Severe myxedema coma and pericardial effusion in a child with Down syndrome: the importance of adherence to levothyroxine therapy.

BACKGROUND: Myxedema coma is a rare, but life-threatening endocrinological emergency. Myxedema is characterized by altered mental status, and is accompanied by hypotension, bradycardia, hypothermia, bradypnea, hyporeflexia, hyponatremia, and hypoglycemia, all stemming from reduced metabolism due to severe hypothyroidism. Additionally, patients may exhibit signs of low cardiac output, edema in the extremities, peripheral circulatory disturbances, shock, and the development of pericardial and pleural effusions, ultimately leading to confusion and coma. We present a successfully treated case of severe myxedema coma with recurrent pericardial effusion and hypotensive shock. This case is characterized by an unusual clinical presentation and required a distinct treatment strategy highlighting its exceptional rarity. CASE: A 2-year-old boy with Down syndrome presented with recurrent pericardial effusion attributed to medication non-adherence. The critically-ill patient, experiencing a severe cardiogenic shock required mechanical ventilation and inotropic infusions in the pediatric intensive care unit. Elevated thyroid stimulating hormone (TSH), and low free T4 (fT4) and free T3 (fT3) levels prompted consideration of myxedema coma. Upon reviewing the patient's medical history, it was ascertained that he had an ongoing diagnosis of primary hypothyroidism, and exhibited non-adherence to the prescribed treatment regimen and failed to attend scheduled outpatient clinic appointments for follow-up assessments. The treatment plan, devised by the pediatric endocrinology team, included the peroral administration of L-thyroxine (L-T4) at a dose of 50 micrograms per day. After beginning regular oral L-T4 treatment, a gradual improvement in the patient's condition was observed. Notably, by the 15th day of oral therapy, the patient had made a full recovery. Contrary to the recommended intravenous treatment for myxedema coma, this patient was successfully treated with oral levothyroxine, due to the unavailability of the parenteral form in Türkiye. CONCLUSIONS: This case report presents an instance of non-adherence to L-T4 therapy, which subsequently progressed to severe myxedema coma. Changes in neurologic status and hemodynamic instability in a patient with a history of hypothyroidism should raise the concern of nonadherence and, though rare, myxedema coma should be in the differential diagnosis.

Tofacitinib Treatment for Pretibial Myxedema.

This case report describes tofacitinib treatment for 2 patients with pretibial myxedema.

Life-Threatening Cardiac Arrhythmias in a Case of Undetected Myxedema Coma: Importance of Early Detection and Medication Adherence.

BACKGROUND Myxedema coma is a rare, life-threatening condition caused by a severe form of hypothyroidism. The dangerously low levels of circulating thyroid hormone can lead to progressive mental status changes and numerous organ dysfunctions, including serious cardiac abnormalities. CASE REPORT We present a case of a 59-year-old woman who presented with altered mental status and fall who was originally thought to have a cerebrovascular accident but was later diagnosed with myxedema coma, after multiple cardiac arrests. It was discovered that the patient had not been taking any of her medications for the last several weeks, after her primary care provider retired from practice. Initial laboratory evaluation was significant for a TSH level of 159.419 mIU/L and an undetectable free T4 level. Complications of the myxedema coma resulted in QTC interval prolongation, causing torsades de pointes and sustained polymorphic ventricular tachycardia, requiring cardioversion. CONCLUSIONS This case demonstrates the importance of early detection and treatment of myxedema coma, as it can cause life-threatening cardiac arrhythmias. It also emphasizes the need to ensure proper medication adherence in patients with chronic medical conditions, as non-compliance can result in dire consequences.

[An integrated approach to the treatment of pretibial myxedema based on pulse therapy with prednisolone and electrical neuromyostimulation (FREMS-therapy) in a patient with Graves' disease and thyroid eye disease].

Graves' disease (GD) is one of the urgent problems of modern endocrinology, characterized by a high frequency, polysystemic damage to the body, a steadily progressive course, diagnostic difficulties, a high degree of disability and often resistance to therapy. The manifestations of the disease include: thyrotoxicosis syndrome with impaired lipid and carbohydrate metabolism, and activation of multiple organ pathology in the form of thyroid eye disease (TED), pretibial myxedema, cardiovascular insufficiency, acropathy, lesions of the nervous, osteoarticular system, and other lesions. The development of multiple organ pathology can have a different sequence, different time intervals and different degrees of severity. Any developments in the direction of clarifying the etiopathogenetic, clinical diagnostic and treatment-rehabilitation measures are of undoubted significance. We present a clinical case of GD, TED and pretibial myxedema, in which an integrated approach was tested in the tactics of treating pretibial myxedema (a combination of pulse therapy with prednisolone and FREMS-therapy), as a result of which positive results were obtained within a short time.

Liquid levothyroxine in the treatment of myxoedema coma.

Not required for Clinical Vignette.

Improvement of Pretibial Myxedema Following Administration of Teprotumumab.

