TREMORS Flashcards
________may be defined as involuntary rhythmic oscillatory movement produced by alternating or irregularly synchronous contractions of reciprocally innervated
muscles
Tremor
embedded i n the motor system.
It is present in all contracting muscle groups and persists throughout the waking state and even in certain phases of sleep
Physiologic Trem o r
Frequency of physiologic tremor
It ranges in frequency between 8 and
13 Hz, the dominant rate being 10 Hz in adulthood and
somewhat less in childhood and old age
Action tremors can be conveniently
divided into two categories: ________action
tremor of the ataxic type related to cerebellar disorders
and ________, which are
either the enhanced physiologic variety or essential
tremor
goal directed
postural tremors
the tremor is absent when the limbs are relaxed but becomes evident when the muscles are activated
postural tremors
Most cases of __________
are characterized by relatively rhythmic bursts of grouped motor neuron discharges that occur not quite synchronously in opposing muscle groups
action tremor
This, the commonest type o f tremor, i s o f lower frequency (4 to 8 Hz) than physiologic tremor and is unassociated with other neurologic changes;
ET
Like most tremors, essential tremor is worsened by ________________
emotion, exercise, and fatigue.
Inheritance of ET
autosomal dominant pattern with high penetrance.
Essential tremor most often makes its appearance
late in the_________ but it may begin in childhood and then persist.
second decade,
in ET..
The tremor practically always begins in the ______
and is usually almost_______ in approximately
________ of patients, however, it may appear first in
the dominant hand.
arms
symmetrical;
15 percent
Most patients with essential tremor will have
identified the amplifying effects of anxiety and the ameliorating
effects of _______ on their tremor.
alcohol
A curious fact about essential tremor o f the typical
(non-alternate-beat) type is that it can be suppressed
by a small amount of alcohol in more than ______percent
of patients
75
essential tremor is
inhibited by the beta-adrenergic antagonist __________ taken orally over a long
period of time.
propranolol
(between 80 and 200 mg per day in divided doses or as
a sustained-release preparation)
The barbiturate drug ________ has also been effective
in controlling essential tremor and may be tried
in patients who do not respond to or cannot tolerate
beta-blocking medications
primidone
Most patients with essential tremor will have
identified the amplifying effects of anxiety and the ameliorating
effects of _______ on their tremor.
alcohol
A curious fact about essential tremor o f the typical
(non-alternate-beat) type is that it can be suppressed
by a small amount of alcohol in more than ______percent
of patients
75
essential tremor is
inhibited by the beta-adrenergic antagonist __________ taken orally over a long
period of time.
propranolol
(between 80 and 200 mg per day in divided doses or as
a sustained-release preparation)
The barbiturate drug ________ has also been effective
in controlling essential tremor and may be tried
in patients who do not respond to or cannot tolerate
beta-blocking medications
The alternate-beat, slow, high-amplitude, kinetic-predominant type of essential tremor is more difficult to suppress but has reportedly responded to_______
primidone
clonazepam
Injections of _________into a portion of a limb
can reduce the severity of essential tremor locally; but the accompanying weakness of arm and hand muscles often proves unacceptable to the patient
botulinum toxin
In resistant cases of essential tremor of the fast or
slow variety, stimulation by electrodes implanted in the
_________ (of the same type used to treat
Parkinson disease) has produced a durable response
over many years; d
ventral medial nucleus thalamus or the internal segment
of the globus pallidus
A coarse action tremor, sometimes combined
with myoclonus, accompanies various types of
_________
meningoencephalitis
EMG of PD Tremor
bursts of activity that alternate between opposing muscle
groups
Parkinsonian tremor is_________ in the sense
that it takes the form of flexion-extension or abductionadduction
of the fingers or the hand; pronation-supination
of the hand and forearm is also a common presentation.
