Medical Image of the Week: Moyamoya Disease

Figure 1. Cerebral angiogram of the brain demonstrating bilateral high-grade stenosis of the anterior and middle cerebral arteries, worse on the left.

Figure 2. Magnetic resonance imaging showing multiple punctate infarcts in the frontal and parietal lobes on the left side.

A 52-year-old, right-handed, Caucasian woman with a history of hypertension and morbid obesity presented with acute onset of word-finding difficulty and slurred speech. Her medical and family history was negative for cerebral vascular event, coronary artery disease or smoking. Computed tomography of the patient's brain showed narrow caliber middle cerebral artery vasculature bilaterally. This abnormal finding prompted further investigation with cerebral angiogram. The angiogram showed bilateral high-grade stenosis of the anterior and middle cerebral arteries, worse on the left (Figure 1). Magnetic resonance imaging revealed multiple left sided punctate infarcts in the frontal and parietal lobes (Figure 2). Diagnosis of ischemic stroke secondary to moyamoya disease was established. This patient was not a candidate for fibrinolytic therapy since it had been more than 4 hours from initial presentation. She was treated with aspirin, clopidogrel, and atorvastatin for secondary prevention of ischemic stroke. Two months after her discharge date, the patient had a middle cerebral artery to superior temporal artery bypass on the left side, followed by a middle cerebral artery to superior temporal artery bypass on the right two months after initial bypass. Patient progressed to an uneventful recovery. Discharge planning included the continuation of aspirin, clopidogrel, and atorvastatin indefinitely.

Moyamoya disease (MMD) is an uncommon vasculopathy of unknown origin associated with diverse risk factors (1). It was first discovered in a Japanese population, and reported more commonly in this sub-population. However, numerous cases were reported across the globe (2). Moyamoya disease associated with other systemic condition such as neurofibromatosis type 1, trisomy 21, thyroid cranial irradiation or thyroid disease is termed moyamoya syndrome (MMS) (1,2). Moyamoya syndrome is a cerebrovasculopathy originating from collateral flow that develops secondary to occlusion of the internal carotid artery and the proximal afferent vessels at the circle of Willis (3). MMS can have abrupt or insidious onset and may progress to diversifying cerebral ischemic stroke or to intracranial hemorrhage, which is a worse prognosis and the primary cause of death in patients with MMD (4). It has been shown that ischemic stroke associated with MDD or MMS usually occurs when compensatory collateral vessels are unable to supply sufficient blood to the brain after occlusion or stenosis of the internal carotid arteries or its tributary vessels (5,6). On the other hand, intracranial hemorrhage occurs secondary to the rupture of abnormal moyamoya vessels (7,8).

It is imperative to differentiate between non-hemorrhagic and hemorrhagic moyamoya. Neuroimaging is the preferred method of diagnosis after high clinical suspicion of MMD or MMS. Intracranial hemorrhage and cerebral infarction can be diagnosed with computed tomography and magnetic resonance imaging/ cerebral angiogram, respectively (8,9). Recent use of magnetic resonance perfusion imaging has been shown to be crucial in diagnostics and medical-surgical decision making. There is no common consensus when it comes to treatment of moyamoya at this time. Initial management is symptomatic with anticoagulants, antiplatelet and corticosteroids (10). Treatment options may also include direct or indirect surgical revascularization as optimal therapy (11,12).

Stella Pak MD, Kokou Adompreh-Fia MD, Damian Valencia MD, Adam Fershko MD, and Jody Short DO.

Department of Medicine

Kettering Medical Center

Kettering, OH USA

References

1. Phi JH, Wang KC, Lee JY, Kim SK. Moyamoya Syndrome: A window of moyamoya disease. J Korean Neurosurg Soc. 2015 Jun;57(6):408-14. [CrossRef] [PubMed]
2. Suzuki J, Takaku A. Cerebrovascular "moyamoya" disease. Disease showing abnormal net-like vessels in base of brain. Arch Neurol. 1969 Mar;20(3):288-99. [CrossRef] [PubMed]
3. Yamamoto, S, Koh M, Kashiwazaki D, Akioka N, Kuwayama N, Noguchi K, Kuroda S. Is Quasi-moyamoya disease a uniform disease entity? A three-dimensional constructive interference in steady state imaging study. J Stroke Cerebrovasc Dis. 2016 Jun;25(6):1509-16. [CrossRef] [PubMed]
4. Baba, T., Houkin, K. Kuroda. Novel epidemiological features of moyamoya disease. J Neurol Neurosurg Psychiatry. 2008 Aug;79(8):900-4. [CrossRef] [PubMed]
5. Miyamoto S, Kikuchi H, Karasawa J, Nagata I, Ihara I, Yamagata S. Study of the posterior circulation in moyamoya disease. Part 2: Visual disturbances and surgical treatment. J Neurosurg. 1986 Oct;65(4):454-60. [CrossRef] [PubMed]
6. Kuroda S, Ishikawa T, Houkin K, Iwasaki Y. Clinical significance of posterior cerebral artery stenosis/occlusion in moyamoya disease. No Shinkei Geka. 2002 Dec;30(12):1295-300. [PubMed]
7. Kang K, Lu J, Zhang D, Li Y, Wang D, Liu P, Li B, Ju Y, Zhao X. Difference in cerebral circulation time between subtypes of moyamoya disease and moyamoya syndrome. Sci Rep. 2017;7(1):2587. [CrossRef] [PubMed]
8. Lui, P, Han C, Li DS, Lv XL, Li YX, Duan L. Hemorrhagic moyamoya disease in children: Clinical, angiographic features, and long-term surgical outcome. Stroke. 2016 Jan;47(1):240-3. [CrossRef] [PubMed]
9. Kellner CP, McDowell MM, Phan MQ, Connolly ES, Lavine SD, Meyers PM, Sahlein D, Solomon RA, Feldstein NA, Anderson RC. Number and location of draining veins in pediatric arteriovenous malformations: association with hemorrhage. J Neurosurg Pediatr. 2014 Nov;14(5):538-45. [CrossRef] [PubMed]
10. Whitaker J. Management of moyamoya syndrome [comment]. Arch Neurol. 2001;58:132. [CrossRef] [PubMed]
11. Golby AJ, Marks MP, Thompson RC, Steinberg GK. Direct and combined revascularization in pediatric moyamoya disease. Neurosurg. 1999;45:50-8. [PubMed]
12. Mizoi K, Kayama T, Yoshimoto T, Nagamine Y. Indirect revascularization for moyamoya disease: is there a beneficial effect for adult patients? Surg Neurol. 1996;45:541-8. [CrossRef] [PubMed] 

