Segmental Myoclonus

Clinical and Pharmacologic Study

Joseph Jankovic, MD, Ricardo Pardo, MD

\s=b\ We observed 37 patients (mean age correct diagnosis of this movement Patient 12
at onset, 48.5 years; range, 13 to 84 disorder is important because it may A 73-year-old hypertensive man had an
years) with segmental myoclonus (18 be a symptom of a serious underlying acute onset of dysarthria, dysphagia, left-
branchial, 19 spinal). Etiologies for bran- disorder, and because pharmacologie arm ataxia, and bilateral leg weakness five
chial myoclonus included brain-stem therapy provides effective relief in years previously. He partially recovered,
demyelination, cerebrovascular disease, most patients. Previous reports but three years later he developed palatal
Meige's syndrome, cerebral arteritis sec- described either one case or a few myoclonus (1 to 2 Hz). There was residual
ondary to bacterial meningitis, central cases, and the clinical-anatomical cor¬ right Horner's syndrome, skew deviation,
nervous system Whipple's disease, acute relation was often lacking. Because ocular dysmetria, coarse sustention and
kinetic tremor of the arms, marked trun-
cervicomedullary trauma, and cerebellar segmental myoclonus has not been kal ataxia, dysphagia, sleep apnea, and
degeneration. Spinal myoclonus was well characterized, we have studied mild quadriparesis, which was worse on
associated with laminectomy, remote the clinical and pharmacologie fea¬ the right side.
effect of cancer, spinal cord injury, post- tures of this disorder in 37 patients. A digital subtraction angiogram was
operative pseudomeningocele, laparoto- normal except for a vestigial left vertebral
my, thoracic sympathectomy, poliomyeli- PATIENTS AND METHODS artery. A computed tomographic (CT) scan
tis, herpes myelitis, lumbosacral radiculo- showed diffuse cerebral atrophy. A poly-
pathy, spinal extradural block, and Thirty-seven patients with typical bran¬ somnogram revealed brief episodes of cen¬
myelopathy due to demyelination, electri- chial or spinal segmental myoclonus were tral apnea and rhythmic horizontal eye
cal injury, acquired immunodeficiency selected from the registry of the Movement movements with synchronous EMG activi¬
Disorder Clinic, Baylor College of Medi¬ ty in the submental area that persisted
syndrome, and cervical spondylosis. The
cine, Houston (Tables 1 and 2). All patients during all stages of sleep and correlated
latency between the predisposing condi- were videotaped, and the myoclonic move¬ with the palatal myoclonus.
tion and the onset of myoclonus ranged ments were recorded by needle or surface Therapy with clonazepam, reserpine
from immediate to 33 years (mean, 2.9 electromyogram (EMG). The following hydrochloride, valproate, and lecithin had
years). In six patients, the myoclonus was seven patients are described to illustrate minimal or no effect on the myoclonus, but
the presenting symptom of a serious the characteristic and some unusual fea¬ tetrabenazine hydrochloride (150 mg/d)
underlying disease. Treatment with clon- tures of segmental myoclonus. markedly improved the patient's nystag-
azepam, tetrabenazine hydrochloride, or
other medications provides a satisfactory
control in most patients.
Table 1.—Branchial Myoclonus
(Arch Neurol 1986;43:1025-1031) Latency Between
Presumed Cause
Distribution of and the Onset of
Myoclonus Presumed Cause Myoclonus
TVTyoclonus isinvoluntary, brief,
an 1/70/M Palate, right Unknown Unknown
jerklike movement produced by sternocleidomastoid
muscle contraction (positive myoclo¬ muscle, pinna
2/68/F Palate, diaphragm, and Cerebellar degeneration
nus) or inhibition (negative myoclo¬ abdomen
2y
nus).13 It may be caused by lesions at 3/45/F Palate, tongue, and Demyeiinating disease Unknown
various levels of the central nervous
hypopharynx
system, including the cortex, brain Demyeiinating disease Unknown
stem, and spinal cord.310 S/62/F Palate Meige's syndrome
Segmental myoclonus refers to 6/21/F Palate Demyeiinating disease
rhythmic arrhythmic involuntary
or
7/84/M Lips, face, and platysma Brain-stem lacunar infarct
contractions of muscle groups (usual¬ 8/55/M Neck and larynx Unknown Unknown
ly agonists) supplied by one or several 9/20/M Palate, left to right Demyeiinating disease Unknown
contiguous segments of the brain 10/54/M Palate Pontine hemorrhage
stem (branchial myoclonus) or spinal Platysma Unknown Unknown
cord (spinal myoclonus). The frequen¬ Palate, ocular Brain-stem infarct Immediate
cy is usually 1 to 3 Hz, but the rate 13/42/M Palate Brain arteritis 2 d
may vary from 1 to 600/min. The 14/48/F Palate Demyeiinating disease
15/53/M Face and platysma Central nervous system Immediate
Whipple's disease,
Accepted for publication June 15, 1986. acquired immunodeficiency
From the Department of Neurology, Baylor syndrome
College of Medicine, Houston (Dr Jankovic) and 16/65/F Palate Meige's syndrome Immediate
the Department of Neurology, University of Tex- 17/49/M Face and Acute
as Medical School at Houston (Dr Pardo). platysma cervicomedullary
trauma
Reprint requests to the Department of Neurol-
ogy, Baylor College of Medicine, 1 Baylor Plaza,
18/34/M Left sternocleidomastoid Craniotomy for frontal lobe Immediate
muscle sarcoma, anoxia
Houston, TX 77030 (Dr Jankovic).

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 Table 2.—Spinal Myoclonus
Patient/Age at Latency Between Presumed Cause
Onset, y/Sex Distribution of Myoclonus Presumed Cause and the Onset of Myoclonus
19/61/M Left to right pectoralis deltoid, Spondylitic cervical myelopathy
and biceps
Right forearm flexors Thoracic sympathectomy 1 y
21/30/M Left biceps, brachioradialis, Spinal-brachlal plexus electrical injury 30 d
deltoid, and trapezius
Bilateral quadriceps, buttocks, Lateral medullary infarct 1.5 y
and hamstrings
23/68/M Right foot extensors Facial pseudomeningocele 90 d
24/70/M Gluteus maximus, bilateral Lumbar laminectomy 2d
25/54/M Left sternocelidomastoid muscle, Oat cell carcinoma
trapezius, pectoralis biceps,
and triceps
26/35/F Left arm, flank, and paraspinals Poliomyelitis 33 y
27/51/F Both legs and feet Spinal extradural block 1 d
28/60/F Both legs and hips Lumbosacral radiculopathy Unknown
29/56/M Right arm Cervical laminectomy for syringomyelia
30/55/F Right leg, intermittent left leg Breast cancer Immediate
31/24/F Right iliopsoas and hamstring Spinal injury
cord 1.5 y
32/52/F Hip extensors Spinal cord injury Unknown
33/73/F Right abdominal muscles Craniotomy for parietal glioblastoma 4 d
34/22/F Both legs and abdomen Laparotomy Immediate
35/77/F Lower abdomen Thoracic-sacral herpes myelitis 20 y
36/34/M Left leg and buttocks Lumbar laminectomy and acquired Immunodeficiency Immediate
syndrome-related myelopathy
37/13/F Left tricep. bicep, brachioradialis Demyeiinating disease Immediate

