MOVEMENT DISORDERS

Kenneth H Silver, MD

DEFINITION
Involuntary movement disorders can be characterised by other too (hypo
kinetic) movement or too much (hyperkinetic). Hypo kinetic problems include
Parkinson and -kinston-like conditions, such as progress supra nuclear palsy,
vascular or trauma-induced Parkinson's and multi system atrophy (which
encompasses related disorders of Shy-Dragger syndrome,degeneration, and
tolivopontocerebellar degeneration) hyperkinetic disorders include parkinson and
non .parkinsonian tremor, tics, Gilles de la, dystonia, dyskinesias, hemifacial
spasm, athetosis, hemiballismus and asterixis Depending on which diagnostic
criteria are used, essential tremor has a prevalence ranging from 0.1% to 22%
roughly 20 times more common than Parkinson disease. Idiopathic Parkinson
disease constitutes approximately 85% of all the Parkinson-like conditions;
neuroleptic induced Parkinson disease (7% to 9%), vascular Parkinson (3%),
multi system atrophy (2.5%), and Progressive supra nuclear palsy (1.5%)
represent much smaller fractions. Relatively rare, Huntington disease occurs with
frequency in the population as low as 0.004% by some.

SYMPTOMS
Parkinson patients commonly show a resting tremor slowness of movement
or bradykinesia, and a from of increased muscle tone called rigidity (see
Chapter 132 for more details). Other common features are reduction in
movements of facial expression resulting in "masked facies," stooped posture, and
reduction of

the amplitude of movements (hypometria). Also seen are changes in speech to a

soft nionotone (hypophonia) and small less legible handwriting (micrographic).
Walking be comes slower, stride length is reduced, and pivotir, is replaced with
a series of small steps (turning enbloc"). 1-4 The following syndromes
typically present with the listed features in addition to the characteristic ,
symptoms of Parkinson disease (tremor and rigidity) ,

Shy-Dragger

syndrome:

autonomic

failure

with

prominent postural hypotension

Progressive supra nuclear palsy: reduction in vertical gaze

and slowing of eye movements

Vascular Parkinsonism: early dementia with brisk tendon

reflexes
Multiple head trauma, "Parkinsonism pugilistic a early

dementia with brisk tendon reflexes

Olivopontocerebellar

degeneration: prominent

intention

tremor, imbalance, and ataxia

Tremors, the most common form of involuntary movement disorders, are
characterised by rhythmic oscillation of a body part. Tremors can be dassified as
to the sitution in which they are most prominent. Is the tremor most
pronounced at rest or with movement? Tremor with movement are subdivided
into those occurring with maintained posture (postural or static tremor, tested by
holding the arms out in front), with movement from point to point (kinetic or
intentional tremor, tested by finger to nose pointing), or only with a specific type
of movement (task-specific tremor). Tremors that are at their worst at rest are
exdusively associated with Parkinson disease or other parkinsonian states (such
as those produced by neuroleptics).6-10
Tics are sustained nonrhythmic muscle contractions that are rapid and
stereotyped, often occurring in the same extremity or body part during times of

stress. The muscles of the face and neck are usually involved, with movement of a
rotational sort away from the body's midline. They are commonly familial and
often seen in otherwise normal children between the ages of 5 and 10 years and
usually disappear by the end of adolescence. Tourette syndrome is characterised
by motor and vocal tics lasting for more than 1 year and may involve involuntary
use of obscenities and obscene gestures, although such behaviour may be mild
and transient and occurs only in a minority of afflicted persons. 2
Dystonia are slow, sustained contractions of muscles that frequently cause
twisting movements or abnormal postures. The disorder resembles athetosis but
shows a more sustained static contraction. When rapid movements are involved,
they are usually repetitive and continuous. Dystonia often increases with
emotional or physical stress, anxiety, pain, or fatigue and disappears with sleep.
The dystonias are further classified as focal, segmental, or multifocal on the basis
of the distribution of muscles affected. Symptoms of hemifacial spasm usually
begin in the orbicularis oculi and later involve other muscles innervated by
cranial nerve VII.
Tardive dyskinesia is a condition characterised by involuntary, choreiform
movements of the face and tongue associated with chronic neuroleptic medication
use. Common movements include chewing, sucking, mc,outhunt licking, "fly-catching
movements," puckering, and smacking (buccal-lingual-mastic_atory syndrome).
Choreiform movements of the trunk and extremities can also occur along with
dystonic movements of the neck and trunk.8
Athetosis is characterised by involuntary, slow, writhing, and repetitious
movements. They are slower than choreiform movements and less sustained than
dystonia. Athetosis may be seen alone or in combination with other movement
disorders and itself leads to bizarre but characteristic postures. Any part of the body
can be affected, but it is usually the face and distal upper extremities that are
involved. Chorea presents as non stereotyped, unpredictable, and jerky movements
that interfere with purposeful motion. The movements are rapid, erratic, and
complex and can be seen in any or all body parts but usually involve the oral

