Challenging Cases in Cerebrovascular Disease

Abstract

Disorders involving the vascular system of the brain are numerous and sundry. Atherosclerotic thromboembolism of large vessels and lacunar infarctions of small vessel disease are well known. Brain infarction due to cardioembolism is common as well, and even more so when diligently sought. Rupture of intracranial blood vessels results in subarachnoid and intraparenchymal hemorrhage. We present four cases of stroke of uncommon cause and remind clinicians to be open minded to the many possible causes of stroke, in particular because early recognition and treatment is often critical. Case 1 discusses a patient with inflammatory cerebral amyloid angiopathy. The presentation, ability to recur, and current treatment considerations are reviewed. Case 2 discusses microangiopathic thrombotic angiopathy. Diagnosis and treatment are considered. An association with interferon therapy and the evolving terminology of this and related conditions are discussed. Case 3 discusses intracranial hemorrhage secondary to acute promyelocytic leukemia. Patients with acute leukemias require aggressive management of their coagulopathy, thrombocytopenia, and the disease itself. Finally, Case 4 discusses ischemic stroke due to a paradoxical embolism in the setting of a patent foramen ovale (PFO). Both medical and surgical management of a PFO for stroke prevention are considered.

Keywords

cerebral amyloid angiopathy - microhemorrhage - thrombotic microangiopathy - interferon therapy - acute promyelocytic leukemia - patent foramen ovale

