Reversible complication of Thyroid storm as a cerebral venous Thrombosis - A Case Report

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1 Reversible complication of Thyroid storm as a cerebral venous Thrombosis - A Case Report Sai Sampathkumar Natuva 1, Sangamithra Gandra 2, Vallampalli Ganesh 3, P.N.R. Prasad 4, 1 Professor, 2, 3, 4 Postgraduate students Department of Neurology,Narayana Medical College,Nellore ,Andhra Pradesh, India Corresponding author : Sai Sampathkumar Natuva, ID: drnssampath123@gmail.com ABSTRACT Thyroid storm causing central venous thrombosis is extremely rare.herewith we report a case of 35 year old man who presented with sudden onset of central venous thrombosis with goiter and Thyroid storm.he showed improvement with anti thyroid drugs and anti coagulants.we conclude that it is a case of central venous thrombosis (CVT) secondary to transient hyper coaguble state due to thyrotoxicosis.after 6 weeks during follow up Factor VIII activity was normalised.we consider reversible complication of Thyroid storm as a cause of cerebral venous thrombosis is unique,hence reported. Key words: Thyroid storm,central Venous Thrombosis, Prothrombotic state Introduction: Cerebral venous thrombosis (CVT) represents only <1 % of all strokes 1.2. The incidence is estimated to be 2-4 per million per year, but the number is probably higher due to undiagnosed cases. Common symptoms of CVT are throbbing headache, blurred vision and acute focal neurological disturbances such as paresis and focal seizures 3.The commonest encountered risk factors in 85% of patients are oral contraceptive use, pregnancy and puerperium that make the disease predominant in female sex 4. In % of patients, the disease remains unprovoked, i.e., occurring in the absence of predisposing factors. Thrombophilic abnormalities either inherited [deficiency of the natural anticoagulant proteins antithrombin, protein C or protein S, mutations in the factor V gene (factor V Leiden) or prothrombin gene (prothrombin G20210A)]5 or acquired (antiphospholipid antibodies) are worthy to be investigated in patients with CVT, as well as hyper homocysteinemia. Magnetic resonance imaging (MRI) with venous angiography is the gold standard for the diagnosis of CVT6. Thyroid storm is an acute, life-threatening emergency. If untreated, thyroid storm is almost invariably fatal in adults (90% mortality rate) and is likely to cause 40

2 a similarly severe outcome in children. Hyperthyroidism or thyroid storm is not a wellknown risk factor for deep CVT. Our case report is of a young man(35yrs) who developed CVT due to thyroid storm. Case report A 35 years male, non alcoholic and non smoker developed sudden onset holocranial throbing headache followed by 2 episodes of vomiting and worsening of sensorium and weakness of left upper and lower limbs which was progressive. History of slurring of speech and facial deviation to right present.. He reported increased appetite(takes 5-6 times of food/day) since 6 months and increased bowel frequency( 3-4 times per day) and excessive sweating and weight loss. On examination, the patient was emaciated, goitre was present (multinodular), Icterus was noted. Patient had hyperpyrexia (Temp :103F), tachycardia( PR: 130/min), tachypnea(respiratory rate: 20/min) and his blood pressure was140/80 mm Hg in supine position, at the time of admission. GCS (E3M5V3) was 11 and patient was irritable. Prominent eye balls were noted with full extra ocular movements.left UMN facial palsy was noted. Spastic left upper and lower limb with best observed motor power(mrc Medical Research council grade) was 1/5 in left upper limb and lower limb and 4/ 5 in right upper limb and lower limb. Brisk deep tendon reflexes with bilateral plantar extensors. There were no meningeal signs. The following investigations were done (Table1.) TABLE 1 INVESTIGATIONS Hb 12.6 g/dl TLC 18400cell/cum.m DC - POLYMORPHS 84% - LYMPHOCYTES 08% - EOSINOPHILS 01% - MONOCYTES 07% - BASOPHILS 00 ESR 31mm/1hr SERUM CREATININE 0.63 mg/dl BLOOD UREA 22mg/dl SERUM SODIUM 128 meq/l SERUM POTASSIUM 4.0 meq/l SERUM CHLORIDE 90 meq/l TOTAL CHOLESTEROL 148 mg/dl HDL 36 mg/dl LDL 84 mg/dl VLDL 28 mg/dl TRIGLYCERIDES 140 mg/dl PROTHROMBINE TIME 14.4 secs CONTROL 13.9 secs INR 1.66 HIV Negative HBsAg Negative HCV Negative TOTAL BILIRUBIN 3.75 mg/dl DIRECT BILIRUBIN 1.16 mg/dl SGOT 21 SGPT 19 ALP 627 TOTAL PROTEIN 7.3 g/dl S.ALBUMIN 3.6 g/dl S. GLOBULIN 3.7 g/dl A/G RATIO 0.9 S.CALCIUM 9.9 mg/dl Factor VIII activity 331% Chest X ray NORMAL USG thyroid Normal sized thyroid with heterogenous echotexture with normal vascularity with 2 nodules 41

