A patient with typical Wallenberg syndrome: a case report and literature review

Min Zhang,Hong You?

1Sino-French Department of Neurological Rehabilitation,Gansu Provincial Hospital,Lanzhou 730000,China.

Abstract Wallenberg syndrome is a special type of medulla oblongata infarction with many and severe clinical dysfunction,which is dorsolateral medullary syndrome.Wallenberg syndrome can have various initial symptoms due to different damaged parts. Typical clinical manifestations of the syndrome include dizziness, vomiting, dysphagia, cross sensory disturbance, ataxia, etc. Based on the complexity of functional anatomy, infarction in the medulla oblongata can produce various types of clinical symptoms or signs depending on the location. We describe the clinical comprehensive management of a 71-year-old man who presented with typical Wallenberg syndrome.Through early diagnosis and comprehensive clinical management, the prognosis of patient can be effectively improved.

Keywords:Wallenberg syndrome,Management,Prognosis,Case report,Literature review

Introduction

Wallenberg syndrome is a special type of medulla oblongata infarction with many and severe clinical dysfunction,which is dorsolateral medullary syndrome[1, 2]. Typical clinical manifestations of the syndrome include dizziness, vomiting, dysphagia, cross sensory disturbance, ataxia, etc [3]. In terms of etiology of Wallenberg’s syndrome, middle-aged and elderly patients mainly suffer from atherosclerosis, and young patients are mainly cardiac embolism. Common risk factors are the same as ischemic stroke, including hypertension, diabetes, hyperlipidemia, coronary heart disease, atrial fibrillation, etc [4]. Analyses of lesions via magnetic resonance imaging (MRI) have revealed that various types of clinical symptoms or signs may be present depending on the location of a lesion in the medulla oblongata [5]. This article reports the clinical experience of comprehensive management of a typical Wallenberg syndrome patient.

Case Presentation

Current medical history

A 71-year-old man was admitted to hospital for“sudden dizziness with dysphagia and unstable standing for 1 month”. One month ago, the patient suffered from sudden dizziness at night, accompanied by unstable standing, unconscious loss at that time, no nausea, vomiting and other discomfort. Then the patient was rushed to the local county hospital. The blood pressure was measured at 190/100 mmHg and no obvious abnormality was found in cranial CT at that time, and the dizziness symptom of patient was relieved after antihypertensive treatment. The patient returned home for recuperation. Next morning, the patient suffered from dizziness again, accompanied by dysphagia, nausea, vomiting, general weakness,unstable standing, etc., and then had blurred consciousness. Therefore, he called for emergency medical help and was admitted to Gansu Provincial Hospital for treatment. No bleeding focus was found after cranial CT. Considering diagnosis of “cerebral infarction”, he was treated with blood circulation promotion, microcirculation improvement, antiplatelet,lipid regulation, stomach protection, dehydration and intracranial pressure reduction. The cranial MRI examination revealed left medulla oblongata infarction.Pulmonary infection occurred during hospitalization,aspiration pneumonia was considered, and the patient improved after indwelling gastric tube and anti-infection treatment. At present, there are still dizziness and discomfort, dysphagia, unstable standing and other functional disorders. For further rehabilitation treatment, we have admitted the patient to our department with “brainstem infarction (medulla oblongata)”.At the time of admission, the patient was conscious, indwelling gastric tube for nasal feeding,poor sleep, no abnormality in stool and urine, and no obvious weight loss.

He had a history of hypertension for more than 10 years, with the highest hypertension being 190/100 mmHg. He did not take medicine regularly and his blood pressure was not monitored regularly. Denial of the history of diabetes, coronary heart disease and other chronic diseases, denial of the history of infectious diseases,denial of major surgery and trauma,denial of blood transfusion history, denial of food and drug allergy history. The vaccination history is unknown. More than 50 years of smoking history,denied the family history of genetic diseases.

Physical examination (including rehabilitation assessment)

(1) Physical examination: T: 36.3°C, P: 68 times/min,R: 17 times/min, BP: 150/90 mmHg. Clear mind,normal development, moderate nutrition, wheelchair pushed into the ward, physical examination cooperation, answer the right question, slurred speech.General physical examination showed no abnormality,while cardiopulmonary examination and abdominal examination showed no abnormality.

