PARASOMNIAS
Propriospinal Myoclonus at the Sleep-Wake Transition: A New Type of
Parasomnia
Roberto Vetrugno MD,1 Federica Provini MD,1 Stefano Meletti MD,1 Giuseppe Plazzi MD,1 Rocco Liguori MD,1 Pietro Cortelli MD,2 Elio Lugaresi MD1 and Pasquale
Montagna MD1
1Institute of Clinical Neurology, University of Bologna, Bologna, Italy
2Institute of Neurology, University of Modena and Reggio Emilia
Study objectives: To describe the clinical, neurophysiological, and
polysomnographic characteristics of propriospinal myoclonus (PSM) at
the sleep-wake transition.
Design: Patients referred for insomnia due to myoclonic activity arising
during relaxed wakefulness preceding sleep, or complaining of muscular
jerks also during intrasleep wakefulness and upon awakening in the morn-
ing were considered.
Setting: All patients underwent EEG-EMG recordings during wakeful-
ness and night sleep. Back-averaging of the EEG activity preceding the
jerks was performed. Somatosensory evoked potentials (SEPs), transcra-
nial magnetic stimulation (TMS) and spinal and cranial MRI were also
done.
INTRODUCTION
PROPRIOSPINAL MYOCLONUS (PSM) IS CHARACTER-
IZED BY FLEXION OR EXTENSION RHYTHMIC-
ARRHYTHMIC JERKS ARISING IN AXIAL MUSCLES AND
SPREADING TO MORE CAUDAL AND ROSTRAL SEG-
MENTS. In PSM a spinal generator is believed to recruit axial
and limb muscles via slowly conducting propriospinal pathways
to produce the myoclonic jerks.1 Several cases of PSM have been
reported and multiple sclerosis, cervical trauma, thoracic herpes
zoster, HIV infection, excision of cervical hemangioblastoma,
thoracic arachnoid cyst, syringomyelia, and ischemic myelopathy
have occasionally been described as associated lesions.1-10 In
many cases, however, PSM remains idiopathic. PSM may be
spontaneous and stimulus-sensitive and may be worse with the
patient leaning back or lying in bed, as an effect of posture.1,11 In
three previous reported cases PSM showed a striking relationship
with vigilance level, since it occurred in a semirrhythmic fashion
only during the wakefulness period preceding sleep onset. In
these patients jerks recurred every 1020 seconds and were of
such intensity as to cause severe sleep-onset insomnia. The jerks
were absent during sleep proper. It was speculated that changes
in supraspinal control peculiar to the pre-dormitum stage set the
spinal generator responsible for PSM into motion.8 This peculiar
relationship of PSM with relaxed wakefulness and the ensuing
disturbance of sleep allowed the proposition that PSM may rep-
resent a disorder of the sleep-wake transition period in some
Accepted for publication July 2001
Address correspondence to: Dr. Roberto Vetrugno, Istituto di Clinica
Neurologica, Via Ugo Foscolo 7, 40123 Bologna, Italy; Tel:++39/051/6442225;
Fax: ++39/051/6442165; E-mail: elugares@neuro.unibo.it
SLEEP, Vol. 24, No. 7, 2001
Participants: Four patients were studied all affected with involuntary jerks
arising when falling asleep, and one with jerks also during sleep and upon
awakening in the morning.
Interventions: N/A
Measurements and Results: Polysomnographic investigations revealed
jerks arising during the sleep-wake transition period. Myoclonic activity
was neurophysiologically documented to be of the propriospinal type.
SEPs, TMS and MRI were normal
Conclusions: PSM may have a peculiar relationship with the state of vig-
ilance and represent a sleep-wake transition disorder. In this regard we
consider PSM a new type of parasomnia.
Key words: Propriospinal myoclonus; wake; sleep; parasomnia; insom-
nia.
patients.
We report here four additional patients with PSM, aged 36
57, in whom we performed videopolysomnographic (VPSG)
recordings aimed at clarifying the relationship of PSM with the
state of vigilance, in particular the pre- and post-dormitum
stages.
PATIENTS AND METHODS
Case 1
This 57-year-old-man had a family history of hyperthy-
roidism. At age 48 he underwent subtotal thyroidectomy for mul-
tiple nodules, with subsequent thyroxine treatment. Since 45
years he has suffered repetitive involuntary jerks of the trunk,
neck and limbs occurring every night and impeding sleep. The
jerks recurred every 3060 seconds approximately, often for
many times before the patient finally succeeded in falling asleep.
The jerks persisted in all positions in bed (supine, prone, on the
side). The patient had tried no drugs but had noted some benefi-
cial effects from alcohol. On admission neurological examination
and routine blood analysis were normal.
Case 2
This 36-year-old-man had a family history positive for breast
carcinoma in the mother. Since age five years he had a progres-
