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- mise à jour
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- 18 novembre
2023
- Neurology:
Clinical Practice
- 2023;13:e200204
- Scholarpedia
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- Parakinesia
Brachialis Oscitans
- in a
Patient With a First
Manifestation
- of
Multiple Sclerosis
- Manuel Salavisa, Bader Mohamed, Kimberley
Allen-Philbey,
- Andrea M. Stennett, Thomas Campion, Klaus
Schmierera
- Centre for Neuroscience (MS,
BM, KA-P, KS), Surgery and Trauma, The Blizard
Institute, Queen Mary University of
London
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- Tous
les articles sur la parakinésie brachiale
oscitante
- All
articles about parakinsia brachialis
oscitans
-
- Abstract
- Parakinesia brachialis oscitans (PBO) is the
involuntary movement of an otherwise paretic
upper limb triggered by yawning. We describe the
first case of PBO in a patient with a first
manifestation of tumefactive multiple sclerosis
(MS).
-
- A 35-year-old man presented to the emergency
department with a first episode of generalized
seizure. Neurologic examination revealed
left-sided spastic hemiparesis, predominantly
affecting his upper limb. Brain MRI showed a
tumefactive right hemisphere lesion consistent
with demyelination. CSF did not document
unmatched oligoclonal bands.
-
- Two weeks after admission and, despite being
unable to voluntarily raise his left arm, the
patient noticed a repeated and reproducible
involuntary raise of this limb upon yawning,
consistent with PBO. In the following weeks, the
phenomenon diminished both in fre- quency and
movement amplitude alongside motor recovery. An
MRI performed 2 months later showed progression
of the demyelinating lesion load and confirmed a
diagnosis of MS.
-
- PBO is an example of autonomic voluntary
motor dissociation and reþects the
interplay between loss of cortical inhibition of
the cerebellum in the setting of functional
spinocerebellar pathways. Clinicians should be
aware of this transient phenomenon which should
not be mistaken as a chronic movement disorder
or focal epileptic seizures.
-
Recognizing the pattern of limb movement in the
context of yawning as its predicable trigger is
key in diagnosing PBO and sets it apart from
potential confounders such as focal epileptic
seizures. PBO may arise from any structural
lesion disrupting descending
cortico-ponto-cerebellar tracts in the setting
of preserved spino- cerebellar pathways.
Résumé
- La parakinésie brachialis oscitans
(PBO) est le mouvement involontaire d'un membre
supérieur parétique
déclenché par un bâillement.
Ces auteurs décrivent le premier cas de
PBO chez un patient présentant une
première manifestation de sclérose
en plaques (SEP) tumorale.
-
- Un homme de 35 ans s'est
présenté aux urgences pour un
premier épisode de crise
épileptique
généralisée. L'examen
neurologique a révélé une
hémiparésie spastique du
côté gauche, affectant
principalement le membre supérieur. L'IRM
cérébrale a montré une
lésion tumorale de
l'hémisphère droit correspondant
à une démyélinisation. Le
LCR n'a pas révélé de
bandes oligoclonales.
-
- Deux semaines après son admission et
bien qu'il soit incapable de lever
volontairement son bras gauche, le patient a
remarqué une élévation
involontaire répétée et
reproductible de ce membre lors d'un
bâillement, ce qui correspond à la
PBO. Au cours des semaines suivantes, le
phénomène a diminué
à la fois en fréquence et en
amplitude de mouvement, parallèlement
à la récupération motrice.
Une IRM réalisée deux mois plus
tard a montré une progression de la
charge lésionnelle
démyélinisante et a
confirmé le diagnostic de SEP.
-
- Le PBO est un exemple de dissociation
motrice volontaire autonome et reflète
l'interaction entre la perte d'inhibition
corticale du cervelet dans le cadre de voies
spinocérébelleuses fonctionnelles.
Les cliniciens doivent être conscients de
ce phénomène transitoire qui ne
doit pas être confondu avec un trouble
chronique du mouvement ou des crises
épileptiques focales.
- Introduction
- In some cases of hemiplegia, the initiation
of yawning is associated with an involuntary
upward movement of the otherwise paralyzed arm,
a phenomenon described in the medical literature
as parakinesia brachialis oscitans.
-
- Previously, this rare phenomenon has been
essentially reported in the context of stroke,
namely those affecting the internal capsule
region. We report a case of parakinesia
brachialis oscitans in a patient presenting with
a pseudotumoral form of multiple sclerosis.