“alternating”
The cogwheel effect, a ratchet-like interruption perceived
by the examiner on passive movement of an
extrernitiy __________
It is said by many authors
to be no more than a palpable tremor superimposed
on rigidity and as such, is not specific for Parkinson
(the Negro sign) .
Cogwheeling can
be brought out by having the patient engage the opposite
limb, such as tracing circles in the air; what sign?
this Froment sign
The tremor of _________ (which is
now virtually extinct) often had greater amplitude and
involved proximal muscles
postencephalitic parkinsonism
Parkinsonian tremor is suppressed to some extent by
the anticholinergic drugs _________________
and other anticholinergic drugs such as ethopropazine,
a phenothizine derivative;
benztropine, trihexyphenidyl,
In PD
Stereotactic lesions or electrical
stimulation in the _______ diminishes or abolishes tremor contralaterally;
other stimulation sites such as the ___________ and the subthalamic nucleus
basal ventrolateral nucleus of the
thalamus
internal segment
of the globus pallidus
Its salient
feature is that it requires for its full expression the performance
of an exacting, precise, projected movement
Ataxic tremor
This is a strongly familial episodic tremor disorder of
the chin and lower lip that begins in childhood and
may worsen with age.
geniospasm
This is a rare but striking tremor isolated to the legs that
is remarkable by its occurrence only during quiet standing
and its cessation almost immediately on walking.
Pri m a ry Orthostatic Trem o r
Tx for primary orthostatic tremor
clonazepam, gabapentin, primidone, or sodium valproate
alone
______ is focal, superimposed, for example on
torticollis, or it may be evident in a hand
Dystonic tremor
usually restricted to a single limb, often in the dominant
hand; they are gross in nature, are less regular than
the common static or action tremors, and diminish in
amplitude or disappear if the patient is distracted as, for example, when asked to make a complex movement with
the opposite hand.
Psych ogenic Trem o r
If the examiner restrains the affected
hand and arm, the tremor may move to a more proximal part of the limb or to another part of the body________
(“chasing
the tremor”)
I n patients with tremor o f either the parkinsonian,
postural, or intention type, Narabayashi has recorded burst in _______
rhythmic burst discharges of unitary cellular activity in
the nucleus intermedius ventralis of the thalamus (as
well as in the medial pallidum and subthalamic nucleus)
in ET, Based on electrophysiologic
recordings in patients, two likely origins of
oscillatory activity are the ______ circuits and the
_____
olivocerebellar and thalamus.
PD lesion
ventromedial tegmentum of the midbrain,
brachium conjunctivum, or a tegmental-thalamic projection, or the
descending limb of the superior cerebellar peduncle
lesion of ataxic tremor
inactivating the
deep cerebellar nuclei or by sectioning the superior cerebellar
peduncle or the brachium conjunctivum below its
decussation.
This i s a rare disorder consisting o f rapid, rhythmic,
involuntary movements of the soft palate.
Pa lata l Trem o r ( “ Pa l ata l Myocl o n u s “ }
2 types of Palatal tremor
One is essential palatal tremor that reflects the rhythmic activation of the _________ it has no known pathologic basis.
second, more common form is a symptomatic palatal
tremor caused by a diverse group of brainstem lesions
that interrupt the ________(s); these columns
contain descending fibers ________________________ (a component of the GuillainMollaret
triangle
tensor veli palatini muscles;
central tegmental tract
from midbrain nuclei to the inferior olivary complex
Tx of palatal myoclonus
Clonazepam (0.25 to 0.5
mg/ d, increasing gradually to 3.0 to 6.0 mg/d), sodium
valproate (250 mg/ d, increasing to 1 ,000 mg/ d), and gabapentin
(up to 2,100 mg)
It consists of arrhythmic lapses of sustained
posture that allow gravity or the inherent elasticity of
muscles to produce a movement, which the patient then
corrects, sometimes with overshoot.
