Visual disturbances, dysartria, rt. hemiparesis

1. Visual disturbances, dysartria, rt. hemiparesis • 59 year-old, right handed female • Borned in USSR • Previously in good health, except Migraine since age 28 • General weakness and weight loss during last year • No family history of cerebrovascular disease • She did not smoke or consume alcohol

2. Visual disturbances, dysarthria, rt. hemiparesis • The patient developed the acute onset of dizziness, right hemiparesis , dysarthria and visual disturbances • By the following morning the patient was taken to the emergency room, there three parenchimal intracerebral hemorrhages with small subarachnoid component were diagnosed by brain CT • The CT angio of brain vessels was interpreted as normal

3. Brain CT showing : A) Lt. occipital hyperdence lesion with mild edema . B) small Lt. periventricular hyperdence lesion. C) Lt. parietal hyperdence lesion with edema D) all lesions are seen on the saggital projection +++++++++++++++++++++++++++++++++++++++\89=8

4. Physical and neurological examination • Cachectic woman with grade II apical systolic murmur • Fully oriented • The cranial nerves were normal except right homonymous hemianopia, decreased visual acuity in the left eye (6/30), left RAPD, mild pallor of the left eye optic disc and weakness in the lower part of right facial musculature • Right hemiparesis with 2/5 strength in the arm and 4/5 strength in the leg. The muscle tone was normal in all four limbs • Brisk, 3+ reflexes on right side • Sensory examination was intact • The coordination was normal

5. laboratory work-up • Normal CBC and blood chemistry • increased CRP (3,6) • Negative VDRL • No evidence of a connective tissue disorder or coagulation abnormality • The fasting lipogram and glucose level were both normal • ECG and chest radiograph were normal. The total body CT was unremarkable.

6. ?

7. Lt Cardiac Myxomaand Cerebral Hemorrhage • At the second day of hospitalization the transthoracic echocardiogram was performed and myxoma was found in the left atrium. • The patient had been receiving a symptomatic treatment during her 26 days long hospitalization in the neurology department. During this period she had substantial improvement in her right hemiparesis, control brain CT showed no new lesions and some improvement in old lesions . Then she was transferred to the thoracic surgery department.

8. Lt Cardiac Myxomaand Cerebral Hemorrhage

9. Cardiac myxomas • There are slowlygrowing intracavitary neoplasms of theheart • They are predominantly benign(75%) • The incidence - 0.05% of all necropsies - less frequent than metastaticcardiac tumors • Cardiac myxoma can be a sporadic lesion (93% of cases) • Cardiacmyxoma can also be a component of AD hereditary syndrome called Carney complex • About 80% are located in the leftatrium

10. Carney complex • Spotty pigmentation (bluenevi and lentigines) • Myxomas (cardiac, cutaneous, andmammary) • Endocrine over-activity (Cushing’s syndromeand acromegaly), testicular tumors • Schwannomas • Cardiac myxomas of Carney complexare histologically indistinguishable from sporadiccardiac myxomas

11. Facial and labial lentigines (small, pigmented macules)

12. Clinical triade: • Intracardiacobstruction • Constitutional symptoms likefever, weight loss, anemia and raised erythrocytesedimentation rate • Embolism, both cerebral and peripheral • Systemic embolism hasbeen reported in 30–50% of patients with left atrialmyxoma, half of them with cerebral involvement

13. Cardiacmyxoma may produce and release interleukin-6 , this cytokine may beresponsible for the constitutional symptomssuch as weight loss and fever • The frequency of recurrences in cardiac myxomasvaries between 3% for sporadic cases and 22% for casesof Carney complex

14. Fusiformaneurysms • The formationof cerebral aneurysm after tumor removal israrely documented, only approximately 40 casescould be found in the literature • Most aneurysms (91%) were ofthe fusiform type

15. No. of patients 34 Sex (female) 24 (71%) Median age of onsetFemale 37.5 (6–68) Male 33.5 (17–44) Median age at first neurological symptoms Female 38.5 (10–69)Male 33.5 (17–51) Preoperative signs: Constitutional symptoms (weight loss, fever, weakness, etc.) 6 (20%) Chest pain 2 (7%) Dyspnea 1 (3%) Palpitations 5 (13%) Migraine-like headache 4 (13%) Embolism - Peripheral vessels 7 (23%) - Cerebral vessels 23 (74%) Cerebral hemorrhage 4 (13%) Cerebral aneurysm 19 (61%) Demographic and clinical data: myxoma andcerebral aneurysm

16. Demographic and clinical data: myxoma andcerebral aneurysm • Delayed neurological complications (n =15) • Time from tumor resection until delayedneurological symptoms (months; median, range) 36.0 (2–300) • Time from tumor resection until aneurysmdetection (months; median, range)36.0 (2–300) • Cerebral infarction 9 (60%) • Cerebral hemorrhage 4 (27%) • Cerebral aneurysm 15 (100%)

17. Left internal carotid angiogram revealed a fusiform aneurysm of the A2 segment of the left anterior cerebral artery with subsequent occlusion

18. Current hypothesis favors that tumor material from cardiac myxoma embolizes into the vasa vasorum of peripheral arteries and subsequently proliferate in the vessel wall leading to a weakening of subintimal tissue such as the internal elastic lamina with subsequent aneurysm formation. Due to the very slow growingbehavior of the myxomatous tissue, there is avarious delay between embolism of the tumormaterial and the destruction of the vessel wall withaneurysm formation

19. Tumorfragments that have metastasized to cerebral vessel wallsmay enlarge, causing vessel occlusion and delayedinfarction or they may penetrate through the vesselwall, forming intra-axial metastases

20. Treatment ? • There is still no definitive treatment • Chemotherapy: results of doxorubicin alone areequivocal • Low-dose radiation therapy inconjunction with chemotherapy revealed moreencouraging results • Cardiac surgery toremove the primary cardiac tumor usually eliminatesearly neurologic symptoms, but cannot completelyabolish the risk of delayed cerebralaneurysm formation, presumably as a result ofmetastatic seeding prior to surgery

21. Recommendations: • In light of the potentiallypreventable nature of these lesions, the diagnosis of myxomatous aneurysms should be considered inany patient with neurologic symptoms and ahistory of cardiac myxoma • clinical and MRI investigations should be performedregularly and combined low-dose radiationand chemotherapy should be considered in patientswith growing aneurysms