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A Case of Creutzfeldt-Jacob Disease with Bilateral Vocal Fold Abductor Paralysis
A Case of Creutzfeldt-Jacob Disease with Bilateral Vocal Fold Abductor Paralysis *Lishu Li, *Hideto Saigusa, †Hiroshi Nagayama, *Tsuyoshi Nakamura, *Iichirou Aino, *Taro Komachi, and *Satoshi Yamaguchi, Tokyo, Japan Summary: Bilateral vocal fold abductor paralysis was seen in a patient with Creutzfeldt-Jacob disease. After tracheotomy, the patient showed disappearance of reduced oxygen saturation with high-pitched inspiratory stridor and pulling phenomenon of the supraclavicular region and larynx. Electromyographic examinations of the intrinsic laryngeal muscles, including the thyroarytenoid and posterior cricoarytenoid muscles, demonstrated that there was no apparent action potential in those muscles during spontaneous respiratory movements, and there was no abnormal potential for those muscles at rest. By pushing the infrasternal region of the patient on the expiration, normal motor unit action potential could be seen in the posterior cricoarytenoid muscle on the next inspiration. Based on those findings, we concluded that bilateral vocal fold abductor paralysis in this case of Creutzfeldt-Jacob disease was not induced by disorders of the degeneration of motor nucleus in the ambiguus as in multiple system atrophy, but by a disorder of the upper motor neuron. Key Words: Bilateral vocal fold abductor paralysis–Creutzfeldt-Jacob disease–Electromyographic examination– Posterior cricoarytenoid muscle.
INTRODUCTION Respiratory distress in patients with Creutzfeldt-Jacob disease has been described in several reports in relation to sleep apnea.1–3 Sleep apnea in Creutzfeldt-Jacob disease has been considered related to abnormal wave patterns on electroencephalogram, such as periodic synchronous discharge (PSD)— non-PSD cycle.1–3 Although bilateral vocal fold abductor paralysis should be considered a life-threatening condition, only a few cases of bilateral vocal fold abductor paralysis in Creutzfeldt-Jacob disease have been published.4–6 And the mechanism of bilateral vocal fold abductor paralysis in Creutzfeldt-Jacob disease has not yet been clarified. Here, we report a case of bilateral vocal fold abductor paralysis in Creutzfeldt-Jacob disease and we examined the conditions of the intrinsic laryngeal muscles by electromyography. Based on these findings, we assessed the mechanism of bilateral vocal fold abductor paralysis in this case of Creutzfeldt-Jacob disease. CASE REPORT A 56-year-old woman had been admitted for progressive or severe cognitive and memory impairment 3 years earlier. One year later, she showed the clinical features of akinetic mutism. Startle responses could easily be elicited by painful stimuli. Electroenecephalographic examination showed a PSD pattern 9 months later. Laboratory examination of cerebrospinal fluid was positive for 14-3-3 protein and the degree of NSE was increased. The analysis for prion gene indicated M/M type Accepted for publication February 7, 2008. From the *Department of Otolaryngology, Nippon Medical School, Tokyo, Japan; and the yDepartment of Neurology, Nippon Medical School, Tokyo, Japan. Address correspondence and reprint requests to Hideto Saigusa, MD, Nippon Medical School, Department of Otolaryngology, 1-1-5 Sendagi, Bunkyo-ku, Tokyo 113-8603, Japan. E-mail: [email protected] Journal of Voice, Vol. 23, No. 5, pp. 635-638 0892-1997/$36.00 Ó 2009 The Voice Foundation doi:10.1016/j.jvoice.2008.02.002