structures, causing abnormal speech and respiratory patens. Hemiballismus is an

uncommon disorder consisting of extremely violent flinging of the arms and legs
on one side of the body.
In cervical dystonia (torticollis), social stigmatisation is a major concern,
as are functional impairments, which include can include driving, reading, and
activities that involve looking down and using the hands. In another focal
dystonia, writer's or occupational cramp, the symptoms present In a certain
posture or position; for instance, patient may be able to write at a blackboard
but not seated at a desk. With lingual involvement in oromandibular dystonia,
the tongue has abnormal movements during speaking or deglutition. The result of
such dystonias is impairment of speech and eating.14 In Huntington chorea, along
with the choreiform movement, progressive dementia and emotional and
behavioural abnormalities are seen. As the disease progresses, the presentation
becomes less choreiform and more parkinsonian and dystonic (i.e., restricted
motions, immobility, and unsteadiness of gait). Intellectual impairment and
psychosis invariably occur and progress rapidly to become the most disabling
features.
DIAGNOSTIC STUDIES
In most cases of movement disorders, such as Parkinson disease, tardive
dyskinesia, essential tremor, and dystonia, The diagnosis is made on the grounds of
dinical examination and history; no one specific test is pathognomonic for the
disease.." However, underlying causes of many of the movement disorders, such as
stroke, traumatic brain injury, tumor, infection, and metabolic or endocrinologic
disease should be evaluated with appropriate tests, in duding head and spinal
magnetic resonance imaging and computed tomography, cerebrospinal fluid
analysis, and blood serum analysis. Electrodiagnostic tests (electromyography and
nerve conduction studies) may be useful in some cases, such as focal dystonia, to
rule

out

coexisting

or

causative

peripheral

nerve

entrapment.

Electroencephalography is often helpful in distinguishing focal seizures from

myoclonus or other repetitive movement presentation More tests may be necessary

to confirm or to exclude the other diagnoses, such as human immunodeficiency virus

-related diseases, central nervous system infection, toxic. exposures, and psychiatric
illnesses.

TREATMENT
Treatment is highly dependent on which specific category of movement
disorder is present. Typically, pharmacologic treatment is initiated when the
symptoms

become

severe

enough to cause

discomfort

or

disability.

Antiparkinson medications either replace dopamine (levodopa), acting as a

postsynaptic (dopamine) ago- nist, or reestablish the dopamine acetylcholine
balance in the striatum (anticholinergics). In addition, The cat-echol 0methyltransferase inhibitors and monoamine oxidase B inhibitors increase the
availability of levodopa or dopamine by preventing their e abolism.
Levodopa in combination with carbidova (sinemet) is the most effective
medication for the relief of Parkinson disease, but it is usually not the first
medication given to a newly diagnosed patient. Loss; Levodopa efficacy
usually develops within 3 to 5 years after the medication is begun, so an effort
is made to manage early Parkinson disease with other medication. A guiding
principle is to start levodopa treatment; in patients with symptoms that
interfere with the performance of daily life functions despite other treatment
anticholinergic drugs such as trihexyphenidyl (Artane) widely used to treat patients
with early Parkinson disease. ise, with tremor as their primary symptom.
Amantadine (Symmetrel) is another useful medication in early parkinson
disease. Although its usefulness in early Parkinson disease is controversial, the
monoamine oxidase inhibitor deprenyl or selegiline (Eldepryl) is widely given
to newly diagnosed patients. Within 1 to 2 years, most patients will have
sufficient difficulty with movement and daily activities to require levodopa.
Gradually over the years, the patient's frequency of dosing will increase and the
total dose needed will increase, along with the need for other supplemental
medication the two most useful are bromocriptine (Parlodel) pergolide.

Propranolol is the most useful medication in treating essential tremor

(the most common symptomatic tremor), task-specific tremor, and action
tremor. Other

