Presentation on theme: "A Stroke from the Bishop’s Cap"— Presentation transcript:
1 A Stroke from the Bishop’s Cap Thomas A. Showalter, III, DOResident, Internal MedicineCPCAugust 10th, 2007
2 The Case CC: ‘my right arm and leg are weak’ HPI: 22 year old male 5 weeks priorRight arm and leg numbness and weaknessMild dysarthriaNight prior beers, smoked pot, taken 2 vicodin Presented to outside ERTreated with IVF for dehydration and sent home.
3 The Case continued… Same day after ER discharge Developed twitching of right side of face and armReturned to ERCT head negativeAll symptoms resolved with 4-5 hoursExcept for mild residual right facial numbness2 days laterReported mild dysarthriaRight facial droop and right facial numbnessReferred to Neurology at S&W
4 The Case continued… HPI: Seen 4 weeks later Denied paresthesias, paresis, involuntary movements or dysarthria.Complained of mild headache and sonophobia.PMHx:Substance abuseSpontaneous Pneumothorax at age 19One episode, 1 year ago with right sided numbness, but no paresis.
5 The Case continued… PSH: Appendectomy as child Chest tube placement for Spontaneous PTxMeds:noneAllergies:
6 The Case continued… Social Hx: Construction worker +Tobacco. +THC weeklyEtoH daily with binges on weekendMethamphetamine once monthlyNo IV drug abuseFamily Hx:Parents healthy. Sister unknown stomach disorder. No early stroke, hypercoagulable disorders, seizures or malignancy.
7 The Case continued… Review of Systems: No fevers, weight loss, night sweats.No arthralgias or myalgias. No rashes.No abdominal pain. No chest pain.No dyspnea. No palpitations.No dysphagia. No melena/hematochezia.No hematuria/dysuria.No syncope. No easy bruising or bleeding.
8 Physical Exam VS: T 95.7 P 70 R 12 BP 105/62 Gen: thin male, NAD, healthy appearing.HEENT: normalNeck: normalHeart: normalChest: normalExtr: normalNeuro: normalSkin: normal
9 The Data Diff: Neut 54% Lymph 27% Mono 7% LFTs Normal Coags Normal 13.68.727339.0Diff:Neut 54%Lymph 27%Mono 7%LFTs NormalCoags Normal
10 The Data continued… CXR: normal EKG: marked sinus bradycardia with sinus arrhythmia, early repolarizationMRI Brain:chronic left MCA infarct and several chronic tiny cerebellar infarctsMRA Head/Neck: normal Circle of Willis and great vessels of head and neck
11 The Data continued… Additional lab obtained… ESR 10 Total Cholesterol 138Anticardiolipin IgG and IgM – negativeLupus anticoagulant – negativeEchocardiography:normal LV function, 1.7 cm x 1.2 cm mass on the atrial side of the anterior leaflet of mitral valve possibly attached by a stalk
12 Hospital Course BC for bacterial and fungal organisms done. Cardiac MRI:mass on atrial surface of mitral valvewith no clear stalkImpression: mass consistent with either tumor or vegetation from infective endocarditis, but more likely endocarditis based on the location and that no stalk was seen on the MRI.A diagnostic procedure was performed.