mus, palatal myoclonus, and his sleep dis¬ ophthalmoparesis, and orofacial myoclo¬ With clonazepam therapy (3 mg/d), there
turbance. nus suggested central nervous system was a moderate improvement of the invol¬
Patient 15
(CNS) Whipple's disease. He had normal untary contractions, and the myoclonus
fat absorption except for abnormal results resumed when the dose of clonazepam was
A 57-year-old bisexual man awakened in from Schilling tests I and II. A CT scan of reduced. The chronic right-arm pain was
September 1982 because of involuntary the abdomen was normal as were two relieved by a cervical cord stimulator,
clenching of his jaws and loud rhythmic small-bowel biopsy specimens. Antibodies which had no effect in the myoclonus.
clicking of his teeth. The jaw spasms for hepatitis A and were positive. The
human T-cell lymphotropic virus type III Patient 24
increased, and, in March 1984, he had
involuntary rhythmic pulling of the right antibody titer to surface antigen was A 70-year-old man had continuous
facial and platysma muscles. He comp¬ present, and the T4/T8 ratio was 1.2 (nor¬
lained of hand clumsiness, small handwrit¬ mal 1.9 ± 0.5). Laboratory tests of the rhythmic contractions of the gluteus maxi-
mi for six years. The contractions began
ing, right-hand tremor, a burning sensa¬ patient's CSF showed the following values: two days after a second lumbar laminecto¬
tion in both feet, and progressive weakness protein, 76 mg/dL (0.76 g/L); glucose, 71 my. The first procedure was performed
in both legs. Two months later, he noted mg/dL (3.94 mmol/L); and 20 leukocytes, because of lumbar stenosis and radiculopa¬
forgetfulness, blurring of vision, difficulty all of which were lymphocytes.
A diagnosis of CNS Whipple's disease thy, and the second procedure was per¬
looking down, and droopy eyelids. A mag¬ formed to repair a CSF leak, a complica¬
netic resonance imaging (MRI) scan was suspected, despite negative results
tion of the first surgery. An EMG showed
showed two small areas of increased densi¬ from a jejunal biopsy specimen, and, there¬
denervation in the L4-S2 paraspinal mus¬
ty in the white matter of the right frontal fore, a biopsy specimen of the right frontal cles and the gluteus minimi, and 3- to 4-Hz
lobe; a cerebral angiogram was normal. lobe was obtained. The biopsy specimen
In September 1984, the patient had a revealed rods that stained positively for rhythmical contractions of the gluteus
maximi without concomitant contraction
low-volume hypokinetic dysarthria, limita¬ periodic acid-Schiff, consistent with the of any other muscles (Figure). These con¬
tion of lateral gaze and of convergence and diagnosis of Whipple's disease with sulfa- tractions were not seen at rest, but only
absent vertical gaze, corrected by oculo- methoxazole and trimethoprim; his neuro¬
with voluntary contraction of the gluteus
cephalic maneuver. There was a continu¬ logic symptoms stablized. maximi in prone and standing positions.
ous, rhythmical 1- to 2-Hz myoclonus of There was no response to therapy with
Patient 21
the face and platysma, more so on the right
side. There was a moderate hypomimia and After an electrical injury to his left arm,
carbidopa-levodopa (Sinemet), diazepam,
or trihexyphenidyl. With tetrabenazine
a marked decrease in blink frequency. this 30-year-old man had shooting pains in
In February 1985, the patient developed a C7-T1 distribution, and moderate weak¬
hydrochloride therapy (200 mg/d), all
retropulsion, a broad-based shuffling gait,
involuntary muscular contractions were
ness and hyporeflexia of the left arm. One
and a decreased arm swing. He complained month later, he noted constant irregular
completely abolished. The involuntary con¬
tractions promptly returned when a place¬
of severe intolerance to cold, a low-grade contractions (10/min) of the left brachio¬ bo was substituted in a double-blind man¬
fever with night sweats, chronic recurrent radialis and biceps. The contractions ner." Therapy with tetrabenezine was
diarrhea, and loss of 6.75 kg in one month. spread to the left deltoid and trapezius resumed, and his buttock movements were
For the first time, he revealed his promis¬ when the arms were outstretched or controlled for 16 months until his death.
cuous homosexual practices for the past abducted.
ten years. Acquired immunodeficiency A CT scan of the brain and spine and Patient 29
syndrome (AIDS) was suspected also brain-stem auditory evoked potential
because of an oral Candida infection. Fur¬ (BAEP) were unremarkable. An EMG A 61-year-old man had right arm and
thermore, the combination of the gastroin¬ revealed denervation potentials in the C6 hand pain and numbness for 15 years. A
testinal tract symptoms, the supranuclear and C7 innervated muscles of the left arm. cervical syringomelia was surgically

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 She had spasticity and hyperreflexia of
1. Right Gluteus Maximus both legs and mild weakness of the foot
2. Right Hamstring extensors. There were 6- to 8-Hz rhythmic
contractions of the legs, more so on the
right. The jerks were present at rest and
were exacerbated by voluntary contraction
of her right-leg muscles. There was mild
spasticity of the legs and weakness of the
#-—( - foot extensors. A right-breast cancer, with
metastasis to the regional lymph nodes,
was diagnosed. A CT scan of the brain, a

-M—ffrH8h~"^HHf—ìfh
; I 270 ms I
myelogram, a BAEP, a somatosensory-
evoked potential, an EEG, an EMG, and
results from CSF studies, including cytolo¬
gy, were
There
all normal.
complete resolution of the
was a
involuntary contractions with clonazepam
u-*J
therapy (1.5 mg/d).
M cm =
200 µ Patient 36