without gene abnormality. MRI findings of the brain demonstrated severe cortical atrophy and dilatation of the lateral ventricles (Figure 1). 123I-IMP SPECT showed severely low distribution of blood flow in the cerebral cortex, basal ganglia, and thalamus, but flow was not as disturbed in cerebellum and brainstem. Based on these findings, she was diagnosed as a solitary type of Creutzfeldt-Jacob disease. She was nutritionally supported via a nasogastric feeding tube. For 3 months, she had presented severe inspiratory stridor with a high-pitched sound, tachypnea, and pulling phenomenon of the supraclavicular region and larynx all day along (Figure 2). Her respiratory pattern was regular. Arterial blood gas analysis under room air showed PCO2 49.9 mm Hg, PO2 55 mm Hg, and oxygen saturation 88.8%. Laryngoscopic findings demonstrated that bilateral vocal folds were fixed in near middle positions (Figure 3), and saliva and secretion in the hypopharynx were drawn through the glottis into the trachea during inspiration. To release the airway distress of the patient, we performed the cricothyrotomy under local anesthesia at first, because the airway distress was severe and the startle responses of the patient could easily be elicited by painful stimuli. Then, the intubation tube was inserted through the space of the cricothyrotomy. Next, the tracheotomy (the tracheal fenestration) was performed under general anesthesia. After tracheotomy, her stridor disappeared and arterial blood gas analysis showed improvements to PCO2 44.1 mm Hg, PO2 102 mm Hg, and oxygen saturation 98.2%. Abnormal space-occupying lesion related to the bilateral vocal fold abductor paralysis could not be detected on chest CT. Then, we examined the neurological conditions of the intrinsic laryngeal muscles by electromyography with a needle electrode via the cricothyroid membrane as reported by Hirano and Ohara.7 Electromyographic examination showed that there was no apparent action potential for the thyroarytenoid and posterior cricoarytenoid muscles during spontaneous respiration, but there was no abnormal potential, that is, fibrillation potential and positive sharp wave, for those muscles at rest (Figures 4A, B). By pushing the infrasternal region of the patient during
636
FIGURE 1. T1-weighted image of MRI showed severe cortical atrophy in the brain and dilatation of the lateral ventricles. expiration, normal motor unit action potential could be seen in the posterior cricoarytenoid muscle on the next inspiration (Figure 4C). DISCUSSION Bilateral vocal fold abductor paralysis could be seen in some neurodegenerative diseases including multiple system atrophy,8 Parkinson’s disease,9 progressive supra bulbar palsy,10 amyotrophic lateral sclerosis,11 Machado-Joseph’s disease,12 familial amyloid neuropathy,12 and chronic inflammatory demyelinated neuropathy.13 There have been a few reports describing bilateral vocal fold abductor paralysis in Creutzfeldt-Jacob disease.4–6 It was considered that the mechanisms of bilateral vocal fold abductor paralysis differed for each pathological condition. In cases of multiple system atrophy with bilateral vocal fold abductor paralysis, selective neurogenic atrophy was seen in the posterior cricoarytenoid muscle without disturbance of the other intrinsic laryngeal muscles. Isozaki
Journal of Voice, Vol. 23, No. 5, 2009
et al14 reported that cell numbers in the middle of the nucleus ambiguus decreased significantly in patients with multiple system atrophy. In cases of amyotrophic lateral sclerosis or Machado-Joseph’s disease with vocal fold abductor paralysis, neurogenic atrophy could be seen not only in the posterior cricoarytenoid muscle, but also in other intrinsic laryngeal muscles with degenerative change of all motor neurons in the nucleus ambiguus.12,14 In cases of familial amyloid neuropathy or chronic inflammatory demyelinated neuropathy, disorder of myelinated fiber could be seen in recurrent laryngeal nerve and neurogenic atrophy could be observed in all intrinsic laryngeal muscles.12 However, in cases of Parkinson’s disease, there was no apparent pathological change in the intrinsic laryngeal muscle.12,15 It was considered that the mechanism of bilateral vocal fold abductor paralysis in the Parkinson’s disease might differ from that in multiple system atrophy. Isozaki et al15 claimed that bilateral vocal fold abductor paralysis in cases of Parkinson’s disease might be caused by persistent overactivity of the intrinsic laryngeal muscles. Iwata et al4 reported two cases of Creutzfeldt-Jacob disease with bilateral vocal fold