Rare case of diffuse large B-cell lymphoma presenting with central pontine myelinolysis

Central pontine myelinolysis (CPM) is commonly associated with osmotic stress and rapid correction of hyponatraemia. It has rarely been reported in conjunction with malignancies. We report a case where CPM was not only associated with a new diagnosis of diffuse large B-cell lymphoma but was also a key presenting feature.

A Review on Central Pontine Myelinolysis and Correction of Hyponatremia in Hospitalized Patients

Pontine myelinolysis (PM) can be a nerve disorder represented by pons demyelination. it is characterized by damage to regions of the brain, most commonly tracts pontine substantia alba, after rapid correction of metabolic disorders such as hyponatremia. PM (Pontine Myelinolysis) is categorized into Central pontine myelinolysis (CPM) and extra pontine myelinolysis (EPM). The various studies revealed that quick correction of hyponatremia plays a vital role in the pathogenesis of ODS. Prevention of ODS must be conducted by gradually increasing sodium concentration of 4–6 mmol/Lin in any 24-h period. PubMed and Medline literature search was done using CPM and hyponatremia as keywords. The principal aim of this review is to encapsulate, the recent evidence from literature about the association between rapid correction of hyponatremia and central pontine myelinolysis. Keywords: Demyelination syndrome, EPM, CPM, Serum tonicity, Hyponatremia, Demyelination

A Stepwise Multidisciplinary Rehabilitation Approach of Central Pontine and Extra Pontine Myelinosis in Locked-in Syndrome: A Case Report

Central Pontine Myelinosis (CPM) and Extra Pontine Myelinosis (EPM) are debilitating conditions, resulting from the rapid correction of hyponatremia. The outcome and prognosis of this medical condition are variable; mainly involving functional decline from neuro-musculoskeletal upset. An intensive, multidisciplinary rehabilitation has played an essential role in facilitating and hasten recovery, promoting a favorable outcome. We reported our experience in rehabilitating a patient with a rare condition of comorbid CPM and EPM. The reported patient presented with disabling Locked-in Syndrome (LIS) and Parkinsonism movement disorder. We highlighted the stepwise rehabilitation approach according to Patterson and Grabois Motor Recovery scale for LIS in preventing and reversing the complications and disabilities caused by CPM and EPM that may facilitate favorable therapeutic outcomes.

Combined heart–liver transplantation in a case of haemochromatosis

Hereditary haemochromatosis results in multiorgan dysfunction secondary to iron overload. Haemojuvelin (HJV)-associated haemochromatosis, is a rapidly progressing form of haemochromatosis caused by mutation in the HJV that frequently results in heart and liver failure. Herein, we describe the successful treatment of a 39-year-old woman with decompensated heart failure and liver cirrhosis requiring extracorporeal membrane oxygenation who was successfully treated with combined heart–liver transplantation. Following her life-saving multiorgan transplantation, she was also noted to have rapid correction of her serum ferritin to normal levels. She remains healthy with excellent allograft function and normal iron and ferratin levels 4 years after the procedure. To our knowledge, this case is the first demonstration that combined heart–liver transplantation is a feasible option for patients with heart and liver failure secondary to HJV-associated haemochromatosis and indeed offers a long-standing corrective solution to treat this condition and restore physiologically normal iron metabolism.

Clinical experience of the efficacy and safety of low dose tolvaptan therapy in a UK tertiary oncology setting

Background In patients with cancer, hyponatraemia is associated with increased morbidity and mortality and can delay systemic therapy. Methods The safety and efficacy of low-dose tolvaptan (7.5 mg) for hospitalized, adult patients with hyponatraemia due to Syndrome of Inappropriate Antidiuresis (SIAD), and co-existing malignancy were retrospectively evaluated in a tertiary cancer centre. Results Fifty-five patients with mean baseline serum sodium (sNa) 117.9±4.6 mmol/L were included. 90.9% had severe hyponatraemia (sNa<125 mmol/L). Mean age was 65.1±9.3 years. Following an initial dose of tolvaptan 7.5 mg, median (range) increase in sNa observed at 24 hours was 9(1-19) mmol/L. Within one week, 39 patients (70.9%) reached sNa≥130 mmol/L and 48 (87.3%) had sNa rise of ≥5 mmol/L within 48 hours. No severe adverse events were reported. Thirty-three (60%) and seventeen (30.9%) patients experienced sNa rise of ≥8 and ≥12 mmol/L/24hrs, respectively. The rate of sNa correction in the first 24 hours was significantly higher among participants that continued fluid restriction after tolvaptan administration (median[quantiles]: 14[9-16] versus 8[5-11] mmol/L, p=0.036). Moreover, in the over-rapid correction cohort (≥12 mmol/L/24hrs) demeclocycline was appropriately discontinued only in 60% compared to 91.7% of the remaining participants (P=0.047). Lower creatinine was predictive of higher sNa correction rate within 24 hours (p=0.01). Conclusion In the largest series to date, although low-dose tolvaptan was demonstrated to be effective in correcting hyponatraemia due to SIAD in cancer patients, a significant proportion experienced over-rapid correction. Concurrent administration of demeclocycline and/or fluid restriction must be avoided due to the increased risk of over-rapid correction.