1. Right Gluteus Maximus This is a 37-year-old homosexual man,

2. Right Gluteus Minimus whose three sexual partners had died of
AIDS. In August 1980, he had radicular
lumbosacral pain while lifting a heavy
object. Partial laminectomy for L5-S1 disk
was performed in September 1980 and in

2.
«fi—4|—^—41—41—14—^h
->»l»n. y li b · - . \** ^W" li.|lt"U.< > litt- » .1 l il >>' * ' «'»-I'«' 4 J l· * ·
January 1981. A total laminectomy was
performed in January 1982. Within a few
days after the last operation, the patient
noted involuntary movements primarily
involving his left leg.
In October 1982, the patient experienced
a 22.5-kg weight loss and generalized
lymphadenopathy. There was 4- to 6-Hz
I 270 ms rhythmic involuntary jerking of the entire
left leg, especially the left buttock and left
1 cm =
200 µ, hamstring. These movements were exacer¬
bated by standing. Additionally, there was
a sudden loss of extensor tone in the left
1. Right Gluteus Maximus
2. Left Gluteus Maximus
leg during a walk (negative myoclonus).
This caused several falls, and the patient
needed extension braces and crutches to
walk. Synchronous movements were occa¬
sionally present in his right leg. A more

-IHN
rapid (7 to 8 Hz) and less coarse resting
pronation-supination tremor was noted in
his right arm two years after the onset of
the left-leg myoclonus. There was no weak¬
ness, hyperreflexia, or clonus. A CT scan of
the brain and an EEG were normal, and
CSF studies showed normal results.
A complete myelogram in August 1983
1^-270 msj showed a nerve root sleeve defect from C7
to Tl and cervical spondylosis. Laboratory
Tl cm =
200 µ tests of the patients CSF showed the fol¬
lowing values: seven lymphocytes; protein,
42 mg/dL (0.42 g/L); and glucose, 52 mg/dL
Case 24. Electromyogram demonstrating 3- to 4-Hz rhythmic contractions lasting about 200 ms.
(2.9 mmol/L).
Top, Contractions in both glutei maximi. Center, Selective involvement of right gluteus maximus In March 1984, the patient had mild
sparing right gluteus minimus. Bottom, Selective involvement of right gluteus maximus sparing cogwheel rigidity of all four extremities
right hamstring muscles. and a supination-pronation tremor at rest
in his right arm. No other parkinsonian
features were seen. He continued to have
drained ten years later. Immediately after On clonazepam (1.5 mg/d) and carbama¬ segmental myoclonus involving predomi¬
his operation, the patient had excruciating zepine (600 mg/d) therapy, the patient had nantly his left leg, particularly when sit¬
pain in his right arm. There were rhythmi¬ "80% improvement in the right arm caus¬
"
ting and standing. An EMG in July 1985
cal contractions of the entire right arm at algia and myoclonus. showed nonsynchronous 5- to 6-Hz con¬
rest, markedly exacerbated when the arms Patient 30
tractions of his right arm and left leg. The
were held in an outstretched and in a T4/T8 ratio was depressed, and the human
"wing-beating" position. An EMG revealed A 55-year-old woman noticed difficulty T-cell lymphotropic virus type III antibody
3- to 4-Hz rhythmical contractions in the arising from a squatting position nine days was positive.
right biceps and brachioradialis. An MRI before her visit to the clinic. Two days The rhythmical contractions improved
of the cervical and thoracic cord, five years later, she developed violent, jerklike con¬ with therapy with clonazepam and tetra-
postoperatively, showed spinal cord atro¬ tractions of her entire right leg, which benazine, but administration of both medi¬
phy. sometimes spread to involve the left leg. cations was discontinued because of

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 adverse reactions. The segmental myoclo¬
nus was then controlled with carbidopa-
Table 3. Segmental Myoclonus: Pharmacologie Response
—

