An unusual case of stroke in a young adult.
* CASE REPORT
A 40-year-old white man developed a sudden change in mental status; he was unable to communicate or follow commands, and was in a state of confusion. Paramedics were summoned to his home and found the patient to have no evidence of trauma, but he had global aphasia with right-sided weakness of the upper extremity more than the lower extremity; his blood pressure was 210/140 mm Hg. The patient had a past medical history for hypertension and hyperlipidemia and was regularly taking antihypertensive medication, ie, lisinopril 20 mg daily. He had no history of diabetes mellitus, heart palpitations, alcohol or drug use, prior thromboembolic episodes, cigarette smoking, or family history of stroke. He was treated for hypertension en route to the emergency department.
In the emergency department, the patient was awake but disoriented. He was aphasic, unable to follow simple commands, and made inappropriate responses to questions posed by emergency department personnel. The patient's blood pressure was 120/56 mm Hg, his pulse 95 beats per minute, oral temperature 96.6[degrees]F (35.9[degrees]C), and respirations 16 per minute. The right side of the patient's face drooped and his tympanic membranes were clear, with no hemotympanum. There was no thyromegaly. No carotid bruits were auscultated. An examination of the heart and lungs revealed no abnormalities. The abdomen was soft and nontender, and there was no hepatosplenomegaly.
A neurologic examination revealed that the pupils were sluggish but reactive to light. Extraocular muscles were intact; accommodation could not be tested. Fundi showed no papilledema, and visual fields were hard to assess. Conjugate gaze was with left preference. There was minimal flattening of the right nasal labial fold. Other cranial nerves could not be assessed. Deep tendon reflexes of the brachial radialis, triceps, and patella were 3+ on the right side and 2+ on the left. The Achilles tendon reflex was 2+ bilaterally with a questionable Babinski sign on the right side. Decreased motor function and muscle tone were greater on the right upper extremity than on the right lower extremity, with strong sensory pain withdrawal on the left side compared with the right.
Laboratory evaluations revealed the following values: white blood cell count (WBC) 8.6 x [10.sup.3]/[mm.sup.3], hemoglobin 14.4 g/dL, hematocrit 42.0%, platelet count 220 x [10.sup.3]/[mm.sup.3], neutrophils 84%, lymphocytes 8%, monocytes 7%, and eosinophils 1%. Sodium level was 135 mEq/L, potassium 3.7 mEq/L, chloride 100 mEq/L, C[O.sub.2] 23 mEq/L, BUN 15 mg/dL, creatinine 1.0 mg/dL, glucose 120 mg/dL, and sedimentation rate 6 mm/h. Total cholesterol was 207 mg/dL and triglycerides 168 mg/dL. The drug and alcohol screening tests were negative. An ANA test was within normal limits. Arterial blood gases on room air showed a pH of 7.4, PC[O.sub.2] at 38 mm Hg, P[O.sub.2] at 96.8 mm Hg, and bicarbonate 25 mEq/L.
A lumbar puncture was within normal limits. A computed tomographic (CT) scan of the brain showed no evidence of hemorrhage. Doppler ultrasongraphy of the carotid artery was negative. Transthoracic echocardiography showed no mass, thrombi, or valvular abnormality. An electrocardiogram (ECG) showed normal sinus rhythm with no ectopy or ST changes. A magnetic resonance imaging scan (MRI) of the brain 2 days later showed basal ganglia and temporal m/d parietal ischemic changes on the left side compatible with a left middle cerebral artery infarct.
Although the standard workup of the patient revealed no abnormalities, including a transthoracic echocardiogram that failed to show any cardiac thrombus or tumor, other conceivable causes had to be investigated before the patient's condition was diagnosed as an idiopathic stroke and he was sent for rehabilitation. (6) In this case, the patient had a transesophageal echocardiogram that revealed a 3.0-cm left atrial myxoma, the cause for his embolic stroke. This was done as a result of wanting to leave "no stone unturned." If the patient had been sent off to rehabilitation without this diagnosis, stroke recurrence would be extremely high with devastating results in the young adult, such as permanent brain necrosis from atrial myxoma showers. (6) The patient was transferred to a tertiary care medical center, where open heart surgery and removal of the benign atrial myxoma was completed without incident.
At present, the patient is not taking any anticoagulation medication, he is in speech and occupational rehabilitation, and doing quite well. He is currently taking lisinopril 10 mg daily, digoxin 0.25 mg daily, metoprolol 150 mg 1/2 twice daily, and hydrochlorothiazide 25 mg/triamterene 37.5 mg daily.
All primary tumors of the heart are rare (0.001% to 0.28% of autopsy cases). (8) Atrial myxomas ate the most common, with 80% of them found in the left atrium. Other primary cardiac tumors include rhabdomyomas, fibrous histiocytomas, hemangiomas, and different types of sarcomas. The more common metastatic tumors to the heart include bronchogenic carcinoma, breast carcinoma, malignant melanoma, lymphomas, renal cell carcinoma, and Kaposi's sarcoma. (8)
Atrial myxomas are usually pathologically benign with the potential to embolize systemically, and are diagnosed more frequently in middle-aged women than men. Diagnosis is usually accomplished with the aid of echocardiography, MRI, (8,10) or cutaneous biopsy of emboli material. (11) Cardiac myxomas have surgical cures, low recurrence, and good long-term survival. (12) Patients may present with fever, weight loss, and malaise, with an increased erythrocyte sedimentation rate and leukocytosis, or with cardiac obstruction of blood flow or signs of peripheral, cerebral, or pulmonary embolization (depending on the tumor location). With cardiac obstruction, the patient may present with right-sided heart failure (right-sided tumors) of transient pulmonary edema exacerbated in the upright position (gravity pulling the tumor into the mitral valve) with left atrial tumors (Figure 1).