blockers have fewer side effects but are less: effective. The

anticonvulsant primidone and the benzodiazepineclonazepam are also effective

antitremor drugs Gaba-pentin and botulinum toxin injections have also been
demonstrated to be effective in tremor manage ment.Tics can be managed with
neuroleptics; pimozide and haloperidol are generally effective,but
sedation limits their use. Newer atypical neuroleptics may have fewer or
different side effects, including risperidone, quetiapine, olanzapine, and
clozapine Other medications shown to be of use include benzodiazepines,
clonazepam, clonidine, calcium channel blockers, and antidepressant agents. 12
Anticholinergic medications, such as trihexyphenidyl and benztropine, are the
most effective oral agents for both generalised and focal dystonia. Baclofen,
carbamazepine and the benzodiazepines, such as diazepam and clonazepam,
are sometimes helpful. Dopamine-blocking or dopa mine-depleting agents
may be used to treat some patients with dystonia. Focal dystonia are now
commonly treated with botulinum toxin injection. 1424 (See the section on
procedures.)
Replacement of the neuroleptic drug with substitute drugs may help
some patients with tardive dyskinesia. The atypical neuroleptics clozapine
and risperidone are useful to control psychosis in patients with tardive.
Other drugs, such as benzodiazepines, adrenergic antagonists, and
dopamine agonists, may also be beneficial for suppression of movements. The
most important step in the treatment of tardive dyskinesia is prevention by
limiting the use of neuroleptic medications. 25 The response to drug therapy has
been poor in patients with ataxia; may agents have been touted as useful
(propanolol, isoniazid, carbamazepine, clonazepam, tryptophan, buspirone,
thyroid-stimulating hormone), but none has demonstrated efficacy. 26 A number
of drugs have been used to treat myoclonus and can be effective in some

situations. These include diazepam, clonazepam, valproate, and levetiracetam

(Keppta) as well as botulinum toxin injections. 27

REHABILITATION
In general, patient with Parkinson disease needs to be counseled to
maintain in a reasonable level of activity at all costs as physical exertion becomes
more difficult and the risk of reconditioning increases. Exercise focus on proper
body alignment (upright posture) and postural reflexes (response to dynamic
balance challenges) as well as limb range of motion and strengthening of proximal
musculature to assist in stair climbing and coming to a stand. Exercises are also
aimed at the restoration of diminished reciprocal limb motions and an increase in
step length and can include treadmill training. The tendency to freeze can be
reduced with visual targets, such as markers on the floor, counting, or marching
rhythmically. The difficulty in rising from sitting surface can be addressed with
elevated sitting surfaces (chair, toilet) and strategically placed grab rails or bars
(bed, bathtub). Although wheeled walkers are useful in assisting ambulation,
particularly by preventing backward instability, patients with significant postural
deficits may prefer more stable devices, such as a supermarket shopping cart or
walking behind a wheelchair. Adaptive equipment is provided when deficits in
upper extremity control limit efficient and safe function. 28-31 Speech therapy is
useful in patients with Parkinson disease to improve articulation and loudness as
well as to diagnose and to manage dysphagia. 32
In tremor, measures to reduce or to alleviate anxiety (e.g., biofeedback,
relaxation exercises) are useful, as are strategy to control oscillation excursion
with weights or other mechanical compensations. 8 Lifestyle changes may include
restriction of caffeine intake or other stimulants that may temporarily augment
symptoms. In addition, alcohol consumption may lead to transient improvement
for may with essential tremor. 9
Stretching exercises may be important for maintenance or recovery of
range of motion for affected joints in a dystonic limb. Certain types of occupation-

based focal limb dystonias (e.g., writers or musicians cramp) may be treated with
muscle reeducation techniques, including biofeedback. A regular program of
stretching exercises may assist individuals in regaining full range of motion after a
botulinum toxin injection has weakened a dystonic muscle. Some patients use socalled sensory tricks to temporarily relieve their symptoms. These commonly
involve touching or stroking a particular spot on the skin, In addition in some
patients, certain types of braces may provide the same stimulation and be equally
effective. 8,14
Ataxic patients may benefit from rehabilitation to help them learn
compensatory techniques for performance of basic self-care and occupational
activities and to assess the benefits of weighted bracelets or similar devices to
damp the oscillations. Gait training and education in the use of assistive devices
for walking can prevent falls and enhance mobility in the ataxic individual. In
disorders involving athetosis, ballismus, or Huntington disease, careful weighting
of the extremities can help at times. Rehabilitation techniques involving
improvement of coactivation and trunk stability, rhythmic stabilisation, and
traditional relaxation techniques including biofeedback have been mentioned as
reasonable strategies. Some have suggested value in oral desensitisation when
hyperactivity to sensory stimulative dyskinesia, but other rehabilitation strategies
are not of proven utility. 8

PROCEDURES
Botulinum toxin injections are beneficial in numerous hyperkinetic
movement disorders including focal dystonia, tremor, and myoclonus, Trigger
point injections may provide relief in painful muscles associated with focal
dystonia (e.g., cervical torticollis).
The muscles selected for botulinum toxin injection are based on
understanding of the primary clinical patterns of spasticity or dystonia.