levodopa (Sinemet). Complete or

RESULTS Excellent Moderate Mildor Transient

There were 19 patients (eight men Control Control Control of

of Symptoms of Symptoms Symptoms No Effect
and 11 women) with spinal myoclonus Drug (Patient No.) (Patient No.) (Patient No.) (Patient No.)
and 18 patients (11 men and seven 13 (2, 4. 5, 6,
women) with branchial myoclonus 20, 22, 25, 8 (16, 21, 25,
(Tables 1 and 2). The mean age at 26, 28, 29,
30, 33, 34)
27, 29, 31,
36, 37) 1 (23) 3 (8, 12, 18)
onset was 50.2 years for branchial Clonazepam
Tetrabenazine hydrochloride 2 (24, 31) 1 (8)
myoclonus (range, 18 to 84 years) and Trihexyphenidyl 1 (35) 2 (14, 24)
46.8 years for spinal myoclonus
Baclofen 1 (9) 1 (20) 2 (12, 15)
(range, 13 to 77 years). The median Carbamazepine 1 (37) 3 (6, 8, 14)
frequency for spinal myoclonus was Carbidopa-levodopa (Slnemet) 1 (36) 3 (7, 24, 15)
3.5 Hz (range, 0.2 to 8 Hz), and for
branchial myoclonus, 2.5 Hz (range, Reserpine hydrochloride 1 (36) 1 (12)
0.1 to 7 Hz). Six patients had myo¬ Valproate 3 (6, 8, 12)
Amantadine hydrochloride 1 (6)
clonic movements of muscles derived
from the branchial clefts without evi¬ Propranolol hydrochloride 2 (6, 12)
Lecithin 1 (12)
dence of palatal myoclonus (patients
Diazepam 2 (23, 24)
7, 8, 11, 15, 17, and 18). In three
Phenytoin hydrochloride 2 (14, 15)
patients with palatal myoclonus, the
movements were associated with syn¬
chronous contractions of the neck,
limb, or trunk muscles (patients 1, 2, along with spinal myoclonus, the lev¬ The segmental myoclonus was the
and 3). Two had palatal myoclonus els of CSF homovanillic acid, 5- presenting symptom of a serious
associated with an action tremor, hydroxyindole acetic acid and 3- underlying disorder in three patients
blepharospasm, and oral-facial-man- methoxy-4-hydroxy-phenylglycol with branchial myoclonus (cerebro¬
dibular dystonic movements (Meige's were normal. In patient 15 who had vascular disease in patient 7, AIDS
syndrome) (patients 5 and 16). Five AIDS, a brain biopsy specimen was with CNS Whipple's disease in patient
patients with palatal myoclonus had diagnostic of CNS Whipple's dis¬ 15, and Meige's syndrome in patient
an audible clicking sound (patients 4, ease.12 16), and in three patients with spinal
5, 6, 9, and 14). Rhythmic contractions were docu¬ myoclonus (breast cancer in patient
All 16 patients with branchial myo¬ mented by EMGs in 14 of 19 patients 30, AIDS in patient 36, and demyeii¬
clonus had CT scans of the head; the with spinal myoclonus. Three studies nating disease in patient 37). Five
CT scans in four patients were abnor¬ showed only denervation (patients 21, patients (patients 3, 4, 6, 9, and 14)
mal and showed cerebellar atrophy 24, and 27). Patient 25, with oat cell with possible demyeiinating disease
(paient 2), diffuse cortical atrophy carcinoma of the lung, had severely had palatal myoclonus as the only
(patient 12), demyelination (patient abnormal sensory potentials without symptom.
9), and pontine hemorrhage (patient motor component, consistent with a The response to pharmacologie
10). The MRI scans showed bilateral dorsal root ganglion degeneration. treatment was assessed by the patient
frontal white matter lesions (patient Eight of nine EEGs were normal; one and by serial neurologic examina¬
15), and small lesions of the white patient exhibited diffuse slowing (pa¬ tions. Such an assessment was possi¬
matter in both cerebral hemispheres tient 7). Two of 12 CT scans of the ble in 54 patient trials (Table 3).
consistent with demyelination (pa¬ head in patients with spinal myoclo¬ Therapy with clonazepam produced
tient 14). Two of 14 electroencephalo¬ nus were abnormal, one CT scan complete or excellent control of symp¬
grams (EEG) showed diffuse slowing showed generalized atrophy (patient toms in 13 (56% ) of 23 patients, mod¬
(patients 2 and 13), but no abnormal 27), and one, a parietal mass lesion erate control of symptoms in six addi¬
discharges associated with the myo¬ (patient 18). Ten patients with spinal tional patients (27%), mild or tran¬
clonus. Brain-stem evoked potentials myoclonus had myelograms of which sient control in one patient (4%) and
and somatosensory evoked potentials four were abnormal and showed a had no effect in three patients (14% ).
were obtained in 12 patients but indi¬ complete block from C7 through Tl Therapy with clonazepam had to be
cated brain-stem abnormality in only (patient 27), a pseudomeningocele and discontinued in one patient because of
one patient (patient 3). Visual evoked lymphoma from L4 through S2 (pa¬ generalized rash (patient 36). In
potentials were normal in 12 patients. tient 23), spondylolysis and postsurgi- patient 16, the dose required for a
Two patients (patients 5 and 12) with cal changes in the cervical and lumbar complete remission caused excessive
palatal myoclonus had polysomno- regions (patient 36), and lumbar ste¬ daytime drowsiness and ataxia. Three
grams that showed the persistence of nosis (patient 24). The MRI scans patients with spinal myoclonus could
the movements during all stages of showed cervical spinal cord atrophy not tolerate clonazepam therapy, and
sleep, and in patient 12 there was (patient 29) and an incidental small all had excellent improvement with
central apnea. Tests of the CSF from infarct in the right hemisphere (pa¬ tetrabenazine therapy, while the two
13 patients showed normal results, tient 32). In two patients, CSF studies patients with branchial myoclonus
except for bacterial meningitis in showed an elevated protein level (pa¬ had a moderate to mild response. Te¬
patient 13 and lymphocytic pleocyto¬ tients 21 and 25). In patient 35, who trabenazine therapy was discontinued
sis in patient 15. In patient 5, with had probable demyeiinating disease of by one patient due to an allergic rash
palatal myoclonus and blepharo¬ the spinal cord, the visual evoked (patient 36) and by another due to
spasm, and in patient 36, who exhib¬ potentials showed a unilateral pre- restlessness and myalgias (patient
ited some mild parkinsonian features chiasmal abnormality. 31).