[FIGURE 1 OMITTED]
On physical examination, a diastolic "tumor plop" sound or a mitral stenosis-like murmur may be auscultated. Numerous cutaneous manifestations may also be present. For example: livedoid macules, (11) an erythematous papular eruption of the extremity associated with claudication, (13) nonblanching lesions of the fingertips, (14) necrosis to the toes, (15) Raynaud's phenomenon, (16) and erythema and petechiae of the hands and feet. (13) Also, complex syndromes where myxomas and cutaneous lesions are included are: NAME syndrome (nevus, atrial myxoma, and neurofibroma ephelides (17)), and LAMB syndrome (lentigines, atrial myxomas, and bine nevi (18)). Tachyarrhythmias with or without ST changes on ECG may also be subtle clues.
Atrial myxomas ate difficult to diagnose given their rarity, and between 5% amd 9% are missed on transthoracic echocardiography. (8,19) However, trans-esophageal echocardiography (TEE) has a 100% diagnosis rate of atrial myxomas, (8) followed by 78.4% by CT and 70% by MRI (8) (Figure 2). There must be a high index of suspicion on the part of the physician and a persistent pursuit to uncover the cause of a stroke in a concise and well-thought-out workup.
[FIGURE 2 OMITTED]
Thanks are extended to Robert M. Wheeler for his technical assistance in preparing the echocardiogram photos.
(1.) Mohr JP, Kase CS, Adams RD. Cerebral vascular diseases. In: Petersdorf RG, Adams RD, Braunwald E, Isselbacher KJ, et al. Harrison's principles of internal medicine. 10th ed. New York, NY: McGraw-Hill, 1993:2028-60.
(2.) Goya W, Shaper AG. Physical activity and stroke in British middle-aged men. BMJ 1992; 304:597.
(3.) Robbins AS, Manson JE, Lee IM, et al. Cigarette smoking and stroke in a cohort of US male physicians. Ann Intern Med 1994; 120:458.
(4.) Holland RW III, Marx JA, Earnest MP, et al. Grand mal seizures temporally related to cocaine use: clinical and diagnostic features. Ann Emerg Med 1992; 21:772.
(5.) Asinger RW, Hart RG, Helgason CM, et al. Predictors of thromboembolism and atrial fibrillation: I. Clinical features of patients at risk. Ann Intern Med 1992; 116:1-5.
(6.) Browne WT, Wijdicks EF, Parisi JE, Viggiano RW. Fulminant brain necrosis from atrial myxoma showers. Stroke 1993; 24:1090-2.
(7.) Broderick JP, Brott T, Tomsick T, et al. The risk of subarachnoid and intracerebral hemorrhages in blacks as compared to whites. N Engl J Med 1992; 326:733.
(8.) Engberding WG, Daniel R, Erbel W, et al. Diagnosis of heart tumours by transesophageal echocardiography; a multicentre study in 154 patients. Eur Heart J 1993; 14:1223-8.
(9.) Massie BM. Heart. In: Tierney LM, McPheee ST, Papadakis MA, eds. Current medical diagnosis and treatment. 35th ed. Stamford, Conn: Appleton & Lange, 1996:380-1.
(10.) Gee GT, Brazan C, Jinkins JR. Imaging of cerebral infarction caused by atrial myxoma. Neural Radiology 1994; 36:270-2.
(11.) Navarro PH, Bravo FP, Gustavo GB. Atrial myxoma with livedoid macules as its sole cutaneous manifestation. J Am Acad Dermatol 1995; 32:881-3.
(12.) De-Carli S, Sechi LA, Ciani R, et al. Right atrial myxoma with pulmonary emboli. Cardiology 1994; 84: 368-72.
(13.) Houston KA, Combs JJ, Lie JT, et al. Left atrial myxoma simulating peripheral vasculitis. Mayo Clin Proc 1978; 53:752-6.
(14.) Byrd WE, Matthews OP, Hunt RT. Left atrial myxoma presenting as a systemic vasculitis. Arthritis Rheum 1980; 23:240-3.
(15.) Kounis NG. Left atrial myxoma presenting with intermittent claudication and Raynaud's phenomenon. Echocardiographic patterns of the tumor size. Angiology 1979; 30:356-60.
(16.) Leonhardt ETG, Kullenberg KPG. Bilateral atrial myxomas with multiple atrial ancurysms. A syndrome mimicking polyarthritis nodosa. Am J Med 1977; 62:792-4.
(17.) Atherton TJ, Pitcher DW, Wells RS, et al. A syndrome of various cutaneous pigmented lesions, nixoid neurofibromata and atrial myxoma; the NAME syndrome. Br J Dermatol 1980; 103:421-9.
(18.) Rhodes AR, Silverman RA, Harris TTJ, et al. Mucocutaneous lentigines, cardiomucocutaneous myxomas, and multiple blue nevi; the LAMB syndrome. J Am Acad Dermatol 1984; 10:72-82.
(19.) Mohsin A, Rossman HS, Grullon C. Transesophageal echocardiography in the evaluation of atrial masses. Angiology J Vase Dis 1995; 46:123-8.
David C. Robinson, DO Murrieta, California
Submitted, revised, January 26, 1997. Requests for reprints should be addressed to David C. Robinson, DO, Walsh Medical Arts Building, 25405 Hancock Avenue, Suite 108, Murrieta, CA 92562.
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|Author:||Robinson, David C.|
|Publication:||Journal of Family Practice|
|Date:||Apr 1, 1997|
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