abductor paralysis. Based on autopsy examination of one case, they indicated that there was no abnormal change in any of the intrinsic laryngeal muscles or in the nucleus ambiguus. Thus, they suggested that bilateral vocal fold abductor paralysis might be caused by disturbance of the upper motor neurons. Kudo et al5 reported that apnea and a loud snore in a case with Creutzfeldt-Jacob disease were improved markedly with improvement of the electroencephalographic findings from a cyclic PSD-non PSD pattern to a continuous PSD pattern after tracheal intubation. They also indicated that there was no obstructive abnormality in the oropharynx. On electromyographic examination of our case, there was no action potential in the posterior cricoarytenoid or thyroarytenoid muscles during spontaneous respiration, and there was no abnormal potential, that is, fibrillation and positive sharp wave at rest. After pushing the infrasternal region of our patient on expiration, a normal motor unit action potential could be seen in the posterior cricoarytenoid muscle on the next inspiration. Pushing the infrasternal region is one of the chest therapy techniques, called ‘‘diaphragmatic breathing exercise’’ used for chronic obstructive pulmonary disease. The technique is as following: First, the therapist pushes the infrasternal region of the
FIGURE 2. Pulling phenomenon of the supraclavicular region (O) and the lower edge of the cricoid cartilage (*) occurred on inspiration. (A) On expiration, (B) on inspiration.
Lishu Li, et al
637
Creutzfeldt-Jacob Disease: A Case Report
FIGURE 3. Laryngoscopic findings demonstrated bilateral vocal fold abductor paralysis.
patient to flat the infrasternal region during the expiration with the gentle power. Next, when at the end of the expiration until the inspiration begins, the therapist pushes the infrasternal region of the patient with more strong power so as not to affect the pain for the patient. After pushing the infrasternal region of the patient, the inspiratory movement could be stronger and the inspiration duration could be longer reflectively than those before the exercise.16,17 The mechanisms of the reflectively movement of the diaphragmatic breathing exercise could be considered a deflation reflex as part of Hering-Breuer’s reflex
via the vagus nerve. It was reported that the activity of the posterior cricoarytenoid muscle increased in response to negative pressure and/or absence of volume feedback. And, it was reported that there was a temporal relationship between the activation of the posterior cricoarytenoid muscle and the inspiratory phase of the respiratory cycle during tidal breathing in humans.18–20 Thus, it could be considered that reflective control of respiratory movement via the vagus nerve in the medulla might have been conserved in our case. Based on these findings, we considered that bilateral vocal fold abductor paralysis of this case might not be caused by a disturbance of nucleus ambiguus or peripheral nerve, but a disturbance of the upper motor neurons. These findings might be consistent with 123I-IMP SPECT findings showing a severely disturbed cerebral cortex with slightly disturbed brain stem. It could be considered that these findings are in agreement with the pathological findings reported by Iwata et al.4 Further clinical and pathophysiological studies including electromyography may be warranted to elucidate bilateral vocal fold abductor paralysis in Creutzfeldt-Jacob disease. It is considered that physicians should be aware of the potential risk of bilateral vocal fold abductor paralysis in Creutzfeldt-Jacob disease, because emergency tracheotomy may be required.
REFERENCES 1. Goto K, Umezaki H, Suetsugu M. Electroencephalographic and clinicopathological studies on Creutzfeldt-Jakob syndrome. J Neurol Neurosurg Psychiatr. 1976;39:931-940. 2. Onoda M, Koike Y, Hibino R, Takahashi A, Sobue I. Sleep apnea in Creutzfeldt-Jakob disease and subacute sclerosing panencephalitis-with reference to periodic synchronous discharge. Clin Neurol. 1978;18:601-607.
