1) A 68-year-old female presented with progressive dysphonia, dysphagia, and dyspnea since 2002. Examinations found laryngeal diplegia but were otherwise normal. 2) In 2017, she was diagnosed with myasthenia based on tests. However, in 2021 she developed additional parkinsonian signs and was found to have multiple system atrophy based on examinations. 3) Vocal fold impairment can be an early sign of multiple system atrophy and may precede diagnosis in some cases. Laryngeal examinations should be considered a diagnostic tool for multiple system atrophy given their ability to detect irregularities that differentiate it from Parkinson's disease.

1. Cas clinique dossier N 3429/17
QUI
trainent
…
qu'est ce que
ca pourrait
etre?

2. AGE: 68 ans
SEXE: Female
ATCD: Dyslipidémie
MOTIF: 3D
EN NOV 2017
DYSPHONIE DYSPHAGIE DYSPNEE

3. DYSPNEE
- installtion progressive
depuis 2002
- laryngé avec tirage sus-
sternale
DYSPHONIE
- installation progressive
depuis 2002
- voix nassonée
DYSPHAGIE
- installation progressive
depuis 2002
- Mixte
- accentuée a la fin de la
journée
NOV 2017

4. Neurologu
e
PNEUMOLOGUE
ORL
Notre équipe

5. Neurologu
e
FIBROSCOPIE
BRONCHIQUE
NORMALE
SCANNER THORACIQUE
NORMALE
PNEUMOLOGU
E
ORL
Notre équipe

6. Neurologu
e
Diplégie laryngé !!!
essayer la corticothérapie
inhalés
mais pas d’amelioration
pneumologue
ORL
Notre équipe

7. FIBROSCOPIE
BRONCHIQUE
NORMALE
SCANNER THORACIQUE
NORMALE
Diplégie laryngé
!!!
PNEUMOLOGU
E
ORL
NEUROLOGU
E
Notre équipe

8. Pas de ptosis
pas de diplopie
Le reste de l’examen
neurologique est sans
particularitées
Pas de fluctutaion
au cours de la journée
NOV 2017
AGE: 68 ans
SEXE: Female
ATCD: Dyslipidémie
MOTIF: 3D
Diplégie
laryngée
Décrement significative a 20KHz
EMG
10-2017

9. NOV 2017

10. MYASTHENIE
DEC 2017
MYASTHENIE
MYASTHENIE

11. Normal
EMG
7-2018
Auto-anticorps anti-récepteurs de l’acetylcholine
Anticorps anti-MUSK
IRM c-
m
1er ½ 2018
Test a la prostigmine
Bilan d’auto-immunité
Sans anomalies
significatives
Score myasthénique 90

12. MYASTHENIE
juillet 2018
MYASTHENIE
MYASTHENIE

13. FIBROSCOPIE
BRONCHIQUE
NORMALE
SCANNER THORACIQUE
NORMALE
Cordotomie
Opéré améliorée
PNEUMOLOGU
E
ORL
NEUROLOGU
E
2019

14. FIBROSCOPIE
BRONCHIQUE
NORMALE
SCANNER THORACIQUE
NORMALE
Cordotomie
Opéré améliorée
PNEUMOLOGU
E
ORL
NEUROLOGU
E
Avril 2021
Rigidité
lenteur
tremblement

15. Avril 2021
AGE: 73 ans
SEXE: Female
ATCD: Dyslipidémie,
cordotomie
Parkinsonisme TAR bilatérale prédominant a
droite
Myoclonie du menton
Stridor Nocturne
Scopa-Aut = 19
-constipation et trouble VS

16. Avril 2021

17. AMS
Avril 2021
AMS
AMS

18. rst, Night stridor
Shiba K, Isono S, Nakazawa K. Paradoxical vocal cord motion: a
review focused on multiple system atrophy. Auris Nasus Larynx.
2007;34:443–452.
Laryngeal dysfunction is common in MSA and initially
manifests as nocturnal stridor due to restricted bilateral
true VF abduction with restricted opening of the glottis during
sleep.
As the disease progresses, restricted glottal dilation
may become apparent during wakefulness with VF abductor
and adductor paralysis causing daytime stridor
NEW SYMPTOM NIGHT
STRIDOR

19. TEST AU
MODOPAR
SCORE DE
BASE 40
Vmin 8
Pourcentage 80 %

20. MPI
Avril 2021
AMS
AMS

21. MSA & Vocal
Fold
Gagnon JF, Postuma RB, Mazza S, et al. Rapid-eye-movement sleep
behaviour disorder and neurodegenerative diseases. Lancet Neurol.
2006;5:424–432
Multiple system atrophy
neurodegenerative disease
a-synucleinopathies (RBD)
cerebellar syndrome
autonomic dysfunction
extrapyramidal signs
Parkinsonism Dystonia or paresis
The laryngeal muscles
vocal fold hypomobility

22. Fanciulli A, Wenning GK. Multiple-system atrophy. N Engl J Med.
2015;372:249–263.
Respiratory disturbances are common in MSA with
development of stridor occurring in 50% of patients, but
this is more common of advanced disease.
However, symptoms
of vocal fold motion impairment (VFMI) may occur
early in MSA or even precede diagnosis
An Early sign or a marker of
advanced disease ?

