Weber syndrome
OVERVIEW
What is Weber syndrome?
Weber syndrome is a neurological disorder characterized by crossed hemiplegia and oculomotor nerve palsy, named after British physician Hermann Weber who first described its features. It is typically caused by infarction of the cerebral peduncle in the midbrain, hence also known as "cerebral peduncle syndrome."
Is Weber syndrome common?
No.
Most cases of Weber syndrome result from isolated midbrain infarction (referring to a relatively localized cerebral infarction in the midbrain), which accounts for only 1.4‰ of all cerebral infarctions. In other words, among 1,000 cerebral infarction patients, there may be 1–2 cases of isolated midbrain infarction, and only a portion of these are Weber syndrome.
Does Weber syndrome affect daily life?
Yes.
Weber syndrome can severely impact a patient's daily life, imposing significant financial and emotional burdens on both the patient and their family.
SYMPTOMS
What are the typical manifestations of Weber syndrome?
Patients with Weber syndrome mainly exhibit the following symptoms:
- Restricted eye movement on the affected side.
- Diplopia (seeing one object as two).
- Dilated pupil on the affected side.
- Ptosis on the affected side (drooping upper eyelid, partially covering the pupil).
- Central facial paralysis and tongue paralysis on the opposite side.
- Paralysis of the upper and lower limbs on the opposite side.
Of course, the actual symptoms of patients depend on the severity and extent of the disease and may not fully match the above descriptions. If any other symptoms occur, inform the doctor promptly to clarify the diagnosis, identify the lesion location, and adjust the treatment plan.
Are Weber syndrome and Sturge-Weber syndrome the same condition?
No. Although their names overlap, they are two distinct diseases.
Weber syndrome is a neurological disorder caused by unilateral midbrain damage, manifesting as oculomotor nerve palsy and crossed hemiplegia.
Sturge-Weber syndrome is a rare neurocutaneous syndrome caused by genetic mutations, commonly seen in newborns. Symptoms include skin birthmarks, glaucoma, seizures, muscle weakness, intellectual developmental disorders, etc.
What other conditions should Weber syndrome be differentiated from?
-
Benedikt syndrome: Also presents with restricted eye movement, dilated pupil, and ptosis on the affected side, but Benedikt syndrome exhibits contralateral limb rigidity, tremors, involuntary movements, and decreased deep sensation (including kinesthesia, position sense, and vibration sense).
-
Claude syndrome: Also presents with restricted eye movement, dilated pupil, and ptosis on the affected side, but Claude syndrome exhibits contralateral limb ataxia (e.g., uncoordinated movements, unsteady gait) and tremors (e.g., uncontrollable shaking of hands or feet).
-
Foville syndrome: Also presents with contralateral limb hemiplegia, but Foville syndrome involves the pons and additionally manifests as peripheral facial paralysis on the affected side (e.g., loss of forehead wrinkles, mouth deviation) and sensory impairment in the opposite limb.
CAUSES
What are the causes of Weber syndrome?
The most common cause of Weber syndrome is isolated midbrain infarction due to occlusion of the "paramedian midbrain artery."
Less common causes include: cerebral hemorrhage, aneurysm, neurological tumors, and demyelinating diseases.
Who is at risk for Weber syndrome?
The risk factors for Weber syndrome are similar to those for stroke and mainly include:
- Hypertension
- High cholesterol
- Smoking
- Obesity
- Diabetes
- History of stroke
If these risk factors are present, they can be managed through lifestyle and dietary changes, and medical help should be sought when necessary.
DIAGNOSIS
How is Weber syndrome diagnosed?
Doctors make a preliminary diagnosis based on typical clinical manifestations and physical examination findings, but accurately locating Weber syndrome is difficult. Some auxiliary tests are necessary to obtain precise results and a definitive diagnosis.
What tests are needed for Weber syndrome?
- Computed tomography (CT) and magnetic resonance imaging (MRI): Help identify the lesion site and determine whether other causes exist, such as intracranial hemorrhage, tumors, or inflammatory lesions.
- MRI angiography or CT angiography: Assess blood flow supply to the "midbrain" and check for vascular stenosis or occlusion.
- Electrocardiogram (ECG): Determine whether cardiogenic embolism (blood clots caused by heart disease) is present.
- Other tests: Such as blood tests and cerebrospinal fluid analysis to check for neurological inflammation.
TREATMENT
Under what circumstances should Weber syndrome require immediate medical attention?