asterixis
EEG and EMG of Asterixis
that a sharp wave, probably generated
in the motor cortex, immediately precedes the period of
EMG silence
Asterixis may also be evoked by _______ and other
anticonvulsants, usually indicating that these drugs are
present in excessive concentrations
phenytoin
unilateral asterixis lesion
frontal lobe (anterior cerebral artery infarction), midbrain, and cerebellum
_______series of rhythmic, monophasic (i.e., unidirectional) contractions and relaxations of a group of muscles, differing in this way from ________ which are always diphasic or bidirectional.
myoclonus
tremors,
A common and benign example of myoclonus, familiar
to many persons, is the _________ that consists of a
jerking of the body, particularly the torso, while falling
asleep or occasionally, just prior to waking
“sleep-start”
One-sided or focal myoclonic
jerks are the dominant feature of a particular form
of childhood epilepsy-so-called_________
benign epilepsy with
rolandic spikes
__________is also one of the notable features
of degenerative neurologic conditions, particularly
corticobasal ganglionic degeneration; it is generally seen
in a limb that is made rigid by this process
Focal simple myoclonus
A more serious
type of myoclonic epilepsy, which in the beginning
may be marked by polymyoclonus as an isolated phenomenon,
is eventually associated with dementia and
other signs of progressive neurologic disease
familial
variety of Unverricht and Lundborg
Another form of stimulus-sensitive (reflex) myoclonus,
inherited as an autosomal recessive trait, begins in
late childhood or adolescence and is associated with neuronal
inclusions ______________
in the cerebral and cerebellar cortex and in brainstem
nuclei.
(Lafora bodies thus Lafora-body disease)
the __________ has a favorable prognosis,
particularly if the seizures are treated with valproic acid
Baltic
variety of myoclonic epilepsy
diffuse, incapacitating
intention myoclonus associated with visual loss
and ataxia.
cherry-red-spot myoclonus
In cherry-red-spot myoclonus,
The specific enzyme
defect appears to be a deficiency of ______________ resulting in the excretion of large
amounts of sialylated oligosaccharides in the urine
lysosomal alpha-neuroaminidase
(sialidase),
similar clinical syndrome of myoclonic epilepsy is seen in a variant form of neuroaxonal dystrophy and in the late childhood-early adult neuronopathic form of Gaucher disease, in which it is associated with _______
supranuclear gaze palsies and cerebellar
ataxia
subacute encephalopathy with diffuse
myoclonus may occur in association with the autoantibodies
that are characteristically a component of
_________ and _________
Hashimoto thyroiditis and also in Whipple disease
An acute onset of polymyoclonus
with confusion occurs with ________ once
ingestion is discontinued, there is improvement (slowly
over days to weeks) and the myoclonus is replaced by
diffuse action tremors,
lithium intoxication;
Diffuse,
severe myoclonus may be a prominent feature of early
________
tetanus and strychnine poisoning
by rapidly progressive dementia, disturbances of gait
and coordination, and all manner of mental and visual
aberrations associated with myoclonus
Creutzfeldt-Jakob disease,
pathology of spongiform enceph
parenchymal
destruction, the cortical tissue shows a fine-meshed
vacuolation
Fast
(ballistic) movements, however, especially when directed
to a target, as in touching the examiner’s finger, elicit a
series of irregular myoclonic jerks that differ in speed and
rhythmicity from intention tremor
I ntent i o n or Act i o n ( Posta n ox i c ) Myocl o n u s
Tx for post anoxic myoclonus
Barbiturates and valproic acid, levitiracetam
almost continuous arrhythmic jerking of
a restricted group of muscles, often on one side of the
body.
Spinal or Segmental Myoclonus
the most restricted lesions
associated with myoclonus are located in the __________
cerebellar
cortex, dentate nuclei, and pretectal region.
