Abnormal Laryngeal Electromyography Findings in Asymptomatic Adults Across the Age Spectrum.

OBJECTIVES/HYPOTHESIS: Laryngeal electromyography (LEMG) is a diagnostic tool for patients with suspected neurogenic abnormalities of the larynx. LEMG is often used with the assumption that any abnormality is symptom-/disease-related. We sought to determine the prevalence of abnormal LEMG findings in a group of healthy asymptomatic adults across a large age spectrum. STUDY DESIGN: Open, prospective study, gender-match and age balanced by decade. METHODS: Forty-six healthy participants (age 20-78) underwent LEMG, including 178 muscles. Participants had no history of voice problems, normal VHI-10, and normal flexible laryngoscopy. Qualitative and quantitative LEMG (bilateral) were performed involving the thyroarytenoid-lateral cricoarytenoid muscle complex (TA-LCA) and cricothyroid (CT) muscles. LEMG parameters included evaluation for fibrillation potentials, sharp waves, reduced recruitment, polyphasic potentials, electrical synkinesis, and measurement of turns per second. RESULTS: Of participants, 4% had at least one abnormal qualitative finding (slightly reduced recruitment or two to three discrete polyphasic potentials). There were no findings of fibrillation potentials or sharp waves. There were no abnormal qualitative findings in the CT muscles tested. Of participants, 16% had at least one abnormal synkinesis finding. LEMG qualitative abnormalities and quantitative abnormalities do not appear to correlate with gender or age. CONCLUSION: Abnormal qualitative and quantitative LEMG findings were uncommon and minor in severity in our group of asymptomatic healthy adults. The likelihood of abnormal LEMG results in asymptomatic adults was 2.2% for qualitative findings, 9.3% for synkinesis, and 5.4% for turns/s. LEVEL OF EVIDENCE: 3 Laryngoscope, 131:2065-2069, 2021.

Paradoxical Frontalis Activation: An Underrecognized Consequence of Facial Palsy.

BACKGROUND: Aberrant reinnervation and synkinesis are common and debilitating after facial palsy. Paradoxical frontalis activation can antagonize eye closure and increase the risk of corneal damage. If recognized, judicious botulinum toxin injection to the affected side may reduce this risk. METHODS: One hundred consecutive patients with synkinesis were identified from a prospective database. Routine facial view photographs were converted to a standardized scale using iris diameter. The vertical distance from the midpoint of the midcanthal line to the inferior border of the eyebrow was measured bilaterally. A value of p < 0.05 was considered significant. RESULTS: Eighty-two patients were included, with a median age of 44 years (interquartile range, 33 to 59 years); 59 of the patients were women. The commonest cause was idiopathic (n = 55). The median time since onset of palsy was 13 months (interquartile range, 6.5 to 27 months). There was less midpoint of the midcanthal line to the inferior border of the eyebrow excursion on the synkinetic side of the face (p < 0.001). Twenty-two patients (27 percent) displayed paradoxical frontalis movement on the affected side of their face, with increased midpoint of the midcanthal line to the inferior border of the eyebrow distance (eyebrow raise) when attempting eye closure compared with eyebrow raise (n = 19), and tight eye closure compared with gentle eye closure (n = 3). CONCLUSIONS: This study highlights the phenomenon of paradoxical frontalis activation during eye closure. This is often underrecognized and may contribute to lagophthalmos. The authors found it to be present in 27 percent of patients with moderate or severe synkinesis. Further dynamic studies are required to understand the relationship among frontalis activity, eye closure, and the effects of its inhibition.

Characteristics of a nationwide cohort of patients presenting with isolated hypogonadotropic hypogonadism (IHH).

OBJECTIVE: Isolated hypogonadotropic hypogonadism (IHH) is a rare disorder with pubertal delay, normal (normoosmic-IHH, nIHH) or defective sense of smell (Kallmann syndrome, KS). Other reproductive and non-reproductive anomalies might be present although information on their frequency are scanty, particularly according to the age of presentation. DESIGN: Observational cohort study carried out between January 2008 and June 2016 within a national network of academic or general hospitals. METHODS: We performed a detailed phenotyping of 503 IHH patients with: (1) manifestations of hypogonadism with low sex steroid hormone and low/normal gonadotropins; (2) absence of expansive hypothalamic/pituitary lesions or multiple pituitary hormone defects. Cohort was divided on IHH onset (PPO, pre-pubertal onset or AO, adult onset) and olfactory function: PPO-nIHH (n = 275), KS (n = 184), AO-nIHH (n = 36) and AO-doIHH (AO-IHH with defective olfaction, n = 8). RESULTS: 90% of patients were classified as PPO and 10% as AO. Typical midline and olfactory defects, bimanual synkinesis and familiarity for pubertal delay were also found among the AO-IHH. Mean age at diagnosis was significantly earlier and more frequently associated with congenital hypogonadism stigmata in patients with Kallmann's syndrome (KS). Synkinesis, renal and male genital tract anomalies were enriched in KS. Overweight/obesity are significantly associated with AO-IHH rather than PPO-IHH. CONCLUSIONS: Patients with KS are more prone to develop a severe and complex phenotype than nIHH. The presence of typical extra-gonadal defects and familiarity for PPO-IHH among the AO-IHH patients indicates a common predisposition with variable clinical expression. Overall, these findings improve the understanding of IHH and may have a positive impact on the management of patients and their families.