-
- Case Report
- A 35-year-old man with a medical history of
autism spec- trum disorder and mild learning
difficulties was brought to the hospital by
ambulance after a first episode of generalized
tonic-clonic seizure. The patient recalled
noticing brief spells of intermittent left arm
twitching in the 3 days leading up to hospital
admission, followed by brief spells of tran-
sient left arm weakness, particularly affecting
his grip and dexterity.
-
- He denied concurrent headaches or any other
symptoms suggestive of acute neurologic change.
His family denied any change in his behavior or
increased cognitive difficulties. On initial
examination, he was alert and complained of
severe back pain.
-
- Neurologic examination revealed left-sided
spastic hemiparesis, predominantly affecting his
upper limb with a Medical Research Council (MRC)
grade 3 weakness noted in left shoulder ab-
duction and grade 2 in left wrist
dorsiþexion. Reþexes were pronounced
on the left, with extensor plantar response. The
sensory and cerebellar examination results were
normal, as was the cranial nerve examination. No
involuntary movements or signs of epileptic
activity were noted at this stage.
-
- Because of the rapid onset of left-sided
hemiparesis, stroke was suspected, and he was
referred for acute CT of the head. This revealed
an ill-defined right hemispheric supra-
tentorial hypodensity with mild rim enhancement
after contrast administration (Figure, A). A
brain tumor or ab- scess was suspected, and the
patient was immediately referred for a brain
MRI. An EEG demonstrated focal right- sided
frontal and temporal slowing with no
epileptiform discharges.
-
- MRI confirmed a 47-mm oval-shaped
hyperintense lesion in the right centrum
semiovale on T2-weighted scans, with incomplete
(C-shaped) peripheral enhancement after
gadolinium- diethylenetriamine penta-acetic acid
injection (Figure, B and C). The lesion exerted
subtle mass effect on the adjacent ven- tricular
system. In addition, a smaller frontal
juxtacortical lesion with the same
characteristics was noted, both sharing a
typical "central vein sign" visible on the
susceptibility-weighted se- quences (Figure, D
and E). Both diffusion-weighted imaging and
apparent diffusion coefficient sequences were
negative for brain ischemia. Spinal MRI imaging
showed no signal changes within the cord
(Figure, F). CSF studies revealed <1 white
blood cells/mm3. Glucose (3.8 mmol/L) and
protein (279 mg/L) were within normal range.
Testing for oligoclonal bands (OCB) was negative
in the serum and CSF. The CSF neurofilament
light (NfL) chain level was significantly
elevated to 2527 pg/mL (age-adjusted reference
<380 pg/mL). Serum AQP4 and MOG antibodies
were negative. The presentation and findings at
this stage were deemed compatible with a
fulminant first manifes- tation of
inþammatory demyelination not fulfilling
McDonald 2017 criteria for a diagnosis of
multiple sclerosis (MS).
-
- A diagnosis of clinically isolated syndrome
of demyelination was made, and the patient was
given oral methylprednisolone 500 mg for 5 days.
He was subsequently enrolled into Attack MS, a
placebo-controlled clinical trial of natalizumab
within 14 days of symptom onset
(NCT05418010).
-
- Two weeks after symptom onset and shortly
after discharge from inpatient admission, the
patient reported that, despite being unable to
hold up is left arm voluntarily, this would
occur spontaneously on yawning (Video 1). He
described that each episode of yawning would
predictably produce an abduction movement of the
arm with associated distal high amplitude
postural left hand tremor, followed by a return
to the resting position on yawning cessation.
There was no associated left lower limb movement
during these episodes. The patient was unable to
reproduce the phenomenon of parakinesia
brachialis oscitans (PBO) voluntarily by
simulating yawning.
-
- Over the following weeks, the patient
noticed a gradual reduction in the frequency and
amplitude of PBO, concurrent with an improvement
of voluntary movements with his left-sided
limbs. However, in the context of subacute
worsening of his neurologic symptoms, PBO
re-emerged. Follow-up brain MRI performed 2
months after clinical onset demonstrated new
lesions accrual in a periventricular and
juxtacortical distribution (Figure, G) in
keeping with a diagnosis of MS. The original
large right hemi- spheric lesion had reduced in
size. The patient continues to be followed up in
the context of his trial participation.
-
- Discussion
- To the best of our knowledge, this is the
first reported case of PBO in a patient with MS.