Direct injection by palpation technique may be appropriate for superficial

muscles; electromyography or electrical stimulation guidance is commonly used
to identify deeper muscles. Each muscles is injected in one more sites, the number
being a function of the size of the muscles. Dosage is variable but typically does
not exceed 400 units total body dose for a 3-month period. Botulinum toxin A is
reconstituted in the vial with preservative-free normal saline, at varying dilutions
depending on the muscle size: very small muscles need 20 units per 0,1 mL;
average size muscle, 10 units per 0,1 mL; and large muscles, 5 units or less per
0,1 mL.
The skin in cleaned with an alcohol or iodine swab and allowed to dry.
When electromyography or electrical stimulation guidance is used, a specialised
needing with an exposed tip connected by wire to the recording or stimulating
device is needed. Needles are typically 37 mm in length and 27-gauge; larger or
smaller needles are often needed, depending on muscle size and depth. In adults,
preanesthetization of the skin is usually unnecessary; in children, local anesthetic
creams are helpful. When botulinum toxin is injected, aspiration of the syringe to
prevent injection into a blood vessel is standard technique. Before the injection,
informed consent is obtained.
SURGERY
Deep brain stimulation, thalamotomy, and pallidotomy have been used
with success in some patients with Parkinson disease as well as the movement
disorders (e.g dystonia and tremor). In addition, peripheral destructive procedures
such as myectomy, rhizotomy, and peripheral nerve denervation are occasionally
performed on individuals with dystonic limbs who have proved refractory to more
conventional management. Cell transplant and gene therapies continue as
experimental tools under investigation. 33,34
POTENTIAL DISEASE COMPLICATIONS
Many of the movement disorders, particularly Parkinson disease, are
progressive and can result in muscle weakness and immobility, severe limb

contractures, aspiration of food and respiratory compromise, social isolation and

depression, and intellectual impairment and dementia.
POTENTIAL TREATMENT COMPLICATIONS
Antiparkinsons medications may have numerous side effects including
nausea

and

other

gastrointestinal

symptoms,

drowsiness,

confusion,

hallucinations, psychosis, and motor dyskinesia. 4,20 Similar adverse medication

effects are described with other agents used to suppress unwanted movement.
Botulinum toxin is generally well tolerated but can cause transient unwanted
weakness in target or adjacent muscles, including dysphagia. Risks associated
with surgical approaches to central nervous system structures are considerable and
need to be properly weighed before there options are selected. 20
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PENYAKIT GANGGUAN GERAK

Kenneth H Silver, MD
DEFINISI
Penyakit gangguan gerak dapat ditandai dengan gerakan yang terlalu
sedikit (hipokinetik) atau gerakan yang terlalu banyak (hiperkinetik). Hipokinetik
mencakup Parkinson dan kinson-like conditions, seperti progress supranuclear
palsy, vascular or trauma-induced parkinsons, dan multisystem atrophy
(Shy-Drager syndrome, degenerasi, dan olivopontocerebellar degeneration).
Gangguan hiperkinetik termasuk parkinson dan tremor non parkinsonian, tics,
Gilles de la, dystonia, diskinesia, kejang, athetosis, hemiballismus dan asteriksis.
Tergantung pada kriteria diagnostik yang digunakan, tremor esensial memiliki
prevalensi berkisar antara 0,1% sampai 22% kira-kira 20 kali lebih umum
daripada penyakit Parkinson. Penyakit Parkinson idiopatik merupakan sekitar
85% dari semua kondisi seperti Parkinson; neuroleptik disebabkan penyakit
Parkinson (7% sampai 9%), pembuluh darah Parkinson (3%), multisistem atrofi
(2,5%), dan Progressive supranuclear palsy (1,5%). Penyakit Huntington relatif
jarang terjadi dengan frekuensi dalam populasi serendah 0.004.
GEJALA