13 The CaseProblem ListDDx of Stroke in a Young PersonDDx of a Left Atrial MassConclusions
14 The Problem List Multiple chronic strokes Left MCA distribution infarctMultiple tiny cerebellar infarctsLeft Atrial Mass (with or without a stalk)Substance abuseHeadache with sonophobiaHistory of…Right arm and leg numbness and weaknessDysarthria and facial numbness and weaknessMuscle spasm of right face and right arm
15 Stroke in Young Adults Usually defined as age < 45 years Worldwide incidence 9-11 per 100,000 Northern Manhattan Stroke Study, Stroke 2002Multiethnic population of 210,000 residentsIn a 4-yr period, 74 cases of young stroke out of 924 incident first ever strokes (8%)Higher incidence rates in Blacks and Hispanics compared to Whites
16 Stroke in Young AdultsItalian epidemiological review by Gandolfo and Conti Neurological Science 2003Western European Countries, less than 5% of all strokes occurred in patients < 45 years of age (yoa).Developing countries had 20-30% of strokes < 45 yoaUnited States, 8-10% of strokes in patients < 45 yoaEstimated lifetime cost of stroke $103,576 for US patients times that is young adult due to longer period of lost productivity 
17 DDx of Stroke in Young Adults Subarachnoid HemorrhageIntracerebral HemorrhageCerebral Ischemic InfarctsSAH - Leading causeRuptured Saccular aneurysmBiller and associates found ruptured aneurysms in 75 of 95 (79%) patients with SAH between years of ageIntracerebral HemorrhageEtiologyRuptured arteriovenous malformationRuptured saccular aneurysmSympathomimetic drug use
18 Cerebral Ischemic Infarct 3% of all cerebral infarcts occur between years of age EtiologiesAtheroscleroticNonatheroscleroticCardioembolicAges 15-35, cardioembolic and nonatherosclerotic causes predominateAfter age 35, traditional atherosclerotic stroke risk factors become prime determinants of stroke
19 Atherosclerotic causes Traditional risk factors:Hypertension, Smoking, Hyperlipidemia, Diabetes MellitusAge > 2%Age > 30-35%Homocystinuria – premature large vessels diseaseCarotid Atheroma formation due to local radiation for laryngeal tumorsCranial radiation produces a radiation vasculopathy
20 The Myriad of Nonatherosclerotic causes DissectionIllicit drug useInfectionProthrombotic StatesMigraineSickle Cell DiseaseGenetic DisordersInborn Errors of MetabolismMoyamoyaHyperestrogenemic StatesVasculitisArteritis due to Neoplasms
21 The Myriad of Nonatherosclerotic causes DissectionIllicit drug useInfectionProthrombotic StatesMigraineSickle Cell DiseaseGenetic DisordersInborn Errors of MetabolismMoyamoyaHyperestrogenemic StatesVasculitisArteritis due to Neoplasms
24 The Problem List Multiple chronic strokes Left MCA distribution infarctMultiple tiny cerebellar infarctsLeft Atrial Mass (with or without a stalk)Substance abuseHeadache with sonophobiaHistory of…Right arm and leg numbness and weaknessDysarthria and facial numbness and weaknessMuscle spasm of right face and right arm
25 DDx of Left Atrial Mass Endocarditis Left Atrial Thrombus Tumor Nonbacterial Thrombotic Endocarditis (Marantic)Libmann Sachs endocarditisBacterial endocarditisLeft Atrial ThrombusTumorMetastaticPrimary, Benign or Malignant
26 Marantic Endocardits Nonbacterial Thrombotic Endocarditis “Sterile” VegetationsMicroscopic to large aggregates of platelets and fibrin on heart valves (usually aortic or mitral)27% of ischemic stroke in patients with cancer Complicates many nonmalignant wasting illnesses, i.e. AIDSContinuum with Trousseau’s SyndromePredisposed by prothrombotic states, valvular endothelial disruption and underlying valve disease
27 Libmann Sachs Endocarditis Verrucous EndocarditisAccumulation of immune complexes, mononuclear cells, hematoxylin bodies, fibrin and platelet thrombiOccurs in minority of Systemic Lupus ErythematosisFewer seen in Antiphospholipid Antibody SyndromeMost commonly the Aortic or Mitral valve, although the Tricuspid may be affectedTypically asymptomatic, but if large enough may embolize
28 Infective Endocarditis Microbial infection of the endocardial surfaceVegetation – platelets, fibrin, microorganisms and inflammatory cellsIn the U.S., incidence of community-acquired native-valve endocarditis = 1.7 to 6.2 cases per 100,000 person-yearsMedian age = yearsInjection drug usersHigher incidence in younger personsIncidence 150 to 2000 per 100,000 person-years
29 Clinical Manifestations of Infective Endocarditis Fever – most common sign and symptomSubacute – anorexia, weight loss, malaise and night sweatsHeart murmur – new or changing, but usually preexistingPetechiae on skin, conjunctivae or oral mucosaSplenomegalyCongestive heart failureSplinter hemorrhages, Osler’s nodes, Janeway’s lesionsNeurologic complications:20-40% will have neurologic complication!65% of embolic phenomena involve the CNS
30 The Duke CriteriaIntroduced by group at Duke University in 1994, modified in 2000Specificity 99%NPV > 92%Criteria integratedFactors predisposing patients to the development of endocarditisBlood culture isolate and persistence of bacteremiaEchocardiographic findingsTTE – Specificity 98%, Sensitivity 60-70%TEE – Specificity 85-98%, Sensitivity 75-95%, NPV > 92%Other clinical and laboratory findingsOnly 5-7% of patients have sterile blood cultures*
32 Left Atrial Thrombus 45% of cardiogenic thromboemboli 13% patients with atrial fibrillation33% patients with rheumatic mitral stenosisMay complicate primary or metastatic tumorsRegional or global wall motion abnormalities increase riskAssociated with the left atrial appendageGenerally, attached to posterior left atrial wall by a broad base, therefore immobileCan be pedunculated and mobile
33 Left Atrial Thrombus Omran in 2000 Sinus Rhythm1% incidence of left atrial thrombus in patients with recent neurologic deficit6/583 patients (1%) had left atrial appendage thrombus3 mitral stenosis, 1 aortic stenosis, 1 dilated cardiomyopathy, 1 coronary artery diseaseLeft atrial thrombi are an infrequent cause of thromboembolism in patients in sinus rhythm and are associated with valvular disease and atrial dysfunction.*Left atrial thrombus is associated with left atrial tumors…
34 Cardiac Tumors Metastatic Primary 20-40 times more common than primary tumorsPericardium > Myocardium > Endocardium10-20% patients with disseminated cancer will have involvement of heart or pericardiumPrimary% incidence in unselected autopsy series1 in 500 cardiac surgical cases, with exception of myxomaBenign -> 75%Myxomas comprise 50% of benignMyxomas comprise 80-90% of left atrial primary tumorsMalignant -> 25%Sarcomas comprise 75% of malignant
35 Frequency of Cardiac Tumors Atlas of Heart Disease: Cardiopulmonary Diseases and Cardiac Tumors. Vol III Philadelphia: Mosby.
