Myasthenia Report of
a
Gravis Focal to the
Larynx
Case
Richard F. Neiman, MD; John R.
Mountjoy, MD; Elizabeth L. Allen, PhD
\s=b\ Myasthenia gravis, focal to the larynx, should be considered in the differential diagnosis of intermittent breathy dysphonia and aphonia. A 20-year-old woman had intermittent breathy dysphonia and aphonia as the sole manifestation of myasthenia gravis. The vocal cords were flaccid, and adducted to the paramedian position for phonation. Improvement of laryngeal function was demonstrated with edrophonium and neostigmine bromide. Therapy with pyridostigmine bromide (Mestinon) maintained normal phonation.
(Arch Otolaryngol 101:569-570, 1975) is in myasthenia gravis, occurring in 27% of pa¬ tients.1 Other bulbar signs, such as nasal régurgitation and dysphagia, usually appear concomitantly.2 Hypernasality caused by weakness of the muscles of the soft palate is also common.15 Observation of the vocal cords has seldom been reported, per¬ haps due to infrequent indirect lar¬ yngoscopy. Our case is unusual in that it illustrates selective involvement of the laryngeal muscles, with breathy dysphonia and aphonia as the sole manifestation of myasthenia gravis.
dysphonia, Dysarthria, including early sign a common
Accepted for publication March 25, 1975. From the departments of neurology (Dr. Neiman) and otolaryngology (Dr. Mountjoy), University Hospitals, and the Department of Speech and Hearing, Wendell Johnson Speech and Hearing Center (Dr. Allen), Iowa City. Dr. Neiman is now with the Lexington Clinic, Lexington, Ky. Reprint requests to the Lexington Clinic, 1221 S Broadway, Lexington, KY 40504 (Dr. Neiman).
REPORT OF A CASE A 20-year-old woman was first seen in December 1972 because of a complaint of intermittent hoarseness of four years1 du¬ ration. Hoarseness would last one to two weeks, then abate. Her voice was usually better in the early morning and deteri¬ orated with use. Between attacks, the voice was normal. There was no history of nasal
régurgitation, dysphagia, ptosis, or gen¬ eral weakness. Physical examination re¬ vealed minimal weakness of the palate and decreased vocal cord tension during phona¬ tion, with incomplete adduction. Voice was characterized by obvious continuous breathy dysphonia without hypernasality or articulatory defects. Intravenous administration of 10 mg of
adrophonium (Tensilon) resulted in a nor¬ mal phonation within two minutes. Dys¬ phonia returned six minutes later. Intrave¬ nously administered aminophylline, used as a control, had no effect. Complete blood cell count, erythrocyte sedimentation rate, antinuclear antibody titer, serum T4 test, serum immunoelectrophoresis, and roentgenographic view of the chest, were normal. Repetitive stimulation at 3 stimuli/sec on the left musculus abductor pollicis brevis failed to demonstrate fatigue. Treatment was begun with 60 mg of orally adminis¬ tered pyridostigmine bromide (Mestinon) every eight hours. Phonation returned to normal, and the vocal cords were noted to adduct normally for phonation. Pyridostigmine bromide therapy was discontinued in January 1973. The dys¬ phonia did not recur until October 1973, but the patient did not return for reexamination until February 1974. Examination at that time revealed mild palatal weaknerve
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and, on direct laryngoscopy, the vocal cords abducted fully but were sluggish on adduction. In the paramedian position, the vocal cords were flaccid. Speech was now whispered or aphonie due to a widened pos¬ terior chink and incomplete adduction. Intramuscular administration of physi¬ ological saline solution produced no effect. Intramuscular injection of 1 mg of neostigmine bromide resulted in an obvious improvement of voice in seven to eight minutes. Palatal movements improved, and vocal cord adduction was normal on phonation. Vocal cord tension improved but remained slightly lax. The patient's pretest dysphonia returned in three to four hours. Tape recordings at 334 inches per second were made as the patient read aloud. Spectrographic analyses of short samples ("estimated at four billion dollars") can be seen in the Figure. In