(2) Rehabilitation assessment: clear mind, nasal feeding diet, slurred speech, bilateral nasolabial sulcus basically symmetrical, tongue extension basically centered, uvula basically centered, pharyngeal reflex weakened, soft palate raised poorly, left side obvious.No obvious abnormality is found in muscle strength and muscle tension of limbs. The left facial numbness,right limb pain and temperature sensation decreased,limb tendon reflex was normal and pathological sign was negative. Bilateral finger-nose test and heel-knee-shin test are satisfactory, but the left side is slightly worse. The mini-mental state examination score was 26 points.Balance check does not cooperate(dizziness cannot cooperate), Watian drinking water test grade 5, assessment of daily living ability score(Modified Barthel Index): 45 points, severe functional defect.

Laboratory and imaging examination

(1) Laboratory examination: routine blood, urine and feces, coagulation function, blood sugar, electrolyte of liver and kidney function and blood lipid are basically normal. (2) Imaging examination: cranial MRI, left medulla oblongata acute infarction (Figure 1); cranial magnetic resonance angiography (MRA), left vertebral artery not shown (Figure 2). The whole brain digital substraction angiography examination was rejected by patient.

Diagnosis

(1) Clinical diagnosis: ① brain stem infarction convalescence (left medulla oblongata), ②hypertension grade 3, extremely high risk. According to the patient’s medical history and imaging findings,the diagnosis is clear.

(2) Functional diagnosis: dysphagia, ataxia and dysfunction of daily living. Watian drinking water test grade 5, pharyngeal reflex weakened, etc., diagnosed as dysphagia. The left side of bilateral finger-nose test and heel-knee-tibia test is slightly worse, and the diagnosis is ataxia. Modified Barthel Index score: 45 points, severe functional defect, diagnosed as dysfunction of daily living.

(3) Clinical syndrome: Wallenberg syndrome. The cranial MRI examination of this patient showed that the left medulla oblongata infarction foci, and the patient had 4 major symptoms [2, 6, 7], including dysphagia,ataxia,numbness of the affected lateral part,and decreased pain and temperature sensation of the healthy limb. The clinical diagnosis was Wallenberg syndrome.Furthermore,it is considered that the causes of this syndrome may be hypertension, smoking history for many years, as well as the age is also a factor.

Differential diagnosis

(1) Brain stem hemorrhage is usually more acute and severe, usually coma occurs earlier, most patients are critically ill, and the mortality rate is extremely high.Obvious hemorrhage foci can be seen in brain stem on cranial CT and can be clearly identified on imaging.(2)Cerebral hemispheric infarction, such as basal ganglia,usually starts with limb weakness, which can be gradually aggravated or accompanied by dysphagia,usually pseudobulbar paralysis and pharyngeal reflex.Cranial MRI examination can clearly identify the infarct site.

Figure 1 Cranial MRI shows left medulla oblongata infarction(black arrow indication)

Figure 2 Cranial MRA shows left vertebral artery not shown(black arrow indication)

Comprehensive management

After admission, patient was thoroughly evaluated(liver function, renal function, electrolyte, prothrombin time activity, blood routine , );monitor patient’s blood pressure, and provide nasal feeding diet, and giveantihypertensive (amlodipine), antiplatelet(aspirin), anti-vertigo (betahistine), and promotion of nerve repair(bossgate) treatments.

For swallowing dysfunction, there are mainly basic training, indirect swallowing training and feeding training [8, 9]. Basic training includes sensory stimulation (including touch, temperature and taste stimulation) and muscle training of mouth and face.Indirect swallowing training is to train patients to swallow related decomposition actions under the condition of no eating, and to achieve the purpose of safe and effective swallowing by means of some specific manipulations and actions. During the feeding training, choose the appropriate body position, and select the appropriate one-mouthful food intake according to the actual swallowing function of the patient, and increase it according to the patient’s reaction during the training [8]. Electric standing bed training can improve the patient’s adaptability to body position changes, enhance the proprioceptive input and balance of lower limbs, and prevent complications.Exercise therapy and balance function training improve limb movement and coordination ability, and improve balance and coordination functions;occupational therapy improves patients’ coordination ability and fine function, and improves their daily activities.