36 Relative Incidence of Primary Malignant Tumors of the Heart Adults (%)Children (%)Angiosarcoma286Rhabdomyosarcoma1141Fibrosarcoma818Malignant fibrous histiocytomaOsteosarcoma7Leimyosarcoma5Myxosarcoma3Other sarcomas*1412Undifferentiated sarcomaLymphoma*Other sarcomas include liposarcomas, synovial and neurogenic sarcomasAdapted from Braunwald’s Heart Disease 7th edition
37 Malignant Cardiac Tumors 25% of all cardiac tumors are invasive or metastatic95% of these are Sarcomas(2nd to myxoma in overall frequency)5% are LymphomasSarcomas derive from mesenchyme, therefore have a wide variety of morphological typesMutations in K-ras were seen in most cardiac sarcomasAny age, but most common third and fifth decadesExcept for rhabdomyosarcomas and fibrosarcomas, distinctly unusual in infants and children
38 Malignant Cardiac Tumors 25-50% patients will have metastatic disease at time of diagnosisMost frequent: lungs, thoracic lymph nodes, mediastinum and vertebral columnLess frequent: liver, kidneys, adrenals, pancreas, bone, spleen and bowelTransesophageal Echocardiography recommended for diagnosisCT and MRI show degree of tumor infiltrationOften endomyocardial or open biopsy needed
39 Treatment of Cardiac Sarcomas Sarcomas proliferate rapidlyDeath due to widespread infiltration of the myocardium, obstruction of flow within the heart or distant metastasis with a few weeks to 2 years after onset of symptomsMedian survival 6-12 monthsSurgical excision considered to achieve local control and relieve symptomsComplete excision - median survival monthsPossible in less than 50% of patientsIncomplete excision – median survival 3-10 months
40 Treatment of Cardiac Sarcomas AutotransplantationCardiac explantation, ex vivo tumor resection, cardiac reconstruction and reimplantationChemotherapeutic benefits are unclearData support anthracycline-based regimens is soft tissue sarcomasAdjuvant chemotherapy and/or radiation therapy usually recommendedOrthotopic heart transplantation in patients with locally unresectable disease without evidence of metastasis66% still die within 1 year either of locally recurrent or metastatic disease
41 Angiosarcoma 30% of primary cardiac sarcomas 3:1 male-to-female ratio Predilection for right atrium, may be either intracavitary and polypoid or diffuse and infiltrativeLater forms have sheet-like covering of pericardiumUsually present with right-sided heart failure or tamponadeSystemic signs such as fever and weight loss
42 Angiosarcoma“Cauliflower” appearance on MRI due to areas of hemorrhage and necrosisTend to be discovered ‘late’, often already metastasizedOften not amenable to complete resectionVery poor prognosisKaposi Sarcoma (HSV 8)Less than 5% of pts with AIDS or solid organ transplant
43 RhabdomyosarcomaMost common cardiac malignancy in infants and children10% of all primary cardiac sarcomasDiffusely infiltrate the ventricular myocardiumMay on occasion form polypoid extension into chamberUsually multiple foci with occasional nodular involvement of pericardiumRhabdomyoblast – histological hallmark
44 Fibromyosarcoma 5-10% of cardiac sarcomas Fibroblastic in differentiation, composed of spindle-shaped cells containing areas of hemorrhage and necrosisExtensively infiltrate the heartOften involving more than one chamber spreading to the pericardium
45 Relative Incidence of Benign Tumors of the Heart Adults (%)Myxoma52Papillary fibroelastoma16LipomaRhabdomyoma1Fibroma3TeratomaHemangioma6Other tumors*5*other tumors include cystic tumors of the atrioventricular node,endocrine tumors and histiocytoid tumorsAdapted from Braunwald’s Heart Disease 7th edition
