Fatal outcome in a Hispanic woman with moyamoya syndrome and Graves’ disease

Summary We describe the case of a young Hispanic female who presented with thyrotoxicosis with seizures and ischemic stroke. She was diagnosed with a rare vasculopathy – moyamoya syndrome. After starting antithyroid therapy, her neurologic symptoms did not improve. Acute neurosurgical intervention had relieved her symptoms in the immediate post-operative period after re-anastomosis surgery. However, 2 post-operative days later, she was found to be in status epilepticus and in hyperthyroid state. She quickly deteriorated clinically and had expired a few days afterward. This is the second case in literature of a fatality in a patient with moyamoya syndrome and Graves’ disease. However, unlike the other case report, our patient had undergone successful revascularization surgery. We believe her underlying non-euthyroid state had potentiated her clinical deterioration. Case studies have shown positive correlation between uncontrolled hyperthyroidism and stroke-like symptoms in moyamoya syndrome. Mostly all patients with these two disease processes become symptomatic in marked hyperthyroid states. Thus, it may be either fluctuations in baseline thyroid function or thyrotoxicosis that potentiate otherwise asymptomatic moyamoya vasculopathy. Learning points: Awareness of the association between Graves’ disease and moyamoya syndrome in younger patients presenting with stroke-like symptoms. Obtaining euthyroid states before undergoing revascularization surgery may protect the patient from perioperative mortality and morbidity. Although moyamoya disease is usually thought to be genetically associated, there are reports that thyroid antibodies may play a role in its pathogenesis and have an autoimmune link. Fluctuations in baseline thyroid function for patients with known Graves’ disease may be a potentiating factor in exacerbating moyamoya vasculopathy.

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Efficacy of Revascularization Surgery for Moyamoya Syndrome Associated With Graves' Disease

Neurologia medico-chirurgica ◽

10.2176/nmc.50.977 ◽

2010 ◽

Vol 50 (11) ◽

pp. 977-983 ◽

Cited By ~ 7

Author(s):

Hidenori ENDO ◽

Miki FUJIMURA ◽

Kuniyasu NIIZUMA ◽

Hiroaki SHIMIZU ◽

Teiji TOMINAGA

Keyword(s):

Graves Disease ◽

Moyamoya Syndrome ◽

Revascularization Surgery

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Carbimazole induced rhabdomyolysis

Endocrinology Diabetes and Metabolism Case Reports ◽

10.1530/edm-21-0006 ◽

2021 ◽

Vol 2021 ◽

Author(s):

Niamh O’Donnell ◽

Aisling McCarthy ◽

Ken Thong

Keyword(s):

Adverse Effect ◽

Creatine Kinase ◽

Side Effects ◽

Temporal Relationship ◽

Early Recognition ◽

Antithyroid Drug ◽

Graves Disease ◽

List Type ◽

Musculoskeletal Complaints ◽

Learning Points

Summary Carbimazole is a commonly used antithyroid drug (ATD), which is associated with several well-established side effects. However, Carbimazole-induced rhabdomyolysis is rarely reported in the literature. We report a 27-year-old male who presented with upper limb myalgia and significantly raised creatine kinase elevation, 1-month post commencement of Carbimazole for Graves’ disease. Carbimazole was ceased with subsequent clinical and biochemical improvement. Though the pathophysiology remains unclear, we hope to raise awareness regarding this rare adverse effect with a view to promote early recognition and prompt discontinuation of the offending medication caused by a commonly used medication in endocrinology. Learning points Musculoskeletal complaints can relate to unidentified and untreated hyperthyroidism. However one must be mindful that the treatment for these disorders can too induce myopathies. ATD-induced myopathy should be considered when there is a temporal relationship between introduction of ATDs and the onset of symptoms. If ATD-induced myopathy is being considered, other causes of myopathy should still be outruled. Prompt discontinuation of potentially offending medications may provide resolution of symptoms and avoid significant consequences.

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Treatment of congenital hypothyroidism in a newborn with malabsorption after subtotal ileum resection

Endocrinology Diabetes and Metabolism Case Reports ◽

10.1530/edm-17-0156 ◽

2018 ◽

Vol 2018 ◽

Author(s):

Charlotte S Schömig ◽

Marie-Ève Robinson ◽

Julia E von Oettingen

Keyword(s):

Thyroid Function ◽

Congenital Hypothyroidism ◽

Small Bowel Resection ◽

List Type ◽

Free T4 ◽

Adverse Health Outcomes ◽

Levothyroxine Replacement ◽

High Doses ◽

The Individual ◽

Learning Points

Summary Congenital hypothyroidism requires prompt treatment to prevent adverse health outcomes. Poor intestinal levothyroxine absorption can complicate management. We present a case of a term female newborn with necrotizing enterocolitis (NEC) requiring subtotal ileum resection. Congenital hypothyroidism was diagnosed by newborn screening. Treatment was complicated by intestinal malabsorption of levothyroxine. Intravenous levothyroxine substitution restored euthyroidism and supraphysiologic PO doses subsequently maintained a euthyroid state. After several months, the required levothyroxine dose was weaned down to typical recommended dosing. In conclusion, small bowel resection secondary to NEC may lead to malabsorption of oral levothyroxine. An intravenous levothyroxine dose of approximately 50% typical PO dosing is effective in providing rapid normalization of free T4 and TSH. High PO doses may be required to maintain euthyroidism. Close thyroid function monitoring and immediate therapy adjustment are essential as the individual absorption may vary widely. Normal absorption levels may be regained due to adaption of the neonatal intestines. Learning points: In neonates with malabsorption after ileum resection intravenous levothyroxine replacement should be used to provide normalization of free T4 and TSH. Very high doses of up to 500% usual oral levothyroxine may be required to maintain euthyroidism. The estimated degree of malabsorption can be used to determine the initial dose. Close thyroid function monitoring and immediate therapy adjustment are essential as the absorption and intestinal adaption may vary widely.

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Euthyroid athyroxinemia – a novel endocrine syndrome

Endocrinology Diabetes and Metabolism Case Reports ◽

10.1530/edm-17-0019 ◽

2017 ◽

Vol 2017 ◽

Author(s):

Nicholas Woodhouse ◽

Fatima Bahowairath ◽

Omayma Elshafie

Keyword(s):

Thyroid Nodule ◽

Thyroid Function ◽

Urinary Iodine ◽

Free Thyroxine ◽

List Type ◽

Thyroid Function Tests ◽

Thyroxine Level ◽

Learning Points ◽

Free Thyroxine Level ◽

Tsh Levels

Summary A 55-year-old female was referred with abnormal thyroid function tests (TFTs); the free thyroxine level (FT4) was undetectable <3.3 pmol/L (normal: 7.9–14.4), while her FT3, TSH and urinary iodine levels were normal. She was clinically euthyroid with a large soft lobulated goitre that had been present for more than thirty years. She received an injection of recombinant human TSH (rhTSH) following which there was a progressive rise of the FT3 and TSH levels to 23 pmol/L and >100 mIU/L respectively at 24 h, The FT4 however remained undetectable throughout. Being on thyroxine 100 µg/day for one month, her FT4 level increased to 15 pmol/L and TSH fell to 0.08 mIU/L. Four years earlier at another hospital, her FT4 level had been low (6.8 pmol/L) with a normal TSH and a raised Tc-99 uptake of 20% (normal<4%). We checked the TFTs and Tc-99 scans in 3 of her children; one was completely normal and 2 had euthyroid with soft lobulated goitres. Their Tc-99 scan uptakes were raised at 17% and 15%, with normal TFTs apart from a low FT4 7.2 pmol/L in the son with the largest thyroid nodule. This is a previously unreported form of dyshormonogenesis in which, with time, patients gradually lose their ability to synthesize thyroxine (T4) but not triiodothyroxine (T3). Learning points: This is a previously unreported form of dyshormonogenetic goitre. This goitre progressively loses its ability to synthesize T4 but not T3. The inability to synthesize T4 was demonstrated by giving rhTSH.

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Graves disease in infancy: a patient presentation and literature review

Endocrinology Diabetes and Metabolism Case Reports ◽

10.1530/edm-20-0162 ◽

2021 ◽

Vol 2021 ◽

Author(s):

Kara Alex-Ann Beliard ◽

Srinidhi Shyamkumar ◽

Preneet Cheema Brar ◽

Robert Rapaport

Keyword(s):

Weight Gain ◽

English Literature ◽

Pediatric Intensive Care ◽

Graves Disease ◽

List Type ◽

Remarkable Recovery ◽

Patient Presentation ◽

Learning Points ◽

Poor Weight Gain ◽

Height For Age

Summary We describe a case of an infant who presented with clinical features of hyperthyroidism. The child was found to be tachycardic, hypertensive and diaphoretic, she was noted to have poor weight gain and difficulty in sleeping. The child was admitted to the pediatric intensive care unit for care. She was found to have biochemical evidence of hyperthyroidism with positive thyroid stimulating immunoglobulin. She responded well to methimazole and propranolol and had a remarkable recovery. She is the youngest patient to be diagnosed with Graves disease in the English literature, at 12 months of life. Learning points Hyperthyroidism must always be considered even at very young age, for patient presenting with poor weight gain and hyperdynamic state. Autoimmune diseases are becoming more common in infancy. Craniosynostosis and increased height for age are well-documented consequences of untreated hyperthyroidism in developing children.

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Catastrophic stroke in a patient with left ventricular non-compaction

Echo Research and Practice ◽

10.1530/erp-18-0015 ◽

2018 ◽

Vol 5 (3) ◽

pp. K59-K62 ◽

Cited By ~ 3

Author(s):

Sothinathan Gurunathan ◽

Roxy Senior

Keyword(s):

Left Ventricular ◽

List Type ◽

Colour Doppler ◽

Mortality And Morbidity ◽

History Of ◽

Dimensional Echocardiography ◽

Two Dimensional Echocardiography ◽

Learning Points ◽

Normal Cardiac Function

Summary We present the case of a 32-year-old man who presented with a remote history of chest pain and was diagnosed with non-compaction cardiomyopathy on echocardiography. On presentation, he was relatively asymptomatic with normal cardiac function. Unfortunately, he presented 1 year later with a catastrophic embolic stroke. Learning points: Left ventricular non-compaction (LVNC) is a myocardial disorder characterised by prominent left ventricular (LV) trabeculae, a thin compacted layer and deep intertrabecular recesses. Two-dimensional echocardiography with colour Doppler is the study of choice for diagnosis and follow-up of LVNC. CMR serves an important role where adequate echocardiographic imaging cannot be obtained. LVNC is associated with high rates of mortality and morbidity in adults, including heart failure, thromboembolic events and tachyarrhythmias.

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Lugol's solution-induced painless thyroiditis

Endocrinology Diabetes and Metabolism Case Reports ◽

10.1530/edm-17-0034 ◽

2017 ◽

Vol 2017 ◽

Cited By ~ 1

Author(s):

Ji Wei Yang ◽

Jacques How

Keyword(s):