3 MRI brain revealed venous infarcts in bilateral thalami and centrum semiovale. MRV showed extensive thrombosis of internal cerebral veins, Vein of Galen, inferior sagittal sinus, straight sinus, and tranverse sinus. (Fig. 182) On the next day patient had persistent rise of temperature of F, tachycardia of 160/ min, tachypnea, with saturation of 80% at room air. In view of worsening of GCS and falling O2 saturation he was intubated and ventilator support was given. His neurological status worsened from hemiparesis to quadriparesis. As the premorbid history and examination were suggestive of hypercatabolic state, we have sent for thyroid profile. It showed elevated thyroid hormone levels FT ( pg/dl),FT4 >5.96 ( ng/dl) and decreased TSH 0.14 ( IU) suggestive of a hyperthyroid state.tpo antibodies were positive. USG thyroid was done which revealed normal sized thyroid with heterogenous echotexture with normal vascularity with 2 nodules.the calculated Burch-Wartofsky- Score was 105. (score? 45 suggestive of thyroid storm) This was highly suggestive of thyroid storm. Workup was done for Factor VIII and other prothrombotic factors. Factor VIII activity was increased (331%). Protein-C, protein-s, Factor V Leiden mutation and homocyteine were normal. Finally we concluded this as a case of Cerbral Venous Thrombosis secondary to transient hypercoagulable state due to thyrotoxicosis. He was treated with anticoagulants and supportive management. Thyroid storm was managed according to Endocrinologist advice with antithyroid drugs(methimazole), cholestyramine, lithium and steroids. He gradually recovered over a period of one week and his thyroid hormone levels steadily decreased. He was kept on maintenance dose of methimazole and oral anticoagulants were continued. During the follow up period after 6 weeks of discharge, factor VIII activity was normalized which is a transient prothrombotic state due to thyroid storm. Discussion Hyperthyroidism is the condition that occurs due to excessive production of thyroid hormone by the thyroid gland.7 Thyrotoxicosis is the condition that occurs due to excessive thyroid hormone of any cause and therefore includes hyperthyroidism.7 Signs and symptoms vary between people and may include irritability, muscle weakness, sleep disturbance, tachycardia, heat intolerance, diarrhea, enlargement of the thyroid, and weight loss. Symptoms are typically less in the old and during pregnancy.8 An 42

4 uncommon complication is thyroid storm in which an event such as an infection results in worsening symptoms such as confusion and a high temperature and often results in death. Graves' disease is the cause of about 50% to 80% of the cases of hyperthyroidism. Other causes include multinodular goiter, toxic adenoma, inflammation of the thyroid, intake of excess iodine, and too much synthetic thyroid hormone. A less common cause is a pituitary adenoma. The diagnosis may be suspected based on signs and symptoms and then confirmed with blood tests. Typically blood tests show a low thyroid stimulating hormone (TSH) and raised T3 or T4.Radioiodine uptake by the thyroid, thyroid scan, and TSI antibodies may help to determine the cause. The Diagnosis of Thyroid storm is primarily clinical and by investigating the thyroid hormone levels. The Burch-Wartofsky- Score(Table-2) is a point scale that helps to assess the probability of thyrotoxicosis independently from the level of thyroid hormones. It is solely based on clinical and physical criteria. The point scale covers body temperature, central nervous system effects, hepato gastrointestinal symptoms, cardiovascular dysfunction and the patient's history Hyperthyroidism is associated with increased incidence of venous thromboembolic events, such as pulmonary embolism and deep venous thrombosis11. Increased level of ft4 is associated with increased synthesis and secretion of factor VIII. Abnormalities in the coagulation system, such as increased fibrinogen, Von Willebrand factor (vwf), Plasminogen activator inhibitor 1 (PAI-1), coagulation factors VIII, IX and X and shorter activated partial thromboplastin time (APTT) are found in hyperthyroid patients12. The hypercoagulability and decreased fibrinolysis in hyperthyroidism is thyroxindependent. In the present case, a 35 yr old male who had prior history suggestive of hyperthyroidism presented with sudden onset of altered sensorium and progressive weakness of all limbs. He also had features suggestive of hyper metabolism. On workup the patient was found to have cerebral venous thrombosis for which treatment with anticoagulants was started. Further investigations were done to find out the cause for hyper coagulable state. Meanwhile the patient was also diagnosed to have thyroid storm. His Factor VIII levels were found to be elevated. The hyperthyroid state may be responsible for the hyper coagulable state which precipitated cerebral venous thrombosis in this patient. On appropriate treatment of the hyperthyroid state the factor VIII levels have reduced gradually. We report this as a case of cerebral venous thrombosis due to a rare reversible cause of hyper coagulability due to 43

