Marchiafava-Bignami disease with haemophagocytic lymphohistiocytosis as a postoperative complication of cardiac surgery

Takei, Kentarou; Motoyoshi, Naotaka; Sakamoto, Kazuhiro; Kitamoto, Tetsuyuki

doi:10.1136/bcr-2019-230368

Cited by 3 publications

(1 citation statement)

References 12 publications

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“…We thought that this finding could be associated with B12 hypovitaminosis. e combination of cerebral and spinal cord involvement has only been reported in two previous MBD cases [3,18]. Histopathological findings of MBD showed demyelination (particularly of corpus callosum central fibers) with myelin breakdown products, foamy macrophages, perivascular lymphocytes, gliosis, and white matter necrosis [19,20].…”

Section: Discussionmentioning

confidence: 99%

Marchiafava–Bignami Disease Associated with Spinal Involvement

Perea¹,

Luis²,

Lázaro³

et al. 2020

Case Reports in Neurological Medicine

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Marchiafava–Bignami disease (MBD) is a rare disorder of unknown etiology, strongly associated with alcoholism and malnutrition. MBD causes primary involvement of the corpus callosum, leading to confusion, dysarthria, seizures, and frequent death. We report the case of a 54-year-old male without a history of alcoholism or known malabsorption disease, who presented with altered consciousness and neurologic impairment. Complex B deficiency was addressed. Magnetic resonance imaging (MRI) showed typical corpus callosum lesions. The clinical features and radiologic images suggested spinal cord involvement. Brain histopathologic findings were consistent with MBD. Despite vitamin replacement therapy, he had a poor outcome.

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Section: Discussionmentioning

confidence: 99%

Marchiafava–Bignami Disease Associated with Spinal Involvement

Perea¹,

Luis²,

Lázaro³

et al. 2020

Case Reports in Neurological Medicine

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Retrospective analysis of 21 cases of Marchiafava-Bignami disease in alcoholic in Southwest China

Wang

et al. 2023

Preprint

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Marchiafava-Bignami disease (MBD) is a rare disease with only a few reports worldwide. To describe clinical features and identify difficulties in the treatment of alcohol-related MBD, we performed a retrospective study of 21 MBD inpatients at a hospital in Southwest China. The interquartile range (IQR) for age was 53-66, with a mean age of 59 years. The IQR for drinking duration was 30-40, with a mean drinking length of 35.5 years. Cognitive impairment and unconsciousness were the most common symptoms (n=13, [61.9%]). Consciousness disorders, delirium, irritability, and ataxia are more prevalent in type A MBD patients; seizures, cognitive impairment, and limb weakness are more common in type B MBD patients. Routine blood and biochemical tests in alcoholic MBD patients may demonstrate orthocytic hypochromic anaemia and impaired liver function. Patients' uric acid (UA), potassium, and sodium levels may be normal. Eight patients received thiamine, while one received steroids. The average stay was 15 days. At the time of discharge, there had been no deaths. One patient died six months after being discharged, while another died two years afterwards. Indeed, MBD is a rare alcohol-related disorder with a variety of clinical symptoms. With thiamine treatment, prognosis is positive.

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The Multifaceted Immunology of Cytokine Storm Syndrome

Lee

Cron

2023

The Journal of Immunology

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Cytokine storm syndromes (CSSs) are potentially fatal hyperinflammatory states that share the underpinnings of persistent immune cell activation and uninhibited cytokine production. CSSs can be genetically determined by inborn errors of immunity (i.e., familial hemophagocytic lymphohistiocytosis) or develop as a complication of infections, chronic inflammatory diseases (e.g., Still disease), or malignancies (e.g., T cell lymphoma). Therapeutic interventions that activate the immune system such as chimeric Ag receptor T cell therapy and immune checkpoint inhibition can also trigger CSSs in the setting of cancer treatment. In this review, the biology of different types of CSSs is explored, and the current knowledge on the involvement of immune pathways and the contribution of host genetics is discussed. The use of animal models to study CSSs is reviewed, and their relevance for human diseases is discussed. Lastly, treatment approaches for CSSs are discussed with a focus on interventions that target immune cells and cytokines.

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Marchiafava-Bignami disease with haemophagocytic lymphohistiocytosis as a postoperative complication of cardiac surgery

Cited by 3 publications

References 12 publications

Marchiafava–Bignami Disease Associated with Spinal Involvement

Marchiafava–Bignami Disease Associated with Spinal Involvement

Retrospective analysis of 21 cases of Marchiafava-Bignami disease in alcoholic in Southwest China

The Multifaceted Immunology of Cytokine Storm Syndrome

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