Moyamoya Syndrome in Middle-Aged Men: Sudden Speech and Movement Impairment Leads to Life-Saving Diagnosis

Imagine a healthy 44-year-old man suddenly struggling to speak or move one side of his body. This was the reality for one patient who came to the hospital with sudden-onset speech difficulties and left-sided weakness. What doctors discovered was not just a stroke, but a rare and progressive cerebrovascular condition known as Moyamoya syndrome—a diagnosis that highlights the importance of early detection and advanced neurosurgical intervention.

Understanding Moyamoya Syndrome: A Rare but Serious Condition

Moyamoya syndrome is a chronic, progressive disorder characterized by the narrowing or blockage of major arteries at the base of the brain, particularly the internal carotid arteries and their branches. As these vessels become obstructed, tiny collateral blood vessels form to compensate for reduced blood flow. On angiograms, these fragile networks resemble a "puff of smoke"—hence the name "Moyamoya," derived from Japanese.

This case involved a previously healthy male patient presenting with acute neurological deficits. After thorough evaluation using MRI, MRA, and digital subtraction angiography (DSA), he was diagnosed with bilateral cerebrovascular abnormalities consistent with Moyamoya disease—more severe on the right hemisphere, where complete occlusion of the middle cerebral artery was observed.

Patient Profile at a Glance

Age & Gender: 44-year-old male
Diagnosis: Moyamoya Syndrome
Hospital: Liaoning Provincial People's Hospital
Date of Admission: April 2021
Treatment Plan: Combined revascularization surgery + medical therapy
Surgical Procedure: Right superficial temporal artery to middle cerebral artery (STA-MCA) bypass with encephalomyosynangiosis (temporal muscle grafting)
Medications: Furosemide injection (for edema and blood pressure control), Oxiracetam injection (neuroprotective agent)
Hospital Stay: 14 days
Outcome: Successful surgical revascularization with significant functional improvement during follow-up

Initial Clinical Presentation: When Symptoms Signal Something Deeper

The patient arrived at the hospital 13 days after experiencing sudden slurred speech and left-sided paralysis. Upon examination, he was alert but exhibited clear aphasia and motor deficits: left upper extremity had zero muscle strength (Grade 0), while the lower limb showed moderate weakness (Grade 3). The right side remained intact (Grade 5).

Brain imaging revealed extensive infarction in the right frontal and parietal lobes, along with multiple small strokes throughout the brain. Magnetic resonance angiography (MRA) showed poor visualization of the right middle cerebral artery, sparse branching of the superficial temporal artery entering the skull, and stenosis in the left middle cerebral artery origin. Three-dimensional arterial spin labeling (3D-ASL) confirmed diminished perfusion in the right frontoparietal, occipital, and temporal regions.

Definitive diagnosis came via cerebral angiography, which demonstrated bilateral anterior cerebral artery narrowing, left middle cerebral artery stenosis, and complete occlusion on the right—classic features of Moyamoya syndrome.

Comprehensive Treatment Approach: Bridging Blood Flow Back to the Brain

Given the patient's young age and high risk of future ischemic events, prompt intervention was critical. The family expressed concern but quickly agreed to surgery once they understood the potential benefits.

A two-pronged surgical strategy was implemented: STA-MCA bypass combined with encephalomyosynangiosis (also known as indirect revascularization through temporal muscle apposition). In this procedure, the superficial temporal artery was carefully dissected and anastomosed end-to-side to a cortical branch of the middle cerebral artery to immediately restore blood flow. Simultaneously, the temporalis muscle was placed directly onto the brain surface to encourage new vessel growth over time.

Postoperatively, the patient received intravenous furosemide to manage cerebral edema and maintain optimal perfusion pressure, alongside oxiracetam to support neuronal metabolism and cognitive recovery.

Positive Outcomes and Recovery Progress

Follow-up CT angiography confirmed successful graft patency, with visible blood flow from the superficial temporal artery into the ischemic territory of the right middle cerebral artery. The patient reported no complications post-surgery and tolerated oral intake well.

He was discharged after suture removal with stable vital signs and improved neurological status. At a six-month follow-up visit, notable progress was evident: speech function had significantly improved, left upper limb strength increased from Grade 0 to Grade 2, and lower limb strength remained at Grade 3. Repeat MRI showed reduced areas of hypoperfusion in the right hemisphere, indicating enhanced cerebral circulation.

Rehabilitation continues with physical and speech therapy, aiming for further gains in mobility and communication skills.

Key Recommendations for Patients Post-Surgery

While the immediate surgical outcome was excellent, long-term success depends heavily on lifestyle management and vigilant monitoring. The newly formed vascular network takes 3 to 6 months to mature fully, making this period especially vulnerable to both ischemic and hemorrhagic events.

To minimize risks during recovery, patients should:

Eat a balanced diet rich in protein and fluids, focusing on soft, easily digestible foods
Prevent constipation, which can increase intracranial pressure
Monitor blood pressure and glucose levels daily
Avoid smoking, alcohol consumption, and sleep deprivation
Dress appropriately for weather changes to prevent colds or infections
Engage in regular, physician-guided rehabilitation exercises

Equally important is emotional support. Family members are encouraged to provide psychological encouragement, helping the patient maintain motivation and reduce anxiety during recovery.

Why Early Detection Matters: A Neurologist's Perspective

Moyamoya syndrome may be rare, but its consequences can be devastating if undiagnosed. Many cases present initially as stroke-like episodes in otherwise healthy individuals—especially those under 50. As seen here, timely diagnosis allowed for preventive surgery before irreversible damage occurred.

I strongly recommend including MR angiography (MRA) as part of routine brain MRI evaluations, especially when unexplained neurological symptoms arise. MRA not only helps detect Moyamoya syndrome but also identifies other silent threats such as aneurysms, arteriovenous malformations, and early-stage stenosis.

Early screening empowers doctors to intervene before catastrophe strikes. For patients like this one, it meant the difference between permanent disability and meaningful recovery.