Patients with Weber syndrome should seek immediate medical attention if they suddenly experience any of the following symptoms:
- Limb weakness
- Double vision
- Drooping eyelid
- Facial paralysis
Symptoms of Weber syndrome always appear suddenly and progress rapidly within hours to days. Time is of the essence—do not delay treatment, as it may be life-threatening.
Which department should Weber syndrome patients visit?
- Neurology
- In emergencies, go to the emergency department.
Does Weber syndrome require hospitalization?
Yes.
How to respond to sudden worsening of Weber syndrome?
If a Weber syndrome patient experiences a sudden deterioration, patients and their families should take the following actions:
- Call for help and immediately transport the patient to the nearest hospital or contact an ambulance.
- Clear any foreign objects from the patient’s mouth to prevent choking.
- Lay the patient flat to improve cerebral blood perfusion.
- Stay calm and monitor changes in the patient’s consciousness.
Emergency responders should take the following actions:
- Administer oxygen promptly to maintain oxygen saturation above 94%.
- Establish intravenous access.
- Initiate cardiac monitoring.
- Transport the patient to the hospital as quickly as possible for further treatment.
What treatments are required for Weber syndrome?
Treatment depends on the underlying cause:
-
If Weber syndrome is caused by atherosclerosis:
- Administer "rtPA" (within 3–4.5 hours) or "urokinase" (within 6 hours) for intravenous thrombolysis after a cerebral infarction.
- If intravenous thrombolysis is not performed within the specified time, start aspirin or clopidogrel as early as possible within 48 hours, and consider dual antiplatelet therapy or even combined anticoagulation if necessary.
- Long-term use of antiplatelet drugs can prevent stroke recurrence.
-
If Weber syndrome is caused by neurological tumors or aneurysms:
- Surgical intervention may be considered.
- Supportive care, psychological therapy, and rehabilitation are also critical components of treatment.
What are the common complications of Weber syndrome during hospitalization?
Common complications of Weber syndrome during hospitalization include:
- Stroke recurrence
- Falls
- Deep vein thrombosis
- Pulmonary embolism
- Pulmonary infection
- Urinary tract infection
- Pain
- Pressure ulcers
- Depression
- Anxiety
DIET & LIFESTYLE
Can Weber syndrome fully recover?
Not necessarily.
Patients with Weber syndrome may experience varying degrees of symptom improvement after treatment as brain edema and inflammation subside. However, depending on the severity of midbrain damage, functional recovery may take weeks, months, or even years, and in some cases, complete recovery may never be achieved.
Therefore, for Weber syndrome, early detection, timely and scientific treatment, adherence to medical advice to prevent complications, and subsequent rehabilitation therapy, occupational therapy, and psychological therapy when necessary are crucial to maximize recovery opportunities.
What should Weber syndrome patients pay attention to in daily life?
The following points should be noted:
- Build confidence in life: Communicate more with family and doctors to help solve problems, share stress, and alleviate anxiety.
- Adhere to rehabilitation exercises: Patients should consult professional doctors to choose appropriate methods for rehabilitation training. Scientific rehabilitation should help restore function and adapt to daily life while avoiding overexertion, which may worsen symptoms.
- Maintain personal hygiene: Patients need to develop and maintain good hygiene habits. However, due to limited mobility, personal hygiene can be challenging, and family members may need to assist.
- Wear suitable clothing: Choose clothes and shoes that are easy to put on and take off. Buttons and shoelaces can be troublesome for hemiplegic patients, so family assistance may be necessary.
- Prevent stroke: Patients should improve lifestyle habits, control blood pressure, blood sugar, and cholesterol, quit smoking, lose weight, engage in moderate exercise, and take antiplatelet or anticoagulant medications as prescribed.
What should family members of Weber syndrome patients do?
-
Provide emotional support: Encourage and help patients gradually accept and adapt to physical and life changes, and share their psychological burden. Seek help from a psychologist if necessary.
-
Supervise and assist with rehabilitation exercises: Family members can help patients with appropriate exercises to restore physical function and better adapt to daily life.
-
Adjust the patient's diet: If hemiplegia occurs on the dominant side, Weber syndrome patients may need time to adapt and recover fine motor skills for eating. Family members can offer easy-to-eat foods and use spoons instead of chopsticks or forks.
-
Assist with personal hygiene: Due to mobility limitations, family members should help maintain the patient's hygiene, which aids in treatment and boosts the patient's confidence in life.
PREVENTION
How to prevent Weber syndrome?
- Quit smoking
- Control diabetes
- Manage blood pressure
- Low-fat diet
- Increase physical exercise
- Lose weight