C A S E R E P O R T JIACM 2014; 15(3-4): 222-3

Thyroid storm: An unusual presentation

NS Neki*

Abstract

A 23-year-old female patient was hospitalised with complaints of fever, diarrhoea, altered sensorium for 3 days with a rapidlydeclining mental status. Before admission to the hospital, her relatives gave history of her being restless, markedly irritable withgeneralised tonic-clonic seizures and a positive history of palpitations. Suspecting the diagnosis of thyroid storm clinically, shewas started on aggressive antithyroid treatment following which she showed marked clinical improvement. Thyroid storm is arare endocrine emergency with increased mortality risk in this otherwise fatal entity. The aim of reporting this case is to make thephysicians aware of this fatal medical disease necessitating prompt intervention. A case of thyroid storm presenting withpredominant neurological manifestations is being presented here for its rarity.

Key words: Thyroid storm, unusual presentation.

*Professor of Medicine, Government Medical College, Amritsar, Punjab, and Trained Endocrinologist, Department ofEndocrinology, PGIMER, Chandigarh; and President, Geriatric Society of India.

IntroductionThyroid storm (accelerated hyperthyroidism) is a raremanifestation of thyrotoxicosis with a wide spectrum ofclinical presentations involving multiple systems. Itgenerally occurs in females from the third to the sixthdecade of life, especially with Graves disease. It is usuallya life-threatening medical emergency and is fatal if leftuntreated. The classic clinical presentation includes fever,tachycardia, hypertension, tremors, nausea, vomiting,diarrhoea, dehydration, arrhythmias, delirium, and coma1.It usually develops in an undiagnosed hyperthyroidpatient who has a major stress or continues withoutantithyroid treatment in addition to other precipitatingfactors like surgery, radio-iodine therapy, trauma, acuteinfection toxaemia of pregnancy, labour, excessivepalpation of the thyroid gland in hyperthyroid patients,pulmonary thromboembolism, severe drug reactions, ormyocardial infarction, etc2. Its early recognition andtreatment is essential in reducing the morbidity andmortality rate in this potentially fatal disease. Only 1 - 2%of hyperthyroid cases manifest as thyroid storm and themortality ranges between 20 - 30% despite treatment3.

Case reportA 23-year-old unmarried female was brought to thehospital with complaints of fever, diarrhoea, lightheadedness, altered sensorium for 3 days with a rapidlydeclining mental status. Before admission, she wasreported to be restless, markedly irritable with generalisedtonic clonic seizures as well as positive history ofpalpitations as narrated by relatives.

There was no history of previous illness or hospitaladmission, major surgery, or antipsychotic drugs intake. On

examination, she was febrile (temp 101F), pulse rate 126/min and regular, BP 110/70 mmHg, respiratory rate 29breaths/mm, oxygen saturation 98% on 3L. Her pupils wereslightly dilated, mucous membranes were moist and theshe had an abrasion on the tongue consistent with biting.JVP was normal; there was no lymphadenopathy.Examination of the neck revealed bilateral thyroidenlargement with diffuse toxic goitre with systolic bruitheard over it. She had a stare with a typical anxious look.There were no signs of meningeal irritation. Fundusexamination was normal. On neurological examination, sheresponded to painful stimuli, with brisk deep tendonreflexes and plantar flexor response. CVS examinationrevealed tachycardia with regular rhythm but no murmurs.Respiratory system and abdominal examination wasunremarkable. ECG showed sinus tachycardia. Herlaboratory results showed no abnormality of haemogram,blood glucose levels, CSF, serum electrolytes, renal and liverfunction tests. X-ray chest showed no active disease. CTbrain was normal. Blood cultures were negative. EEGrevealed bilateral frontal slow waves. Technetium scan ofthyroid showed hyperfunctioning gland with diffuse traceruptake (36% at 20 minutes). The constellation of signs andsymptoms suggested that this could be a case of thyroidstorm. Based on the diagnostic criteria of Burch andWartofsky4, she had a total score of 60, which was stronglysuggestive of thyroid storm (a score > 25 is suggestive ofthyroid storm). The patient was put on antithyroid drugsawaiting thyroid function tests T3, T4, and TSH. She wasstarted on propylthiouracil 400 mg tid and propranolol 40mg tid, IV fluids, hydrocortisone IV (later changed to oralsteroids). Her thyroid profile revealed TSH 0.02 mIU/ml, T428 g/dl (N 4 - 14 g/dl); T3 40 ng/dl (N = 0.8 - 2 ng/dl).Thyroid peroxidase autoantibody was negative. Based on

clinical presentation and later on confirmed by results,Lugols iodine 8 drops 8 hourly was given for 3 days. Thefever subsided, diarrhoea improved, and agitationdecreased. After considerable improvement in hercondition in about 8 days time, she was discharged in aneuthyroid state, conscious, well oriented, without tremorsand fever, and advised to continue on propylthiouracil andpropranolol. On follow-up at 3 months, she was carryingour all routine activities with a positive frame of mind.