After nearly one month of rehabilitation treatment,the patient’s swallowing function was significantly improved.The Watian drinking water test was grade 2.The gastric tube was pulled out and the oral intake was good. Sitting balance level 3, standing balance level 2.Berg Balance Scale score: 31 points. Patient can walk a short distance with the help of family members.Modified Barthel Index score: 75 points, mild functional defect.

Discussion

Wallenberg syndrome, also known as posterior inferior cerebellar artery (PICA) syndrome and dorsolateral medullary syndrome, is the most common posterior circulation ischemic stroke syndrome [2]. In 1895,German neurologist Adolf Wallenberg described the clinical manifestations of the syndrome. Of all ischemic stroke patients, about 20% are posterior circulation stroke, and about half of them can be represented as syndrome, which is mostly male and is more common in the middle-aged and elderly population [10-12].About 75% of patients are caused by atherosclerosis, 17% by cardiac embolism, and 8%by vertebral artery dissection [10, 13, 14]. In patients with dorsolateral medulla oblongata syndrome, only a few patients are caused by PICA occlusion. Most of the causes of PICA ischemia are stenosis or occlusion of intracranial segment or origin of vertebral artery, or thrombus shedding at origin of vertebral artery[10,15,16]. Cranial MRA of this patient showed a loss of left vertebral artery signal. Although no further digital substraction angiography examination of the whole brain was performed, it could still reflect that there were some problems in the vertebral artery.Considering that the responsible vessel for this occurrence is the left vertebral artery, the etiological type is macroatherosclerosis, and qualitative diagnosis is ischemic cerebrovascular disease.

Wallenberg syndrome can have various initial symptoms due to different damaged parts, but vertigo/dizziness is common in clinic [3]. Dizziness is the first symptom of this patient, which indicates vestibular nucleus is involved. Nausea and vomiting,dysphagia, ataxia, and cross sensory disturbance gradually appear as the disease progresses [17, 18].At the initial stage of the disease, due to incomplete symptoms or careless physical examination, it is easy to be misdiagnosed as posterior circulation ischemia,delay relating examination and treatment, and even cause sudden death of patients due to respiratory or cardiac arrest.

There are 8 types of Wallenberg syndrome sensory disturbance [19]: (1) lateral contralateral limb sensory disturbance of lesion, which is crossed sensory disturbance; (2) the pain and temperature sensation of bilateral face and contralateral lesion decreased; (3)sensory disturbance of the lesion to the lateral part and hemi-body; (4) sensory disturbance only at the lateral part of the lesion; (5) somatosensory disturbance on opposite side of lesion only; (6) bilateral somatosensory disorders; (7) bilateral facial sensory disturbance; (8) lesion-to-lateral sensory disturbance.Combined with the clinical symptoms and signs of this patient, the type of sensory disturbance of this patient is analyzed as typical cross sensory disturbance. The previous reports of patients with dorsolateral medulla oblongata syndrome presenting as pure sensory disturbance [20]. In addition, this patient has no consciousness disorder,but there are also patients with consciousness disorder, which may be caused by gradual aggravation of edema after infarction,compression of brainstem and influence on cerebrospinal fluid circulation[21].

If the patient lacks typical clinical manifestations,some scholars suggest that the possibility of Wallenberg syndrome can be indicated when the patient meets the following two conditions: (1) cranial MRI indicates that the focus is in medulla oblongata,and dysarthria and dysphagia must be one of them; (2)cranial MRI indicates that the lesion is located at the dorsolateral medulla oblongata, pain and temperature sensation disorder, ataxia and Horner syndrome must be one of them [22]. This patient met the above requirements and was diagnosed accordingly.

To sum up, this case extends our knowledge of clinical characteristics of Wallenberg syndrome, and increases awareness of its etiologies, diagnosis, and comprehensive management, and effectively improves the prognosis of patient.