A Case of Postural Orthostatic Tachycardia Syndrome Associated with Migraine and Fibromyalgia
A 51-year-old woman presented with a 4-month history of presyncope and sometimes syncope on standing. These episodes occurred with premonitory signs of nausea, lightheadedness, orthostatic visual blurring, and tachycardia. They were all relieved in recumbency. She also complained of severe throbbing headache in the right occipitotemporal area preceded by photopsia, which was accompanied by vomiting, unilateral tinnitus, photophobia and phonophobia. Headache impact test-6 score was 78 []. She had widespread body pain, chronic fatigue, muscle tenderness, and difficulty sleeping. The diagnosis of fibromyalgia was made according to the ACR 2010 criteria []. Widespread pain index and symptom severity scale score were 9 and 12 respectively. Fibromyalgia impact questionnaire score was 87 []. Beck depression inventory score [] was 47 which reflected a state of severe depression.\nShe smoked for 6 years. Her medical history including recent viral infections was not significant except for migraine. She denied the use of medications. She had laparoscopic assisted vaginal hysterectomy for a uterine myoma 2 years ago.\nExamination between the attacks revealed a blood pressure (BP) of 120/70 mmHg, regular pulse at 76 bpm, no carotid or supraclavicular bruits, and no cardiac murmurs. Her physical examination was normal except for 13 specific points of tenderness on palpation; left and right occiput, trapezius, supraspinatus muscles, lower cervical spaces, second ribs, knees, and left lateral epicondyle. Cranial nerve examination was normal and there was no motor or sensory deficit. Cerebellar function and gait were normal.\nBlood tests including electrolyte, epinephrine and norepinephrine, thyroid function test, Venereal Disease Research Laboratory (VDRL) test, HIV, rheumatoid factor, lupus anticoagulant, antiphospholipid antibody, antinuclear antibody, antineutrophil cytoplasmic antibody, and anti-Ro/La antibody were unremarkable. 24-hour Holter monitoring, an echocardiogram, an electroencephalogram, and brain CT angiogram were normal. BP and HR responses during lying to standing were characterized by increase in HR from 78 to 135 bpm without a significant decrease of BP (). On the autonomic function test, HR variance at deep breathing was decreased to 5.04 bpm (normal > 12 bpm) and the expiration-to-inspiration (E:I) ratio was within normal range. During Valsalva maneuver, the ratio between HR in phases II and IV was normal. The sympathetic skin response (SSR) was unremarkable. On the head-up tilt table test (HUTT) at 70° for 30 minutes, changes in systolic BP, diastolic BP, and HR were calculated with respect to basal values. Very low dose isoproterenol (2 µg) infusion test demonstrated excessive tachycardia of HR increment of 49 bpm without a significant decrease of BP (). HUTT was interrupted because of the patient's intense malaise, lightheadedness, and palpitations as she recognized these symptoms as prodromes of her episodes of transient loss of consciousness.\nThe patient has been treated with topiramate, antidepressants and antiepileptic GABAergic drugs with gradual improvement of the headache and widespread pain over one month. She was commenced on propranolol with no symptomatic improvement. She was subsequently treated with midodrine and fludrocortisone and advised to increase salt and fluid intake. Application of an abdominal binder, aerobic exercise and physical counter-maneuvers [] were recommended to help with autonomic conditioning. They resulted in a reduction of supine and erect HR to 76 and 92 bpm, respectively, along with symptomatic improvement over 2 months ().