DiscussionThyroid storm a dramatic exacerbation of existinghyperthyroidism, of sudden onset associated with fever,tachycardia, and CNS symptomatology remains a life-threatening medical emergency if left untreated. Being arare endocrine emergency, all clinicians must be aware ofits clinical features and treatment so that morbidity andmortality can be avoided. About 1 - 2% of patients withhyperthyroidism progress to thyroid storm and the 100%mortality reported earlier has now come down to 20 - 30%with better recognition and treatment. It might be difficultto distinguish between thyroid storm and infection inthyrotoxic patients as tachycardia and fever might bepresent in both. On account of an overlapping of thesymptoms, precipitating conditions and complications, aclinical diagnosis is not easy and is often made too late. Thedefinitive criteria of thyroid storm laid down by Burch andWartotsky4 are useful. The triggering factors for thyroidstorm include surgery, major stress, noncompliance toantithyroid drugs, infection, radio-iodine, etc2. Treatmentof thyroid storm should not be delayed if there is a highindex of suspicion, and empirical treatment should bestarted on clinical grounds awaiting laboratory reports,which was evident in our case5. Urgent thyroid function testsis a confirmatory diagnosis. Hyperglycaemia,hypercalcaemia, leucocytosis may co-exist. Deranged liverfunctions mainly alkaline phosphatase may occur due toincreased osteoblastic activity in response to high boneresorption. Serum thyroid hormone levels would typicallyshow hyperthyroidism, but due to an abrupt rise of thryoidhormone secondary to triggering factors, the patient canno longer adapt to the sudden metabolic stress6. An acuteelevation of FT3 or FT4 in thyrotoxic patients may produceacute decompensation. However, no absolute levels ofserum T3 or T4 exist above which thyroid storm developsinevitably.7 Earlier, cases of thyroid storm have been wellreported where treatment of thyroid storm was startedimmediately awaiting thyroid function tests8,9. T4 may rarelybe normal or even decreasing because of co-existingnonthyroidal illness10.

In our case, the patient presented with altered sensoriumwithout signs of raised intracranial tension and focalneurological deficit. Differential diagnosis includes

neuroleptic malignant syndrome, anticholinergic poisoning,sympathomimetic toxicity, alcohol withdrawal syndrome,toxic/metabolic encephalopathy, hypertensiveencephalopathy, meningitis, etc. But the clinical features ofour patient in the form of hypermetabolic state madethyroid storm a definitive diagnosis; and possibly thetriggering factor for thyroid storm in this case could befebrile illness. Predominant neurological manifestation inthe form of altered mental status and history of tonic clonicgeneralised seizures was an unusual feature in our case.

Treatment of thyroid storm includes correction of severethyrotoxicosis, precipitating illness, and associated activethyroid eye disease. Patients should be monitored in theintensive care units in the early phase. Diuretics may begiven for congestive cardiac failure (CHF). Drugs likethionamides block hormone synthesis; iodine solutionblocks the release of thyroid hormone, beta-blockerscontrol adrenergic symptoms, and steroids reduce T4 to T3conversion. Beta-blockers should be used cautiously in thepresence of CHF. Among thionamides, propylthiouracil ispreferred over methionazole as it blocks peripheral T4 toT3 conversion. Iodinated radiocontrast iopanoic acid,cholestyramine can also be used. Peritoneal dialysis andplasmaphresis are used to reduce the high levels ofcirculating T4 and T3 in a thyroid storm5,9,10.

ConclusionDiagnosis may be missed on account of variablepresentation. Treatment should never be delayed. A highindex of suspicion is required for prompt recognition andeffective management of unusual presentation of thyroidstorm in order to reduce the morbidity and mortality ofthis life-threatening medical disorder.

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Journal, Indian Academy of Clinical Medicine Vol. 15, No. 3 & 4 July-December, 2014 223