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 Of the other drugs administered continuous, flowing, unpredictable many other peripherally induced
either during the study or prior to the movement, randomly distributed movement disorders that may resem¬
patient's referral, trihexyphenidyl, throughout the body. Dystonia is dis¬ ble segmental myoclonus. These
baclofen, carbamazepine, and L- tinguished from myoclonus because include the syndrome of painful legs
DOPA provided varying degrees of the contractions are typically more (or arm) and moving toes (or fin¬
improvement (Table 3). The following sustained and have a twisting charac¬ gers),3536 pseudoathetosis secondary to
drugs were ineffective in relieving ter. The term myoclonic dystonia has proprioceptive deficit,37·38 rhythmic
symptoms of myoclonus: valproate been recently introduced to describe stump movements after amputa¬
(three patients), phenytoin hydrochlo¬ the rapid "jerklike" movements some¬ tion,39·40 and focal dystonic movements
ride (two patients), diazepam (two times seen in dystonia and in tic disor¬ associated with peripheral nerve inju¬
patients), propranolol hydrochloride ders.2·2326 Some patients with dystonia ry.41·42
(two patients), and lecithin (one may have an associated myoclonus in Palatal myoclonus is the most com¬
patient). body parts other than those involved mon form of segmental myoclonus,
with dystonia: the dystonia-myoclonus occurring in 12 (67%) of 18 patients
COMMENT with branchial myoclonus. It is due to
syndrome.2"21
Segmental myoclonus is differenti¬ Rhythmic myoclonus is sometimes a disconnection in the dentato-rubro-
ated from other types of myoclonus in difficult to distinguish from tremor. olivary pathway (the Guillain-Mollar-
that it involves muscles or groups of Tremor is characterized by a sinusoi¬ et triangle).4345 If the palatal move¬
muscles supplied by contiguous seg¬ dal oscillatory movement, produced ment is unilateral, the lesion is usual¬
ments of the spinal cord or brain stem by synchronous or alternating con¬ ly in the ipsilateral dentate nucleus or
without accompanying EEG dis¬ tractions of reciprocally innervated the contralateral central tegmental
charges.2·3·8·1314 In contrast, cortical antagonist muscles.27 In contrast, tract. The movement consists of
myoclonus is usually multifocal and rhythmic myoclonus has a square rhythmic contractions of the soft pal¬
affects predominantly distal muscles. wave configuration with a pause ate and pharynx at 2.5 Hz (range, 0.5
It occurs spontaneously, in response between each contraction. The con¬ to 5 Hz). The contractions persist dur¬
to a variety of afferent inputs (corti¬ tractions in segmental myoclonus ing sleep,46 but a disappearance during
cal reflex myoclonus), during move¬ involve the agonist muscles only or sleep has been reported.47·48 While the
ment (cortical action myoclonus), or both agonist and antagonist muscles rate is usually constant, the move¬
as a manifestation of an epileptic dis¬ simultaneously. The persistence of the ment may be voluntarily suppressed,
charge (epilepsia partialis continua, rhythmical movement during sleep triggered reflexively by specific stim¬
simple motor seizures).710 Subcortical and the improvement with serotoner- uli and modified or interrupted by
myoclonus is usually generalized and gic drugs are characteristics of seg¬ oral-pharyngeal maneuvers and limb
it probably originates in the brain mentai myoclonus that further differ¬ positions.49 Olivary enlargement, of¬
stem reticular formation (reticular entiate it from tremor. The only other ten asymmetrical, may be evident on
myoclonus). It is either spontaneous, involuntary movements occurring MRI scan.50 Palatal myoclonus has
reflexive (reticular reflex myoclonus), during sleep, besides segmental myo¬ been attributed to a neuronal cyto¬
or precipitated by fast, voluntary clonus and the cardiorespiratory plasmic vacuolation and astrocytic
movement (ballistic overflow myoclo¬ movements, are nystagmus, tics, fas¬ hyperplasia due to a transsynaptic
nus and oscillatory myoclonus).57 ciculations, seizures, and periodic leg degeneration from a lesion in the
Oscillatory myoclonus occurs also at movements.22·2830 afferent fibers to the inferior olive.44
rest and is distinguished from the Heilman31 introduced the term However, not all patients with palatal
other forms of subcortical myoclonus orthostatic tremor to describe rapid, myoclonus exhibit olivary hypertro¬
by paroxysmal, short (1- to 60-s) irregular, and asynchronous move¬ phy, and vice versa.
bursts of 3- to 6-Hz alternating activ¬ ments primarily involving the legs The most common cause of palatal
ity in antagonist muscles.15 Opsoclo¬ and trunk, occurring while standing. myoclonus is brain-stem infarction,
nus-myoclonus, a syndrome consist¬ This disorder responded to clonaze¬ but the condition has been described
ing of rapid, chaotic, involuntary eye pam therapy and was associated with also in multiple sclerosis, trauma,
movements, and generalized myoclo¬ loss of extensor tone in the legs (nega¬ tumor, brain-stem angioma, progres¬
nus may be associated with neuro¬ tive myoclonus) in one patient. Since sive bulbar palsy, encephalitis, hem¬
blastoma, other tremors, or viral some of our patients with leg myoclo¬ orrhage, syringobulbia, and Meige's
infections.16·17 Segmental myoclonus nus had similar clinical features, this syndrome.16·46·51·52 While the MRI scan
should be differentiated from fascicu¬ orthostatic tremor may, in some often shows the cause, the BAEP is
lations, minipolymyoclonus, and spon¬ patients, represent a form of spinal usually normal.53
taneous myoclonus associated with myoclonus.32 Palatal myoclonus may be associ¬
chronic spinal muscular atrophy.1819 Hemifacial spasm is another form ated with ataxia, synchronous eye
Segmental myoclonus is often mis- of segmental myoclonus. While movements, blepharospasm, and con¬
diagnosed as one of the other hyperki- ephaptic transmission between adja¬ tractions of larynx, neck, diaphragm,
netic movement disorders or as a pul¬ cent axons of the facial nerve has been trunk, and limbs.49·50·5458 Palatal myo¬
satile mass.20·21 Tics, in contrast to implicated in the pathophysiology of clonus has been also described with
myoclonus, consist of more coordi¬ this movement disorder, electrophysi¬ "myorhythmia," which consists of 2 to
nated movements. They can be par¬ ologic recordings have shown that the 3 Hz of rhythmic alternating move¬
tially controlled by an effort of will, facial motor nucleus may be involved ments in the limbs, face, head, jaw,
are usually accompanied by more in hemifacial spasm.33·34 Because of its neck, tongue, eyes, or trunk.59 Patient
complex movements or vocalizations, distinct and easily recognizable char¬ 15, with CNS Whipple's disease, devel¬
and are associated with an urge to acteristics, patients with hemifacial oped 1- to 2-Hz rhythmical move¬
move.22 Like segmental myoclonus, spasm were not included in this ments of the face and platysma.
tics may occur during sleep. The main study. Similar movements named "oculo-
distinguishing feature of chorea is the Besides hemifacial spasm, there are masticatory myorhythmia," have