Pretibial myxedema (PTM) is a rare complication of Graves' disease. It is characterized by non-pitting edema with hyperpigmented hyperkeratotic papules and plaques on bilateral lower legs. Effective treatments for patients with PTM are lacking. The etiology of PTM is unknown; however, it may be similar to the mechanism of thyroid-associated ophthalmopathy (TAO). Activated fibroblasts produce inflammatory cytokines and synthesize excessive glycosaminoglycans (GAG) that accumulate in the dermis and subcutaneous tissue. A recent, novel pathway implicates IGF-1 receptor as a mediator in this process. We present two patients with refractory PTM that improved following treatment with teprotumumab, an IGF-1 receptor inhibitor approved for use in TAO. J Drugs Dermatol. 2022;21(11):1252-1254. doi:10.36849/JDD.6854.

Sustained remission of psychotic symptoms secondary to hypothyroidism (myxedema psychosis) after 6 months of treatment primarily with levothyroxine: a case report.

BACKGROUND: Psychotic symptoms associated with hypothyroidism, also known as "myxedema psychosis," are a treatable cause of psychosis often associated with complete recovery. While most cases receive both thyroxine and a short course of antipsychotics, some reports indicate that symptoms can resolve without antipsychotic treatment, though follow-up in these cases has often been short or not reported. This is one of the first case reports demonstrating sustained remission of psychotic symptoms at 6 months in a case of myxedema psychosis treated with minimal antipsychotic medication. CASE PRESENTATION: We describe the case of a 40-year-old Caucasian woman who was brought to hospital with a 7-day history of anxiety and decreased sleep and 1 day of disorganized speech, paranoid delusions, and auditory hallucinations. After being admitted to psychiatry for management, screening blood work revealed elevated thyroid-stimulating hormone. The patient was initiated on treatment with levothyroxine and low doses of antipsychotics. Her symptoms resolved on the third day of her admission with ongoing symptomatic remission at 6 months follow-up. CONCLUSIONS: The identification of myxedema psychosis is important owing to the implications on treatment and prognosis of the disorder. Our case suggests that sustained symptom resolution may occur with little to no antipsychotic treatment, though these findings are preliminary and additional study is needed before definitive conclusions on the optimal approach can be made.

Central hypothyroidism with myxoedema: a less known but clinically challenging presentation.

Myxoedema can have a variety of presentations, from mild cognitive impairment to psychosis, to overt coma. While majority of cases have primary hypothyroidism as the underlying aetiology, very few cases have central hypothyroidism. We report two patients who presented with myxoedema and were diagnosed with central hypothyroidism. A man in his 50s with a history of panhypopituitarism presented with hypotension, slurring of speech and psychosis that worsened to coma. He was initially treated as adrenal crisis, and on failing to improve he was later treated correctly as myxoedema coma. A woman in her 30s presented with bradykinesia and shock and was diagnosed with Sheehan's syndrome based on hormonal and imaging features. Both patients improved with a loading dose of oral thyroxine and intravenous steroids. Central hypothyroidism presenting with myxoedema is often complicated by coexisting pituitary hormone deficiencies. A high index of suspicion is required for better treatment outcomes.

Myxedema coma: Recognition and treatment.

ABSTRACT: Myxedema coma (MC) develops from a long-standing, unrecognized, or untreated hypothyroidism. This article discusses the pathophysiology, clinical manifestations, treatment, and nursing considerations for patients with MC.

A case of fatal myxedema coma with electrocardiogram Osborne J-wave in a patient initially diagnosed with hypothyroidism.

Myxedema coma is a life-threatening endocrine emergency with a high mortality rate resulting from severe insufficiency of thyroid hormones. Intravenous levothyroxine replacement is considered the standard therapy for myxedema coma in many countries. In Japan, however, although there are diagnostic criteria highly suggestive or diagnostic for myxedema coma, no management strategy has been established, despite the availability of levothyroxine. Here we report a 75-year-old man with a history of Alzheimer's disease and schizophrenia who developed somnolence and generalized edema. Except for a pulse rate of 60 bpm, his vital signs and blood oxygen level were stable. Thyroid studies showed an elevated serum thyrotropin level of 219.2 µU/mL and a decreased serum free-thyroxine level of 0.15 ng/dL. On this basis he was diagnosed as having hypothyroidism rather than being highly suggestive for myxedema coma. Daily oral levothyroxine 25 µg was initiated and increased to 50 µg 3 days later. Seven days after being started on levothyroxine, the patient suddenly developed impaired consciousness, hypoxemia, hypotension, hypothermia, and hyponatremia. Electrocardiography revealed junctional bradycardia with Osborne J-wave. Myxedema coma was therefore diagnosed. He went into cardiac arrest in the emergency room but was resuscitated. Despite subsequent intravenous administration of hydrocortisone and levothyroxine, as well as intensive supportive care, he eventually died 12 hours after hospital admission. This case illustrates some of the challenges associated with the management of patients with signs highly suggestive/diagnostic of myxedema coma, including the optimal loading dosage and intervention timing of thyroid hormone replacement.

Case Report: Myxedema Coma Caused by Immunoglobulin A Vasculitis in a Patient With Severe Hypothyroidism.