Aside from exaggerated forms of the normal startle
reflex, the commonest isolated syndrome is so-called startle
disease, also referred to as___________
hyperexplexia or hyperekplexia
The most common mutation is in the 1-subunit of the
inhibitory _________(Shiang et al) but other
glycine receptor related genes have been implicated in
other cases.
glycine receptor GLRA1
diseases where startle can be seen
response may be a manifestation
of other neurologic diseases. It is a prominent feature of
Tay-Sachs disease, SSPE, and of some cases of the “stiffman
syndrome.”
localization of startle
pontine reticular nuclei,
with transmission to the lower brainstem and spinal motor
neurons via the reticulospinal tracts.
treatment of startle
Clonazepam controls the startle disorders t o varying
degrees. Levetiracetam has reportedly been helpful in
some patients
focal or segmental dystonias
intermittent, brief or prolonged spasms or contractions of a group of adjacent muscles that places the body part in a
forced and unnatural position
the laryngeal
muscles may be involved, imparting a high-pitched,
strained quality to the voice
spasmodic dysphonia
This, the most frequent form o f restricted dystonia, is
localized to the neck muscles. It usually begins in early
to middle adult life (peak incidence in the fifth decade),
is somewhat more common in women
Spasm odic Tortico l l is (Id iopath ic
Cervical Dysto n i a )
cause of Idiopathic cerv dystonia
With the exception of the finding of DYTl
gene abnormality in a few patients, it is idiopathic
tremor of dystonia
the tremor tends to beat in the direction of the dystonic
movement
msucles affected by ICD/torticolis
The most prominently affected muscles are the sternocleidomastoid,
levator scapulae, and trapezius
About ________percent of patients with torticollis
also have oral, mandibular, or hand dystonia
15
The periodic ____________injection of small
amounts of botulinum toxin directly into several sites in
the affected muscles is by far the most effective form of
treatment.
(every 3 to 6 months)
All but _________ of patients with torticollis have had some degree of relief
from symptoms with this treatment
10 percent
Adverse effects of botox
(excessive weakness of injected muscles, local pain, and
dysphagia-the latter from a systemic effect of the toxin)
are usually mild and transitory
_____________of
patients eventually become resistant to repeated injections
because of the development of neutralizing antibodies
to the toxin
Five to 10 percent
DBS targets of torticolis
The internal segments of the globus
pallidus and the subthalamic nuclei have been used
as targets.
Intractable torticolis Tx
sectioning
of the spinal accessory nerve and of the first three cervical
motor roots bilaterally has been successful in reducing
spasm of the muscles without totally paralyzing them
Patients in mid and late adult life, predominantly
women, may present with the complaint of inability
to keep their eyes open that is a manifestation of
involuntary closure of the eyelids
blepharospasm
Pathology of blepharospasm
No neuropathologic lesion
or neurochemical profile has been established in any of
these disorders
Tx for blepharo
The most effective treatment consists o f the injection of
botulinum toxin into several sites in the orbicularis oculi
and adjacent facial muscles
Tx for resistant blepharo
Thermolytic destruction
of part of the fibers in the branches of the facial
nerves that innervate the orbicularis oculi muscles is
reserved for the most resistant and disabling cases.
A homolateral blepharospasm has also been observed
with a small __________infarct
thalamomesencephalic
Difficulty in speaking
and swallowing (spastic or spasmodic dysphonia)
and blepharospasm are also frequently conjoined, and
occasionally patients with these disorders
Brueghel syndrome/Meige
A form of focal dystonia that affects only the jaw muscles
has been described____________
(masticatory spasm of Romberg
The performance of other highly skilled motor acts,
such as playing the piano or fingering the violin, may
acquire a similar highly task-dependent spasm (“musician’s
cramp”, now commonly called “_________)
or in the past, __________
musician’s dystonia”
telegrapher ‘s palsy
In monkeys, Byl and colleagues found that sustained,
rapid, and repetitive highly stereotypical movements
greatly ___________ the area of cortical representation
of sensory information from the hand
expand
__________ of the
forearm in 20-minute sessions has a modest effect for Writer’s cramp
Transcutaneous electrical stimulation (TENS)
it refers specifically
to movements induced by the use of neuroleptic
drugs, often, but not always phenothiazines, which are
delayed in onset from the initiation of drug therapy and
persist after the drugs are withdrawn
These movements
are distinguished from acute dystonic reactions that
occur in the first few days of exposure to medications,
are aborted by anticholinergic drugs, ________
D ru g - I n d u ced Ta rd ive ( D e l ayed ) Dys k i n es i
and do not persist
drugs causing tardive dyskinesia
metoclopramide, pimozide, amoxapine,
and clebopride, risperidone
phenomenology of tardive dyskinesia
One highly characteristic pattern
combines retrocollis, backwards arching of the trunk,
internal rotation of the arms, extension of the elbows, and
flexion of the wrists simulating an opisthotonic posture
Blockade and subsequent unmasking of the ________
receptor have been specifically linked to the development of
the tardive syndromes.