Pasien Parkinsonian umumnya menunjukkan bradikinesia dan kekakuan

(Lihat Bab 132 untuk informasi lebih lanjut). Lainnya adalah kurangnya gerakan
ekspresi wajah yang mengakibatkan facies mask, postur membungkuk, dan
kurangnya gerakan amplitudo (hypometria). Perubahan dalam berbicara untuk
(hypophonia) dan tulisan tangan yang kurang terbaca (micrographia). Berjalan
menjadi lebih lambat, panjang langkah berkurang, dan pivotir, diganti dengan
serangkaian langkah-langkah kecil. 1-4 Sindrom berikut biasanya terdapat pada
penyakit Parkinson:
Shy - Drager syndrome : kegagalan otonom dengan hipotensi postural menonjol.
Progresif supranuclear palsy : penurunan tatapan vertikal dan memperlambat
gerakan mata.
Parkinsonisme Vascular : demensia dini dengan refleks tendon cepat.
Trauma Beberapa kepala , "Parkinsonisme pugilistica" demensia dini dengan
refleks tendon cepat
Degenerasi olivopontocerebellar : tremor, ketidakseimbangan, dan ataksia.
Tremor, bentuk paling umum dari gangguan gerakan yang tidak
disengaja, yang karakternya oscillation rhythmic dari bagian tubuh. Apakah
tremor yang paling menonjol saat istirahat atau dengan gerakan? Tremor dengan
gerakan dibagi lagi menjadi orang-orang yang terjadi dengan postur dipelihara
(postural atau tremor statis, diuji dengan memegang tangan di depan), dengan
gerakan dari titik ke titik (kinetik atau sengaja tremor, diuji oleh jari ke hidung
menunjuk), atau hanya dengan jenis tertentu gerakan (tugas-spesifik tremor).
Tremor yang berada di terburuk mereka saat istirahat yang exdusively
berhubungan dengan penyakit Parkinson atau parkinsonian lainnya (seperti yang
pro-diproduksi oleh neuroleptik) Gerakan yang berkelanjutan kontraksi otot
nonrhythmic yang cepat dan stereotip, sering terjadi di ekstremitas atau bagian
tubuh yang sama selama masa stres. Otot-otot wajah dan leher biasanya terlibat,
dengan gerakan semacam rotasi jauh dari garis tengah tubuh. Mereka umumnya
keluarga dan sering terlihat pada anak-anak dinyatakan normal antara usia 5 dan

10 tahun dan biasanya menghilang pada akhir adolescence. Sindrom Tourette

ditandai oleh motor dan tics vokal yang berlangsung selama lebih dari 1 tahun dan
dapat melibatkan penggunaan paksa kata-kata kotor dan gerakan cabul, meskipun
perilaku seperti itu mungkin ringan dan sementara dan occuis hanya sebagian
kecil orang yang menderita. Dystonias lambat, kontraksi otot berkelanjutan yang
sering menyebabkan gerakan memutar atau postur abnormal. Kelainan
menyerupai athetosis tetapi menunjukkan kontraksi statis lebih berkelanjutan,
mereka biasanya berulang dan kontra-kontinyu. Dystonia sering meningkat
dengan stres emosional atau fisik, kecemasan, nyeri, atau kelelahan dan
menghilang dengan tidur. Para dystonias diklasifikasikan lebih lanjut sebagai
focal, segmental, atau multifokal berdasarkan distribusi otot yang terkena. Gejala
kejang spasm biasanya dimulai pada oculi orbicularis dan kemudian melibatkan
otot-otot lain diinervasi oleh saraf kranial VII.
Tardive dyskinesia adalah kondisi yang ditandai oleh involuntary,
movernents choreiform wajah dan lidah yang terkait dengan penggunaan obat
neuroleptik kronis. Gerakan yag palig sering termasuk mengunyah, menghisap,
menjilati, (sindrom bukal-lingual-mastic_atory). Gerakan Choreiform pada batang
tubuh dan ekstremitas juga dapat terjadi seiring dengan gerakan distonik leher dan
trunk.
Athetosis ditandai dengan lambat, menggeliat, dan gerakan berulangulang. Athetosis dapat dilihat sendiri atau dalam kombinasi dengan gangguan
gerakan lain dan dirinya sendiri. Setiap bagian tubuh dapat terpengaruh, tetapi
biasanya wajah dan ekstremitas atas distal yang terlibat. Chorea menyajikan
sebagai nonstereotyped, gerakan yang unpredictable. Gerakan yang cepat, tidak
menentu, dan kompleks dan dapat dilihat pada salah satu atau semua bagian
tubuh, tetapi melibatkan struktur mulut , namun bicara normal. Hemiballismus
adalah gangguan uncommon terdiri dari melemparkan sangat keras pada lengan
dan kaki pada satu sisi tubuh.
Dalam cervical dystonia (tortikolis), stigmatisasi sosial merupakan
perhatian utama, seperti gangguan fungsional, yang meliputi dapat mencakup
mengemudi, membaca, dan kegiatan yang melibatkan melihat ke bawah dan

