Objective. To describe the epidemiologic, clinical, neuroimaging, and laboratory features; treatment; and outcome in a cohort of children with acute disseminated encephalomyelitis (ADEM). Methods. A 6-year retrospective chart review of children with the diagnosis of ADEM was conducted. Results. Eighteen cases were identified. Sixteen patients (88%) presented in either winter or spring. Thirteen children (72%) had a recent upper respiratory tract illness. Patients presented most often with motor deficits (77%) and secondly with altered consciousness (45%). Spinal fluid abnormalities occurred in 70%. Despite rigorous microbiologic testing, a definite microbiologic diagnosis was established only in 1 child with Epstein-Barr virus disease and probable or possible diagnoses in 3 children with Bartonella henselae, Mycoplasma pneumoniae, or rotavirus disease. Brain magnetic resonance imaging identified lesions in the cerebral cortex in 80%, in subcortical white matter in 93%, in periventricular white matter in 60%, in deep gray matter in 47%, and in brainstem in 47% of patients. Eleven patients (61%) were treated with corticosteroids, and 2 were treated with intravenous immunoglobulins. All patients survived. Three patients (17%) had long-term neurologic sequelae. Conclusions. Epidemiologic evidence from this study suggests an infectious cause for ADEM. The agent is most likely a difficult-to-diagnose winter/spring respiratory virus. Magnetic resonance imaging was the neuroimaging study of choice for establishing the diagnosis and for following the course of the disease. Prognosis for survival and outcome was excellent. Recurrent episodes of ADEM must be differentiated from multiple sclerosis.

https://pediatrics.aappublications.org/content/pediatrics/110/2/e21.full.pdf

Acute Disseminated Encephalomyelitis in Children

Post-infectious or post-vaccinal demyelinating encephalomyelitis and neuritis may be due to immunological cross-reactions evoked by specific viral antigenic determinants (epitopes) that are homologous to regions in the target myelins of the central and peripheral nervous systems. Such homologies have been found by computer searches in which decapeptides in two human myelin proteins were compared with proteins of viruses known to infect humans. These viruses include measles, Epstein-Barr, influenza A and B, and others that cause upper respiratory infections. Several regions identified in myelin basic protein and P2 protein can be related to experimental allergic encephalomyelitis or neuritis in laboratory animals.

https://science.sciencemag.org/content/sci/229/4710/282.full.pdf

Sequence homology between certain viral proteins and proteins related to encephalomyelitis and neuritis.

Massive periarticular calcinosis of the soft tissues is a unique but not rare radiographic finding. On the contrary, tumoral calcinosis is a rare familial disease. Unfortunately, the term tumoral calcinosis has been liberally and imprecisely used to describe any massive collection of periarticular calcification, although this term actually refers to a hereditary condition associated with massive periarticular calcification. The inconsistent use of this term has created confusion throughout the literature. More important, if the radiologist is unfamiliar with tumoral calcinosis or disease processes that mimic this condition, then diagnosis could be impeded, treatment could be delayed, and undue alarm could be raised, possibly leading to unwarranted surgical procedures. The soft-tissue lesions of tumoral calcinosis are typically lobulated, well-demarcated calcifications that are most often distributed along the extensor surfaces of large joints. There are many conditions with similar appearances, including the calcinosis of chronic renal failure, calcinosis universalis, calcinosis circumscripta, calcific tendonitis, synovial osteochondromatosis, synovial sarcoma, osteosarcoma, myositis ossificans, tophaceous gout, and calcific myonecrosis. The radiologist plays a critical role in avoiding unnecessary invasive procedures and in guiding the selection of appropriate tests that can result in a conclusive diagnosis of tumoral calcinosis.

https://pubs.rsna.org/doi/pdf/10.1148/rg.263055099

Tumoral calcinosis: pearls, polemics, and alternative possibilities.

Mechanism of ethanol induced hepatic injury.

Regulation of monocyte chemoattractant protein-1 expression in adult human non-neoplastic astrocytes is sensitive to tumor necrosis factor (TNF) or antibody to the 55-kDa TNF receptor

Excerpt Nonketotic hyperosmolar coma is a syndrome found mostly in diabetic patients with marked hyperglycemia; the relative pathogenetic roles of hyperglycemia, hyperosmolality, and cellular (espe...

Hyperosmolar Coma: Cellular Dehydration and the Serum Sodium Concentration

Focal encephalomyelitis in infectious mononucleosis. A report with pathological description.

tumors. Another form of tumoral calcinosis is seen in patients without advanced renal failure, is familial, and is thought to result from a defect in phosphate handling in the proximal tubules (6). We present a case report of a patient who was not hyperparathyroid, yet devel o_ massive soft-tissue calcifications three years after the onset of hemodialysis. The calcifications were as sociated with aplastic bone disease and severe hypercal cemia. These soft-tissue calcifications, highly metabol ically active on bone scintigraphy, combined with the aplastic bone disease on bone biopsy, imply that the increased calcium turnover from the soft tissues and the inability of the aplastic bone to incorporate the excess calcium may have been a potential step in the pathogenesis of hypercaicemia in this patient. We present a case of a 58-yr-old male to illustrate the scintigraphic, roentgenographic, clinical,and pathologic features of penarticulartumoralcalcinosisthat occurred in a hemodialysispatient.Soft-tissuecalcificationsdeveloped 3 yr after onset of hemodialysis,became progressively larger during the ensuing five years, and culminated in voluntarywithdrawalfrom dialysisbecauseof the extreme discomfort and lack of mobility that resulted from the calcinosis. Histologically, an aplastic disorder was present withverylowboneformation. Onbonescintigraphy, in tensecalciumuptakein soft tissuesimpliedthat it was metabolicallyactive. We hypothesizethat this high meta bolic activity contributed to the persistent hypercalcemia observed during the patient's last year of life.

http://jnm.snmjournals.org/content/31/6/1099.full.pdf

Periarticular tumoral calcinosis and hypercalcemia in a hemodialysis patient without hyperparathyroidism: a case report.

Acid-base disorders in hyperglycemia of insulin-dependent diabetic patients on chronic dialysis

https://www.ajkd.org/article/S0272-6386(01)78945-7/pdf

Antonios H. Tzamaloukas

Papers

Hyperosmolar Coma: Cellular Dehydration and the Serum Sodium Concentration

Focal encephalomyelitis in infectious mononucleosis. A report with pathological description.

Periarticular tumoral calcinosis and hypercalcemia in a hemodialysis patient without hyperparathyroidism: a case report.

Acid-base disorders in hyperglycemia of insulin-dependent diabetic patients on chronic dialysis

Creatinine excretion in continuous peritoneal dialysis: A systematic error of the Cockroft-Gault formula