sive drooping of the eyelids and since age 15 years nocturnal
cramps of the legs. Neurological examination revealed bilateral
eyelid ptosis with restriction of vertical and horizontal eye move-
ments. Creatine phosphokinase (CPK) was elevated (190 and 330
I.U.; n.v. < 80 I.U.) and blood lactate level after standardized
physical effort increased. EMG showed abnormal myopathic
835 Propriospinal MyoclonusVetrugno et al
 F4 - C4
P3 - 01
Mylohyoid
R.Masset.
R.S.C.M.
R.Pect.
R.Interc.
R.Bic.Br.
R.Tric.Br.
R.Rect.abd.
R.tl.Parasp.
L.Rect.abd.
L.tl.Parasp.
R.Rect.fem.
R.Gastr.
S.S. 55 yrs
Figure 1Polysomnographic recordings in Case 1 illustrating the quasi-periodic recurrence of myoclonus during relaxed wakefulness, causing sudden arousal.
Mylohyoid, mylohyoideous; Masset, masseter; SCM, sternocleidomastoideus; Pect, pectoralis; Interc, intercostalis; Bic Br, biceps brachi; Tric Br, triceps brachi; Rect
abd, rectus abdominis; Parasp, thoraco-lumbar paraspinalis; Rect fem, rectus femoris; Gastr, gastrocnemius.
potentials, and SFEMG of the left orbicularis oculi muscle
revealed an abnormal jitter. Tensilon test was negative. Biopsy of
the left deltoideus muscle showed mild scattered hypotrophic
fibers with fiber type I predominance without ragged-red fibers
(RRF). The findings were consistent with ocular myopathy.
At age 35 he began complaining of sudden involuntary jerks
of the trunk and limbs arising during relaxed wakefulness, in par-
ticular when lying in bed. The jerks recurred many times, one to
two hours prior to falling asleep, and reappeared briefly during
nocturnal arousals and in the morning upon awakening.
Case 3
This 55-year-old-man had a family history positive for cere-
bellar neoplasm in the son. At age 53 he noticed the occurrence
of sudden involuntary isolated jerks of the head when trying to
fall asleep. The jerks were concomitant with a click sensation
in the head and involved the trunk and limbs. At the beginning
the jerks occurred only three to four times when the patient was
relaxed and ready to fall asleep. After six months, myoclonic
activity increased, recurring in clusters at intervals of 30 seconds
to one minute, making falling asleep impossible. Diazepam in the
evening reduced the myoclonic jerks and allowed the patient to
sleep. On neurological examination, lower limb hypopallesthesia
was associated with asymmetric brisk tendon reflexes.
Case 4
This 43-year-old-man developed, at age 32, involuntary jerks
involving the trunk and limbs. The jerks occurred during relaxed
wakefulness preceding falling asleep and were concomitant with
an inner sensation of electrical shock in the head and/or the chest.
The jerks recurred 1030 times per hour, making sleep onset
SLEEP, Vol. 24, No. 7, 2001
3 sec.
very difficult. Neurological examination was normal. Laboratory
tests revealed hepatitis C.
Neurophysiological Studies
All patients underwent 24-hour videopolysomnographic
(VPSG) recordings. Detailed study of myoclonic activity was
done by means of bipolar silver/silver chloride electrodes placed
2 cm apart longitudinally over the relevant muscle bellies (1024
Hz sampling frequency and low-pass of 300 Hz and high-pass of
30 Hz). In particular EMG activity was recorded from electrodes
placed on right masseter, right and left sternocleidomastoideus,
right pectoralis, right and left thoracolumbar intercostalis, right
and left biceps brachi and triceps brachi, right and left rectus
abdominis and thoracolumbar (T10-L2) paraspinalis, right rectus
femoris, right biceps femoris, right tibialis anterior and right gas-
trocnemius (Cases 1 and 2); and right and left masseter, right and
left sternocleidomastoideus, left trapezius, left deltoideus, right
and left thoracolumbar intercostalis, right and left rectus abdomi-
nis and thoracolumbar (T10-L2) paraspinalis, right and left
biceps femoris and rectus femoris, right and left tibialis anterior
(Cases 3 and 4).
Back-averaging of the EEG activity preceding the jerks was
performed off-line (10-20 International System, with EEG band
pass of 1-70 Hz)12 (Shibasaki, 1988). Data were acquired on a
Grass polygraph (mod. 78 E) and then stored in a computerized
system (Neuroscan, SCAN 3.0) for analysis. All patients under-
went mental and sensory stimulations aimed at evoking the
myoclonic jerks. The day after VPSG, all patients underwent
standard somatosensory evoked potentials (SEPs) with stimula-
tion of the median and tibial nerves13 and transcranial magnetic
stimulation (TMS) with recording from the median and peroneal
innervated muscles. Specifically, TMS to the abductor pollicis
836 Propriospinal MyoclonusVetrugno et al
 Wake
Stage 1
Stage 2
Stage 3
Stage 4
REM