Publication History

Accepted Manuscript online:
23 November 2022

Article published online:
09 January 2023

• References

• 1 Wu JJ, Yao M, Ni J. Cerebral amyloid angiopathy-related inflammation: current status and future implications. Chin Med J (Engl) 2021; 134 (06) 646-654
• 2 Auriel E, Charidimou A, Gurol ME. et al. Validation of clinicoradiological criteria for the diagnosis of cerebral amyloid angiopathy–related inflammation. JAMA Neurol 2016; 73 (02) 197-202
• 3 Renard D, Tatu L, Collombier L. et al. Cerebral amyloid angiopathy and cerebral amyloid angiopathy-related inflammation: comparison of hemorrhagic and DWI MRI features. J Alzheimers Dis 2018; 64 (04) 1113-1121
• 4 Crosta F, Orlandi B, De Santis F. et al. Cerebral amyloid angiopathy-related inflammation: report of a case with very difficult therapeutic management. Case Rep Neurol Med 2015; 2015: 483020
• 5 Regenhardt RW, Thon JM, Das AS. et al. Association between immunosuppressive treatment and outcomes of cerebral amyloid angiopathy–related inflammation. JAMA Neurol 2020; 77 (10) 1261-1269
• 6 Antolini L, DiFrancesco JC, Zedde M. et al. Spontaneous ARIA-like events in cerebral amyloid angiopathy-related inflammation: a multicenter prospective longitudinal cohort study. Neurology 2021; 97 (18) e1809-e1822
• 7 Goldberg RJ, Nakagawa T, Johnson RJ, Thurman JM. The role of endothelial cell injury in thrombotic microangiopathy. Am J Kidney Dis 2010; 56 (06) 1168-1174
• 8 Hofer J, Rosales A, Fischer C, Giner T. Extra-renal manifestations of complement-mediated thrombotic microangiopathies. Front Pediatr 2014; 2: 97
• 9 Noorbakhsh-Sabet N, Pulakanti VC, Zand R. Uncommon causes of cerebral microbleeds. J Stroke Cerebrovasc Dis 2017; 26 (10) 2043-2049
• 10 Sonneveld MA, de Maat MP, Portegies ML. et al. Low ADAMTS13 activity is associated with an increased risk of ischemic stroke. Blood 2015; 126 (25) 2739-2746
• 11 Allinovi M, Cirami CL, Caroti L. et al. Thrombotic microangiopathy induced by interferon beta in patients with multiple sclerosis: three cases treated with eculizumab. Clin Kidney J 2017; 10 (05) 625-631
• 12 Gulleroglu K, Fidan K, Hançer VS, Bayrakci U, Baskin E, Soylemezoglu O. Neurologic involvement in atypical hemolytic uremic syndrome and successful treatment with eculizumab. Pediatr Nephrol 2013; 28 (05) 827-830
• 13 Elshazzly ME, Hammo B, Buhtoiarov IN. A case of subacute brain hemorrhage and disseminated intravascular coagulation secondary to acute promyelocytic leukemia in a pediatric patient. Cureus 2021; 13 (05) e14922
• 14 Sanz MA, Montesinos P. Advances in the management of coagulopathy in acute promyelocytic leukemia. Thromb Res 2020; 191 (Suppl 1): S63-S67
• 15 Del Prete C, Kim T, Lansigan F, Shatzel J, Friedman H. The epidemiology and clinical associations of stroke in patients with acute myeloid leukemia: a review of 10,972 admissions from the 2012 National Inpatient Sample. Clin Lymphoma Myeloma Leuk 2018; 18 (01) 74-77 .e1
• 16 Ferro JM, Infante J. Cerebrovascular manifestations in hematological diseases: an update. J Neurol 2021; 268 (09) 3480-3492
• 17 Cornelissen LL, Kreuger AL, Caram-Deelder C. et al. Thrombocytopenia and the effect of platelet transfusions on the occurrence of intracranial hemorrhage in patients with acute leukemia - a nested case-control study. Ann Hematol 2021; 100 (01) 261-271
• 18 Hakman EN, Cowling KM. Paradoxical embolism. In: StatPearls. Treasure Island, FL: StatPearls Publishing; 2021
• 19 Tobis MJ, Azarbal B. Does patent foramen ovale promote cryptogenic stroke and migraine headache?. Tex Heart Inst J 2005; 32 (03) 362-365
• 20 Kasner SE, Lattanzi S, Fonseca AC, Elgendy AY. Uncertainties and controversies in the management of ischemic stroke and transient ischemic attack patients with patent foramen ovale. Stroke 2021; 52 (12) e806-e819
• 21 Elgendy AY, Saver JL, Amin Z. et al. Proposal for the updated nomenclature and classification of potential causative mechanism in patent foramen ovale-associated stroke. JAMA Neurol 2020; 77 (07) 878-886
• 22 Meacham III RR, Headley AS, Bronze MS, Lewis JB, Rester MM. Impending paradoxical embolism. Arch Intern Med 1998; 158 (05) 438-448
• 23 Saremi F, Emmanuel N, Wu PF. et al. Paradoxical embolism: role of imaging in diagnosis and treatment planning. Radiographics 2014; 34 (06) 1571-1592
• 24 Ren P, Li K, Lu X, Xie M. Diagnostic value of transthoracic echocardiography for patent foramen ovale: a meta-analysis. Ultrasound Med Biol 2013; 39 (10) 1743-1750
• 25 Collado FMS, Poulin MF, Murphy JJ, Jneid H, Kavinsky CJ. Patent foramen ovale closure for stroke prevention and other disorders. J Am Heart Assoc 2018; 7 (12) e007146
• 26 Kleindorfer DO, Towfighi A, Chaturvedi S. et al. 2021 Guideline for the prevention of stroke in patients with stroke and transient ischemic attack: a guideline from the American Heart Association/American Stroke Association. Stroke 2021; 52 (07) e364-e467
• 27 Al-Nouri ZL, Reese JA, Terrell DR, Vesely SK, George JN. Drug-induced thrombotic microangiopathy: a systematic review of published reports. Blood 2015; 125 (04) 616-618
• 28 Reese JA, Bougie DW, Curtis BR. et al. Drug-induced thrombotic microangiopathy: experience of the Oklahoma Registry and the BloodCenter of Wisconsin. Am J Hematol 2015; 90 (05) 406-410
• 29 Saleem R, Reese JA, George JN. Drug-induced thrombotic microangiopathy: an updated systematic review, 2014-2018. Am J Hematol 2018; 93 (09) E241-E243