FIGURE 4. The raw electromyographic curves of the thyroarytenoid and posterior cricoarytenoid muscles. There was no apparent action potential or abnormal potential, including fibrillation potential or positive sharp wave, at the thyroarytenoid and posterior cricoarytenoid muscles during spontaneous respiration. After pushing the infrasternal region of the patient on the expiration, normal action potential was seen at the posterior cricoarytenoid muscle on the next inspiration. (A) Thyroarytenoid muscle on the left side during normal respiratory movement, (B) posterior cricoarytenoid muscles on the right side during normal respiratory movement, (C) posterior cricoarytenoid muscles of the right side after pushing the infrasternal region of the patient on the expiration.
638 3. Mamdani MB, Masdeu J, Ross E, Ohara R. Sleep apnea with unusual EEG changes in Jakob-Creutzfeldt disease. Electroencephalogr Clin Neurophysiol. 1983;55:411-416. 4. Iwata S, Nakamura A, Nakamura N, Sakashita I, Fukunaga H. 2 Cases of Creutzfeldt-Jacob disease with bilateral vocal cord paralysis. Clin Neurol. 1988;28:333-337. 5. Kudo Y, Tamaru F, Motomura N, Yamadori A. Disappearance of sleep apnea by tracheal intubation in Creutzfeldt-Jakob disease. Electroencephalogr Clin Neurophysiol. 1984;58:226-229. 6. Isozaki E. Vocal fold paralysis in neurodegenerative diseases. Otorhinolaryngology. 1998;41:393-403 [in Japanese]. 7. Hirano M, Ohara J. Use of hooked-wire electrodes for electromyography of the intrinsic laryngeal muscles. J Speech Hear Res. 1969;12:362-373. 8. Isozaki E, Naito A, Horiguchi S, Kawamura R, Hayashida T, Tanabe H. Early diagnosis and stage-classification of vocal cord abductor paralysis in multiple system atrophy. J Neurol Neurosurg Psychiatr. 1996;60: 399-402. 9. Plasse HM, Lieberman AN. Bilateral vocal cord paralysis in Parkinson’s disease. Arch Otolaryngol. 1981;107:252-253. 10. Yokoji H, Nakamura S, Ikeda T. A case of progressive supranuclear palsy associated with bilateral vocal cord abductor paralysis. Rinsho Shinkeigaku. 1997;37:523-525. 11. Palesse N, Marelli A, Legge MP. Bilateral abductor paralysis of the vocal cords in the course of neurological diseases: report of 5 cases. Ital J Neurol Sci. 1988;9:59-62. 12. Isozaki E, Hayashi M, Hayashida T, Oda M, Hirai S. Myopathology of the intrinsic laryngeal muscles in neurodegenerative diseases, with reference to the mechanism of vocal cord paralysis. Rinsho Shinkeigaku. 1998;38: 711-718.
Journal of Voice, Vol. 23, No. 5, 2009 13. Yamamoto T, Ohnishi A, Miyoshi T, Hashimoto T, Murai Y. A case of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) with bilateral recurrent nerve palsy and primary alveolar hypoventilation—comparative studies of the histological findings of the two sural nerve biopsies with 9 years interval. Rinsho Shinkeigaku. 1994;34: 712-716. 14. Isozaki E, Matsubara S, Hayashida T, Oda M, Hirai S. Morphometric study of nucleus ambiguus in multiple system atrophy presenting with vocal cord abductor paralysis. Clin Neuropathol. 2000;19:213-220. 15. Isozaki E, Shimizu T, Takamoto K, et al. Vocal cord abductor paralysis (VCAP) in Parkinson’s disease: difference from VCAP in multiple system atrophy. J Neurol Sci. 1995;130:198-202. 16. Brach BB, Chao RP, Sgroi VL, Minh VD, Ashburn WL, Moser KM. 133Xenon washout patterns during diaphragmatic breathing. Studies in normal subjects and patients with chronic obstructive pulmonary disease. Chest. 1977;71:735-739. 17. Miller WF. A physiologic evaluation of the effects of diaphragmatic breathing training in patients with chronic pulmonary emphysema. Am J Med. 1954;17:471-477. 18. Brancatisano TP, Dodd DS, Engel LA. Respiratory activity of posterior cricoarytenoid muscle and vocal cords in humans. J Appl Physiol. 1984;57:1143-1149. 19. Mathew OP, Sant’Ambrogio FB, Woodson GE, Sant’Ambrogio G. Respiratory activity of the cricothyroid muscle. Ann Otol Rhinol. 1988;97: 680-687. 20. Sant’Ambrogio FB, Mathew OP, Clark WD, Sant’ Ambrogio G. Laryngeal influences on breathing pattern and posterior cricoarytenoid muscle activity. J Appl Physiol. 1985;58:1298-1304.