23. vocal fold motion
impairment preceding
diagnosis
01 04
05
02
a series of four patients diagnosed with MSA
who initially presented with respiratory symptoms and
VF
dysfunction
Vocal Fold "Paralysis": An Early Sign in Multiple System Atrophy
J voice 2020 Nov;34(6):940-944.

24. A 70-year-old gentleman presented to the otolaryngology
clinic with a diagnosis of bilateral true VF paresis and
stridor.
He reported several years of dysphonia and dyspnea.
He had also been experiencing tremor for a few months
accompanied by erectile dysfunction and constipation
that
led an outside neurologist to making a diagnosis of MSA.
His neurological examination was notable
for parkinsonism characterized by bradykinesia, rigidity,
tremor, and postural instability that was poorly responsive
to a trial of levodopa. Cerebellar dysfunction was evidenced
by gait and limb ataxia, as well as oculomotor dysfunction.
Autonomic reflex screen (ARS) revealed severe,
generalized autonomic dysfunction (CASS 8/10) including
orthostatic hypotension.
0
1
Vocal Fold "Paralysis": An Early Sign in Multiple System Atrophy
J voice 2020 Nov;34(6):940-944.

25. A 76-year-old man presented to our otolaryngology
clinic with a two-year history of hoarseness and stridor.
Laryngoscopy revealed left true VF immobility and right
true VF hypomobility.
Evaluation by a movement specialist at our institution
revealed postural instability, gait ataxia, limb ataxia,
anterocollis,
and cerebellar oculomotor dysfunction. Subjective
reporting of urinary incontinence prompted ARS, which
revealed severe, generalized autonomic dysfunction
(CASS
8/10) including orthostatic hypotension, which precluded
treatment with levodopa. MRI of the brain showed moderate
pontocerebellar degeneration. The patient was diagnosed
with probable cerebellar MSA (MSA-C) .
0
2
Vocal Fold "Paralysis": An Early Sign in Multiple System Atrophy
J voice 2020 Nov;34(6):940-944.

26. A 63-year-old woman presented to our otolaryngology
clinic with a two-year history of stridor, dysphonia, dysphagia,
, gait instability with dizziness.
She was previously diagnosed with bilateral true VF
paralysis
and underwent evaluations by multiple specialists, including
a neurologist, resulting in no diagnosis.
Evaluation revealed rigidity, postural instability, ataxic dysarthria,
limb ataxia, and cerebellar oculomotor dysfunction. The
patient reported urinary incontinence and her subjective
“dizziness”
was determined to be due to orthostatic hypotension
detected ARS, which revealed moderately severe autonomic
failure (CASS 6/10) involving adrenergic and cardiovagal
responses. Orthostatic hypotension precluded initiation of a
levodopa trial. The patient was diagnosed
with probable MSA-C
0
3
Vocal Fold "Paralysis": An Early Sign in Multiple System Atrophy
J voice 2020 Nov;34(6):940-944.

27. A 57-year-old woman presented reporting a two-year history of breathing
difficulties
which were attributed to bilateral true VF paralysis
resulting in tracheostomy despite an undetermined etiology.
About one-year later, she began experiencing difficulties
with balance. She also endorsed urinary incontinence and
experiencing several episodes concerning for orthostatic
hypotension while her husband reported her dream-enactment RBD
for several years.
The patient’s neurological examination was notable for
bradykinesia, postural instability, gait ataxia, limb ataxia,
and cerebellar oculomotor dysfunction. MRI of the brain
showed atrophy of the pons and, to a lesser degree, of the
cerebellum. MRI of the brain showed a faint T2-weighted
hyperintense cross pattern, the so-called “hot cross bun
sign,” within the brainstem.
0
4
Vocal Fold "Paralysis": An Early Sign in Multiple System Atrophy
J voice 2020 Nov;34(6):940-944.

