Hereditary neuropathy with liability to pressure palsies (HNPP) is an autosomal dominant peripheral neuropathy characterized by recurrent episodes of paralysis of peripheral nerves, usually after a minor trauma to topographies prone to compression 1,2 . Involvement of the cranial nerves has rarely been described in these patients [3][4][5][6][7][8] . Reports of swallowing dysfunction in patients with HNPP are even rarer.We report a patient diagnosed with HNPP confirmed by molecular testing who presented with recurrent episodes of swallowing dysfunction (SD). CaSeA 41-year-old man who presented with recurrent episodes of acute mononeuropathies affecting mainly the ulnar, radial and peroneal nerves followed by spontaneous improvement since the age of 12 years. In the years prior to diagnosis, the patient started experiencing sudden episodes of reversible SD without a specific trigger, but it worsening with progressive increased of volume and solid consistency of bolus food. The SD had a spontaneous improvement after few weeks, but with residual dysfunction when he drinking liquid. Some of his relatives (sisters and nephew) had similar symptoms, but none had SD. He denied episodes of aspiration (airway protection), exposure to drugs or toxic agents.Cranial nerves showed mild weakness in posterior tongue during the swallow. Gag reflex, taste and sensation of tongue and oropharynx were normal. Mild asymmetrical muscle atrophy was observed in both hands and forearms while weakness was detected in the left first dorsal interosseus dorsal and flexor carpi ulnaris (grades 3 and 4, respectively, according to the Medical Research Council scale). Tendon reflexes were absent in both biceps and left brachioradial and ankle tendon reflexes were also absent. Pain, pinprick and light touch sensory impairment were detected on the territories of the left median, bilateral sural and superficial peroneal nerves.Nerve conduction studies showed a nonuniform demyelinating neuropathy, mainly the ulnar, radial and peroneal nerves, together with evidence of asymmetrical acute and/or chronic denervation affecting predominantly the first dorsal interosseus, tibialis anterior and gastrocnemius muscles. Nerve conduction studies with similar findings were observed in the patient's sisters and nephew.Laboratory tests as well as serological tests were normal. Biopsy of the sural nerve showed a reduction in the number of large myelinated fibers and focal thickening of the myelin sheath (formation of tomaculae). Biopsy of the gastrocnemius muscle biopsy showed recent and chronic denervation.Phonoaudiological evaluation, videoendoscopic and fluoroscopic examination of deglutition revealed normal oral (preparatory and transport) phase; swallowing test (liquid, semisolid and solid consistencies) presented mild nasal escape, normal oral transit time, residual bolus in pyriform sinuses and valleculae cleared by dry swallow and cough, episodes of laryngeal penetration only after great volume of liquid, but with normal coughing reflex, and absence of asp...

Properties of signals captured by contact accelerometers assist the evaluation of human organs involved in the swallowing process. Here we present a novel method to account the alignment between two different swallowing signals based on two steps: first, a scaled cross-correlation determines an anchor point of maximum similarity between the signals, dividing both signals in two parts; next, dynamic time warping aligns the left and right parts of both signals. Additional filtering procedures are used smooth the swallowing signals due to their low SNR. The results show that this technique can be used to assess similarities between liquid and solid boluses of a subject.

RESUMO Objetivo Investigar a percepção auditiva de juízes leigos quanto ao gênero de mulheres com edema de Reinke, relacionada com o grau do edema e a frequência fundamental da voz. Método Estudo observacional, analítico e transversal. Participaram 46 juízes leigos que analisaram 48 vozes disfônicas - 24 vozes de mulheres com Edema de Reinke (Grupo Edema de Reinke - GER) e 24 vozes de mulheres e homens com outros tipos de disfonias (Grupo Controle - GC). Os juízes analisaram a contagem de números de 1 a 10 e julgaram as vozes como pertencentes a homem ou mulher, além de descreverem também se tinham certeza ou dúvida quanto à resposta. Os resultados do GER foram associados ao Grau do Edema (1, 2 ou 3) e à frequência fundamental média (F0), analisada por meio da vogal /Ɛ/. Resultados Observou-se que a porcentagem de erros em relação ao gênero foi maior no GER quando comparada à do GC, o percentual de certezas quanto ao gênero foi maior no GC. No GER, edemas de grau 1 ocasionaram menos erros quando comparados aos graus 2 e 3. A média da F0 das mulheres cujas vozes foram julgadas como masculinas (141 Hz) foi menor do que as identificadas corretamente (149 Hz). Conclusão Por meio da avaliação de juízes leigos, as vozes das mulheres com edema de Reinke são identificadas como vozes masculinas. O aumento do grau do edema e a redução da F0 estão relacionados à maior quantidade de erros e/ou dúvidas em relação à identificação do gênero de mulheres com edema de Reinke .

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Introduction: Medical literature shows consistent data on the use of botulinum toxin for the treatment of drooling, especially in patients with neurological disorder. However, any trial evaluated the reduction of sialorrhea through laryngotracheoscopy. Objective: To evaluate the reduction of salivation and aspiration in children with cerebral palsy submitted to intraparotid injection of botulinum toxin. Methods: A prospective cohort study including 31 pediatric patients with cerebral palsy who complained of sialorrhea and were submitted to an intraparotid injection of botulinum toxin type A. It was analysed the reduction of the number of bibs and the amount of saliva in the larynx and trachea through a laryngotracheoscopic examination. Results: There was a reduction in the number of bibs from 7.35 to 4 after application of BoNT/A, with p-value < 0.001. The age of the patients did not influence on the number of bibs. 71% of the patients reduced the amount of saliva in laryngotracheoscopic examination. The number of normal exams increased from 3.2% to 38.7% and aspiration fell from 38.7% to 12.9%. Conclusion: Application of botulinum toxin type A is a good method for temporary control of salivation in children with neurological disorders.