65-Year-Old Woman Diagnosed with Moyamoya Syndrome After Sudden Limb Weakness, Recovers with Timely Treatment

In this case study, we explore the journey of a 65-year-old woman who suddenly developed right-sided limb weakness and was later diagnosed with Moyamoya syndrome—a rare cerebrovascular disorder. Initially misdiagnosed and treated for standard ischemic stroke, her condition worsened until a thorough neurological evaluation revealed the true underlying cause. With targeted medical therapy and early rehabilitation, she made significant progress. This article highlights the importance of accurate diagnosis, timely intervention, and comprehensive care in managing complex neurological conditions like Moyamoya syndrome.

Understanding Moyamoya Syndrome: A Rare but Serious Condition

Moyamoya syndrome, also known as Moyamoya disease when no secondary cause is found, is a progressive cerebrovascular disorder characterized by chronic narrowing or blockage of the internal carotid arteries at the base of the brain. As these major vessels become obstructed, the brain attempts to compensate by forming tiny collateral blood vessels that appear "hazy" or "like a puff of smoke" on imaging—hence the name "Moyamoya," which means "puff of smoke" in Japanese.

While relatively rare, its incidence appears to be rising globally, particularly in East Asian populations, though cases are increasingly being recognized in Western countries as well. The condition can affect both children and adults, often presenting with transient ischemic attacks (TIAs), strokes, seizures, or cognitive decline.

Patient Profile and Initial Presentation

Age & Gender: 65-year-old femaleHospital: Hubei Provincial Third People's HospitalDate of Admission: December 2020Primary Diagnosis: Moyamoya Syndrome with Acute Ischemic StrokeTreatment Plan: Combination of neuroprotective agents, antiplatelet therapy, and structured rehabilitationDuration: 10-day inpatient care followed by one month of outpatient follow-up

The patient initially presented with sudden-onset right-sided weakness, impaired balance, and difficulty walking. Within 24 hours, her symptoms progressed dramatically—she could no longer sit upright unassisted, began slurring her speech, experienced frequent choking while drinking water, and struggled to eat. Notably, she did not report headache, dizziness, nausea, or vomiting, which may have delayed initial suspicion of a serious cerebrovascular event.

Initial Evaluation and Diagnostic Challenges

At a local hospital, an MRI scan revealed acute infarcts in the left frontal lobe, basal ganglia, and corona radiata—areas critical for motor control and language. She was started on aspirin for antiplatelet therapy and atorvastatin to stabilize plaque formation, standard treatments for ischemic stroke.

However, despite this treatment, her neurological deficits continued to deteriorate. She developed altered consciousness and worsening hemiparesis, prompting transfer to a tertiary stroke center. Upon arrival, our team suspected a more complex etiology than typical atherosclerotic stroke due to the rapid progression and lack of traditional vascular risk factors.

Uncovering the True Cause: Advanced Imaging Reveals Moyamoya

A detailed family history revealed that the patient had experienced occasional headaches for years and a prior episode of transient left leg weakness several years earlier, which resolved after intravenous vasodilator therapy ("expanding blood vessel injections"). Her relatives believed she had always been healthy, masking potential warning signs.

We ordered a CT angiography (CTA) to evaluate cerebral vasculature. The results were striking—severe stenosis of the terminal internal carotid arteries with a dense network of fragile collateral vessels resembling a tangled web. This classic "puff-of-smoke" appearance confirmed the diagnosis of Moyamoya syndrome.

To confirm the findings, digital subtraction angiography (DSA)—the gold standard for diagnosing Moyamoya—was performed. It clearly demonstrated bilateral occlusive changes in the distal internal carotid arteries and extensive compensatory collateral circulation, solidifying the diagnosis.

Targeted Treatment Approach and Recovery Progress

Recognizing that conventional stroke protocols were insufficient, we adjusted her treatment plan to specifically address the pathophysiology of Moyamoya syndrome:

Neuroprotection: Infusion of Edaravone Dexborneol Injection to reduce oxidative stress and protect neurons from ischemia-reperfusion injury.
Improved Collateral Circulation: Administration of Butylphthalide in Sodium Chloride Injection, a compound shown to enhance microcirculation and promote cerebral blood flow in compromised areas.
Antiplatelet Therapy: Continued use of enteric-coated aspirin to prevent further thrombotic events without increasing hemorrhagic risk.
Early Rehabilitation: Initiated physical and occupational therapy focusing on muscle strength, coordination, and gait training.

Within ten days, the patient showed remarkable improvement. She regained full alertness, communicated clearly, and achieved partial recovery of motor function in her affected limbs. By discharge, she was able to stand and walk short distances with assistance.

Follow-Up and Long-Term Outlook

One month after discharge, during an outpatient visit, she reported sustained progress. Using a cane for support, she could walk independently across rooms and perform basic self-care tasks. Her chronic headaches had diminished significantly, and she described feeling mentally sharper than before the incident.

She continues to take oral butylphthalide soft capsules to support collateral circulation for a planned duration of two months, alongside ongoing home-based rehabilitation exercises.

Key Recommendations for Patients and Caregivers

This case underscores several crucial points for long-term management:

Adherence to Medication: Never discontinue or modify prescribed medications—especially anticoagulants or neuroprotective drugs—without consulting your physician. Consistency is vital in preventing recurrent strokes.

Maximize the Golden Window of Recovery: The first 30 days post-stroke are considered the most effective period for neurological recovery. Daily, focused exercise targeting each muscle group in the paralyzed limb can significantly improve outcomes.

Lifestyle Modifications: Avoid smoking and alcohol consumption, manage emotional stress, and ensure adequate rest. These factors can trigger vasoconstriction and increase the risk of ischemic episodes.

Surgical Consideration: Once stable, patients with confirmed Moyamoya syndrome should consider revascularization surgery (such as EDAS or STA-MCA bypass), which can restore blood flow and reduce future stroke risk by up to 70%.

Clinical Insights and Broader Implications

Moyamoya syndrome remains underdiagnosed, especially in non-Asian populations. Its association with genetic predisposition means that individuals with a family history of stroke or unexplained neurological symptoms should undergo proactive cerebrovascular screening—even in the absence of major risk factors like hypertension or diabetes.

Early detection saves brain tissue. Every minute during a stroke, approximately 1.9 million neurons die. Prompt referral to a certified stroke center ensures access to advanced diagnostics and time-sensitive therapies that can halt progression and preserve function.

This patient's favorable outcome was largely due to timely recognition of atypical stroke features, rapid escalation to specialized care, and initiation of syndrome-specific treatment. It serves as a powerful reminder that not all strokes are the same—and precision medicine matters.

For healthcare providers, maintaining a high index of suspicion for rare causes of stroke, particularly in patients with progressive or recurrent neurological symptoms, is essential. For patients and families, understanding the signs of cerebrovascular compromise and seeking expert care quickly can make the difference between disability and recovery.