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Abstract: Objective: Neurologic disease is the most common cause of bilateral vocal fold paralysis in children. Arnold-Chiari malformations (ACM) account for the majority. Early decompression results in the resolution of preoperative symptoms in the majority of patients. The purpose of present study is to describe the time to vocal fold function recovery following neurosurgical management of children with vocal fold paralysis secondary to ACM. Methods: Prospective case series. Inclusion criteria included diagnoses of both ACM and vocal fold paralysis. All children were followed with office flexible laryngoscopy at two-month intervals following diagnosis until complete return of vocal fold motion was noted. Return of recurrent laryngeal nerve function was confirmed with intraoperative laryngeal electromyography (EMG); one child additionally underwent EMG during partial return of vocal fold function. Results: Four patients met inclusion criteria. Mean age at neurosurgical decompression was 3.1 months (range 1–7.5). Three subjects demonstrated bilateral paralysis; one had a left-sided paralysis. Three subjects, including the child with unilateral paralysis, required tracheotomy. Initial return of vocal fold motion was seen at a mean of 5.75 months after decompression (range 4–8). Complete return of function was seen at a mean of 9.5 months (range 7–12). One child underwent EMG when vocal fold function was initially seen to return, with low-amplitude activity seen. The patient demonstrated full amplitude when EMG was repeated following return of full abduction. EMG likewise confirmed return of motion in the three other subjects. All tracheotomized patients were decannulated without further surgical procedures. Conclusion: Methods of airway management that do not result in permanent alteration of laryngeal anatomy are preferred. Families may be counseled that return of vocal fold function is expected within one year of neurosurgical decompression. The presence of electrical activity on laryngeal EMG may have a role in predicting return of vocal fold motion before complete function is visible on flexible laryngoscopy. [Copyright &y& Elsevier]

Objectives: We sought to determine whether serial intraoperative laryngeal electromyography (L-EMG) or evoked L-EMG predicts vocal fold (VF) recovery following iatrogenic injury. Methods: Six beagles were sedated, and bipolar needle electrodes were inserted into each thyroarytenoid (TA) muscle. Endotracheal tube surface electrodes were also placed. As the sedation lightened. L-EMG activity was recorded from all electrodes with an intraoperative nerve monitoring system. The neck was opened, and direct recurrent laryngeal nerve (RLN) stimulation was performed. Subsequently, 4 animals underwent crush injury of the left RLN, and 2 animals underwent nerve transection. The L-EMG procedures were repeated every 1 to 2 weeks until left VF motion was observed in the dogs that suffered RLN crush injury. At each time point, the neck was opened and both RLNs were stimulated. Results: Fibrillation potentials were detected in ail animals after RLN injury. A change to electrical silence was seen in the animals in the crush injury group that were evaluated during the week preceding VF recovery. Fibrillation potentials and VF immobility persisted in the transection group throughout the complete time course of these experiments. The first appearance of an evoked response coincided with occurred after the return of left VF motion in the crush injury group. The threshold, latency, and amplitude differed from those of the controls and approached normal values over time. No response was detected in the transected nerves. Conclusions: The disappearance of fibrillations on intraoperative L-EMG was noted in the animals tested the week before the return of VF motion, and the return of motor unit action potentials was seen along with return of VF function. Evoked L-EMG was not helpful in predicting the return of VF mobility, but it may help quantify degrees of RLN injury and predict the speed of recovery. [ABSTRACT FROM AUTHOR]

Abstract: Sensory neuronopathy associated with Sjögren’s syndrome (SS) usually has a subacute or chronic onset. We report the case of a 37-year-old woman who presented with an unusual hyperacute form of SS ganglionopathy. She initially developed paresthesias of her fingertips and rapidly became severely ataxic. Nerve conduction studies revealed abnormal sensory but normal motor functions. Lip biopsy showed findings consistent with SS. Sural nerve biopsy showed severe axonal loss. The patient showed modest improvement with immunosuppressive therapies. [Copyright &y& Elsevier]

Abstract: A 49-year-old man developed simultaneously a Guillain Barré Syndrome (GBS) and a nephrotic syndrome (NS). The patient relapsed twice, despite treatment with intravenous immunoglobulins (IVIg) after a full or partial recovery, and became resistant to IVIg. Renal biopsy revealed focal segmental glomerulosclerosis (FSGS). He responded to plasmapheresis and corticosteroids with simultaneous recovery of his GBS and NS, suggesting a common pathogenesis of the two conditions. [Copyright &y& Elsevier]