menggunakan tangan. Dalam dsytonia fokus lain, penulis atau kram kerja, gejala
dapat hadir dalam postur atau posisi tertentu; misalnya, pasien mungkin dapat
menulis di papan tulis, tetapi tidak duduk di meja. Dengan keterlibatan bahasa di
dystonia oromandibular, lidah memiliki gerakan abnormal selama berbicara atau
penelanan. Hasil nias dysto tersebut adalah gangguan berbicara sedang.14 Di
Huntington chorea, bersama dengan gerakan choreiform, demensia progresif dan
kelainan emosi dan perilaku terlihat. Sebagai penyakit berlangsung, presentasi
menjadi kurang choreiform dan lebih parkinsonian dan distonik (yaitu, gerakan
terbatas, immobility, dan kegoyangan kiprah). Gangguan intelektual dan psikosis
selalu dapat terjadi.
STUDI DIAGNOSIS
Dalam kebanyakan kasus gangguan gerak, seperti penyakit Parkinson,
tardive dyskinesia, tremor esensial, dan dystonia, diagnosis dibuat atas dasar
anamnesis dan pemeriksaan fisik; tidak ada satu tes khusus yang patognomonik
untuk penyakit. Namun, dapat disebabkan oleh banyak penyebab, seperti stroke,
cedera otak, tumor, infeksi, dan metabolik atau penyakit endokrinologi harus
dievaluasi dengan tes yang sesuai, di kepala dan tulang belakang pencitraan
resonansi magnetik, analisis cairan serebrospinal, dan analisis serum darah. Tes
elektrodiagnostik (electromyography) mungkin berguna dalam beberapa kasus,
seperti distonia fokal, untuk menyingkirkan hidup bersama atau penyebab saraf
perifer jebakan. Electroencephalograpy sering bermanfat terhadap kejang fokal
yang membedakan dari mioklonus atau gerakan berulang hasil presentatition
lainnya mungkin diperlukan untuk mengkonfirmasi atau terkecuali diagnosis lain,
seperti immunodeficiency virus -terkait penyakit manusia, infeksi sistem nereous
pusat, beracun. eksposur, dan penyakit kejiwaan.
TREATMENT
Pengobatan sangat tergantung pada kategori yang spesifik gangguan
gerakan yang hadir. Biasanya, pengobatan farmakologi dimulai ketika gejala
menjadi cukup berat sehingga menyebabkan ketidaknyamanan atau cacat. Obat

antiparkinson antidopamin (Levodopa), bertindak sebagai postsynaptic (dopamin)

lalu-NIST, atau membangun kembali keseimbangan dopamine asetilkolin di
striatum (antikolinergik). Dalam addidtion, Theinhibitor echol 0-methyltransferase
dan monoamine inhibitor oksidase B meningkatkan ketersediaan levodopa atau
dopamin dengan mencegah theit e abolism. Levodopa dalam kombinasi dengan
carbidova (Sinemet) adalah obat yang paling efektif untuk menghilangkan dari
penyakit Parkinson, tetapi biasanya bukan obat pertama yang diberikan kepada
pasien yang baru didiagnosis. khasiat levodopa biasanya berkembang dalam
waktu 3 sampai 5 tahun setelah pengobatan dimulai, sehingga upaya yang
dilakukan untuk mengelola penyakit Parkinson awal dengan obat lain. Prinsip
guiding adalah untuk memulai pengobatan levodopa; pada pasien dengan gejala
yang mengganggu kinerja fungsi kehidupan sehari-hari meskipun perlakuan
lainnya anticholinergic seperti trihexyphenidyl (Artane) sangat luas digunakan
untuk mengobati pasien dengan penyakit Parkinson dini. Seperti penyakit dengan
tremor sebagai gejala utama mereka. Amantadine (Symmetrel) adalah obat yang
berguna pada penyakit parkinson awal. Meskipun kegunaannya pada awal
Parkinson disease kontroversial, yang monoamine oxidase inhibitor deprenyl atau
selegiline (Eldepryl) secara luas diberikan kepada pasien yang baru didiagnosis.
Dalam 1 sampai 2 tahun, sebagian besar pasien akan mengalami kesulitan yang
cukup dengan aktivitas gerakan dan setiap hari memerlukan levodopa. GraduaIly
selama bertahun-tahun, frekuensi pasien dari dosis akan meningkat dan total dosis
yang dibutuhkan akan meningkat, seiring dengan kebutuhan untuk obat tambahan
lainnya dua yang paling berguna adalah bromocriptine (Parlodel)
Propranolol adalah obat yang paling berguna dalam mengobati tremor
esensial. Antikonvulsan primidone dan odiazepine benzclonazepam juga obat
antitremor efektif. Gabapentin dan suntikan toksin botulinum juga telah terbukti
efektif dalam management. Tics tremor dapat dikelola dengan neuroleptik;
pimozide

dan haloperidol

umumnya

efektif,

namun

sedasi membatasi

penggunaannya. Neuroleptik atipikal yang lebih baru mungkin memiliki lebih

sedikit atau berbeda efek samping, termasuk risperidone, quetiapine, olanzapine,
dan clozapine obat lain terbukti penggunaan termasuk benzodiazepines,