Myocl./min. 7
6
5
4
3
2
1
0
60 120 180 240 300 360 420 480 min
S.S. 57 yrs

Figure 2Histograms illustrating the occurrence of myoclonic jerks (bottom) in relation to the sleep-wake cycle in Case 1. Peaks in the lower traces express the num-
ber of jerks per 1-minute intervals. Jerks are limited to the drowsiness period preceding sleep onset.

brevis and tibialis anterior muscles was performed with the coil
center positioned above vertex and 46 cm frontally and 23
R.Mass. cm contralaterally respectively. Relaxed motor evoked potentials
(threshold MEPs) and contracted MEPs (facilitated MEPs) were
evaluated. 35 consecutive trials were recorded and onset laten-
cies were read from the earliest MEP. MEPs were analysed for
Mylo.
onset latency (cortical latency, CL), peak-to-peak amplitude and
duration, the former expressed as ratios related to M-wave ampli-
tude and duration. Root stimulation was performed with coil
windings overlying the appropriate nerve roots, C3-4 and S3
R.S.C.M.
spinous processes, with obtained MEPs analyzed for onset laten-
cy only. Central conduction time (CCT) was evaluated consider-
ing CL and the latency of the earliest reproducible wave of 20 F
waves elicited by supramaximal median and peroneal distal
R.Pectoralis
nerve stimulation, according to standard formula14. Brain and
total spinal MRI were also performed in all patients.

R.t.I.Parasp. RESULTS
Case 1
The spontaneous jerks were restricted to the prehypnic wake
L.t.I.Parasp. period and appeared only after the EEG alpha rhythm disap-
peared when the patient was falling asleep, causing sudden
arousal (Figure 1). Myoclonic jerks recurred at quasi-periodic
intervals (range 1530 sec) and this pattern was repeated over a
R.Rectus.Fem. hundred times (Figure 2). Sleep structure was abnormal, charac-
terized by increased sleep stages 12 (74%, n.v. 50%),
decreased deep sleep stages 34 (10%, n.v. 25%) and REM
sleep (16%, n.v. 25%), and increased number of arousals (Fig. 1).
100 V Mental exercise (arithmetic calculations) caused EEG desyn-
-200 -100 0 100 200 300 400 500 600 msec. chronization and made the jerks disappear. The jerks could not
S.S. 55 yrs be evoked by sudden auditory and sensory stimuli. Myoclonic
activity constantly involved the sternocleidomastoideus (SCM),
Figure 3EMG recording of a single myoclonic jerk in Case 1 showing a rostro-
caudal propagation of muscular activity starting in the right sternocleidomas-
mylohyoideus, biceps brachi, pectoralis, intercostal, thoraco-
toideus. Mass, masseter; Mylo mylohyoideus, SCM, sternocleidomastoideus; tl lumbar paraspinalis, and rectus abdominis muscles bilaterally.
Parasp, thoraco-lumbar paraspinalis, Rectus Fem, rectus femoris. Masseter and rectus femoralis were often involved while the tib-

SLEEP, Vol. 24, No. 7, 2001 837 Propriospinal MyoclonusVetrugno et al

 Wake
Stage 1
Stage 2
Stage 3
Stage 4

REM
Myocl./min.
6
5
4
3

2
1
0
60 120 180 240 300 360 420 min
C.A. 36 yrs

Figure 4Histograms illustrating the occurrence of myoclonic jerks (bottom) in relation to the sleep-wake cycle in Case 2. Peaks in the lower traces express the
number of jerks per 1-minute intervals. Jerks are not limited to the drowsiness period preceding sleep but reappear also during intra-sleep wakefulness and upon
awakening in the morning.