the Figure, panel A shows the pretreatment condition. The energy distribution of the lower part of the panel (wide band spectrogram) verifies that speech articulation was normal, but total energy was extremely diffuse throughout the frequency range. In the up¬ per part of the panel, the amplitude trace shows very low intensity for the utterance except for fricative ("s") and plosive ("t") consonants, as in the word "estimated." Panel B, which was recorded folowing the administration of saline, shows no change from the pretreatment condition. Percep¬ tual evaluation of these two samples indi¬ cated aphonie (whispered) vocal production with good articulation. The third sample, panel C, recorded after administration of neostigmine bromide, shows essentially the same energy articulation distribution as the first two samples. However, in this sample, the high-energy bands are more ness
COMMENT Disorders of phonation, resonation, and articulation are seen as the ini¬ tial manifestation of myasthenia gravis in 27% of patients.1 Even¬ tually, 62% of myasthénie patients will develop dysarthria.' Speech changes are usually characterized by progressive decrement of volume and increased hypernasality with repeti¬ tive vocal use. Initially, articulation is distinct but becomes imprecise with continued use.6·7 Psychogenic dysphonia was consid¬ ered in the differential diagnosis of our patient. However, the character¬ istic fatigue of speech and the response to edrophonium and neo¬ stigmine bromide strongly supported the diagnosis of myasthenia gravis. Improvement with pyridostigmine bromide therapy has been maintained to date. Myasthenia gravis, focal to the larynx, should be considered in the differential diagnosis of in¬ termittent breathy dysphonia and
aphonia.
This study was supported by the Neurology Department and the Neurosensory Center, Uni¬ versity Hospitals, Iowa City. The Neurosensory Center is supported by program-project National Institutes of Health grant NS 3354. Maurice W. Van Allen, MD, gave assistance and advice in this study.
References and treatment conditions: Panel A, pretreatment condition; panel B, after administration of saline; panel C, after administration of neostigmine bromide; panel D, after continued use of pyridostigmine bromide. Lower part of each display shows speech energy distribution; above is ampli¬ tude tracing.
Spectrographic analyses of pretreatment
prominent, and the amplitude trace indi¬ cates a near-normal intensity level. The voice was normal, with clinically insignifi¬ cant breathiness.
Oral administration of 60 mg of pyrido¬ stigmine bromide every four hours during the day produced normal voice. At the time of the patient's last examination in April 1974, while she was taking pyridostigmine bromide, she had normal phonation and
palatal function. Again, a recording was made as she read aloud. Spectrographic analysis of the recorded sample ("that he had a fortune of four billion dollars") appear in panel D of the
Figure.
It is clear that
intensity
was
mide-treated condition. The voice was sidered to be entirely normal.
con¬
greater than in the levels previously achieved, even in the neostigmine bro¬
Downloaded From: http://archotol.jamanetwork.com/ by a New York University User on 06/09/2015
1. Osserman KE, Genkins G: Studies in myasthenia gravis: Review of 20 year experience in over 1,000 patients. Mt Sinai J Med 38:497-537, 1971. 2. Grob D: Myasthenia gravis: Current status of pathogenesis, clinical manifestations and management. J Chronic Dis 8:536-566, 1958. 3. Aronson AE: Early motor unit disease masquerading as psychogenic breathy dysphonia: A clinical case presentation. J Speech Hear Disord 36:115-124, 1971. 4. Ball JR, Lloyd JH: Myasthenia gravis as hysteria. Med J Aust 1:1018-1020, 1971. 5. Maxwell S, Lockey JL: Voice in myasthenia gravis. Laryngoscope 79:1902-1905, 1969. 6. Ferguson FR: A critical review of the clinical features of myasthenia gravis. Proc R Soc Med 55:49-52, 1962. 7. Schwab RS, Viets HR: Myasthenia gravis: Neuromuscular disorder. Res Publ Assoc Res Nerv Ment Dis 38:624-643, 1961.
a
Gravis Focal to the
Larynx
Case
Richard F. Neiman, MD; John R.