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 been described in other patients with bursts of the myoclonus, and the activity in the ipsilateral spinal seg¬
CNS Whipple's disease.60 The myo¬ somatosensory evoked potentials were ments adjacent to the deafferented
rhythmia probably represents a form normal.79 The mechanism of the seg¬ dorsal horn.8285 This segmental reflex
of segmental myoclonus, a more mentai myoclonus in these patients is hyperactivity and spontaneous neuro¬
appropriate term for these rhythmi¬ unclear, but the rhythmical contrac¬ nal "burst" firing in the deafferented
cal movements. tions may be an expression of sponta¬ cord segments is presumably due to
Spinal myoclonus consists of invol¬ neous spinal neuronal discharges due some disturbance of normal inhibito¬
untary, rhythmic contractions of mus¬ to a disinhibition from a supraspinal ry interneuronal function. Some
cle groups innervated by a limited control. investigators have also postulated pri¬
spinal cord region.4·13-61 The reported The spinal myoclonus has a fre¬ mary overactivity of the 7-motor neu¬
causes of spinal myoclonus include quency of 1 to 3 Hz (range, 0.2 to 8 Hz), rons.86·87 These studies suggest that
spinal tumor,17·62 vacuolar degenera¬ and the contractions may be increased segmental myoclonus arises from
tion of anterior horn cells,63 spinal in certain positions, with fatigue, and abnormal discharges in the interneu-
root lesion,64 myelitis,65·66 spinal anes¬ with emotional stress.13 They are usu¬ rons and the motor neurons due to a
thesia,67 penicillin,68·69 arteriovenous ally unaffected by sensory stimuli or disturbance in the normal segmental
malformation,70 herpes zoster and motor activity, but stimulus-sensitive or suprasegmental inhibition.
other infections,1·7173 meningomyelo- spinal myoclonus has been reported.80 Although the underlying biochemi¬
cele,74 injury to the spine or the bran¬ The contractions usually cease with cal mechanisms are unknown, seg¬
chial plexus,75-76 spondylosis, and vari¬ general or spinal anesthesia, but they mentai myoclonus is remarkably sen¬
ous neurodegenerative pro- may persist during sleep. The EEG is sitive to medical therapy. Therapy
cesses.1'3·4·13·17,77'78 In our series, the usually normal.77 with clonazepam,88 valproate,89'91 car¬
causes of spinal myoclonus were quite The pathophysiology of segmental bamazepine,92·93 L-5-hydroxy-trypto-
diverse (Table 2). Besides the reported myoclonus is unknown. Rhythmical phan,72·94·95 and tetrabenazine1·11 have
causes, there were some other previ¬ involuntary contractions showing been reported to control the myoclonic
ously unrecognized causes including many of the characteristics of seg¬ contractions. Therapy with baclofen
thoracic sympathectomy, electrical mentai myoclonus have been produced may improve nocturnal leg myoclo¬
injury, laparotomy, pseudomeningo- experimentally by inoculation of the nus96 and cranial cervical myoclonus.97
cele, poliomyelitis, AIDS-related my¬ Newcastle disease virus in cats.81 The In this study, we found clonazepam
elopathy, syringomyelia, oat cell car¬ virus caused either generalized or seg¬ and tetrabenazine to be the most
cinoma, and breast cancer. mentai myoclonus depending on the effective agents in the treatment of
In three patients with branchial route of inoculation. Inoculation of segmental myoclonus. Other drugs
myoclonus and in three patients with the brain stem or the spinal cord useful in some patients include trihex-
spinal myoclonus, the involuntary produced rhythmic, bilaterally syn¬ yphenidyl, reserpine, and L-DOPA.
movement was the presenting symp¬ chronous 0.7- to 2-Hz contractions in Segmental myoclonus is an uncom¬
tom of an underlying serious disease the affected segments. The myoclonus mon, often misdiagnosed, and some¬
as follows: stroke, Whipple's disease, remained confined to the affected seg¬ times disabling movement disorder.
Meige's syndrome, breast cancer, and ments or spread gradually to the con¬ The recognition of segmental myoclo¬
demyeiinating disease. tiguous segments. It was unaffected nus is important because it may be the
In another three patients (two with after surgical isolation from the high¬ presenting symptom of an occult, and
spinal and one with branchial myoclo¬ er segments, or by brain-stem or spi¬ potentially serious, underlying disor¬
nus) the segmental myoclonus was nal cord transection, indicating spinal der. Medical therapy, while empiric,
associated with a suprasegmental cord origin. Semirhythmic segmental provides satisfactory control of symp¬
lesion (patients 18, 22, and 33). The myoclonus has been also produced by toms in most patients.
myoclonus in these patients did not fit direct application of penicillin to the
the clinical or electrophysiologic cri¬ spinal cords of cats and rats.6869
teria for cortical or reticular myoclo¬ Deafferentation of the spinal cord J. P. Glass, MD, University of Texas MD
in cats or humans, by dorsal rhizoto- Anderson Hospital and Tumor Institute at Hous¬
nus. The myoclonus was not stimulus
ton and the University of Texas Health Science
sensitive, there were no EEG dis¬ my or hemicordotomy, causes an Center, Houston, kindly referred patient 23. J.
charges associated with the EMG increase in the spontaneous neuronal Killian, MD, performed the EMG studies.
References
1. Hoehn MM, Cherington M: Spinal myoclo- Reticular reflex myoclonus: A physiological type patients with acquired immune deficiency syn-
nus. Neurology 1977;27:942-946. of human posthypoxic myoclonus. J Neurol Neu- drome (AIDS). Neurology, in press.
2. Marsden CD, Hallett M, Fahn S: The nosol- rosurg Psychiatry 1977;40:253-264. 13. Aigner BR, Mulder DW: Myoclonus: Clini-
ogy and pathophysiology of myoclonus, in Mars- 7. Hallett M, Chadwick D, Marsden CD: Corti- cal significance and an approach to classifica-
den CD, Fahn S (eds): Movement Disorders. cal reflex myoclonus. Neurology 1979;29:1107\x=req-\ tion. Arch Neurol 1960;2:600-615.
Stoneham, Mass, Butterworth Publishers Inc, 1125. 14. Swanson PD, Luttrell CN, Magladery JW:
1982, pp 196-248. 8. Halliday AM: The neurophysiology of myo- Myoclonus: A report of 67 cases and review of the
3. Fahn S, Marsden CD, Van Woert MH: Defi- clonic jerking: A reappraisal, in Charlton MM literature. Medicine 1962;41:339-356.
nition and classification of myoclonus, in Fahn S, (ed): Myoclonic Seizures. Princeton, NJ, Excerp- 15. Obeso JA, Lang AE, Rothwell JC, et al:
Marsden CD, Van Woert MH (eds): Advances in ta Medica, 1975, pp 1-29. Postanoxic symptomatic oscillatory myoclonus.
Neurology. New York, Raven Press, 1986, vol 43, 9. Kinsbourne M, Rosenfield DB: Nonprogres- Neurology 1983;33:240-243.
Definition and Classification of Myoclonus, pp sive myoclonus, in Charlton MH (ed): Myoclonic 16. Kuban KC, Ephros MA, Freeman RL, et al:
1-5. Seizures. Princeton, NJ, Excerpta Medica, 1975, Syndrome of opsoclonus-myoclonus caused by
4. Silfverskiold BP: Rhythmic myoclonias pp 30-59. Coxsackie B3 injection. Ann Neurol 1983;13:69\x=req-\
including spinal myoclonus, in Fahn S, Marsden 10. Obeso JA, Rothwell JC, Marsden CD: The 71.
CD, Van Woert MH (eds): Advances in Neurolo- spectrum of cortical myoclonus. Brain 1985; 17. Dropcho E, Payne R: Paraneoplastic opso-
gy. New York, Raven Press, 1986, vol 43: Myoclo- 108:193-124. clonus-myoclonus: Association with medullary
nus, pp 243-253. 11. Jankovic J: Treatment of hyperkinetic thyroid carcinoma and review of the literature.
5. Hallett M, Chadwick D, Marsden CD: Ballis- movement disorders with tetrabenazine: Double- Arch Neurol 1986;43:410-415.
tic movement overflow myoclonus: A form of blind crossover study. Ann Neurol 1982;11:41\x=req-\ 18. Frenken CWGH, Korten JJ, Gabreels FJM,
essential myoclonus. Brain 1977;100:299-312. 47. et al: Spinal myoclonus. Clin Neurol Neurosurg
6. Hallett M, Chadwick D, Adam J, et al: 12. Nath A, Jankovic J: Movement disorders in 1974;77:44-53.