5 thyroid storm. To the best of our knowledge we believe that probably this may be the first Case report of thyroid storm as the precipitating cause for cerebral venous thrombosis. Table 2 Burch - Wartofsky score Diagnostic criteria for Thyroid storm precipitating cause of CVT in our patient. CVT should be suspected and MRI with venous angiography be performed when unusual headache alone or in combination with neurological deficits occurs in patients with hyperthyroidism. Thyroid profile should be considered apart from Thrombotic profile for aetiology of CVT in young individuals.cvt and hyperthyroidism/thyroid storm should be treated as soon as possible as mortality rate of these cases is very high. References 1. 1.Einhäupl K, Stam J, Bousser MG, De Bruijn SF, Ferro JM, et al. (2010) EFNS guideline on the treatment of cerebral venous and sinus thrombosis in adult patients. Eur J Neurol 17: Coutinho JM, Stam J (2010) How to treat cerebral venous and sinus thrombosis. J Thromb Haemost 8: Conclusions The cause of CVT may be multi-factorial. Studies indicate that a high thyroxin level causes a hypercoagulable condition. We conclude TRANSIENT HYPER COAGULABLE STATE DUE TO THYROTOXICOSIS as the main 3. Ferro JM, Canhão P, Stam J, Bousser MG, Barinagarrementeria F, et al.(2004) Prognosis of cerebral vein and dural sinus thrombosis: results of the International Study on Cerebral Vein and Dural Sinus Thrombosis (ISCVT). Stroke 35: Coutinho JM, Ferro JM, Canhão P, Barinagarrementeria F, Cantú C, et al.(2009) Cerebral venous and sinus thrombosis in women. Stroke 40:

6 5. Madroñero-Vuelta AB, Sanahuja-Montesinos J, Bergua-Llop M, Araguás- Arasanz C (2004) [Cerebral venous thrombosis associated to subacute De Quervain's thyroiditis in a carrier for the G20210A mutation of the prothrombin gene]. Rev Neurol 39: Rizzo L, Crasto SG, Rudà R, Gallo G, Tola E, et al. (2010) Cerebral venous thrombosis: role of CT, MRI and MRA in the emergency setting. Radiol Med 115: Bahn Chair, RS; Burch, HB; Cooper, DS; Garber, JR; Greenlee, MC; Klein, I; Laurberg, P; McDougall, IR; Montori, VM; Rivkees, SA; Ross, DS; Sosa, JA; Stan, MN (June 2011). "Hyperthyroidism and other causes of thyrotoxicosis: management guidelines of the American Thyroid Association and American Association of Clinical Endocrinologists.". Thyroid. 21 (6): doi: / thy pmid "Hyperthyroidism". July Retrieved Klubo-Gwiezdzinska, Joanna; Wartofsky, Leonard (March 2012). "Thyroid emergencies". Medical Clinics of North America 96 (2): doi: / j.mcna pmid Burch, HB; Wartofsky, L (June 1993). "Lifethreatening thyrotoxicosis. Thyroid storm.". Endocrinology and metabolism clinics of North America 22 (2): PMID Verberne HJ, Fliers E, Prummel MF, Stam J, Brandjes DP, et al. (2000) Thyrotoxicosis as a predisposing factor for cerebral venous thrombosis. Thyroid 10: Maes J, Michotte A, Velkeniers B, Stadnik T, Jochmans K (2002) Hyperthyroidism with increased factor VIII procoagulant protein as a predisposing factor for cerebral venous thrombosis. J Neurol Neurosurg Psychiatry 73: