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Central nervous system
Published in Brian J Pollard, Gareth Kitchen, Handbook of Clinical Anaesthesia, 2017
A preoperative history of disease progress, symptoms and functional ability is important to predict postoperative course. Assessment of respiratory function, including details of recent chest infection or evidence of hypoventilation, is crucial. A 30% fall in vital capacity from standing to lying suggests significant diaphragm involvement and postoperative ventilation may be necessary. A history of drooling, choking and a nasal voice suggests bulbar impairment. Severe bulbar or respiratory impairment precludes sedation for procedures such as endoscopic gastrostomy insertion.
Nasalance in adolescents with autism spectrum disorders
Published in International Journal of Speech-Language Pathology, 2020
Rachel Kasthurirathne, Karen Forrest, Jared Ross, Rita Patel
The large age-range of speakers that were included in the Shriberg et al. (2001) study and the variation in the phonetic content of the conversational speech sample obtained from diagnostic interviews suggest the need for a more focussed investigation of nasal resonance in the speech of people with ASD. These issues helped motivate Smerbeck’s (2015) investigation of “nasal voice” in boys with ASD between the ages of 6 and 13 years and age-matched neurotypical controls. Smerbeck investigated isolated neutral words produced by the speakers with and without ASD that were elicited from written and pictorial stimuli in an effort to limit the emotional content of spontaneous speech that could alter voice or resonance. A group of naïve listeners who underwent perceptual training listened to each word and then rated its nasality on a 6-point scale. A small but reliable between-group difference was found such that the speakers with ASD were rated as being more nasal than the control group. An examination of individual speakers found that 14 of 29 speakers in the ASD group were perceived to be highly nasal whereas only 5 of the 29 typical speakers received this rating. Smerbeck (2015) also found that listeners attributed negative social attributes (e.g. whining) to individuals with ASD, especially if the speakers sounded “highly” or “excessively” nasal.
Myasthenia gravis and chronic inflammatory demyelinating polyneuropathy in the same patient – a case report
Published in International Journal of Neuroscience, 2018
Weiwei Quan, Junhui Xia, Qiuling Tong, Jie Lin, Xiaolu Zheng, Xuezhi Yang, Dewei Xie, Yiyun Weng, Xu Zhang
About 10 days after admission, we found the symptoms of dysarthria and nasal voice a little fluctuating. Overlap of MG was suspected. Further repetitive nerve stimulation (RNS) testing showed a significant decrement (11%–13%) at 3–5 Hz stimulation in the deltoid muscle and orbicularis oculi muscle. Prostigmine test result was probable positive. The subsequent positive result of acetylcholine receptor (AchR) antibody (>8 nmol/L, normal <0.4 nmol/L) confirmed this diagnosis. Imaging of brain and mediastinum was normal. Antiganglioside antibodies and antibodies related to paraneoplastic neurological syndrome in serum were all negative. He was treated with pyridostigmine bromide, prednisone and mycophenolate mofetil. Good clinical results were observed, followed by consequent improvement of ptosis, dysphagia and dysarthria, and improvement of limb weakness. His muscle strength in four limbs was proximally grade 5/5 at discharge.
Bilateral Sixth Nerve Palsy and Nasal Voice in Two Sisters as a Variant of Guillan–Barré Syndrome
Published in Neuro-Ophthalmology, 2018
Francesco Pellegrini, Margaret Wang, Napoleone Romeo, Andrew G. Lee
A 6-year-old girl presented with acute, horizontal, binocular double vision associated with hypernasal speech. She was febrile and had a 2-day history of a sore throat, but systemic examination was otherwise unremarkable. She was seen by her paediatrician, diagnosed with “pharyngitis,” and given amoxicillin 12.25 mg per kg two times per day. One week later, ophthalmological examination revealed uncorrected visual acuity of 20/20 and normal anterior and posterior segment in both eyes (OU). There was no ptosis and pupils were equal. Extraocular motility revealed a bilateral abduction deficit and a moderate incomitant esotropia (ET) measuring 25 prism diopters (PDs) in primary position at a far distance and 6 PD ET at a near distance (Figure 1). The neurological examination was remarkable for a hypernasal voice (rhinolalia) caused by bilateral soft palate paralysis. Brain and orbit magnetic resonance imaging (MRI) with contrast was normal. Pharyngeal swab resulted positive for Streptococcus pyogenes and cerebrospinal fluid (CSF) analysis showed cytoalbuminologic dissociation (elevated CSF protein but no white blood cells) with CSF protein levels of 78 mg/dL (normal < 45 mg/dL). Acetylcholine receptor (AChR) antibody testing was negative. On further questioning, the relatives admitted that the 10-year-old older sister suffered from double vision and nasal voice after an episode of acute pharyngitis just 1 month before. She had been evaluated in another hospital where the neurologist noted rhinolalia aperta caused by palate palsy and bilateral weakness of lateral rectus muscles. AChR antibody testing resulted negative and symptoms regressed spontaneously in a few days. Testing for anti-GQ1b antibodies in both the patient and her sister was negative. Our patient was diagnosed with polyneuritis cranialis and given a course of daily intravenous immune globulin (40 mg for 5 days) and intravenous methylprednisolone (40 mg for 3 days) followed by oral taper. She gradually recovered and at 1 month follow-up, both voice and extraocular motility (Figure 2) were back to normal. Stereopsis (Lang test) was also normal.