Downloaded From: http://archneur.jamanetwork.com/ by a University of British Columbia Library User on 06/18/2015

 19. Jankovic J, Rivera VM: Hereditary myoclo- 46. Herrmann C, Brown JW: Palatal myoclo- 73. Baruah JK: Spinal myoclonus: A case
nus and progressive distal muscular atrophy. nus: A reappraisal. J Neurol Sci 1967;5:473-492. report. J Bone Joint Surg Am 1984;66A:304-305.
Ann Neurol 1979;6:227-231. 47. Jacobs L, Newman RP, Bozian JD: Disap- 74. Snyder RD, Appenzeller O: Segmental
20. Marsden CD: The pathophysiology of pearing palatal myoclonus. Neurology 1981; myoclonus in meningomyelocele.
Trans Am Neu-
movement disorders, in Jankovic J (ed): Neuro- 31:748-751. rol Assoc 1971;96:97-100.
logic Clinics. Philadelphia, WB Saunders Co, 48. Tanaka M, Suzuki M, Kawarabayashi I, et 75. Short MP, Banks G, Nielsen VK, et al:
1984, vol 2, pp 435-460. al: Palatal myoclonus disappears in sleep. Neu- Brachial plexus myoclonus. Neurology 1984;
21. Gehris CW, Hendler N: Myoclonus presen- rology 1984;34:406-407. 34(suppl 1):226.
ting as a pulsatile neck mass. Trans Am Acad 49. Soso MJ, Nielen VK, Jannetta PJ: Palatal 76. Wooten GF, Bennett JP: Isolated branchial
Ophthalmol Otolaryngol 1977;84:129-131. myoclonus: Reflex activation of contractions. myoclonus secondary to paraneoplastic encepha-
22. Jankovic J, Fahn S: The phenomenology of Arch Neurol 1984;41:866-869. lopathy. Ann Neurol 1984;16:133.
tics. Move Disorder 1986;1:17-26. 50. Sperling MR, Herrmann C Jr: Syndrome of 77. Hopkins AP, Michael WF: Spinal myoclo-
23. Obeso JA, Rothwell JC, Lange AE, et al: palatal myoclonus and progressive ataxia. Neu- nus. J Neurol Neurosurg Psychiatry 1974;
Myoclonic dystonia. Neurology 1983;33:825-830. rology 1985;35:1212-1214. 37:1112-1115.
24. Fahn S: The varied clinical expressions of 51. Jankovic J: Blepharospasm associated with 78. Daniel DG, Webster DC: Spinal segmental
dystonia, in Jankovic J (ed): Neurologic Clinics. palatal myoclonus and communicating hydro- myoclonus: Successful treatment with cervical
Philadelphia, WB Saunders Co, 1984, vol 2, pp cephalus: A reply. Neurology 1984;34:1522-1524. spinal decompression. Arch Neurol 1984;41:898\x=req-\
541-555. 52. Jankovic J: Cranial-cervical dyskinesias, in 899.
25. Feinberg TE, Shapiro AK, Shapiro E: Pa- Appel SH (ed): Current Neurology. Chicago, Year 79. Rothwell JC, Obeso JA, Marsden CD: On
roxysmal myoclonic dystonia with vocalizations: Book Medical Publishers, 1986, vol 6, pp 153\x=req-\ the significance of giant somatosensory-evoked
New entity or variant of preexisting syndromes. 176. potentials in cortical myoclonus. J Neurol Neu-
J Neurol Neurosurg Psychiatry 1986;49:52-57. 53. Westmoreland BF, Sharbrough W, Stock- rosurg Psychiatry 1984;47:33-42.
26. Kurlan R, Behr J, Miller C, et al: Inherited ard JJ, et al: Brain-stem auditory evoked poten- 80. Davis SM, Murray NMF, Diengdoh JV, et
myoclonic dystonia. Ann Neurol 1985;18:162\x=req-\ tials in 20 patients with palatal myoclonus. Arch al: Stimulus-sensitive spinal myoclonus. J Neu-
163. Neurol 1983;40:155-158. rol Neurosurg Psychiatry 1981;44:884-888.
27. Quinn NP, Marsden CD: Myoclonic dyston- 54. Nagaoka M, Narabayashi M: Palatal myo- 81. Luttrell CN, Bang FB, Luxenberg K: New-
ia, in Fahn S, Calne DB, Marsden CD (eds): clonus: Its remote influence. J Neurol Neurosurg castle disease encephalomyelitis in cats: II. Phys-
Dystonia. New York, Raven Press, in press. Psychiatry 1984;47:921-926. iological studies on rhythmnic myoclonus. Arch
28. Jankovic J, Fahn S: Physiologic and patho- 55. Phillips JR, Eldridge FL: Respiratory myo- Neurol Psychiatry 1959;81:285-291.
logic tremors. Ann Intern Med 1980;93:460-465. clonus (Leeuwenhoek's disease). N Engl J Med 82. Eccles JC, McIntyre AK: The effects of
29. Montplaisir J, Godbout R, Boghen D, et al: 1973;289:1390-1395. disuse and of activity on mammalian spinal
Familial restless legs with periodic movement in 56. Nakada T, Kwee IL: Oculopalatal myolo- reflexes. J Physiol 1953;121:492-516.
sleep: Electrophysiologic, biochemical, and phar- nus. Brain 1986;109:431-441. 83. Eccles JC, Eccles RM, Shealy CN: An inves-
macologic study. Neurology 1985;35:130-134. 57. Keane JR: Acute vertical ocular myoclo- tigation into the effect of degenerating primary
30. Ohanna N, Peled R, Rubin A-HE, et al: nus. Neurology 1986;36:86-89. afferent fibers on the monosynaptic innervation
Periodic leg movements in sleep: Effect of clon- 58. Zegers Beyl D, Flament-Durand J, Boren- of motoneurons. J Neurophysiol 1962;25:544\x=req-\
azepam treatment. Neurology 1985;35:408-411. stein JS, et al: Occular bobbing and myoclonus in 559.
31. Heilman KM: Orthostatic tremor. Arch central pontine myelinolysis. J Neurol Neuro- 84. Loeser JD, Ward AA: Some effects of deaf-
Neurol 1984;41:880-881. surg Psychiatry 1983;46:564-565. ferentation on neurons of the cat spinal cord.
32. Young RR: Movement disorders and objec- 59. Masucci EF, Kurtze JF, Saini N: Myo- Arch Neurol 1967;17:629-636.
tive measurements. Arch Neurol 1985;42:932. rhythmia: A widespread movement disorder: 85. Loeser JD, Ward AA, White LE: Chronic
33. Moller AR, Jannetta PJ: Hemifacial Clinicopathological correlations. Brain 1984; deafferentation of human spinal cord neurons.