INTRODUCTION Respiratory distress in patients with Creutzfeldt-Jacob disease has been described in several reports in relation to sleep apnea.1–3 Sleep apnea in Creutzfeldt-Jacob disease has been considered related to abnormal wave patterns on electroencephalogram, such as periodic synchronous discharge (PSD)— non-PSD cycle.1–3 Although bilateral vocal fold abductor paralysis should be considered a life-threatening condition, only a few cases of bilateral vocal fold abductor paralysis in Creutzfeldt-Jacob disease have been published.4–6 And the mechanism of bilateral vocal fold abductor paralysis in Creutzfeldt-Jacob disease has not yet been clarified. Here, we report a case of bilateral vocal fold abductor paralysis in Creutzfeldt-Jacob disease and we examined the conditions of the intrinsic laryngeal muscles by electromyography. Based on these findings, we assessed the mechanism of bilateral vocal fold abductor paralysis in this case of Creutzfeldt-Jacob disease. CASE REPORT A 56-year-old woman had been admitted for progressive or severe cognitive and memory impairment 3 years earlier. One year later, she showed the clinical features of akinetic mutism. Startle responses could easily be elicited by painful stimuli. Electroenecephalographic examination showed a PSD pattern 9 months later. Laboratory examination of cerebrospinal fluid was positive for 14-3-3 protein and the degree of NSE was increased. The analysis for prion gene indicated M/M type Accepted for publication February 7, 2008. From the *Department of Otolaryngology, Nippon Medical School, Tokyo, Japan; and the yDepartment of Neurology, Nippon Medical School, Tokyo, Japan. Address correspondence and reprint requests to Hideto Saigusa, MD, Nippon Medical School, Department of Otolaryngology, 1-1-5 Sendagi, Bunkyo-ku, Tokyo 113-8603, Japan. E-mail: [email protected] Journal of Voice, Vol. 23, No. 5, pp. 635-638 0892-1997/$36.00 Ó 2009 The Voice Foundation doi:10.1016/j.jvoice.2008.02.002
without gene abnormality. MRI findings of the brain demonstrated severe cortical atrophy and dilatation of the lateral ventricles (Figure 1). 123I-IMP SPECT showed severely low distribution of blood flow in the cerebral cortex, basal ganglia, and thalamus, but flow was not as disturbed in cerebellum and brainstem. Based on these findings, she was diagnosed as a solitary type of Creutzfeldt-Jacob disease. She was nutritionally supported via a nasogastric feeding tube. For 3 months, she had presented severe inspiratory stridor with a high-pitched sound, tachypnea, and pulling phenomenon of the supraclavicular region and larynx all day along (Figure 2). Her respiratory pattern was regular. Arterial blood gas analysis under room air showed PCO2 49.9 mm Hg, PO2 55 mm Hg, and oxygen saturation 88.8%. Laryngoscopic findings demonstrated that bilateral vocal folds were fixed in near middle positions (Figure 3), and saliva and secretion in the hypopharynx were drawn through the glottis into the trachea during inspiration. To release the airway distress of the patient, we performed the cricothyrotomy under local anesthesia at first, because the airway distress was severe and the startle responses of the patient could easily be elicited by painful stimuli. Then, the intubation tube was inserted through the space of the cricothyrotomy. Next, the tracheotomy (the tracheal fenestration) was performed under general anesthesia. After tracheotomy, her stridor disappeared and arterial blood gas analysis showed improvements to PCO2 44.1 mm Hg, PO2 102 mm Hg, and oxygen saturation 98.2%. Abnormal space-occupying lesion related to the bilateral vocal fold abductor paralysis could not be detected on chest CT. Then, we examined the neurological conditions of the intrinsic laryngeal muscles by electromyography with a needle electrode via the cricothyroid membrane as reported by Hirano and Ohara.7 Electromyographic examination showed that there was no apparent action potential for the thyroarytenoid and posterior cricoarytenoid muscles during spontaneous respiration, but there was no abnormal potential, that is, fibrillation potential and positive sharp wave, for those muscles at rest (Figures 4A, B). By pushing the infrasternal region of the patient during