Wake
Stage 1

Stage 2

Stage 3-4

REM

Myocl./min. 4

0
60 120 180 240 300 360 420 min
C.F. 55 yrs

Figure 5Sleep-wake histogram (upper trace) in relation to myoclonic activity per 1-minute intervals (lower trace) in Case 3. Note the long sleep latency due to the
repetitive occurrence of PSM.

ialis anterior and gastrocnemius rarely and minimally. The first
activated muscle was always the right sternocleidomastoideus
with a variable delay to the most caudal and rostral muscles (right
masseter and right gastrocnemius) ranging from 16 ms to 155 ms
(Figure 3). A crude estimation of the propagation velocity along
the spinal cord, obtained by dividing the length from C1 to L1
spinous processes by the mean latency between the right SCM
and right gastrocnemius, gave values of 46 m/s. Back averag-
ing of the EEG, triggered on the right SCM, disclosed no time-
locked cortical potential in the one second preceding the jerks.
Median and tibial nerves somatosensory evoked potentials
(SEPs), trancranial magnetic stimulation (TMS), and spinal and
cranial MRI were negative. Clonazepam (1 mg/day at bedtime)
was effective in reducing the jerks.

SLEEP, Vol. 24, No. 7, 2001 838 Propriospinal MyoclonusVetrugno et al

 Cz01
Mylohyoid
R.Masset.
L.Masset.
R.S.C.M.
L.S.C.M.
L.Trapez.
L.Delt.
R.Interc.
L.Interc.
R.tl.Parasp.
L.tl.Parasp.
R.Rect.abd.
L.Rect.abd.
L.Rect.fem.
L.Bicep.fem.

R.Rect.fem.

R.Bicep.fem.

C.F. 55 yrs 50 V 50 V
1 sec 1 sec

Figure 6EEG-EMG recording of PSM in Case 3. Left and right panels illustrate the same jerk at different velocities of recording. Mylohyoid, mylohyoideus; Masset,
masseter; SCM sternocleidomastoideus; Trapez, trapezius; Delt, deltoideus; Interc, intercostalis; tl Parasp, thoraco-lumbar paraspinalis; Rect abd, rectus abdominis;
Rect fem, rectus femoris; Bicep fem, biceps femoris.

Wake
Stage 1

Stage 2
Stage 3

Stage 4

REM

Myocl./min. 4
3

2
1
0
0 120 240 300 360 420 min
60 180
C.L. 24 yrs

Figure 7 Histogram illustrating the occurrence of the myoclonic jerks (number of jerks per 1-minute) (bottom) in relation to the sleep-wake cycle in Case 5.
* indicates transient suppression of PSM by mental exercise.

SLEEP, Vol. 24, No. 7, 2001 839 Propriospinal MyoclonusVetrugno et al

 Case 2

*patient with ocular myopathy; #patient in addendum; patients previously reported (Montagna et al., Movement Disorders 1997); Treatment responses +, ++, and +++: mild, great, and complete abolition
The jerks arose in a semirrhythmic fashion during relaxed

Treatment and Response

wakefulness; the patient did not fall asleep for a full hour after
lights out. There was a clear relationship between alpha EEG
activity fragmentation and the emergence of the jerks. PSM reap-

Clonazepam +++
Clonazepam +++
peared briefly during intra-sleep wakefulness and upon awaken-

Clonazepam ++
Clonazepam +

Clonazepam +

Clonazepam +
Barbiturate +
ing in the morning (Figure 4). Sleep structure was abnormal:

Opiates ++
stages 12 were 68% (n.v. 50%), stages 34 21% (n.v. 25%),
and REM sleep 11% (n.v. 25%) (Fig. 4). Sleep latency was
increased to 172 minutes; sleep efficiency was decreased (60%,
n.v.>85%). Mental exercise prevented the appearance of the
jerks. Sudden auditory and sensory stimuli, including peripheral
nerve electrical stimulation, did not evoke any jerk.
T8-T9 arachnoid cyst Polymyographic analysis documented that the originating muscle
was the left biceps brachi with subsequent involvement of the
C5-C6 bulging

SCM and then the truncal and lower limb muscles. The delay
between the first activated and the most caudal and rostral mus-
Normal

Normal
Normal

Normal

Normal
Normal

cles was 34110 ms, giving an intraspinal conduction velocity

MRI

of 516 m/s. EEG back-averaging, triggered on the left bicep

brachi, did not show any cortical jerk-related potential. SEPs,
TMS, spinal and cranial MRI were negative. Clonazepam (1.5
Spinal propagation velocity

mg/day at bedtime) dramatically abolished the jerks.

Case 3
Repeated jerks appeared during relaxed wakefulness every
two to five minutes for two hours, preventing the patient from
falling asleep (Figure 5). The myoclonic contractions emerged
5-16 m/s
5-10 m/s

4-13 m/s
2-16 m/s
4-6 m/s

5-7 m/s

2-3 m/s

when alpha activity became diffuse on the EEG, evident also on

8 m/s

the anterior scalp regions and intermixed with theta activity.

Sleep structure was abnormal, characterized by increased light
R. Rectus Abdominis

sleep stages 12 (67%, n.v. 50%), slightly decreased deep sleep

L. Biceps Brachialis

stages 34 (21%, n.v. 25%) and decreased REM sleep (11%,

Starting muscle

L. Paraspinalis

n.v. 25%), with increased arousals. Sleep latency was increased

L. Intercostalis

to 205 minutes and sleep efficiency reduced (72%, n.v. >85%).

Mental exercise prevented the emergence of the myoclonus caus-
R. SCM

L. SCM

L. SCM

L. SCM

ing EEG desynchronization. Somaesthetic and acoustic stimula-

Table 1Clinical and laboratory findings of propriospinal myoclonus

tion was ineffective in causing jerks. The left intercostalis was the
muscle first involved in the myoclonic jerks, with subsequent
caudal and rostral propagation (Figure 6) with a delay of 5887
sleep post-dormitum

sleep postdormitum
pre-dormitum infra-

pre-dormitum infra-

ms, giving an intraspinal conduction velocity of 510 m/s. Jerk-

related EEG back-averaging, triggered on the left intercostalis,
pre-dormitum

pre-dormitum
pre-dormitum

pre-dormitum
pre-dormitum
pre-dormitum

did not show any cortical jerk-related potential. TMS disclosed

increased motor latency to the left abductor pollicis brevis with
of PSM; SCM: sternocleidomastoideus; R: right; L: left.

a prolonged central conduction time. Lumbar SEPs potentials

State

were prolonged in latency and reduced in amplitude from the

posterior tibial nerves. A C5-C6 disc protrusion impinging on the
cord was present on spinal MRI. Clonazepam (2 mg/day at bed-
Onset (yrs)

time) abolished the jerks.

Case 4
45
35

53
32
23

40
30
37

The jerks arose only during sleep-wake transition when alpha

activity became diffuse over the EEG, causing arousals. Sleep
efficiency was reduced (79%, n.v.>85%) and sleep structure
2) male, 36 yrs*

5) male, 24 yrs#

6) male, 71 yrs
7) male, 50 yrs
8) male, 41 yrs
1) male, 57 yrs

4) male, 43 yrs
3) male, 55yrs

demonstrated increased light sleep stages 1-2 (65%, n.v. 50%),

decreased deep sleep stages 3-4 (18%, n.v. 25%) and decreased
Patients

REM sleep (17%, n.v. 25%). When the patient was mentally acti-
vated, with EEG desynchronization, myoclonic jerks never
occurred. Auditory and somatosensory stimulation did not evoke
SLEEP, Vol. 24, No. 7, 2001 840 Propriospinal MyoclonusVetrugno et al
 Table 2Clinical, neurophysiological and therapeutic characteristics of propriospinal myoclonus (PSM), periodic limb movement during sleep (PLMS), restless legs syndrome
(RLS), "painful legs and moving toes" syndrome, spinal myoclonus and cortical myoclonus.