clonazepam, clonidine, calcium channel blockrs, dan agents.1 antidepresan " 2

Antircholinergic obat, seperti trihexyphenidyl dan benztropine, adalah obat oral
yang paling efektif untuk kedua dystonias umum dan fokus. Baclofen,
carbamazepine dan benzodiazepin, seperti diazepam dan clonazepam, kadangkadang membantu. Dopamin-blocking atau agen dopamine-depleting dapat
digunakan untuk mengobati beberapa patients dengan dystonia. Distonia fokal
sekarang diobati dengan toksin botulinum injection.14 24 (Lihat bagian tentang
prosedur.)
Penggantian obat neuroleptik dengan obat pengganti dapat membantu beberapa
pasien dengan tardive dyskinesia. Atipikal neuroleptik clozapine dan resperidone
berguna untuk mengendalikan psikosis pada pasien dengan tardive.
Obat lain, seperti benzodiazepin, antagonis adrenergik, dan agonis
dopamin, juga bermanfaat untuk menekan gerakan. Langkah yang paling penting
dalam pengobatan tardive dyskinesia adalah pencegahan dengan membatasi
penggunaan obat neuroleptik. 25 Respon terhadap terapi obat lebih sedikit pada
pasien

dengan

ataksia;

seperti

(propranolol,

isoniazid,

carbamazepine,

clonazepam, triptofan, buspirone, thyroid-stimulating hormone), tetapi tidak ada

yang menunjukkan keampuhan. 26 Sejumlah obat telah digunakan untuk
mengobati mioklonus dan dapat efektif dalam beberapa situasi termasuk
diazepam, clonazepam, valproate, dan levetiracetam (Keppra) serta suntikan
toksin botulinum. 27
REHABILITASI
Secara umum, pasien dengan penyakit Parkinson perlu dinasihati untuk
mempertahankan aktivitas di tingkat yang wajar. Latihan fokus pada keselarasan
tubuh (postur tegak) dan refleks postural (respon terhadap tantangan
keseimbangan dinamis) serta berbagai anggota gerak dan penguatan otot
proksimal untuk membantu memanjat tangga. Latihan juga ditujukan untuk
pemulihan gerakan ekstremitas dan dapat mencakup pelatihan treadmill.
Kecenderungan untuk freeze dapat dikurangi dengan target visual, seperti penanda
di lantai, menghitung, atau berbaris berirama. Kesulitan dalam naik dari duduk

dapat diatasi dengan permukaan tinggi duduk (kursi, toilet) dan rel ambil
ditempatkan secara strategis atau bar (tempat tidur, bak mandi). Meskipun pejalan
kaki roda berguna dalam membantu ambulasi, terutama dengan mencegah
ketidakstabilan mundur, pasien dengan defisit postural yang signifikan dapat
memilih perangkat yang lebih stabil, seperti keranjang belanja supermarket atau
berjalan di belakang kursi roda. Peralatan adaptif disediakan ketika defisit dalam
batas kendali ekstremitas atas fungsi yang efisien dan aman. 28-31 Terapi wicara
berguna pada pasien dengan penyakit Parkinson untuk meningkatkan artikulasi
dan kenyaringan serta mendiagnosa dan mengelola disfagia. 32
Dalam tremor, langkah-langkah untuk mengurangi atau meringankan
kecemasan (misalnya, biofeedback, latihan relaksasi) yang berguna, seperti
strategi untuk mengendalikan osilasi perjalanan dengan bobot atau kompensasi
mekanis lainnya. 8 Perubahan gaya hidup dapat mencakup pembatasan asupan
kafein atau stimulan lainnya yang mungkin sementara meningkatkan gejala.
Selain itu, konsumsi alkohol dapat menyebabkan peningkatan sementara untuk
Mei dengan tremor esensial. 9
Latihan peregangan mungkin penting untuk pemeliharaan atau pemulihan
berbagai gerakan untuk sendi yang terkena di dahan distonik. Beberapa jenis
distonia tungkai fokus berbasis pekerjaan (emg, penulis atau kram musisi) dapat
diobati dengan teknik biofeedback. Sebuah program reguler latihan peregangan
dapat membantu individu dalam mendapatkan kembali berbagai gerak setelah
injeksi toksin botulinum telah melemahkan otot distonik. Beberapa pasien
menggunakan apa yang disebut trik sensorik untuk meringankan sementara gejala
mereka. Ini biasanya melibatkan menyentuh atau membelai tempat tertentu pada
kulit, Selain pada beberapa pasien, beberapa jenis kawat gigi dapat memberikan
stimulasi yang sama dan sama-sama efektif. 8,14
Pasien ataksia dapat mengambil manfaat dari rehabilitasi untuk membantu
mereka belajar teknik kompensasi untuk kinerja perawatan diri dasar dan kegiatan
kerja dan untuk menilai manfaat gelang tertimbang atau perangkat serupa untuk
meredam osilasi. Pelatihan Kiprah dan pendidikan dalam penggunaan alat bantu
untuk berjalan dapat mencegah jatuh dan meningkatkan mobilitas pada individu

