Multidisciplinary Care of Patients with Facial Palsy: Treatment of 1220 Patients in a German Facial Nerve Center

To determine treatment and outcome in a tertiary multidisciplinary facial nerve center, a retrospective observational study was performed of all patients referred between 2007 and 2018. Facial grading with the Stennert index, the Facial Clinimetric Evaluation (FaCE) scale, and the Facial Disability Index (FDI) were used for outcome evaluation; 1220 patients (58.4% female, median age: 50 years; chronic palsy: 42.8%) were included. Patients with acute and chronic facial palsy were treated in the center for a median of 3.6 months and 10.8 months, respectively. Dominant treatment in the acute phase was glucocorticoids ± acyclovir (47.2%), followed by a significant improvement of all outcome measures (p < 0.001). Facial EMG biofeedback training (21.3%) and botulinum toxin injections (11%) dominated the treatment in the chronic phase, all leading to highly significant improvements according to facial grading, FDI, and FaCE (p < 0.001). Upper eyelid weight (3.8%) and hypoglossal–facial-nerve jump suture (2.5%) were the leading surgical methods, followed by improvement of facial motor function (p < 0.001) and facial-specific quality of life (FDI, FaCE; p < 0.05). A standardized multidisciplinary team approach in a facial nerve center leads to improved facial and emotional function in patients with acute or chronic facial palsy.

Recurrent Bilateral Lower Motor Neuron Type of Facial Palsy with Hearing Impairment: Hyperphosphatemic Familial Tumoral Calcinosis

Hyperphosphatemic familial tumoral calcinosis (HFTC) presents with varied neurological manifestations that have been reported in the literature like facial palsy, vision and hearing impairment, stroke, and headache. In this article, we reported a 12-year-old girl child patient with recurrent facial weakness with bilateral hearing impairment and multiple ulcerative lesions on lower limbs and elbows. On examination, she had lower motor neuron (LMN) facial palsy with conductive hearing loss. The investigations showed hyperphosphatemia (9.3 mg/dL) with normal serum calcium (10.4 mg/dL), alkaline phosphatase (147.9 U/L), parathyroid hormone (23.12 pg/mL), and renal function tests. Elevated serum calcium and phosphorus product (96.72 mg2/mL2) and elevated renal tubular reabsorption of phosphate (TMPxGFR) value (9.16) were noted. Skeletal survey showed hyperostosis in the long bone diaphysis, vertebrae, ribs, pelvic bone, skull, and facial bones with narrowing of cranial ostium, characteristically without any peri-articular soft tissue calcifications. An angiogram showed multiple intravascular calcifications. She was managed with a low-phosphate diet, sevelamer, niacinamide, acetazolamide, sucroferric oxyhydroxide to lower serum phosphate level, and topical sodium thiosulfate ectopic cutaneous calcification. Exome sequencing showed novel homozygous inframe deletion of ACG in FGF23 gene exon 3 at c.374_376 delins position (p. Asp125del) in the proband and a mutation in the heterozygous state in the mother and elder sibling, thus confirming a molecular diagnosis of HFTC. Our case had a unique neurological presentation of recurrent bilateral lower motor nerve facial palsy, hearing loss, multiple ectopic cutaneous calcifications without peri-articular deposits, multiple intravascular, intracranial, and vertebral endplate calcification, which has not been reported earlier. The proband showed a novel pathogenic variant suggesting an expanding phenotype of HFTC.

A Case of Unrelenting Facial Swelling

Melkersson-Rosenthal syndrome (MRS) is an uncommon disorder with presenting symptoms that typically involve the face and orofacial structures. It is a difficult diagnosis to make, as it may present with a protracted course of seemingly unrelated dermatological, ocular, and neurological findings. This case report reviews the presentation, workup, and diagnosis of a 75-year-old woman who presented with orofacial swelling, facial palsy, and tongue fissuring that had intermittently recurred over 10 years without a unifying diagnosis. Extensive medical history, photography, laboratory workup, and radiographic imaging were performed to identify the diagnosis of MRS in this patient. Our case highlights the challenge and importance of critically evaluating and consolidating a patient’s history of their present illness, physical examination, and ancillary testing to successfully establish a unifying diagnosis, especially when the diagnosis is relatively rare and diverse in its range of affected populations and symptomatology.

The Offprint of an Abnormal Pre-Parotidectomy Electrodiagnostic Finding in a Normally Functioning Facial Nerve: Correlation with Intraoperative Findings, Histology and Postoperative Facial Nerve Function

The aim of this study was to search for associations between an electrodiagnostically abnormal but clinically normal facial nerve before parotidectomy and the intraoperative findings, as well as the postoperative facial nerve function. The records of all patients treated for parotid tumors between 2002 and 2021 with a preoperative House–Brackmann score of grade I but an abnormal electrophysiologic finding were studied retrospectively. A total of 285 patients were included in this study, and 222 patients had a benign lesion (77.9%), whereas 63 cases had a malignant tumor (22.1%). Electroneurographic facial nerve involvement was associated with nerve displacement in 185 cases (64.9%) and infiltration in 17 cases (6%). In 83 cases (29.1%), no tumor–nerve interface could be detected intraoperatively. An electroneurographic signal was absent despite supramaximal stimulation in 6/17 cases with nerve infiltration and in 17/268 cases without nerve infiltration (p < 0.001). The electrophysiologic involvement of a normal facial nerve is not pathognomonic for a malignancy (22%), but it presents a rather rare (~6%) sign of a “true” nerve infiltration and could also appear in tumors without any contact with the facial nerve (~29%). Of our cases, two thirds of those with an anatomic nerve preservation and facial palsy had already directly and postoperatively recovered to a major extent in the midterm.