Progressive bilateral peripheral facial palsy

L.P.H. van de Rijt1 and K.J.A.O. Ingels1

  1. Department of otorhinolaryngology and Head and Neck Surgery, Radboudumc, Nijmegen, the Netherlands

A 60-year old woman was referred to our center for a progressive right-sided peripheral facial paralysis. She reported an ipsilateral sensory neural hearing loss and associated vertigo. Her history revealed systemic sclerosis and hypertension. At the time of presentation, her physical examination showed a right-sided facial paralysis (House-Brackmann Grade 6), a positive Head-Impulse-Test to the right side and right-sided deafness (Fig. 1). Further workup including lumbal puncture was carried out to exclude any autoimmune, metabolic, or infectious causes, none of them demonstrating any abnormality.

Figure 1. Audiogram of 60-year old patient, demonstrating right-sided deafness. On the Y-axis Hearing Loss in dB.

 

Magnetic resonance imaging (MRI) with gadolinium contrast of the cerebello-pontine angle (CPA) was performed (Fig. 2B) and compared to a 4 month previous MRI (Fig. 2A). It demonstrated an increased nodular enhancement of the cranial nerves VII and VIII in the right internal auditory meatus.

At this moment, differential diagnosis included systemic sclerosis giving the presenting complaint was unilateral facial and vestibulocochlear nerve paralysis.

 

Figure 2.  Axial T2-weighted MRI-scans of CPA.

  1. A) showing no abnormalities in the right internal auditory meatus (green arrow). B) 4 months later; showing a nodular enhancement of the cranial nerves VII and VIII in the right-sided internal auditory meatus (red arrow), expressing itself in the cerebrospinal fluid.

1 month later she developed a left-sided facial paralysis (House-Brackmann Grade 6) and right-sided foot-drop. A MRI-spine with gadolinium contrast was performed and showed a nodular enhancement of the sacral nerves at the level of the cauda equina (Fig. 3).

 

Figure 3. Sagittal T2-weighted MRI showing an abnormal nodular enhancement at the cauda equina (red arrow).

A new lumbal puncture was performed and showed positive TFF1 (Trefoil Factor 1) levels in the CSF, suggesting an unknown adenocarcinoma. MRI showed now abnormal thickening of the nerves in bilateral internal auditory canals (Fig. 4).

 

Figure 4. Showing an increased nodular enhancement of the cranial nerves VII and VIII in the right-sided (A) and left-sided (B) internal auditory meatus (red arrow).

To detect an unknown primary tumor a positron-emission-tomography-computed tomography (PET-CT) with a diagnostic CT was performed. The PET-CT showed right-sided hilar masses, with fluor-18-deoxyglucose (FDG)-accumulation in that same area (Fig. 5). Fine needle aspiration (FNA) of a mediastinal node (level 7) revealed an adenocarcinoma of the lung.

 

Figure 5. A) Axial CT-scan, demonstrating a right-sided hilar mass (red arrow). B) FDG accumulation in the right hilar area.

The progressive bilateral facial paralysis as a first symptom of an adenocarcinoma of the lung due to leptomeningeal metastases is very rare. Diagnosis is normally made in patients with advanced cancer. The three most common primary tumors that are found are breast cancer, melanoma and lung cancer. Leptomeningeal metastases develop in approximately 2–5% of cancer patients. In conclusion, progressive bilateral facial paralysis needs thorough neurological and internal work-up to exclude an undiagnosed primary tumor.