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July 2010

Bilateral Vocal Cord Paralysis: A Rare Onset of Amyotrophic Lateral Sclerosis

Author Affiliations

Author Affiliations: Neurology Unit, Department of Neuroscience, University of Modena and Reggio Emilia, Nuovo Ospedale Civile S. Agostino Estense di Modena, Modena, Italy.

Arch Neurol. 2010;67(7):897-899. doi:10.1001/archneurol.2010.141

We read with great interest the neurological review about vocal cord dysfunction in amyotrophic lateral sclerosis (ALS).1 We agree that vocal cord dysfunction, including hoarseness and stridor, may be part of the clinical picture of ALS but in our experience they are quite rare at onset; of 350 patients diagnosed with ALS in the province of Modena from 1990 to the present, only 1 patient had stridor at onset. This 74-year-old man without a family history of ALS presented to the emergency department after a 6-month history of persistent hoarseness. He had dyspnea associated with hoarseness and stridor. Neurological examination revealed fasciculations of the tongue and 4 limbs. Dysarthria, dysphagia, and sensory, extrapyramidal, autonomic, and cerebellar signs were absent. Tendon reflexes were brisk. The laryngologist detected bilateral vocal cord abductor paralysis. Results of nerve-conduction studies were normal, and electromyograph revealed widespread acute and chronic denervation in the 4 limbs and bulbar region. Clinical and neurophysiological findings were consistent with ALS. Other diseases were excluded with appropriate tests. During his stay in hospital, respiratory distress increased with severe stridor. Noninvasive ventilation and partial cordotomy were ineffective. The patient agreed to perform tracheostomy to recover from acute respiratory distress. Spirometry showed mildly reduced forced vital capacity, arterious emogasanalysis was normal, and the patient did not need mechanical ventilation.

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