Clinical Findings Neurophysiology Treatment

SLEEP, Vol. 24, No. 7, 2001

PSM Axial muscle jerks with slow rostral and caudal prop- Duration of EMG burst: 100-300 ms. Benzodiazepines
agation. Jitter of intermuscle latencies: 16-200 ms.
Intraspinal conduction velocity: 2-16 m/s.

PLMS State-dependent periodic (T:20-30 s) dorsiflexion of Sustained polyclonic EMG activity with inconstant Dopa Agonists
great toe, foot, and/or flexion of entire leg, with fre- muscular recruitment pattern and extremely variable Benzodiazepines
quent involvement of upper limbs. intermuscle latencies (> 2 s). Opioids

RLS Incessant bedtime dysesthesia relieved by agitated As in PLMS; patients may get out of the bed and walk Dopa Agonists
around. Benzodiazepines
motor activity with legs and arms flexing, stretching
Opioids
and crossing.

Painful Legs & Moving Toes Severe pain of feet with burning sensation and repet- Irregular EMG bursts in small muscles of the foot and Dopa Agonists
itive semicontinuous movements of toes, not neces- leg.
sarily worse at night or relieved by activity.

841
Spinal Myoclonus Segmental jerks of trunk as well as of limb muscles, Rhythmic contractions of the same segmentally Benzodiazepines
involving only one or two adiacent spinal segments innervated muscles with EMG-burst duration of 100- Baclofen
and not propagated. 200 ms. Carbamazepine
Tetrabenazine

Cortical Myoclonus Irregular and asynchronous jerks involving mainly dis- EEG spikes leading the jerk by about 20 ms (hand Antiepileptic drugs
tal limb and facial muscles. muscles) with duration of the myoclonic EMG dis-
charges usually less than 50 ms.

