Moyamoya syndrome in hemoglobin E-beta thalassemia: A rare presentation and association

Journal of Postgraduate Medicine
P N DoctorR H Merchant

Abstract

Moyamoya disease is an idiopathic, nonatherosclerotic, noninflammatory, chronic progressive cerebrovascular disease characterized by bilateral stenosis or occlusion of the arteries around the circle of Willis, typically the supraclinoid internal carotid arteries, followed by extensive collateralization, which are prone to thrombosis, aneurysm, and hemorrhage. Secondary moyamoya phenomenon or moyamoya syndrome (MMS) occurs in a wide range of clinical scenarios including prothrombotic states such as sickle cell anemia, but the association with other hemoglobinopathies is less frequently observed. We describe a case of a 25-year-old female with hemoglobin E-beta thalassemia who had a rare presentation of MMS in the form of choreoathetoid movements in the left upper and lower extremities. We describe this association, primarily to emphasize thalassemia as an extremely rare but a potential etiology of MMS. Since MMS is a progressive disease, it is important to diagnose and initiate treatment to prevent worsening of the disease and recurrence of stroke.

References

Jan 1, 1983·Stroke; a Journal of Cerebral Circulation·J Suzuki, N Kodama
Aug 1, 1994·Neuroradiology·K TsuchiyaS Furui
Aug 14, 1998·Journal of Magnetic Resonance Imaging : JMRI·K HasuoT Matsushima
Nov 13, 2008·Pediatric Blood & Cancer·Torrey M ParkerRobert J Klaassen
Apr 2, 2009·Neurosurgical Focus·Ali A BaajHarry van Loveren
Apr 20, 2010·Journal of Clinical Neuroscience : Official Journal of the Neurosurgical Society of Australasia·Basak Karakurum GökselSuheyl Asma
Jul 16, 2010·Movement Disorders : Official Journal of the Movement Disorder Society·Jong Sam Baik, Myung Sik Lee

❮ Previous
Next ❯

Citations

Dec 22, 2019·Indian Journal of Pediatrics·Anika AgrawalMonica Juneja
Jul 10, 2020·Neurology India·Boby Varkey Maramattom
Aug 20, 2019·Journal of Neurology·Shambaditya DasMarkus Kraemer
Feb 17, 2021·Annals of Hematology·Linet NjueAlicia Rovó

❮ Previous
Next ❯

Related Concepts

Related Feeds

Anemia

Anemia develops when your blood lacks enough healthy red blood cells. Anemia of inflammation (AI, also called anemia of chronic disease) is a common, typically normocytic, normochromic anemia that is caused by an underlying inflammatory disease. Here is the latest research on anemia.

Blood And Marrow Transplantation

The use of hematopoietic stem cell transplantation or blood and marrow transplantation (bmt) is on the increase worldwide. BMT is used to replace damaged or destroyed bone marrow with healthy bone marrow stem cells. Here is the latest research on bone and marrow transplantation.

Aneurysm

Aneurysms are outward distensions or bulges that occurs in a weakened wall of blood vessels. Discover the latest research on aneurysms here.

Related Papers

Gaceta médica de México
José Luis Gutiérrez-MoralesLeoncio Miguel Rodríguez-Guzmán
Iranian Journal of Child Neurology
Payam SasannejadEhsan Zarepur
Neuropathology : Official Journal of the Japanese Society of Neuropathology
Y Hosoda, E Ikeda
© 2021 Meta ULC. All rights reserved