BEZOLD JARISCH REFLEX DURING HALO-GRAVITY TRACTION: CASE REPORT

ABSTRACT Halo-gravity traction is an option that can be used in the treatment of severe spinal deformities. The author reports a complication not yet described in the literature in which rapid correction of the deformity triggered the Bezold Jarisch reflex. Level of evidence IV; Case Series.

Rare case of pancrelipase therapy-induced neutropaenia

A 61-year-old man was transferred to our facility from an outside hospital due to refractory neutropaenia of unknown aetiology. The patient presented to the referring hospital with a 5-day history of worsening diarrhoea and abdominal pain. Initial lab results at presentation showed severe neutropaenia with an absolute neutrophil count of 0. Investigations included a bone marrow biopsy which showed slightly hypocellular marrow with near absence of granulocytic precursors. A CT without contrast showed evidence of chronic pancreatitis and acute colitis. The patient’s neutropaenia persisted despite granulocyte colony-stimulating factor therapy. The patient was, thus, transferred to our facility for a higher level of care. At our facility, the patient had rapid correction of neutropaenia after discontinuation of pancrelipase therapy. The patient’s abdominal pain and diarrhoea also improved while off pancrelipase. Neutropaenia has completely resolved 6 weeks after discharge without any further therapy.

Osmotic Demyelination Syndrome after Primary Hypoadrenocorticism Crisis Management

Background: Primary hypoadrenocorticism is a rare condition resulting from immune-mediated destruction of the adrenal cortices. It can also occur due to necrosis, neoplasms, infarctions and granulomas. The clinical and laboratory changes are due to deficient secretion of glucocorticoids and mineralocorticoids, which leads to electrolyte disorders associated with hyponatremia and hyperkalemia. These disorders can cause hypotension, hypovolemia and shock, putting a patient's life at risk if inadequate hydroelectrolytic supplementation and hormone replacement is provided. Nevertheless, rapid sodium chloride supplementation is contraindicated due to the risk of central pontine myelinolysis induction. The present study aims to describe a thalamic osmotic demyelination syndrome after management of a primary hypoadrenocorticism crisis in a 2-year-old, female West White Highland Terrier. Case: The patient had a presumptive diagnosis of hypoadrenocorticism already receiving oral prednisolone and gastrointestinal protectants in the last 2 days. After prednisolone dose reduction the dog presented a severe primary hypoadrenocorticism crisis treated with intravenous sodium chloride 0.9% solution along with supportive therapy. Four days after being discharged from the hospital, the patient showed severe neurological impairment and went back to the clinic where a neurological examination revealed mental depression, drowsiness, ambulatory tetraparesis and proprioceptive deficit of the 4 limbs, postural deficits, and cranial nerves with decreased response. Due to these clinical signs, a magnetic resonance imaging was performed. It showed 2 intra-axial circular lesions, symmetrically distributed in both thalamus sides, with approximately 0.8 cm in diameter each without any other anatomical changes on magnetic resonance imaging. The images were compatible with metabolic lesions, suggesting demyelination. Furthermore, liquor analysis did not show relevant abnormalities, except for a slight increase in density and pH at the upper limit of the reference range. After treatment, the patient had a good neurological evolution secondary to standard primary hypoadrenocorticism treatment, without sequelae. Discussion: In the present case report, primary hypoadrenocorticism gastrointestinal signs seemed to be triggered by a food indiscretion episode, not responsive to the symptomatic therapies employed. The patient´s breed and age (young West White Highland Terrier bitch) is in accordance with the demographic profile of patients affected by the disease, where young females are frequently more affected. Regarding the probable thalamic osmotic demyelination syndrome documented in this case, is important to notice that myelinolysis or demyelination is an exceedingly rare noninflammatory neurological disorder, initially called central pontine myelinolysis, which can occur after rapid correction of hyponatremia. It has already been observed in dogs after correction of hyponatremia of different origins, including hypoadrenocorticism and parasitic gastrointestinal disorders. Currently, the terms "osmotic myelinolysis" or “osmotic demyelination syndrome" are considered more suitable when compared to the term "central pontine myelinolysis" since it has been demonstrated in dogs and humans the occurrence of demyelination secondary to the rapid correction of hyponatremia in distinct regions of the central nervous system including pons, basal nuclei, striatum, thalamus, cortex, hippocampus and cerebellum. The present case report emphasizes the difficulties for hormonal confirmation of primary hypoadrenocorticism in a patient already on corticosteroid treatment, as well as proposes that the current term osmotic demyelination syndrome replace the term “central pontine myelinolysis” in veterinary literature related to the management of hypoadrenocorticism crisis.Keywords: Addison Syndrome, hyponatremia, osmotic myelinolysis, magnetic resonance imaging.