Myoclonus occurs as a result of excessive discharge from a

group of neuronsthen spreads up and down the neuraxis through

1. rapidly conducting pathways as in cortical reflex myoclonus
(Hallett et al 1979)
2. or slowly conducting pathways as in propriospinal myoclonus
(Brown et al 1991).
The spread is usually so fast that the details cannot be appreciated
by the naked eye, necessitating neurophysiologic studies.
Drugs inducing myoclonus include
1. anticonvulsants, Newer AED such as lamotrigine (Janszky et al
2000) and gabapentin (Asconape et al 2000; Holtkamp et al
2006),
2. levodopa, Amantadine has been reported to cause cortical
myoclonus (Matsunaga 2001), and selegiline
3. Tricyclic antidepressants may cause an encephalopathy and
myoclonus with EEG changes that may be confused with
Creutzfeldt-Jakob disease (Foerstl et al 1989).
4. lithium multifocal action myoclonus of cortical origin without
epileptiform abnormalities on routine EEG at toxic and
therapeutic doses
5. Fentanyl
6. Propofoltransient cortical reflex myoclonus that requires no
treatment (Dearlove and Dearlove 2002; Nimmaanrat 2005).
7. Mefloquine, used in chloroquine-resistant P falciparum malaria,
caused multifocal myoclonus in one patient. (Jimenez-Huete et
al 2002).
8. Tardive myoclonus has been described following exposure to
long-term neuroleptics (Little and Jankovic 1987).
9. carvedilol (a beta-blocker) (Fernandez and Friedman 1999)
10.
and antibiotics such as gatifloxacin (Marinella 2001) have
been reported to induce myoclonus.
Differential diagnosis
The differential diagnosis of an isolated myoclonic jerk includes
1. chorea
2. and tic.
3. The term myoclonic dystonia refers to a combination of
myoclonus and dystonia in other muscles (Obeso et al 1983).

4. Terminal kinetic tremor and cerebellar ataxia may be severe

enough to be confused with myoclonus. The two may coexist as
in progressive myoclonic ataxia, and only after the treatment of
myoclonus can the true severity of the underlying ataxia be
appreciated.
5. Some patients with postural tremor may have changes in
amplitude, giving the impression of myoclonus, but rarely do
these two conditions coexist
Isolated choreic jerk choreic jerk is
1. not stimulus sensitive
2. and tends to be not as rapid as myoclonus.
3. In its fully developed form, chorea results in continuous
random, flowing, quick, and arrhythmic movements.
4. Further, motor impersistence occurs such as inability to keep
the tongue protruded and waxing and waning of the grip
strength (milkmaid's grip).
Tic (simple or complex).
In contrast to myoclonus, tics may be
- preceded by premonitory sensations,
- and their execution may be delayed or suppressed.
- The voluntary suppression results in a buildup of inner tension
that is relieved by the execution of the movement.
.
Management
1. Metabolic derangements or other underlying conditions, if
found, need to be treated.
2. symptomatic control of myoclonus.
- The most effective drugs are clonazepam (4 mg to 10 mg
per day),
- sodium valproate (250 mg to 4500 mg per day),
- and piracetam (10 g to 24 g per day).
- Somewhat less effective drugs include lisuride,
acetazolamide, and carbamazepine (Obeso et al 1989).
3. In Lance Adams syndrome, a combination of
- 5-hydroxytryptophan (600 mg to 2000 mg per day)
- and carbidopa (100 mg to 200 mg per day) can be effective
(Van Woert et al 1977).
- Recently, levetiracetam has been reported to alleviate

1. posthypoxic,
2. spinal,
3. and postencephalitic myoclonus (Genton and Gelisse
2000; Krauss et al 2001; Keswani et al 2002).
Progressive myoclonic epilepsy may be worsened by phenytoin
(Eldridge et al 1983).
Acetazolamide may be helpful in myoclonus of Ramsay Hunt
syndrome.
Essential myoclonus
- often responds to alcohol
- and may be improved by anticholinergics (Chokroverty et al
1987).
Palatal myoclonus is usually resistant to therapy, but in some
cases,
1. anticholinergics (Jabbari et al 1989),
2. 5-hydroxytryptophan (Magnussen et al 1977),
3. and carbamazepine (Sakai et al 1981) have been helpful.
Spinal myoclonus may respond to
1. removal of compressing lesion (Daniel and Webster 1984)
2. and effective drugs have included clonazepam and
tetrabenazine (Jankovic and Pardo 1986) 4limbs=tetrabenaz.
Negative myoclonus
1. often resolves with the correction of the responsible metabolic
derangement,
2. and ethosuximide may be particularly useful in the symptomatic
treatment (Shirasaka and Mitsuyoshi 1999). Several data have
demonstrated the effectiveness of ethosuximide in the
treatment of epileptic negative myoclonus in children suffering
from idiopathic partial epilepsy (Oguni et al 1998; Capovilla et al
1999; 2000; Shirasaka and Mitsuyoshi 1999; Kubota et al
2005). These evidences have suggested that this drug,
specifically acting as T-type Ca2+ blocker on thalamic neurons
and related cortical assemblies, may modulate at the thalamo-

cortical level the physiopathogenic mechanisms possibly

involved in the genesis of epileptic negative myoclonus.
3. Another drug that has been recently reported to control epileptic
negative myoclonus is levetiracetam, however, more extensive
studies are needed to confirm these preliminary findings
(Gelisse et al 2003).
4. Epileptic negative myoclonus associated with symptomatic or
cryptogenic epilepsies is usually less responsive to common
antiepileptic treatments.
Several data have demonstrated the induction or worsening of
epileptic negative myoclonus by
1. carbamazepine in children suffering from partial epilepsies
(Caraballo et al 1989;(Nanba e Maegaki 1999; Shirasaka and
Mitsuyoshi 1999; Parmeggiani et al 2004).
2. valproic acid (Aguglia et al 1995),
3. phenytoin (Young and Shahani 1986; Chi et al 2000),
4. lamotrigine (Cerminara et al 2004)
5. and oxcarbazepine (Hahn et al 2004).
In uncontrolled studies, sodium oxybate improved posthypoxic
myoclonus and myoclonus-dystonia (Frucht et al 2005).
In addition, 1 patient with myoclonus-dystonia syndrome improved
about 80% with neurostimulation of the ventral intermediate thalamic
nucleus without any significant change in the dystonic symptoms
(Kupsch 2001).
Finally, another patient showed significant improvement in myoclonus
and dystonia after medial pallidum stimulation (Liu 2002).
opsoclonus-myoclonus.
1. Conventional management consists of ACTH or corticosteroids
2. though some concomitant treatment with PE (Sheela and
Mani 2003) or IVIG (Aysun et al 2004).
3. Rituximab, a monoclonal antibody directed to CD20, targets
mature B cells for apoptosis and has been reported to improve
opsoclonus-myoclonus (Pranzatelli et al 2005).