Mountjoy, MD; Elizabeth L. Allen, PhD
\s=b\ Myasthenia gravis, focal to the larynx, should be considered in the differential diagnosis of intermittent breathy dysphonia and aphonia. A 20-year-old woman had intermittent breathy dysphonia and aphonia as the sole manifestation of myasthenia gravis. The vocal cords were flaccid, and adducted to the paramedian position for phonation. Improvement of laryngeal function was demonstrated with edrophonium and neostigmine bromide. Therapy with pyridostigmine bromide (Mestinon) maintained normal phonation.
(Arch Otolaryngol 101:569-570, 1975) is in myasthenia gravis, occurring in 27% of pa¬ tients.1 Other bulbar signs, such as nasal régurgitation and dysphagia, usually appear concomitantly.2 Hypernasality caused by weakness of the muscles of the soft palate is also common.15 Observation of the vocal cords has seldom been reported, per¬ haps due to infrequent indirect lar¬ yngoscopy. Our case is unusual in that it illustrates selective involvement of the laryngeal muscles, with breathy dysphonia and aphonia as the sole manifestation of myasthenia gravis.
dysphonia, Dysarthria, including early sign a common
Accepted for publication March 25, 1975. From the departments of neurology (Dr. Neiman) and otolaryngology (Dr. Mountjoy), University Hospitals, and the Department of Speech and Hearing, Wendell Johnson Speech and Hearing Center (Dr. Allen), Iowa City. Dr. Neiman is now with the Lexington Clinic, Lexington, Ky. Reprint requests to the Lexington Clinic, 1221 S Broadway, Lexington, KY 40504 (Dr. Neiman).
REPORT OF A CASE A 20-year-old woman was first seen in December 1972 because of a complaint of intermittent hoarseness of four years1 du¬ ration. Hoarseness would last one to two weeks, then abate. Her voice was usually better in the early morning and deteri¬ orated with use. Between attacks, the voice was normal. There was no history of nasal
régurgitation, dysphagia, ptosis, or gen¬ eral weakness. Physical examination re¬ vealed minimal weakness of the palate and decreased vocal cord tension during phona¬ tion, with incomplete adduction. Voice was characterized by obvious continuous breathy dysphonia without hypernasality or articulatory defects. Intravenous administration of 10 mg of
adrophonium (Tensilon) resulted in a nor¬ mal phonation within two minutes. Dys¬ phonia returned six minutes later. Intrave¬ nously administered aminophylline, used as a control, had no effect. Complete blood cell count, erythrocyte sedimentation rate, antinuclear antibody titer, serum T4 test, serum immunoelectrophoresis, and roentgenographic view of the chest, were normal. Repetitive stimulation at 3 stimuli/sec on the left musculus abductor pollicis brevis failed to demonstrate fatigue. Treatment was begun with 60 mg of orally adminis¬ tered pyridostigmine bromide (Mestinon) every eight hours. Phonation returned to normal, and the vocal cords were noted to adduct normally for phonation. Pyridostigmine bromide therapy was discontinued in January 1973. The dys¬ phonia did not recur until October 1973, but the patient did not return for reexamination until February 1974. Examination at that time revealed mild palatal weaknerve
Downloaded From: http://archotol.jamanetwork.com/ by a New York University User on 06/09/2015
and, on direct laryngoscopy, the vocal cords abducted fully but were sluggish on adduction. In the paramedian position, the vocal cords were flaccid. Speech was now whispered or aphonie due to a widened pos¬ terior chink and incomplete adduction. Intramuscular administration of physi¬ ological saline solution produced no effect. Intramuscular injection of 1 mg of neostigmine bromide resulted in an obvious improvement of voice in seven to eight minutes. Palatal movements improved, and vocal cord adduction was normal on phonation. Vocal cord tension improved but remained slightly lax. The patient's pretest dysphonia returned in three to four hours. Tape recordings at 334 inches per second were made as the patient read aloud. Spectrographic analyses of short samples ("estimated at four billion dollars") can be seen in