Babinski–Nageotte syndrome

Overview

Babinski–Nageotte syndrome is a rare neurological condition caused by damage to the dorsolateral medulla oblongata and adjacent areas in the brainstem. First described by Joseph Babinski and Jean Nageotte in the early 20th century, this syndrome presents a combination of features seen in both Wallenberg syndrome and corticospinal tract involvement. It is considered a variant of lateral medullary syndrome with additional pyramidal signs due to more extensive brainstem involvement. Though uncommon, it provides crucial insights into the complex vascular and anatomical layout of the medulla.

Causes

Babinski–Nageotte syndrome is typically caused by ischemic stroke due to occlusion of the vertebral artery or its branches, particularly the posterior inferior cerebellar artery (PICA). Less common causes include:

• Brainstem tumors

• Demyelinating diseases such as multiple sclerosis

• Traumatic brainstem injury

• Vascular malformations or aneurysms

• Infectious or inflammatory lesions affecting the medulla

Symptoms

The clinical presentation of Babinski–Nageotte syndrome reflects damage to multiple tracts in the medulla:

• Ipsilateral cranial nerve dysfunction, especially involving the 9th (glossopharyngeal), 10th (vagus), and sometimes the 11th (accessory) nerves

• Contralateral hemiparesis or hemiplegia due to involvement of the corticospinal tract

• Contralateral loss of pain and temperature sensation in the body from spinothalamic tract damage

• Ipsilateral Horner’s syndrome (ptosis, miosis, anhidrosis)

• Ataxia and coordination difficulties on the same side as the lesion

• Dysphagia and dysarthria, often severe

The combination of brainstem motor tract signs with lateral medullary symptoms is the hallmark of this syndrome.

Diagnosis

Diagnosis is primarily clinical, supported by neuroimaging and other tests:

• Neurological examination to assess for cranial nerve deficits, motor weakness, sensory loss, and ataxia

• Magnetic Resonance Imaging (MRI) of the brainstem is the gold standard to visualize infarcts or lesions in the medulla

• Magnetic Resonance Angiography (MRA) or CT angiography to identify vertebral artery or PICA occlusion

• Blood work to assess risk factors like hyperlipidemia, clotting disorders, or autoimmune markers

Treatment

Management focuses on addressing the underlying cause and relieving symptoms:

• Acute ischemic stroke management (e.g., thrombolysis or thrombectomy) if within the therapeutic window

• Antiplatelet or anticoagulant therapy to prevent future strokes

• Rehabilitation therapy including physical, occupational, and speech therapy for recovery of function

• Symptomatic treatments such as medications for spasticity, swallowing support (e.g., feeding tube), and pain control

Treatment of non-vascular causes (e.g., tumors, infections) would be specific to the etiology.

Prognosis

The prognosis for Babinski–Nageotte syndrome varies depending on the cause, extent of brainstem damage, and speed of intervention:

• Partial recovery is possible, especially with early rehabilitation and stroke management

• Severe disability may occur in cases with extensive damage or delay in treatment

• Mortality risk is higher in brainstem strokes compared to cortical strokes, due to involvement of vital centers

Long-term outcome often includes some degree of persistent neurological deficit, though quality of life may improve with multidisciplinary support.