Haroon Kisana, MD
Resident
HonorHealth PM&R Program
Scottsdale, Arizona, United States
Brian Khin, DO
Resident
KPC Health Transitional Year Program
Arcadia, California, United States
Joanna Israel, DO
Medical Director of HonorHealth Osborn Medical Center
HonorHealth PM&R Program
Scottsdale, Arizona, United States
A 26-year-old female with past medical history significant for Absence Seizures since the birth of her first child and recent autoimmune connective tissue disorder with Raynaud’s Phenomenon since the birth of her second child which was treated with steroids and Azathioprine. Two months prior, the patient presented to the with right hand numbness but was sent home with diagnosis of anxiety. The patient was admitted to the hospital two weeks later for pancytopenia and became altered. MRI demonstrated multiple cortical infarcts and diffuse leptomeningeal enhancement. The patient was given methylprednisolone for five days and was discharged home. The following morning, the patient suddenly became mute and confused, leading to re-hospitalization. Neurological exam was significant for receptive and expressive aphasia, right-sided hemiplegia and hemisensory deficits. Brain imaging demonstrated substantial blood vessel collateralization consistent with chronic MoyaMoya Syndromeas as the etiology of left-sided hemispheric strokes.
Discussions: MoyaMoya Syndrome is a cerebrovascular condition characterized by narrowing of large intracranial arteries with the development of small-vessel collaterals. MoyaMoya is progressive and causes ischemic or hemorrhagic strokes. Vascular stenosis due to intimal thickening can lead to extracranial and systemic arterial involvement. While etiology remains unknown, the disease has a higher incidence in East Asian countries with 10-15% of cases with familial occurrence. Onset is bimodal in distribution. MoyaMoya is associated with conditions such as hematologic, genetic, and autoimmune diseases.
The patient presented to the rehabilitation hospital with severe expressive aphasia, moderate receptive aphasia, right-sided hemiplegia, impaired trunk control, and impaired oral manipulation, requiring Maximal Assist. From rehab, she discharged home with supervision or touching assistance.
Conclusions: This is a unique presentation of MoyaMoya Syndrome in the setting of an autoimmune mixed connective tissue disorder. It demonstrates the importance of performing thorough a diagnostic work-up for patients.
