March/April - West Virginia State Medical Association

Scientific Article |Moyamoya in a Non-Asian Patient: A Case Report andReview of the LiteratureDaniel Felbaum, MS-IVMarshall University Joan C. Edwards School of MedicineElise Henning, MS-IIIMarshall University Joan C. Edwards School of MedicineBryan R. Payne, MDProfessor and ChairmanMarshall University Joan C. Edwards School ofMedicine, Department of NeuroscienceAbstractMoyamoya disease (MMD) hashistorically been diagnosed in theJapanese population. However, newerstudies have demonstrated worldwidedistribution. Of note, patients ofAppalachian descent with no known Asianancestry have presented with MMD. Weare presenting a case of MMD in anAppalachian, non-Asian patient whopresented to the neurosurgical servicewith a severe headache of four daysduration. The patient was found to havemultiple hemorrhagic infarcts on CT andwas admitted to the ICU. Cerebralangiography findings confirmed thediagnosis of MMD. Our case providesinformation regarding signs andsymptoms, diagnostic neuroimagingfindings, and treatment modalities forMMD.Case ReportCHIEF COMPLAINTHeadacheHISTORY OF PRESENT ILLNESSD.B. is a 46-year-old Caucasianfemale of Appalachian descentwho presented to the EmergencyDepartment (ED) with an unremittingheadache of four days duration.Headache came on suddenly aftermoderate physical activity. Headachewas described as severe, global andcontinuous and dull. The patient tookover-the-counter NSAIDs and restedfor four days but did not experiencerelief of her symptoms. She wentto the ED after the fourth day ofunrelenting headache. Additionalcomplaint was difficulty walkingfrom lower extremity weakness. CTwithout contrast was performed uponadmission that revealed moderateintraventricular hemorrhage, smallintraparenchymal hemorrhage, andmild subarachnoid hemorrhage.PAST MEDICAL HISTORYMultiple sclerosisMajor depressive disorderSeizuresPost-surgical thrombophlebitisAppendectomyTotal hysterectomySOCIAL HISTORYCigarette smoking – 1pack per day for 20 yearsThree cups of coffee per dayMEDICATIONSCitalopram 40mg by mouth1 tablet every nightROS: Negative per HPIPHYSICAL EXAMPatient was intact neurologically.She was alert and oriented to person,place, and time. Cranial nerves2-12 were grossly intact. Strengthwas rated 5/5 in upper and lowerextremities. No sensory deficits to pinprick and light touch. Reflexes were2+ in four extremities. No dysmetriaand dysdiadochokinesia present.TREATMENT PLAN RENDERED:The patient was admitted to theICU. She received an angiogramto localize areas of hemorrhage.Angiogram showed bilateralocclusion of the middle and anteriorcerebral arteries, with mild stenosisof the left and right common carotidarteries. Rete mirabile – numerousbilateral collaterals and tangled,malformed arteries secondaryto increased collateral flow wasdemonstrated. Because she wasneurologically intact and presentedlate in the disease course, medicalmanagement was the agreed upontreatment. Surgical management viarevascularization was considered notappropriate because of the patient’swell-developed collateral circulation.The patient was counseled aboutMMD and instructed to modifyvascular risk factors, includingsmoking cessation and properdiet and exercise behaviors. Shewas discharged home whenheadache abated and was laterseen in clinic for follow-up.DiscussionOur patient is a middle-agedfemale who presented with an acuteintracranial hemorrhage. She isCaucasian and Appalachian with noknown Asian ancestry. The patientpresented to the ED after enduring afour-day headache. She complainedof weakness in her extremitiesbut denied other symptoms. Herprior history of seizures andmigraines could be attributed tominor hemorrhagic causes.MMD is derived from the Japaneseword “puff of smoke” due to theappearance of numerous collateralvessels seen on angiography. Inaddition to multiple collaterals,bilateral occlusion occurs in theterminal segment of the internalcarotid. 1 MMD tends to be bimodal,affecting children around 5 years16 West Virginia Medical Journal