636
FIGURE 1. T1-weighted image of MRI showed severe cortical atrophy in the brain and dilatation of the lateral ventricles. expiration, normal motor unit action potential could be seen in the posterior cricoarytenoid muscle on the next inspiration (Figure 4C). DISCUSSION Bilateral vocal fold abductor paralysis could be seen in some neurodegenerative diseases including multiple system atrophy,8 Parkinson’s disease,9 progressive supra bulbar palsy,10 amyotrophic lateral sclerosis,11 Machado-Joseph’s disease,12 familial amyloid neuropathy,12 and chronic inflammatory demyelinated neuropathy.13 There have been a few reports describing bilateral vocal fold abductor paralysis in Creutzfeldt-Jacob disease.4–6 It was considered that the mechanisms of bilateral vocal fold abductor paralysis differed for each pathological condition. In cases of multiple system atrophy with bilateral vocal fold abductor paralysis, selective neurogenic atrophy was seen in the posterior cricoarytenoid muscle without disturbance of the other intrinsic laryngeal muscles. Isozaki
Journal of Voice, Vol. 23, No. 5, 2009
et al14 reported that cell numbers in the middle of the nucleus ambiguus decreased significantly in patients with multiple system atrophy. In cases of amyotrophic lateral sclerosis or Machado-Joseph’s disease with vocal fold abductor paralysis, neurogenic atrophy could be seen not only in the posterior cricoarytenoid muscle, but also in other intrinsic laryngeal muscles with degenerative change of all motor neurons in the nucleus ambiguus.12,14 In cases of familial amyloid neuropathy or chronic inflammatory demyelinated neuropathy, disorder of myelinated fiber could be seen in recurrent laryngeal nerve and neurogenic atrophy could be observed in all intrinsic laryngeal muscles.12 However, in cases of Parkinson’s disease, there was no apparent pathological change in the intrinsic laryngeal muscle.12,15 It was considered that the mechanism of bilateral vocal fold abductor paralysis in the Parkinson’s disease might differ from that in multiple system atrophy. Isozaki et al15 claimed that bilateral vocal fold abductor paralysis in cases of Parkinson’s disease might be caused by persistent overactivity of the intrinsic laryngeal muscles. Iwata et al4 reported two cases of Creutzfeldt-Jacob disease with bilateral vocal fold abductor paralysis. Based on autopsy examination of one case, they indicated that there was no abnormal change in any of the intrinsic laryngeal muscles or in the nucleus ambiguus. Thus, they suggested that bilateral vocal fold abductor paralysis might be caused by disturbance of the upper motor neurons. Kudo et al5 reported that apnea and a loud snore in a case with Creutzfeldt-Jacob disease were improved markedly with improvement of the electroencephalographic findings from a cyclic PSD-non PSD pattern to a continuous PSD pattern after tracheal intubation. They also indicated that there was no obstructive abnormality in the oropharynx. On electromyographic examination of our case, there was no action potential in the posterior cricoarytenoid or thyroarytenoid muscles during spontaneous respiration, and there was no abnormal potential, that is, fibrillation and positive sharp wave at rest. After pushing the infrasternal region of our patient on expiration, a normal motor unit action potential could be seen in the posterior cricoarytenoid muscle on the next inspiration. Pushing the infrasternal region is one of the chest therapy techniques, called ‘‘diaphragmatic breathing exercise’’ used for chronic obstructive pulmonary disease. The technique is as following: First, the therapist pushes the infrasternal region of the
FIGURE 2. Pulling phenomenon of the supraclavicular region (O) and the lower edge of the cricoid cartilage (*) occurred on inspiration. (A) On expiration, (B) on inspiration.