Involuntary raising of an otherwise paretic arm
during yawning has been described in case re-
ports dating back to 1844. However, the term
parakinesia brachialis oscitans was only coined
by Walusinski in 2005, in a study describing 4
patients presenting with ischemic strokes, all
affecting the internal capsular region.1
-
- Our patient had several presenting features
unusual for MS, including epileptic seizures at
presentation, the tumefactive nature of brain
lesions detected, and the absence of OCB in the
CSF. These are, however, not uncommon in
patients with MS presenting with tumefactive
demyelination, as previously described
elsewhere.2,3 Further clinical and MRI follow-up
in the context of a controlled clinical trial
eventually ruled out potential differential
diagnoses while fulfilling the McDonald 2017
diagnostic criteria for this condition.
-
- PBO may occur within the first day after
cerebral infarction or as late as 4 months after
stroke onset2 and has been observed in paretic
limbs during both the þaccid and spastic
stages of post- stroke recovery. These
involuntary and patterned movements usually
include a combination of abduction of the
shoulder,
- þexion of the elbow, and extension of
the fingers. Variants in- clude lower limb
involvement3 and associated tremor4 such as in
our case. Some patients can deliberately
suppress their PBO,5 which tends to disappear
with recovery of motor function in the affected
limb, usually within 6 months, although
persistence of PBO beyond this point has been
observed.3
-
- The pathophysiology of PBO remains
unclear.6,7 The most widely accepted explanation
is that PBO reþects a pro- prioceptive
loop activation, whereby the strong contraction
of respiratory muscles during yawning generates
a pro- prioceptive signal through the
spinocerebellar tract that reaches the medullary
lateral reticular nucleus. This in turn induces
a motor signal through extrapyramidal pathways
to cervical anterior horn cells, resulting in
the involuntary movement of the affected upper
limb. The interruption of the
cortico-pontocerebellar pathway is thought to be
es- sential for the manifestation of PBO because
it enables proprioceptive loop
disinhibition.
-
- Although previously reported in the context
of stroke only, our case demonstrates that PBO
may also occur with large inþammatory
demyelinating lesions. Mechanistically, we
hypothesize that our patient's tumefactive
lesion in the right hemisphere temporarily
disrupted cortico-ponto-cerebellar and
corticospinal pathways releasing subcortical
structures from cortical inhibition, thereby
enabling the proprioceptive loop disinhibition
necessary for the spontaneous yawning-
associated upper limb movement to be initiated
through intact spinocerebellar pathways.
-
- PBO is clinically relevant because it can
potentially be mis- taken for a focal epileptic
seizure, particularly in an acute setting.
Recognizing the highly stereotyped limb movement
pattern in the context of yawning as its
predicable trigger is key in diagnosing this
condition.
-
- References
-
- 1. Walusinski O, Quoirin E, Neau JP. La
parakin´esie brachiale oscitante
[Parakinesia
- brachialis oscitans]. Rev Neurol
(Paris). 2005;161(2):193-200. doi:10.1016/s0035-
3787(05)85022-2
- 2. Li J-Y, Wu L, Sun L-Q, Xiong J-M.
Clinical and radiological characteristics of
hemiplegic arm raising related to yawning in
stroke patient. Med J Chin PLA. 2018;
43(3):229-233.
doi:10.11855/j.issn.0577-7402.2018.03.09
- 3. de Lima PM, Munhoz RP, Becker N, Teive
HA. Parakinesia brachialis oscitans: report of
three cases. Parkinsonism Relat Disord.
2012;18(2):204-206. doi:10.1016/
j.parkreldis.2011.09.020
- 4. Farah M, Barcellos I, Boschetti G,
Munhoz RP. Parakinesia brachialis oscitans: a
case report. Mov Disord Clin Pract.
2015;2(4):436-437. doi:10.1002/mdc3.12234
- 5. Chowdhury A, Datta AK, Biswas S, Biswas
A. Parakinesia brachialis oscitans &endash; a
rare post-stroke phenomenon. Tremor Other
Hyperkinetic Mov (N Y). 2022;12(1):6. doi:
10.5334/tohm.680
- 6. To¨pper R, Mull M, Nacimiento W.
Involuntary stretching during yawning in
patients with pyramidal tract lesions: further
evidence for the existence of an independent
emotional motor system. Eur J Neurol.
2003;10(5):495-499. doi:10.1046/j.1468-
1331.2003.00599.x
- 7. Walusinski O, Neau JP, Bogousslavsky J.
Hand up! Yawn and raise your arm. Int J Stroke
2010;5(1):21-27.
doi:10.1111/j.1747-4949.2009.00394.x
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