spasm: Results of electrophysiologic recording 107:53-79. J Neurosurg 1968;29:48-50.
during microvascular decompression operations. 60. Schwartz MA, Harris JK, Campbell WW, 86. Eccles RM, Kozar W, Westerman RA:
Neurology 1985;35:969-974. et al: Oculomasticatory myorhythmia: A sign of Enhancement of spinal monosynaptic reflex
34. Esteban A, Molina-Negro P: Primary hem- Whipple's disease. Ann Neurol 1985;18:135-136. responses after denervation of synergic hind
ifacial spasm: A neurophysiological study. 61. Nohl M, Doose H, Gross-Selbeck G, et al: limb muscles. Exp Neurol 1962;6:451-464.
J Neurol Neurosurg Psychiatry 1986;49:58-63. Spinal myoclonus. Eur Neurol 1978;17:129-135. 87. Eadie MJ: Electrophysiological observa-
35. Schoenen J, Gonce M, Delwaide PJ: Painful 62. Garcin R, Rondot P, Guiot G: Rhythmic tions in a patient with segmental myoclonus.
legs and moving toes: A syndrome with different myoclonus of the right arm as presenting symp- Proc Aust Assoc Neurol 1969;6:65-69.
physiopathologic mechanisms: Neurology 1984; tom of cervical cord tumor. Brain 1968;91:75-84. 88. Gledhill RF, Wiles CM: Clonazepam and
38:1108-1112. 63. Shivapour E, Teasdall RD: Spinal myoclo- branchial myoclonus. Ann Neurol 1977;1:306\x=req-\
36. Verhagen WIM, Horstink MWIM, Noter- nus with vacuolar degeneration of anterior horn 307.
mans SLH: Painful arm and moving fingers. cells. Arch Neurol 1980;37:451-453. 89. Rollinson RD, Gilligan BS: Postanoxic
J Neurol Neurosurg Psychiatry 1985;48:384-389. 64. Sotaniemi KA: Paraspinal myoclonus due action myoclonus (Lance-Adams syndrome)
37. Adams JA: Feedback theory of how joint to spinal root lesion. J Neurol Neurosurg Psychi- responding to valproate. Arch Neurol 1979;36:44\x=req-\
receptors regulate the timing and positioning of atry 1985;48:722-723. 45.
a limb. Psychol Revue 1977;84:540-523. 65. Campbell AMG, Garlan H: Subacute myo- 90. Fahn S: Posthypoxic action myoclonus:
38. Tatton WG, Bruce IC: Comment: A schema clonic spinal neuronitis. J Neurol Neurosurg Review of the literature and report of two new
for the interaction between motor programs and Psychiatry 1956;19:268-274. cases with response to valproate and estrogen, in
sensory input. Can J Physiol Pharmacol 1981; 66. Koike H, Yoshino Y: Spinal myoclonus Fahn S, Davis N, Rowland LP (eds): Advances in
59:691-699. with dermal and retinal changes affected by Neurology. New York, Raven Press, 1984, vol 25:
39. Steiner JL, DeJesus PV, Mancall ER: Pain- myelitis. Arch Neurol 1977;34:383-385. Cerebral Hypoxia and Its Consequences, pp 49\x=req-\
ful jumping amputation stumps: Pathophysiolo- 67. Fox EJ, Villanueva R, Schutta HS: Myoclo- 84.
gy of 'sore circuit.' Trans Am Neurol Assoc nus following spinal anesthesia. Neurology 1979; 91. Lefkowitz D, Harpold G: Treatment of
1974;99:253-255. 29:379-380. ocular myoclonus with valproic acid. Ann Neurol
40. Jankovic J, Glass JP: Metoclopramide- 68. Lothman EW, Somjen GG: Motor and elec- 1985;17:103-104.
induced phantom dyskinesia. Neurology 1985; trical signs of epileptiform activity induced by 92. Rahko T, H'akkinen V: Carbamazepine in
35:432-435. penicillin in the spinal cord of decapitated cats. the treatment of objective myoclonus tinnitus.
41. Marsden CD, Obeso JA, Traub MM, et al: Electroencephalogr Clin Neurophysiol 1976; J Laryngol Otol 1979;9:123-127.
Muscle spasms associated with Sudeck's atrophy 41:237-252. 93. Sakai T, Shiraishi S, Murakiami S: Palatal
after injury. Br Med J 1984;288:173-176. 69. Kao LI, Crill WE: Penicillin-induced seg- myoclonus responding to carbamazepine. Ann
42. Schott GD: The relationship of peripheral mental myoclonus: I. Motor responses and intra- Neurol 1983;9:199-200.
trauma and pain to dystonia. J Neurol Neuro- cellular recording from motoneurons. Arch Neu- 94. Magnussen I, Dupont E, Prange-Hansen A,
surg Psychiatry 1985;48:698-701. rol 1980;37:451-453. et al: Palatalmyoclonus treated with 5-hydroxy-$
43. Guillain G: The syndrome ofsynchronous 70. Levy R, Plassche W, Riggs J, et al: Spinal tryptophan and a decarboxylase-inhibitor. Acta
and rhythmic palato-pharyngo-laryngo-oculo-$ myoclonus related to an arteriovenous malfor- Neurol Scand 1977;55:251-253.
diaphragmatic myoclonus. Proc R Soc Med 1938; mation: Response to clonazepam therapy. Arch 95. Van Woert MH: Myoclonus and L-5\x=req-\
31:1030-1038. Neurol 1983;40:254-255. hydroxytryptophan (L-5HTP), Prog Clin Biol Res
44. Lapresle J: Palatal myoclonus, in Fahn S, 71. Dhaliwal GS, McGreal DA: Spinal myoclo- 1983;127:43-52.
Marsden CD, Van Woert MH (eds): Advances in nus in association with herpes zoster infection. 96. Guilleminault C, Flagg W: Effect of baclo-
Neurology. New York, Raven Press, 1986, vol 43: Can J Neurol Sci 1974;239-241. fen on sleep-related periodic leg movements. Ann
Myoclonus, pp 265-273. 72. Williams A, Goodenberger D, Calne DB: Neurol 1984;15:234-239.
45. Matsuo F, Ajax ET: Palatal myoclonus and Palatal myoclonus following herpes zoster ame- 97. Meldrum BS: 7-Aminobutyric acid and the
denervation supersensitivity in the central ner- liorated by 5-hydroxytryptophan and carbidopa. search for new anticonvulsant drugs. Lancet
vous system. Ann Neurol 1979;5:72-78. Neurology 1978;28:358-359. 1978;2:304-306.

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