interactive segmentation system applied to MR brain quantification

Signoroni¹,

Carletti²,

Grazioli³

et al. 2008

4th IET International Conference on Advances in Medical, Signal and Information Processing (MEDSIP 2008)

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“…Lastly, in 2002, Ziegner et al reported several cases of progressive neurodegeneration in patients with primary immunodeficiency who were receiving immunoglobulin replacement therapy [20]. It was speculated whether use of IVIg might be playing a role in these cases.…”

Section: Fig 2 Ucsf Metagenomic Deep Sequencing Protocol For Identifmentioning

confidence: 99%

Neurologic Complications of Common Variable Immunodeficiency

et al. 2016

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Common variable immunodeficiency is a rare disorder of immunity associated with a myriad of clinical manifestations including recurrent infections, autoimmunity, and malignancy. Though rare, neurologic complications have been described in a small number of case reports and case series of CVID patients. In this article, we present a patient with CVID who suffered significant neurologic morbidity and categorize the reported range of neurologic complications associated with CVID. Our case highlights the complex nature of neurologic manifestations in CVID patients, and our review of the current database suggests that infection and inflammatory neurologic disorders are the cause of most neurologic presentations.

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“…The most important late side effects are acute renal failure and thromboembolic events [118]. Other late adverse events include aseptic meningitis, stroke [119], progressive neurodegeneration [120], neutropenia, autoimmune haemolytic…”

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confidence: 99%

Immunoglobulin treatment in primary antibody deficiency

Maarschalk-Ellerbroek

Hoepelman

Ellerbroek

2011

International Journal of Antimicrobial Agents

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The primary antibody deficiency syndromes are characterised by recurrent respiratory tract infections and the inability to produce effective immunoglobulin (Ig) responses. The best known primary antibody deficiencies are common variable immunodeficiency (CVID), X-linked agammaglobulinaemia (XLA), immunoglobulin G (IgG) subclass deficiency, and selective antibody deficiency with normal immunoglobulins (SADNI).Therapy in these patients consists of prophylactic antibiotics and/or Ig replacement therapy. Diagnostic delay remains common owing to limited awareness of the presenting features and may result in increased morbidity and mortality. Replacement therapy with immunoglobulins increases life expectancy and reduces the frequency and severity of infections, but the effect on end-organ damage is still unknown. Both intravenous immunoglobulin (IVIg) and subcutaneous immunoglobulin (SCIg) treatment appear to be safe, with comparable efficacy. A starting dose of 300-400 mg/kg/month in IVIg and 100 mg/week for SCIg is recommended. IgG trough levels should be >5 g/L for patients with agammaglobulinaemia and 3 g/L greater than the initial IgG level for patients with CVID; however, the clinical response should be foremost in choosing the dose and trough level. Infusion-related adverse reactions are generally mild owing to improved manufacturing processes. In this paper, aspects of Ig replacement therapy in primary antibody-deficient patients will be addressed.

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Progressive Neurodegeneration in Patients with Primary Immunodeficiency Disease on IVIG Treatment

Cited by 62 publications

References 9 publications

Accurate and fast 3D interactive segmentation system applied to MR brain quantification

Accurate and fast 3D interactive segmentation system applied to MR brain quantification

Neurologic Complications of Common Variable Immunodeficiency

Immunoglobulin treatment in primary antibody deficiency

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