Lishu Li, et al
637
Creutzfeldt-Jacob Disease: A Case Report
FIGURE 3. Laryngoscopic findings demonstrated bilateral vocal fold abductor paralysis.
patient to flat the infrasternal region during the expiration with the gentle power. Next, when at the end of the expiration until the inspiration begins, the therapist pushes the infrasternal region of the patient with more strong power so as not to affect the pain for the patient. After pushing the infrasternal region of the patient, the inspiratory movement could be stronger and the inspiration duration could be longer reflectively than those before the exercise.16,17 The mechanisms of the reflectively movement of the diaphragmatic breathing exercise could be considered a deflation reflex as part of Hering-Breuer’s reflex
via the vagus nerve. It was reported that the activity of the posterior cricoarytenoid muscle increased in response to negative pressure and/or absence of volume feedback. And, it was reported that there was a temporal relationship between the activation of the posterior cricoarytenoid muscle and the inspiratory phase of the respiratory cycle during tidal breathing in humans.18–20 Thus, it could be considered that reflective control of respiratory movement via the vagus nerve in the medulla might have been conserved in our case. Based on these findings, we considered that bilateral vocal fold abductor paralysis of this case might not be caused by a disturbance of nucleus ambiguus or peripheral nerve, but a disturbance of the upper motor neurons. These findings might be consistent with 123I-IMP SPECT findings showing a severely disturbed cerebral cortex with slightly disturbed brain stem. It could be considered that these findings are in agreement with the pathological findings reported by Iwata et al.4 Further clinical and pathophysiological studies including electromyography may be warranted to elucidate bilateral vocal fold abductor paralysis in Creutzfeldt-Jacob disease. It is considered that physicians should be aware of the potential risk of bilateral vocal fold abductor paralysis in Creutzfeldt-Jacob disease, because emergency tracheotomy may be required.
REFERENCES 1. Goto K, Umezaki H, Suetsugu M. Electroencephalographic and clinicopathological studies on Creutzfeldt-Jakob syndrome. J Neurol Neurosurg Psychiatr. 1976;39:931-940. 2. Onoda M, Koike Y, Hibino R, Takahashi A, Sobue I. Sleep apnea in Creutzfeldt-Jakob disease and subacute sclerosing panencephalitis-with reference to periodic synchronous discharge. Clin Neurol. 1978;18:601-607.
FIGURE 4. The raw electromyographic curves of the thyroarytenoid and posterior cricoarytenoid muscles. There was no apparent action potential or abnormal potential, including fibrillation potential or positive sharp wave, at the thyroarytenoid and posterior cricoarytenoid muscles during spontaneous respiration. After pushing the infrasternal region of the patient on the expiration, normal action potential was seen at the posterior cricoarytenoid muscle on the next inspiration. (A) Thyroarytenoid muscle on the left side during normal respiratory movement, (B) posterior cricoarytenoid muscles on the right side during normal respiratory movement, (C) posterior cricoarytenoid muscles of the right side after pushing the infrasternal region of the patient on the expiration.