46 Relative Incidence of Benign Tumors of the Heart Adult (%)Children (%)Myxoma5217Papillary fibroelastoma16LipomaRhabdomyoma142Fibroma318Teratoma12Hemangioma65Other tumors*4*other tumors include cystic tumors of the atrioventricular node,endocrine tumors and histiocytoid tumorsAdapted from Braunwald’s Heart Disease 7th edition
47 Rhabdomyoma Most common cardiac tumors in infants and children ¾ occur in patients younger than 1 yearLeft and right ventricular and septal myocardium1/3 involve either or one atriaNearly all are multipleSmall, lobulatedDiameter range 2 mm to 2 cm
48 RhabdomyomaMost common presentation is heart block or other arrhythmiasEcho: multiple small, lobulated, homogenous, hyperechoic intramural tumorsAssociation with Tuberous Sclerosis (80%)HamartomasEpilepsyMental deficiencyAdenoma sebaceum
49 Lipoma Rare Occur at any age with equal male/female ratio Diameter 1-15 cmSessile or polypoidOccur in the subendocardium or subpericardium,¼ are completely intramuscularMost common chambers affected are the left ventricle, right atrium and interatrial septum
50 Primary Cardiac Valve Tumors Retrospective study by Edwards et al 1991from 1932 to 1990Walter Reed and Brooke Army Medical Centers53 patients had 56 primary cardiac valve tumorsAge range 2 to 88 yearsAverage 52 years of age – 79% maleAortic valve most commonly affectedTumor size ranged 3 mm to 7 cmAverage size = 1.15 cm
51 Primary Cardiac Valve Tumors 52/56 (93%) tumors were benign41 papillary fibroelastomas5 myxomas4 fibromas1 hamartoma1 hemangiomaMitral valve most commonly symptomatic8/53 (15%) patients had neurologic symptoms6 had mitral valve tumors3 myxomas and 3 malignancies
52 Comparison with Nonvalvular Cardiac Tumors Most common nonvalvular -> MyxomaMost common valvular -> Papillary fibroelastomaEdwards et al “Primary Cardiac Valve Tumors” Ann Thorac Surg 1991; 32:1131
53 Papillary Fibroelastoma Most common tumor of the cardiac valvesAverage age 60 at detection (range neonates to 92 yoa)Men = WomenMost have concomitant valvular disease suggesting this may predispose to papillary fibroelastoma90% solitary
54 Papillary Fibroelastoma Median diameter = 8 mm, largest reported 4 cmAny valve, aortic and mitral most commonlyarterial side of semilunar valvesatrial side of AV valvesShort pedicle 50% of time
55 Papillary Fibroelastoma Path Grossly, a frond-like appearance resembling a sea anemoneHistological, numerous papillary fronds consisting of a collagen core surrounded by elastic fibers and loose connective tissue, all covered by endocardial endothelium
56 Myxomas 3rd and 6th decades of life Mean age at presentation = 50 yearsAge range = newborn to 95 years2/3’s females
57 Myxomas Location 75% Left Atria 15-20% Right Atria 3-4% Left Ventricle 3-4% Right VentricleAttachmentInteratrial SeptumLimbus of Fossa OvalisPosterior Atrial wallAnterior Atrial wallAtrial appendageRarely endocardial
58 Myxomas Average size 5-6 cm in diameter Size Range < 1 to > 15 cmEcho shows a mobile,distensible tumor connectedto the interatrial septum by a narrow stalkPolypoidUsually pedunculatedRound or oval with smooth or lobulated surface
59 Myxoma Path Embryonic mesenchymal cells with multipotent capability Myxoid matrix of acid- mucopolysaccharide- rich stromaImmunohistochemical studiesVimentinneuroendocrine markers (S-100)gene product 9.5 and calretinin
60 Myxomas Surface of tumor is often covered with thrombi Embolism occurs in 30-40% of patientsUsually systemic, majority are cerebralInfected myxomas have been describedGreater danger of systemic embolismExcised Villous Left Atrial Myxoma
61 Myxomas Intracardiac obstruction Systemic embolization 70% have heart failure or syncopeDyspnea, pulmonary edema, sudden deathSystemic embolization30% of patients with myxoma with 2/3 cerebral25% with emboli have evidence of multiple embolic eventsConstitutional symptomsUnique to myxoma, 30-40% of patientsSynthesis of interleukin – 6Fatigue, fever, erythematous rash, arthralgia, myalgia and weight loss
