# Rogers Syndrome and Callosal Disconnection in the Setting of Moyamoya Disease

## TL;DR

This paper reports the first case of a young woman with both Rogers syndrome and moyamoya disease, leading to a rare stroke presentation requiring complex treatment.

## Contribution

The paper presents the first documented case of Rogers syndrome co-occurring with moyamoya disease.

## Key findings

- A 22-year-old female presented with altered mental status, left-sided weakness, and delayed speech due to corpus callosum infarction.
- The patient had callosal disconnection syndrome from moyamoya disease and Rogers syndrome.
- Combined revascularization and rehabilitation were used to manage the complex condition.

## Abstract

Rogers syndrome, also known as thiamine-responsive megaloblastic anemia (TRMA), is a rare autosomal recessive disorder characterized by diabetes mellitus (DM), sensorineural hearing loss, and megaloblastic anemia responsive to thiamine supplementation. Moyamoya disease is a progressive steno-occlusive cerebrovascular disorder involving stenosis of the terminal internal carotid arteries with subsequent formation of fragile collateral vessels, resulting in ischemic or hemorrhagic strokes. We present the first documented case of the co-occurrence of Rogers syndrome and moyamoya disease, involving a 22-year-old female who presented with altered mental status, left-sided weakness, and delayed speech. Neuroimaging revealed acute infarction of the corpus callosum and bilateral watershed territories, manifesting as callosal disconnection syndrome. The patient underwent combined direct and indirect revascularization procedures followed by multidisciplinary rehabilitative care. This report underscores the complex interplay of rare genetic, metabolic, and vascular factors leading to atypical stroke presentations and highlights the importance of thorough evaluation and coordinated multidisciplinary management in young patients with syndromic cerebrovascular disease.