Myxedema coma is a critical disorder with high mortality rates. Disruption of the compensatory mechanism for severe and long-term hypothyroidism by various causes leads to critical conditions, including hypothermia, respiratory failure, circulatory failure, and central nervous system dysfunction. Infectious diseases, stroke, myocardial infarction, sedative drugs, and cold exposure are considered the main triggers for myxedema coma. A 59-year-old Japanese woman presented with bilateral painful purpura on her lower legs. She was diagnosed with coexisting immunoglobulin A (IgA) vasculitis and severe IgA vasculitis with nephritis and was consequently treated with intravenous methylprednisolone (125 mg/day). However, she rapidly developed multiple organ failure due to the exacerbation of severe hypothyroidism, i.e., myxedema. Her condition improved significantly following oral administration of prednisolone along with thyroxine. There was a delayed increase in the serum free triiodothyronine level, while the serum free thyroxine level was quickly restored to normal. Rapid deterioration of the patient's condition after admission led us to diagnose her as having myxedema coma triggered by IgA vasculitis. Hence, clinicians should be aware of the risks of dynamic exacerbations in patients with hypothyroidism. Furthermore, our study suggested that combination therapy with thyroxine and liothyronine might prove effective for patients with myxedema coma, especially for those who require high-dose glucocorticoid administration.

Myxedema Coma Precipitated by Iohexol: Case Report and Brief Literature Review on the Wolff-Chaikoff Effect.

Background: Failure to escape from the Wolff-Chaikoff effect (WCE) causes hypothyroidism. Methods: This is the first report of myxedema coma after iohexol administration. The failure of the escape phenomenon in this patient was longer than existing reports. Results: The patient received 42,000 mg of iodine in iohexol cumulatively and developed myxedema coma after 16 days. She was subsequently found to have pre-existing primary hypothyroidism that was treated with levothyroxine 50 µg daily, but had defaulted treatment. She was discharged with levothyroxine 100 µg daily and this was weaned to 50 µg daily over 12 months. Conclusions: Iodine-based contrast media (ICM) can aggravate primary hypothyroidism. In severe cases, it may precipitate myxedema coma. Patients with thyroid disorders should be informed to monitor for aggravation of their symptoms after ICM administration. Long-term follow-up of thyroid function may be needed in patients who fail to escape from the WCE.

Myxedema crisis and ovarian hyperstimulation in a child with Down syndrome.

OBJECTIVES: Myxedema crisis, a fatal complication of severe hypothyroidism, is extremely rare in children and treatment guidelines are lacking. Since availability of intravenous levothyroxine is limited in resource poor settings, myxedema crisis can be treated with oral levothyroxine and/or oral liothyronine (if necessary), in the absence of cardiac risk factors, thus hastening the recovery and significantly decreasing the associated morbidity and mortality. In the background of untreated hypothyroidism, a possible association of ovarian hyperstimulation syndrome (OHSS) and reactive pituitary hyperplasia should be kept in mind, thus preventing unnecessary interventions. CASE PRESENTATION: A 13-year-old girl child with Down syndrome, presented with myxedema crisis, as initial presentation of untreated hypothyroidism. CONCLUSIONS: Annual screening, timely diagnosis of hypothyroidism, and early initiation of thyroid hormone supplementation will prevent associated physical and neurocognitive morbidity in children, especially those with Down syndrome. Importance of oral liothyronine supplementation in myxedema crisis, has been highlighted in this case report.

Bilateral breast myxedema caused by Graves' disease and responsive to multipoint subcutaneous injection of long-acting glucocorticoid: Case report.

RATIONALE: With the absence of ophthalmopathy, thyroid dermopathy especially lesions at atypical locations is a very rare presentation. We herein report an original case of bilateral breast myxedema caused by Grave's disease. PATIENT CONCERNS: A 21-year-old unmarried woman presented with a 4-month history of Grave's disease and a 1-month history of progressive bilateral breast enlargement. She had symmetrical bilateral breast enlargement with redness and nonpitting thickening of the skin, diffusely enlarged thyroid glands, and no exophthalmos. DIAGNOSIS: Ultrasonography, magnetic resonance imaging scan, and skin biopsy confirmed the diagnosis of bilateral breast myxedema. INTERVENTIONS: The patient was treated with multipoint subcutaneous injections of triamcinolone acetonide in each breast every month. OUTCOMES: The bilateral breast returned approximately to its normal size after therapy for 6 months. CONCLUSIONS: Our case illustrates that multipoint subcutaneous injection of glucocorticoids is beneficial for bilateral breast myxedema.

Laryngeal myxoedema: a literature review of an uncommon complication of hypothyroidism.

A 59-year-old woman presented to the emergency department with shortness of breath. She had significant oropharyngeal swelling obstructing her upper respiratory tract. A diagnosis of laryngeal myxoedema was made, which was attributed to severe hypothyroidism. She required invasive ventilation and subsequently a tracheostomy. She was treated with levothyroxine and hydrocortisone. She completely recovered with this therapy. In this review, we will discuss similar cases and different therapeutic options. This case also highlights the fact that establishing a diagnosis of laryngeal myxoedema, a condition that can potentially have lethal outcomes and can be challenging.