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 en-

compasses related disorders of Shy-Dragger syndrome,degeneration, and tolivo-

pontocerebellar 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; neu-

roleptic 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 Chap-

ter 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 typi-

cally 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, imbal-

ance, 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 pro-

nounced at rest or with movement? Tremor with movement are subdivided into

those occurring with maintained posture (postural or static tremor, tested by hold-

ing the arms out in front), with movement from point to point (kinetic or inten-

tional 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 exdu-

sively 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 of-

ten 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 emo-

tional 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). Chor-

eiform 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 dis-

orders 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 in-

volved. Chorea presents as non stereotyped, unpredictable, and jerky movements

that interfere with purposeful motion. The movements are rapid, erratic, and com-

plex 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 dys-

tonia, 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 be-

havioural 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 fea-

tures.

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 anal-

ysis, 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 of-

ten 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 dis-

order is present. Typically, pharmacologic treatment is initiated when the symp-

toms 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 0-methyltransferase

inhibitors and monoamine oxidase B inhibitors increase the availability of

levodopa or dopamine by preventing their e abolism. Levodopa in combina-

tion 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 lev-

odopa treatment; in patients with symptoms that interfere with the perfor-

mance of daily life functions despite other treatment anticholinergic drugs such

as trihexyphenidyl (Artane) widely used to treat patients with early Parkinson dis-

ease. ise, with tremor as their primary symptom. Amantadine (Symmetrel) is an-

other useful medication in early parkinson disease. Although its usefulness in

early Parkinson disease is controversial, the monoamine oxidase inhibitor de-

prenyl 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 in-

crease, 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 anti-

convulsant primidone and the benzodiazepineclonazepam are also effective an-

titremor drugs Gaba-pentin and botulinum toxin injections have also been

demonstrated to be effective in tremor management.Tics can be managed with

neuroleptics; pimozide and haloperidol are generally effective,but se-

dation limits their use. Newer atypical neuroleptics may have fewer or differ-

ent 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 dopamine-depleting agents

may be used to treat some patients with dystonia. Focal dystonia are now com-

monly treated with botulinum toxin injection.14•24 (See the section on proce-

dures.)

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 limit-

ing 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-stimulat-

ing 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 in-

clude 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 main-

tain 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 muscu-

lature 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 rhythmi-

cally. The difficulty in rising from sitting surface can be addressed with elevated

sitting surfaces (chair, toilet) and strategically placed grab rails or bars (bed, bath-

tub). 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, re-

laxation exercises) are useful, as are strategy to control oscillation excursion with

weights or other mechanical compensations. 8 Lifestyle changes may include re-

striction 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., writer’s or musician’s 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 so-

called sensory tricks to temporarily relieve their symptoms. These commonly in-

volve touching or stroking a particular spot on the skin, In addition in some pa-

tients, certain types of braces may provide the same stimulation and be equally ef-

fective. 8,14

Ataxic patients may benefit from rehabilitation to help them learn compen-

satory techniques for performance of basic self-care and occupational activities

and to assess the benefits of weighted bracelets or similar devices to damp the os-

cillations. Gait training and education in the use of assistive devices for walking

can prevent falls and enhance mobility in the ataxic individual. In disorders in-

volving athetosis, ballismus, or Huntington disease, careful weighting of the ex-

tremities 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 move-

ment disorders including focal dystonia, tremor, and myoclonus, Trigger point in-

jections may provide relief in painful muscles associated with focal dystonia (e.g.,

cervical torticollis).

The muscles selected for botulinum toxin injection are based on under-

standing 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; aver-

age 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 de-

vice 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 dis-

orders (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 experi-

mental tools under investigation. 33,34

POTENTIAL DISEASE COMPLICATIONS

Many of the movement disorders, particularly Parkinson disease, are pro-

gressive and can result in muscle weakness and immobility, severe limb contrac-

tures, aspiration of food and respiratory compromise, social isolation and depres-

sion, and intellectual impairment and dementia.

POTENTIAL TREATMENT COMPLICATIONS

Antiparkinsons medications may have numerous side effects including

nausea and other gastrointestinal symptoms, drowsiness, confusion, hallucina-

tions, 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 tar-

get or adjacent muscles, including dysphagia. Risks associated with surgical ap-

proaches 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 dari-

pada 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 ku-

rangnya gerakan amplitudo (hypometria). Perubahan dalam berbicara untuk (hy-

pophonia) dan tulisan tangan yang kurang terbaca (micrographia). Berjalan men-

jadi 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 re-

fleks tendon cepat

• Degenerasi olivopontocerebellar : tremor, ketidakseimbangan, dan ataksia.

Tremor, bentuk paling umum dari gangguan gerakan yang tidak disen-

gaja, 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 neurolep-

tik) 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 kon-

traksi 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 kemu-

dian 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 berulang-

ulang. Athetosis dapat dilihat sendiri atau dalam kombinasi dengan gangguan ger-

akan lain dan dirinya sendiri. Setiap bagian tubuh dapat terpengaruh, tetapi bi-

asanya 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 gang-

guan uncommon terdiri dari melemparkan sangat keras pada lengan dan kaki pada

satu sisi tubuh.