IncobotulinumtoxinA treatment of facial nerve palsy after neurosurgery.

BACKGROUND: This study evaluates the effect of incobotulinumtoxinA in the acute and chronic phases of facial nerve palsy after neurosurgical interventions. METHODS: Patients received incobotulinumtoxinA injections (active treatment group) or standard rehabilitation treatment (control group). Functional efficacy was assessed using House-Brackmann, Yanagihara System and Sunnybrook Facial Grading scales, and Facial Disability Index self-assessment. RESULTS: Significant improvements on all scales were seen after 1month of incobotulinumtoxinA treatment (active treatment group, р<0.05), but only after 3months of rehabilitation treatment (control group, р<0.05). At 1 and 2years post-surgery, the prevalence of synkinesis was significantly higher in patients in the control group compared with those receiving incobotulinumtoxinA treatment (р<0.05 and р<0.001, respectively). CONCLUSIONS: IncobotulinumtoxinA treatment resulted in significant improvements in facial symmetry in patients with facial nerve injury following neurosurgical interventions. Treatment was effective for the correction of the compensatory hyperactivity of mimic muscles on the unaffected side that develops in the acute period of facial nerve palsy, and for the correction of synkinesis in the affected side that develops in the long-term period. Appropriate dosing and patient education to perform exercises to restore mimic muscle function should be considered in multimodal treatment.

Epidemiologic Overview of Synkinesis in 353 Patients with Longstanding Facial Paralysis under Treatment with Botulinum Toxin for 11 Years.

BACKGROUND: Patients with longstanding facial paralysis often exhibit synkinesis. Few reports describe the prevalence and factors related to the development of synkinesis after facial paralysis. Botulinum toxin type A injection is an important adjunct treatment for facial paralysis-induced asymmetry and synkinesis. The authors assessed the clinical and epidemiologic characteristics of patients with sequelae of facial paralysis treated with botulinum toxin type A injections to evaluate the prevalence of synkinesis and related factors. METHODS: A total of 353 patients (age, 4 to 84 years; 245 female patients) with longstanding facial paralysis underwent 2312 botulinum toxin type A injections during an 11-year follow-up. Doses used over the years, previous treatments (electrical stimulation, operations), and how they correlated to postparalysis and postreanimation synkinesis were analyzed. RESULTS: There was a significant association between cause and surgery. Most patients with facial paralysis caused by a congenital defect, trauma, or a tumor underwent reanimation. There were no sex- or synkinesis-related differences in the doses used, but the doses were higher in the reanimation group than in the no-surgery group. Synkinesis was found in 196 patients; 148 (41.9 percent) presented with postparalysis synkinesis (oro-ocular, oculo-oral) and 58 (16.4 percent) presented with postreanimation synkinesis. Ten patients presented with both types. CONCLUSIONS: This study determined the high prevalence (55.5 percent) of synkinesis in patients with longstanding facial paralysis. Postparalysis synkinesis was positively associated with infectious and idiopathic causes, electrical stimulation, facial nerve decompression, and no requirement for surgery. Postreanimation synkinesis was present in 28.2 percent of reanimated patients and was significantly associated with microsurgical flaps, transfacial nerve grafting, masseteric-facial anastomosis, and temporalis muscle transfers.

Mirror movements in parkinsonism: evaluation of a new clinical sign.

BACKGROUND: Mirror movements (MM) are not widely appreciated in parkinsonism and no report has evaluated this clinical sign in detail. OBJECTIVES: To define the parkinsonian clinical features associated with MM in patients with early, asymmetric parkinsonism. METHODS: Twenty seven patients with early Parkinson's disease were evaluated using a standardised videotaping protocol. MM were scored from blinded video assessment using a clinical scale that rates the amplitude, distribution, and proportion of mirroring in the less affected limb. Parkinsonian features were combined into axial and lateralised scores using related items of the Unified Parkinson's Disease Rating Scale. RESULTS: MM were present in 24 of 27 patients. There was a significant linear correlation between the degree of asymmetry of motor deficits and MM on the less affected side. The effect of asymmetry was greater when the proportional rather than the absolute motor difference between sides was largest. Asymmetry in leg rigidity was the most important examination feature in the prediction of contralateral foot mirroring. CONCLUSIONS: MM are a clinical feature of the unaffected or less affected side in mild asymmetric parkinsonism. Their presence may be a useful clinical finding in early parkinsonism.