Thyroid Stimulating Hormone ◽

Thyroid Storm ◽

Graves Disease ◽

List Type ◽

Free T4 ◽

Iodine Excess ◽

Thyroid Glands ◽

Painless Thyroiditis ◽

Learning Points ◽

Painful Thyroiditis

Summary Lugol’s solution is usually employed for a limited period for thyroidectomy preparation in patients with Graves’ disease and for the control of severe thyrotoxicosis and thyroid storm. We describe a rare case of Lugol’s solution-induced painless thyroiditis. In November 2014, a 59-year-old woman was prescribed Lugol’s solution four drops per day for the alleviation of menopausal symptoms. She was referred to our clinic in June 2015 for fatigue, hair loss, and a 20-lb weight loss without thyroid pain or discomfort. Physical examination revealed a normal thyroid gland. On 7 May 2015, laboratory tests revealed a suppressed thyroid-stimulating hormone (TSH) 0.01 U/L with elevated free T4 3.31 ng/dL (42.54 pmol/L). Repeat testing on 25 May 2015 showed spontaneous normalization of the free thyroid hormone levels with persistently low TSH 0.10 U/L. Following these results, a family physician prescribed methimazole 10 mg PO TID and very soon after, the TSH concentration rose to >100 U/L along with subnormal free T4 and T3 levels. Methimazole was promptly discontinued, namely within 18 days of its initiation. Over the course of the next few months, the patient spontaneously achieved clinical and biochemical euthyroidism. To our knowledge, this is a unique case of painless thyroiditis induced by Lugol’s solution, which has not been reported before. Lugol’s solution is a short-term medication given for the preparation of thyroidectomy in patients with Graves’ disease and for the control of severe thyrotoxicosis. Iodine excess can cause both hyperthyroidism and hypothyroidism. Rarely, Lugol’s solution can cause acute painless thyroiditis. Learning points: Lugol’s solution is used for thyroidectomy preparation in patients with Graves’ disease and for the control of severe thyrotoxicosis and thyroid storm. Iodine excess can cause both hypothyroidism and thyrotoxicosis. Thyroid glands with an underlying pathology are particularly susceptible to the adverse effect of iodine. The prolonged off-label use of Lugol’s solution can be harmful. Rarely, Lugol’s solution can cause acute painful thyroiditis.

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Abstract #1202: A Case of Graves’ Disease With Fluctuating Thyroid Function

Endocrine Practice ◽

10.1016/s1530-891x(20)44852-9 ◽

2016 ◽

Vol 22 ◽

pp. 305-306

Author(s):

Sheela Metgud ◽

Anup Kumar ◽

Erin Drever ◽

Tahira Yasmeen

Keyword(s):

Thyroid Function ◽

Graves Disease

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Graves' disease and radioiodine therapy

Nuklearmedizin ◽

10.3413/nukmed-0145 ◽

2008 ◽

Vol 47 (04) ◽

pp. 153-166 ◽

Cited By ~ 10

Author(s):

I. Weber ◽

W. Eschner ◽

F. Sudbrock ◽

M. Schmidt ◽

M. Dietlein ◽

...

Keyword(s):

Success Rate ◽

Thyroid Function ◽

Therapeutic Dose ◽

Radioiodine Therapy ◽

Antithyroid Drugs ◽

Graves Disease ◽

Inclusion Criteria ◽

End Point ◽

Point Measure ◽

The Impact

SummaryAim: This study was performed to analyse the impact of the choice of antithyroid drugs (ATD) on the outcome of ablative radioiodine therapy (RIT) in patients with Graves' disease. Patients, material, methods: A total of 571 consecutive patients were observed for 12 months after RIT between July 2001 and June 2004. Inclusion criteria were the confirmed diagnosis of Graves' disease, compensation of hyperthyroidism and withdrawal of ATD two days before preliminary radioiodine-testing and RIT. The intended dose of 250 Gy was calculated from the results of the radioiodine test and the therapeutically achieved dose was measured by serial uptake measurements. The end-point measure was thyroid function 12 months after RIT; success was defined as elimination of hyperthyroidism. The pretreatment ATD was retrospectively correlated with the results achieved. Results: Relief from hyperthyroidism was achieved in 96 % of patients. 472 patients were treated with carbimazole or methimazole (CMI) and 61 with propylthiouracil (PTU). 38 patients had no thyrostatic drugs (ND) prior to RIT. The success rate was equal in all groups (CMI 451/472; PTU 61/61; ND 37/38; p=0.22). Conclusion: Thyrostatic treatment with PTU achieves excellent results in ablative RIT, using an accurate dosimetric approach with an achieved post-therapeutic dose of more than 200 Gy.

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