Dalam cervical dystonia (tortikolis), stigmatisasi sosial merupakan per-

hatian utama, seperti gangguan fungsional, yang meliputi dapat mencakup menge-

mudi, membaca, dan kegiatan yang melibatkan melihat ke bawah dan menggu-

nakan 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 reso-

nansi magnetik, analisis cairan serebrospinal, dan analisis serum darah. Tes elek-

trodiagnostik (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 ger-

akan yang hadir. Biasanya, pengobatan farmakologi dimulai ketika gejala menjadi

cukup berat sehingga menyebabkan ketidaknyamanan atau cacat. Obat antiparkin-

son 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 di-

lakukan untuk mengelola penyakit Parkinson awal dengan obat lain. Prinsip guid-

ing adalah untuk memulai pengobatan levodopa; pada pasien dengan gejala yang

mengganggu kinerja fungsi kehidupan sehari-hari meskipun perlakuan lainnya an-

ticholinergic seperti trihexyphenidyl (Artane) sangat luas digunakan untuk men-

gobati pasien dengan penyakit Parkinson dini. Seperti penyakit dengan tremor se-

bagai gejala utama mereka. Amantadine (Symmetrel) adalah obat yang berguna

pada penyakit parkinson awal. Meskipun kegunaannya pada awal Parkinson dis-

ease 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 den-

gan 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 lain-

nya dua yang paling berguna adalah bromocriptine (Parlodel)

Propranolol adalah obat yang paling berguna dalam mengobati tremor

esensial. Antikonvulsan primidone dan odiazepine benzclonazepam juga obat an-

titremor efektif. Gabapentin dan suntikan toksin botulinum juga telah terbukti

efektif dalam management. Tics tremor dapat dikelola dengan neuroleptik; pi-

mozide dan haloperidol umumnya efektif, namun sedasi membatasi penggunaan-

nya. Neuroleptik atipikal yang lebih baru mungkin memiliki lebih sedikit atau

berbeda efek samping, termasuk risperidone, quetiapine, olanzapine, dan clozap-

ine obat lain terbukti penggunaan termasuk benzodiazepines, clonazepam, cloni-

dine, calcium channel block¬rs, 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 benzodi-

azepin, seperti diazepam dan clonazepam, kadang-kadang membantu. Dopamin-

blocking atau agen dopamine-depleting dapat digunakan untuk mengobati beber-

apa patients dengan dystonia. Distonia fokal sekarang diobati dengan toksin bo-

tulinum 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 peng-

gunaan 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 menun-

jukkan keampuhan. 26 Sejumlah obat telah digunakan untuk mengobati miok-

lonus 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 keseimban-

gan dinamis) serta berbagai anggota gerak dan penguatan otot proksimal untuk

membantu memanjat tangga. Latihan juga ditujukan untuk pemulihan gerakan ek-

stremitas 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 per-

mukaan tinggi duduk (kursi, toilet) dan rel ambil ditempatkan secara strategis atau

bar (tempat tidur, bak mandi). Meskipun pejalan kaki roda berguna dalam mem-

bantu ambulasi, terutama dengan mencegah ketidakstabilan mundur, pasien den-

gan defisit postural yang signifikan dapat memilih perangkat yang lebih stabil,

seperti keranjang belanja supermarket atau berjalan di belakang kursi roda. Perala-

tan 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 ke-

cemasan (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, kon-

sumsi 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 dis-

tonia tungkai fokus berbasis pekerjaan (emg, penulis atau kram musisi) dapat dio-

bati 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 bi-

asanya 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 Hunt-

ington, bobot hati-hati ekstremitas dapat membantu di kali. Teknik rehabilitasi

yang melibatkan peningkatan coactivation dan stabilitas batang, stabilisasi beri-

rama, dan teknik relaksasi tradisional termasuk biofeedback telah disebutkan se-

bagai strategi yang masuk akal. Beberapa telah mengusulkan nilai dalam desensi-

tisasi oral ketika hiperaktif untuk sensorik stimulasi dyskinesia, tetapi strategi re-

habilitasi 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 bi-

asanya tidak melebihi 400 unit dosis total tubuh untuk jangka waktu 3 bulan. Bo-

tulinum toxin A dilarutkan dalam botol dengan bebas pengawet saline normal, di

berbagai pengenceran tergantung pada ukuran otot: otot yang sangat kecil mem-

butuhkan 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 ker-

ing. 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 kedala-

man. Pada orang dewasa, preanesthetization kulit biasanya tidak diperlukan; pada

anak-anak, krim anestesi lokal sangat membantu. Ketika toksin botulinum disun-

tikkan, 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 digu-

nakan 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 di-

antaranya 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 be-

sar dan perlu ditimbang dengan benar sebelum ada pilihan yang dipilih. 20

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