D2
Tx of tardive dyskinesia
The dystonias also
respond to anticholinergic drugs (trihexyphenidyl 2.5 mg
once or twice daily, increased by small increments weekly
up to 12.5 mg) if high enough doses can be tolerated.
Stereotypy and irresistibility are the main identifying
features of these phenomena
tics and habit spasms
An interesting feature of many tics is that
they correspond to_________
coordinated acts that normally serve
some purpose to the organism
Children between _________of age are especially
likely to develop these habit spasms
5 and 10 years
Multiple tics-sniffing, snorting, involuntary vocalization,
and troublesome compulsive and aggressive
impulses-constitute the rarest and most severe tic syndrome
G i l les de Ia Tou rette SyndromeAQ
It is the __________
of motor and vocal tics that distinguish the disorder from
the more benign, restricted tic disorders
multiplicity of tics and the combination
Explosive and involuntary cursing and the
compulsive utterance of obscenities ___________ are the
most dramatic manifestations
(coprolalia)
IN G i l les de Ia Tou rette Syndrome, An ______________, or both are said to be evident
at some time in the course of the illness, and these
interfere to a greater degree than do the tics with progress
in school
attention-deficit hyperactivity disorder obsessive-compulsive traits
Hyperactive children who have
been treated with __________appear to be at increased
risk of developing or exacerbating
stimulants
Wolf and colleagues have
found that differences in________binding
in the head of the caudate nucleus reflected differences
in the phenotypic severity of Gilles de la Tourette
syndrome.
D2 dopamine receptor
Using the model o f Sydenham chorea, a recent line of
investigation has implicated _________ in
the genesis of abruptly appearing Tourette syndrome
and of less-generalized tics in children.
This is also called _________
streptococcal infection
under the acronym
PANDAS (pediatric autoimmune neuropsychiatric disorders
with streptococcal infection).
Tx for Guilles de Touret
The alpha2-
adrenergic agonists clonidine and guanfacine
Dose of drugs for Guilles de Touret
The initial dose is 0.5 to 1 mg given at bedtime and gradually
increased as needed to a total dose of 4 mg. Clonidine
is started as a bedtime dose of 0.05 mg and increased every
several days by 0.05 mg until a total dose of about 0.1 mg
three times daily; guanfacine is given as 1 mg at bedtime
and increased by 1 mg monthly, up to 3 mg if neede
Tx for Guilles de Touret
The
neuroleptics_______, and tiapride
have proved to be effective therapeutic agents but should
be used only in severely affected patients and usually
after the adrenergic agents have been tried.
haloperidol, pimozide, sulpiride
The addition of
_________ at the outset of treatment
may help to prevent the adverse motor effects of
haloperidol.
benztropine mesylate (0.5 mg daily)
Another interesting approach has been to inject
_________in muscles affected by prominent focal
tics, including the vocal ones
botulinum toxin
the hyperactivity component of
the Tourette syndrome can be treated safely with __________without fear of worsening the tics
methylphenidate
or clonidine