ataxic. Pada gangguan yang melibatkan athetosis, ballismus, atau penyakit

Huntington, bobot hati-hati ekstremitas dapat membantu di kali. Teknik
rehabilitasi yang melibatkan peningkatan coactivation dan stabilitas batang,
stabilisasi berirama, dan teknik relaksasi tradisional termasuk biofeedback telah
disebutkan sebagai strategi yang masuk akal. Beberapa telah mengusulkan nilai
dalam desensitisasi oral ketika hiperaktif untuk sensorik stimulasi dyskinesia,
tetapi strategi rehabilitasi lain tidak utilitas terbukti. 8
PROSEDUR
Suntikan toksin botulinum yang bermanfaat dalam berbagai gangguan
gerak hiperkinetik termasuk dystonia fokus, tremor, dan mioklonus, Pemicu titik
suntikan dapat memberikan bantuan pada otot yang menyakitkan terkait dengan
dystonia fokal (misalnya, tortikolis serviks).
Otot-otot yang dipilih untuk injeksi toksin botulinum didasarkan pada
pemahaman tentang pola klinis utama spastisitas atau dystonia.
Injeksi langsung dengan teknik palpasi mungkin cocok untuk otot dangkal;
elektromiografi atau bimbingan rangsangan listrik umumnya digunakan untuk
mengidentifikasi otot yang lebih dalam. Setiap otot disuntikkan dalam satu situs
yang lebih, jumlah menjadi fungsi dari ukuran otot. Dosis bervariasi tetapi
biasanya tidak melebihi 400 unit dosis total tubuh untuk jangka waktu 3 bulan.
Botulinum toxin A dilarutkan dalam botol dengan bebas pengawet saline normal,
di berbagai pengenceran tergantung pada ukuran otot: otot yang sangat kecil
membutuhkan 20 unit per 0,1 mL; Rata-rata otot ukuran, 10 unit per 0,1 mL; dan
otot-otot besar, 5 unit atau kurang per 0,1 mL.
Kulit di dibersihkan dengan alkohol atau yodium swab dan dibiarkan
kering. Ketika elektromiografi atau bimbingan stimulasi listrik digunakan, khusus
perlu dengan ujung terbuka dihubungkan dengan kawat rekaman atau merangsang
perangkat yang dibutuhkan. Jarum biasanya 37 mm dan 27-gauge; lebih besar
atau lebih kecil jarum sering diperlukan, tergantung pada ukuran otot dan
kedalaman. Pada orang dewasa, preanesthetization kulit biasanya tidak
diperlukan; pada anak-anak, krim anestesi lokal sangat membantu. Ketika toksin

botulinum disuntikkan, aspirasi jarum suntik untuk mencegah injeksi ke dalam

pembuluh darah adalah teknik standar. Sebelum injeksi, informed consent harus
dilakukan.
PEMBEDAHAN
Stimulasi dalam otak, thalamotomy, dan pallidotomy telah berhasil
digunakan pada beberapa pasien dengan penyakit Parkinson serta gangguan gerak
(misalnya distonia dan tremor). Selain itu, prosedur seperti myectomy, rhizotomy,
dan denervasi saraf perifer kadang-kadang dilakukan pada pasien dengan distonia
ekstremitas bawah yang telah terbukti refrakter terhadap manajemen yang lebih
konvensional. Transplantasi sel dan terapi gen dapat dilakukan sebagai alat
eksperimental dalam penyelidikan. 33,34
KOMPLIKASI PENYAKIT
Banyak gangguan gerak, terutama penyakit Parkinson, yang progresif dan
dapat menyebabkan kelemahan otot imobilitas, kontraktur tungkai berat, aspirasi
makanan, gangguan pernapasan, isolasi sosial, depresi, gangguan intelektual, dan
demensia.
KOMPLIKASI PENGOBATAN
Obat antiparkinson mungkin memiliki banyak efek samping termasuk
diantaranya nausea dan gejala gastrointestinal lainnya, mengantuk, kebingungan,
halusinasi, psikosis, dan motorik tardive. 4,20 Efek obat serupa yang merugikan
dijelaskan dengan agen lain yang digunakan untuk menekan gerakan yang tidak
diinginkan. Toksin botulinum umumnya ditoleransi dengan baik, tetapi bisa
menyebabkan kelemahan yang tidak diinginkan, termasuk disfagia. Risiko yang
terkait dengan pendekatan bedah untuk struktur sistem saraf pusat yang cukup
besar dan perlu ditimbang dengan benar sebelum ada pilihan yang dipilih. 20
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