28. hot cross bun sign
elective degeneration of transverse
pontocerebellar tracts and median pontine
raphe nuclei, described in a variety
of neurodegenerative diseases

29. Misdiagnosis and orientation
to other specialist
Koga S, Aoki N, Uitti RJ, et al. When DLB, PD, and PSP masquerade
as MSA. Neurology. 2015;85:1–9
Like many neurodegenerative diseases,
MSA can be difficult to diagnosis
Misdiagnosis rates have been reported
as high as 38%,
with definitive diagnosis currently only possible
by postmortem assessment of the brain.
38%

30. Dark Prognosis
Yamaguchi M, Arai K, Asahina M, et al. Laryngeal stridor in multiple
system atrophy. Eur Neurol. 2003;49:154–159.
The laryngeal dysfunction is further complicated by the development of
central apnea and subsequent nocturnal oxygen desaturation in
advanced MSA.
Which contribute to the risk of sudden nocturnal death,
which is the most common cause of death in MSA.

31. ossible palliative treatment
Shimohata T, Ozawa T, Nakayama H, et al. Frequency of nocturnal
sudden death in patients with multiple system atrophy. J Neurol.
2008;255:1483–1485.
However some treatments including
- CPAP
- Unilateral botulinum toxin (BoNT) injection to the VF
adductors,
- Cordotomie
- tracheostomy may be beneficial.

32. Laryngeal Movement Disorders in Multiple System Atrophy:
A Diagnostic Biomarker
Mov Disord 2020 Dec;35(12):2174-2183. doi: 10.1002/mds.28220. Epub 2020 Aug 5.
Laryngeal Movement Disorders in Multiple System Atrophy: A Diagnostic Biomarker?
comparaison
MSA
- 57 patients
- had a shorter disease duration
4 years VS
- higher disease severity
- 43.9% showed CLINICALLY overt laryngeal
dysfunction with inspiratory stridor.
- During ENDOSCOPIC task assessment
93% of patients with MSA demonstrated laryngeal
dysfunction
- Irregular arytenoid cartilages
movements were present in 91.2%.
PD
57 patients
7 years
only 1.8% of patients with PD
0 % in patients with PD.

33. Mov Disord 2020 Dec;35(12):2174-2183. doi: 10.1002/mds.28220. Epub 2020 Aug 5.
Laryngeal Movement Disorders in Multiple System Atrophy: A Diagnostic Biomarker?
This research’s data suggest that :
irregular arytenoid cartilage movements
could be used as a clinical marker to delineate MSA
from PD
with a
specificity of 1.0
sensitivity 0.9
Laryngeal Movement Disorders in Multiple System Atrophy:
A Diagnostic Biomarker

34. 2020 Sep;176(7-8):608-613. doi: 10.1016/j.neurol.2020.01.351. Epub 2020 Mar 10.
Prevalence and characterisation of vocal fold motion impairment (VFMI) in patients with Multiple system atrophy compared with Parkinson's

35. 2020 Sep;176(7-8):608-613. doi: 10.1016/j.neurol.2020.01.351. Epub 2020 Mar 10.
Prevalence and characterisation of vocal fold motion impairment (VFMI) in patients with Multiple system atrophy compared with Parkinson's
The table’s data support that :
1
vocal fold motion impairments
may be encountered in
two-thirds of MSA (68%)
VS 0 in PD
Prevalence and characterisation of vocal fold impairment in
MSA
MSA VFMI+ MSA VFMI-
68%

36. 2020 Sep;176(7-8):608-613. doi: 10.1016/j.neurol.2020.01.351. Epub 2020 Mar 10.
Prevalence and characterisation of vocal fold motion impairment (VFMI) in patients with Multiple system atrophy compared with Parkinson's
2
=>Laryngological examination should be considered as
A supplementary tool for the diagnosis and
prognosis of MSA
Prevalence and characterisation of vocal fold impairment in
MSA

37. 2020 Sep;176(7-8):608-613. doi: 10.1016/j.neurol.2020.01.351. Epub 2020 Mar 10.
Prevalence and characterisation of vocal fold motion impairment (VFMI) in patients with Multiple system atrophy compared with Parkinson's
3
VFMI in (particular VFAM, VFABD and BVFP)
should be discussed as an additional possible
Red flag even at an early stage of MSA and
could help discriminate
Prevalence and characterisation of vocal fold impairment in
MSA
MSA VS PD

38. Take home message 1
1
When patients present with VF hypomobility !!!,
practitioners should carefully screen for RBD and
other elements of autonomic dysfunction.
If identified, MSA DIAGNOSIS SHOULF BE CONSIDERED.

39. Take home message 2
2
IN MSA patients with VF hypomobility are at increased
risk for SUDDEN DEATH and should be identified
earlyso that respiratory intervention may be expedited
and a multidisciplinary approach to symptoms can be
initiated.

40. THANKS