Cite as: Pak S, Adompreh-Fia K, Valencia D, Fershko A, Short J. Medical image of the week: moyamoya disease. Southwest J Pulm Crit Care. 2017;15(5):227-9. doi: https://doi.org/10.13175/swjpcc136-17 PDF

Medical Image of the Week: Lemierre Syndrome

Figure 1. CT scan of chest (axial image) demonstrating peripheral cavitating lesion (arrow) with multifocal ground glass opacities and bilateral pleural effusions.

Figure 2. CT scan of neck, soft tissue (coronal [A], axial [B] image) demonstrating a partially occlusive thrombus in the left internal jugular vein (coronal red arrows, axial green arrow).

A previously healthy 18-year-old girl was evaluated at an urgent care center for a three day history of sore throat, fever, nausea, vomiting, diarrhea, and myalgias; the diagnosis of influenza was made at that time. Four days later, she presented to our Emergency Department with sore throat, left sided neck pain and swelling, productive cough, fever, worsening dyspnea, and pleuritic chest pain. On examination her temperature was 36.9 °C, heart rate was 142 beats per minute, and respiratory rate was 18 breaths per minute. She had enlarged tonsils without exudates, cervical and submandibular lymphadenopathy, and tenderness of her left lateral neck. Lung examination showed increased work of breathing with decreased breath sounds at the bases. Laboratory evaluation revealed an elevated white count (17,000 cells/µL) with 91% neutrophils, elevated blood urea nitrogen (21 mg/dL), creatinine (1.6 mg/dL), and venous lactate (4.0 mMol/L). Initial chest radiograph showed no evidence for acute cardiopulmonary process.  She was admitted, blood cultures were drawn, and treatment for sepsis with vancomycin, xeftriaxone, and azithromycin was initiated. Subsequent chest radiograph demonstrated an ill-defined airspace opacification in the right lower lobe.

Computed tomographic (CT) imaging of the chest showed multifocal ground glass opacities and areas of consolidation with cavitation, consistent with septic embolic disease (Figure 1). Blood cultures grew Streptococcus anginosus and Fusobacterium necrophorum. CT imaging of the neck showed a partially occlusive thrombus in the left internal jugular vein (Figure 2).  The diagnosis of Lemierre’s syndrome was made. The patient required chest tube drainage for bilateral empyema and was treated with 3 weeks of ampicillin followed by 3 weeks of high dose amoxicillin-clavulanate; she recovered completely.

Lemierre's syndrome, or anaerobic postanginal sepsis, was first described by Andre Lemierre in 1936. It is characterized by thrombophlebitis of the internal jugular vein and bacteremia caused by organisms of the normal oropharyngeal bacterial flora, classically Fusobacterium necrophorum. Lemierre’s syndrome is most commonly preceded by pharyngitis or tonsillitis, but can also be associated with odontogenic infections or otitis media. The primary infection progresses from the oropharynx and invades the lateral pharyngeal space, eventually leading to thrombophlebitis of the internal jugular vein. A majority of patients develop septic emboli, as seen in our patient, with the lungs and large joints being the most common sites of metastasis. Lemierre’s syndrome predominantly affects previously healthy children, adolescents, and young adults with most cases presenting in the second decade of life. Common physical findings include severe pharyngitis, cough/hemoptysis, dyspnea, and tenderness and swelling over the internal jugular vein. Diagnosis is confirmed by the presence of thrombophlebitis of the internal jugular vein and anaerobic organisms such as F. necrophorum in the bloodstream.

Elisa Phillips BA, BS*, Ziad Shehab MD**, and Daniela Lax MD***

*The University of Arizona College of Medicine; **Department of Pediatrics, Division of Infectious Disease; and ***Banner – University Medical Group, Pediatric Cardiology

University of Arizona

Tucson, AZ USA

References

1. Bliss SJ, Flanders SA, Saint S. Clinical problem-solving. A pain in the neck. N Engl J Med. 2004 Mar 4;350(10):1037-42. [CrossRef] [PubMed]
2. Kuppalli K, Livorsi D, Talati NJ, Osborn M. Lemierre's syndrome due to Fusobacterium necrophorum. Lancet Infect Dis. 2012 Oct;12(10):808-15. [CrossRef] [PubMed]
3. Eilbert W, Singla N. Lemierre's syndrome. Int J Emerg Med. 2013 Oct 23;6(1):40. [CrossRef] [PubMed]

Cite as: Phillips E, Shehab Z, Lax D. Medical image of the week: Lemierre syndrome. Southwest J Pulm Crit Care. 2017;15(5):223-4. doi: https://doi.org/10.13175/swjpcc135-17 PDF