Propriospinal MyoclonusVetrugno et al
any jerk. The left SCM was the muscle first involved in the
myoclonic jerks. Latency delay between the SCM and the most
caudal muscle involved (i.e., left tibialis anterior) was 81115
ms, with an intraspinal conduction velocity of 57 m/s. EEG
back-averaging triggered on the left SCM, disclosed no related
cortical potentials in the one second preceding the jerks. SEPs,
TMS, and spinal cord MRI were normal. Barbesaclone (200
mg/day at bedtime) reduced the intensity and frequency of the
jerks.
DISCUSSION
Our four patients had neurophysiologically documented PSM
occurring solely at the transition from wake to sleep, or during
intrasleep arousals or upon awakening. There were no clinical or
neurophysiological features suggesting a cortical or reticular ori-
gin of the myoclonus. In particular, there were no time-locked
cortical correlates in back-averaged EEG activity preceding the
spontaneous jerks, which, moreover did not involve distal mus-
cles, as is typical of cortical myoclonus. It is more difficult to
exclude a reticular origin of the myoclonus, since cranial muscles
(masseter and mylohyoideus) could sometimes be activated in an
ascending fashion. However, cranial nerve involvement was not
obligatory, and the jerks were not elicited by auditory or
somatosensory stimuli, as usually happens in reticular brainstem
myoclonus. The diffusion of the jerks to involve multiple spinal
segments, their origin in axial muscles, the long duration of the
EMG bursts (100-300 ms, sometimes with polymyoclonic
shape), the marked jitter in intermuscle latencies and the low
spinal conduction velocity are characteristic of axial myoclonus
of propriospinal origin.1 In all of our cases the jerks had a clear
relationship with the sleep-wake transition period. The jerks
arose in a semirrhythmic fashion only during the relaxation phase
prior to sleep, and disappeared with the earliest stages of sleep
and throughout all sleep stages. In one case PSM reappeared
briefly during intra-sleep wakefulness and upon awakening in the
morning. Mental and sensory stimulation during relaxed wake-
fulness stopped the jerks concomitantly with the disappearance
of the EEG alpha activity and independently of any postural
changes. Myoclonus thereafter reappeared as the patients were
left undisturbed and the EEG alpha activity returned. All of these
four patients had an altered sleep structure and complained of
insomnia. There seemed to be no relevant single causative mech-
anism in these patients, and we consider it possible that the ocu-
lar myopathy and cervical myelopathy observed in Cases 2 and 3
respectively were fortuitous associations. Our present report
therefore confirms the occurrence of PSM jerks at the transition
from wakefulness to sleep in a pattern quite similar to the cases
previously reported by us (Table 1) and others.8,15 Accordingly,
we propose PSM as a new sleep-wake transition disorder causing
insomnia and altered sleep structure. On polygraphic recordings
PSM can be readily distinguished from PLMS and Restless Legs
Syndrome, the painful legs and moving toes syndrome and seg-
mental and epileptic myoclonus (Table 2). As in some of these
disorders, however, Clonazepam afforded some degree of relief
in most of our patients with PSM.
Notably, seven out of the total eight patients with PSM at the
transition from wake to sleep8,15 were males, a finding compara-
ble to the striking and as yet unexplained male prevalence in
some NREM and REM parasomnias such as REM Behavior
SLEEP, Vol. 24, No. 7, 2001
Disorders,16 and in Nocturnal Frontal Lobe Epilepsy.17
The term sleep-wake transition disorders refers to a group of
parasomnias that occur during the transition from wakefulness to
sleep or from one sleep stage to another. Rhythmic movements
disorders, sleep-talking, and nocturnal legs cramp belong to the
sleep-wake transition disorders in the International
Classification of Sleep Disorders.18 Sleep starts (hypnic jerks) in
particular are non-periodic myoclonic movements, usually
involving the trunk and all extremities simultaneously, occurring
at sleep onset and frequently associated with a perception of
falling.19 Most normal adults indicate that they have experienced
hypnic jerks at the transition between waking and sleep (but is a
real blow of luck to record one of them polygraphically). In same
patients, however, they may be so severe and frequent to cause a
sleep-onset insomnia.18,20,21 The polymyographic characteristics
of the physiological sleep starts, in particular whether they show
a propriospinal propagation pattern, are unknown. PSM in our
cases was indeed reminiscent of the sleep starts encountered in
the general population, but we cannot substantiate our suggestion
in the absence of polygraphic data on sleep starts.
Vigilance level is an important factor for the manifestation and
variability of many movement disorders. The pre-dormitum and
post-dormitum periods in particular are characterized by vigi-
lance level fluctuations which modulate the state-dependent
motor behavior. In our cases, PSM was precipitated by drowsi-
ness preceding nocturnal sleep. The fact that PSM appeared to be
specifically related to sleep inducing mechanisms acting espe-
cially during the pre-dormitum stage argues for the neurophysio-
logical independence of these peculiar states of vigilance, the
pre- and post-dormitum stages. Pre- and post-dormitum are usu-
ally lumped all together into unspecified wakefulness but
instead possess intrinsic cerebral metabolic patterns on PET stud-
ies22 and mental23 and neurophysiological24,25 characteristics. In
our opinion the pre-dormitum and post-dormitum, with the rela-
tive changes in firing patterns of many neuronal supra-spinal
populations related to the control of motor activity, could act to
release a still unknown spinal pacemaker (propriospinal
motoneurons?)26 responsible for PSM.
Addendum
Case 5
A 24-year-old man presented with a one-year history of sud-
den involuntary jerks of the head, axial, and limb muscles aris-
ing during relaxed wakefulness and drowsiness and impeding
falling asleep. Neurological examination and routine tests were
normal. VPSG recorded over one hundred massive jerks recur-
ring in a semirrhythmic fashion during relaxed wakefulness prior
to falling asleep and dramatically disappearing as soon as spin-
dles and K-complexes appeared on the EEG. Isolated jerks could
reappear during intra-sleep arousals and repetitively upon awak-
ening in the morning (Fig. 7). Mental exercise stopped the jerks.
Sleep structure was nearly normal: stages 12 56% (n.v. 50%),
stages 34 29% (n.v. 25%), and REM sleep 15% (n.v. 25%),
sleep latency 40 minutes with normal sleep efficiency (88 %, n.v.
>85%). EMG analysis indicated that the left SCM was the start-
ing muscle with subsequent involvement of the truncal and lower
limb muscles. The delay between the first activated and the most
caudal muscles was 42-132 ms, giving an intraspinal conduction
842 Propriospinal MyoclonusVetrugno et al
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