62 Myxomas The Physical Exam The “Wrecking Ball Effect” Murmur heard > 50%Diastolic due to obstructed filling of the ventricleSystolic due to interference with closure of the AV valveS1 often loud and widely splitDelay in closure of the AV valve“Tumor Plop” in 33% of patientsA diastolic murmur heard 80 to 150 msec after the second heart soundPericardial friction rubRight atrial tumorsThe “Wrecking Ball Effect”Recurrent collision with the pedunculated myxoma and the mitral valve may cause permanent damage
63 Myxomas Familial Myxomas Carney Complex 10% or less of all myxomas Autosomal dominantMedian age 20 yearsAtypical locations, often multiple and recurrent tumorsCarney ComplexMyxomas, Spotty skin pigmentation and endocrine overactivity
64 Treatment of Benign Cardiac Tumors Operative excision under direct vision using cardiopulmonary bypassSchaff and Mullany, 2000Orthotopic heart transplantAutotransplantation
65 DiscussionMyxomas are source of most tumor emboli because of their friable consistency and intracavitary location, but other types may embolize.“An embolic stroke in a young person without evidence of cerebrovascular disease, particularly in the presence of sinus rhythm, should raise the suspicion of intracardiac myxoma, as well as infective endocarditis.” – Braunwald’s Heart Disease, 7th Ed.
66 Discussion Multiple Strokes Left Atrial Mass Tumor Myxoma Papillary FibroelastomaTumor with or without thrombus?Tumor with or without infection?
68 ReferencesGandolfo, C. and M. Conti. “Stroke in young adults: epidemiology” Neurological Science 2003; 24: S1-S3.Jacobs, B.S., et al. “Stoke in the young in the Northern Manhattan Stroke Study” Stroke 2002; 33:Hart, R.G. et al., “Diagnosis and management of ischemic stroke. II. Selected controversies” Current Problems in Cardiology 1983; 8(7):Birgitte, H. et al. “Stroke in Young Adults and Children” Current Neurology and Neuroscience Reports 2001; 1:Martin, P.J., et al. “Causes of ischaemic stroke in the young” Postgraduate Medical Journal 1997; 73: 8-16.Guillon, B. et al., “Internal carotid artery dissection: an update” Journal of Neurologic Science 1998; 153:Sloan, M.A., “Illicit drug-associated ischemic stroke in the Baltimore-Washinton Young Stroke Study” Neurology 1998; 19:Biller, J. et al., “Spontaneous subarachnoid hemorrhage in young adults” Neurolosurgery 1987; 21(5):Mylonakis, E. and Calderwood, S.B., “Infective Endocarditis in Adults” NEJM 2001; 345(18):Cerebral Embolism Task Force. “Cardiogenic Brain Embolism. The Second Report of the Cerebral Embolism Task Force” Archives of Neurology 1989; 46:Klein, A.L. et al., “Use of transesophageal echocardiography to guide cardioversion in patients with atrial fibrillation” NEJM 2001 May 10; 344(19):Srimannaraya, J. et al., “Prevalence of left atrial thrombus is rheumatic mitral stenosis with atrial fibrillation and its response to anticoagulation: a transesophageal echocardiographic study” Indian Heart Journal Jul-Aug; 55(4):Omran, H. et al., “Incidence of left atrial thrombi in patients in sinus rhythm and with a recent neurologic deficit” American Heart Journal 2000; 140(4):Reardon, M.J. and Smythe, W.R. Cardiac Surgery in the Adult. 2nd Ed Chapter 58 Cardiac Neoplasms. New York: McGraw-Hill.Sabatine, M.S. et al. Braunwald’s Heart Disese: A Textbook of Cardiovascular Medicine 7th Ed Chapter 63 Primary Tumors of the Heart. Saunders.Edwards, F.H., et al. “Primary Cardiac Valve Tumors” Annals of Thoracic Surgery 1991; 52:Reynen, M.D. “Cardiac Myxomas” NEJM 1995; 333(24):The Grand TetonsThe Showalter Family
69 Special Thanks Dr. Chiles Dr. Elieson Dr. Sibbitt Dr. Mock Dr. Fillmore