638 3. Mamdani MB, Masdeu J, Ross E, Ohara R. Sleep apnea with unusual EEG changes in Jakob-Creutzfeldt disease. Electroencephalogr Clin Neurophysiol. 1983;55:411-416. 4. Iwata S, Nakamura A, Nakamura N, Sakashita I, Fukunaga H. 2 Cases of Creutzfeldt-Jacob disease with bilateral vocal cord paralysis. Clin Neurol. 1988;28:333-337. 5. Kudo Y, Tamaru F, Motomura N, Yamadori A. Disappearance of sleep apnea by tracheal intubation in Creutzfeldt-Jakob disease. Electroencephalogr Clin Neurophysiol. 1984;58:226-229. 6. Isozaki E. Vocal fold paralysis in neurodegenerative diseases. Otorhinolaryngology. 1998;41:393-403 [in Japanese]. 7. Hirano M, Ohara J. Use of hooked-wire electrodes for electromyography of the intrinsic laryngeal muscles. J Speech Hear Res. 1969;12:362-373. 8. Isozaki E, Naito A, Horiguchi S, Kawamura R, Hayashida T, Tanabe H. Early diagnosis and stage-classification of vocal cord abductor paralysis in multiple system atrophy. J Neurol Neurosurg Psychiatr. 1996;60: 399-402. 9. Plasse HM, Lieberman AN. Bilateral vocal cord paralysis in Parkinson’s disease. Arch Otolaryngol. 1981;107:252-253. 10. Yokoji H, Nakamura S, Ikeda T. A case of progressive supranuclear palsy associated with bilateral vocal cord abductor paralysis. Rinsho Shinkeigaku. 1997;37:523-525. 11. Palesse N, Marelli A, Legge MP. Bilateral abductor paralysis of the vocal cords in the course of neurological diseases: report of 5 cases. Ital J Neurol Sci. 1988;9:59-62. 12. Isozaki E, Hayashi M, Hayashida T, Oda M, Hirai S. Myopathology of the intrinsic laryngeal muscles in neurodegenerative diseases, with reference to the mechanism of vocal cord paralysis. Rinsho Shinkeigaku. 1998;38: 711-718.
Journal of Voice, Vol. 23, No. 5, 2009 13. Yamamoto T, Ohnishi A, Miyoshi T, Hashimoto T, Murai Y. A case of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) with bilateral recurrent nerve palsy and primary alveolar hypoventilation—comparative studies of the histological findings of the two sural nerve biopsies with 9 years interval. Rinsho Shinkeigaku. 1994;34: 712-716. 14. Isozaki E, Matsubara S, Hayashida T, Oda M, Hirai S. Morphometric study of nucleus ambiguus in multiple system atrophy presenting with vocal cord abductor paralysis. Clin Neuropathol. 2000;19:213-220. 15. Isozaki E, Shimizu T, Takamoto K, et al. Vocal cord abductor paralysis (VCAP) in Parkinson’s disease: difference from VCAP in multiple system atrophy. J Neurol Sci. 1995;130:198-202. 16. Brach BB, Chao RP, Sgroi VL, Minh VD, Ashburn WL, Moser KM. 133Xenon washout patterns during diaphragmatic breathing. Studies in normal subjects and patients with chronic obstructive pulmonary disease. Chest. 1977;71:735-739. 17. Miller WF. A physiologic evaluation of the effects of diaphragmatic breathing training in patients with chronic pulmonary emphysema. Am J Med. 1954;17:471-477. 18. Brancatisano TP, Dodd DS, Engel LA. Respiratory activity of posterior cricoarytenoid muscle and vocal cords in humans. J Appl Physiol. 1984;57:1143-1149. 19. Mathew OP, Sant’Ambrogio FB, Woodson GE, Sant’Ambrogio G. Respiratory activity of the cricothyroid muscle. Ann Otol Rhinol. 1988;97: 680-687. 20. Sant’Ambrogio FB, Mathew OP, Clark WD, Sant’ Ambrogio G. Laryngeal influences on breathing pattern and posterior cricoarytenoid muscle activity. J Appl Physiol. 1985;58:1298-1304.