the Figure. In the Figure, panel A shows the pretreatment condition. The energy distribution of the lower part of the panel (wide band spectrogram) verifies that speech articulation was normal, but total energy was extremely diffuse throughout the frequency range. In the up¬ per part of the panel, the amplitude trace shows very low intensity for the utterance except for fricative ("s") and plosive ("t") consonants, as in the word "estimated." Panel B, which was recorded folowing the administration of saline, shows no change from the pretreatment condition. Percep¬ tual evaluation of these two samples indi¬ cated aphonie (whispered) vocal production with good articulation. The third sample, panel C, recorded after administration of neostigmine bromide, shows essentially the same energy articulation distribution as the first two samples. However, in this sample, the high-energy bands are more ness
COMMENT Disorders of phonation, resonation, and articulation are seen as the ini¬ tial manifestation of myasthenia gravis in 27% of patients.1 Even¬ tually, 62% of myasthénie patients will develop dysarthria.' Speech changes are usually characterized by progressive decrement of volume and increased hypernasality with repeti¬ tive vocal use. Initially, articulation is distinct but becomes imprecise with continued use.6·7 Psychogenic dysphonia was consid¬ ered in the differential diagnosis of our patient. However, the character¬ istic fatigue of speech and the response to edrophonium and neo¬ stigmine bromide strongly supported the diagnosis of myasthenia gravis. Improvement with pyridostigmine bromide therapy has been maintained to date. Myasthenia gravis, focal to the larynx, should be considered in the differential diagnosis of in¬ termittent breathy dysphonia and
aphonia.
This study was supported by the Neurology Department and the Neurosensory Center, Uni¬ versity Hospitals, Iowa City. The Neurosensory Center is supported by program-project National Institutes of Health grant NS 3354. Maurice W. Van Allen, MD, gave assistance and advice in this study.
References and treatment conditions: Panel A, pretreatment condition; panel B, after administration of saline; panel C, after administration of neostigmine bromide; panel D, after continued use of pyridostigmine bromide. Lower part of each display shows speech energy distribution; above is ampli¬ tude tracing.
Spectrographic analyses of pretreatment
prominent, and the amplitude trace indi¬ cates a near-normal intensity level. The voice was normal, with clinically insignifi¬ cant breathiness.
Oral administration of 60 mg of pyrido¬ stigmine bromide every four hours during the day produced normal voice. At the time of the patient's last examination in April 1974, while she was taking pyridostigmine bromide, she had normal phonation and
palatal function. Again, a recording was made as she read aloud. Spectrographic analysis of the recorded sample ("that he had a fortune of four billion dollars") appear in panel D of the
Figure.
It is clear that
intensity
was
mide-treated condition. The voice was sidered to be entirely normal.
con¬
greater than in the levels previously achieved, even in the neostigmine bro¬
Downloaded From: http://archotol.jamanetwork.com/ by a New York University User on 06/09/2015
1. Osserman KE, Genkins G: Studies in myasthenia gravis: Review of 20 year experience in over 1,000 patients. Mt Sinai J Med 38:497-537, 1971. 2. Grob D: Myasthenia gravis: Current status of pathogenesis, clinical manifestations and management. J Chronic Dis 8:536-566, 1958. 3. Aronson AE: Early motor unit disease masquerading as psychogenic breathy dysphonia: A clinical case presentation. J Speech Hear Disord 36:115-124, 1971. 4. Ball JR, Lloyd JH: Myasthenia gravis as hysteria. Med J Aust 1:1018-1020, 1971. 5. Maxwell S, Lockey JL: Voice in myasthenia gravis. Laryngoscope 79:1902-1905, 1969. 6. Ferguson FR: A critical review of the clinical features of myasthenia gravis. Proc R Soc Med 55:49-52, 1962. 7. Schwab RS, Viets HR: Myasthenia gravis: Neuromuscular disorder. Res Publ Assoc Res Nerv Ment Dis 38:624-643, 1961.
