Kelainan neuromuscular

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dr Ludmila SpS
 Movement Disorders
Neurologic syndromes in which there is either an excess
of movement, or a paucity of voluntary and automatic
movements unrelated to weakness or spasticity.
Most movement disorders are associated with pathologic
alterations in the
basal ganglia or their connections.
EXTRA PYRAMIDAL SYSTEM
 EXTRA PYRAMIDAL SYSTEM

 Influences spinal motor activity

 Consists of : the cerebral cortex

the basal ganglia

the mid brain

But disorders of the
• Cerebellum or its pathways
• Cerebral cortex
• Thalamus
• Brain stem
• Spinal cord
• Peripheral nerves
may also cause several movement disorders
Sistem ekstrapiramidal :
- Sistem motorik di luar sistem piramidalis
- Rantai sinambung dari sistem saraf termasuk nukleus-nukleus,
sirkuit-sirkuit dan jaras-jaras desendens
- Fungsi utama : mengatur gerakan-gerakan asosiatif, postural dan
integrasi otonomik
- Lesi pada salah satu bagian sistem ekstrapiramidal  muncul
gerakan involunter, gangguan tonis otot dan postural
- Gerakan involunter ; gerakan motorik yang terjadi spontan, tidak
dapat dikendalikan dengan kemauan, gerakan tidak bertujuan
MENCAKUP :
1. Korteks ekstrapiramidalis (area 4,6 dan 8)
2. Ganglia basalis, terdiri dari :
- korpus striatum : Nc. Kaudatus
Nc. Lentiformis : globus pallidus, putamen
- corpus Luysi (Nc. Subtalamikus)
- substansia nigra
- talamus (Nc. Ventrolateralis talami)
3. Nc. Ruber
4. Substansia retikularis batang otak
5. Serebelum
Sistem ekstrapiramidalis  sebagian besar = GANGLIA BASALIS
Yang paling berperan : Nc. Kaudatus, Nc.lentiformis
 Basal ganglia
subcortical nuclei:
caudate, putamen &
globus pallidus are
named corpus
striatum
Caudate &putamen are
named striatum
caudate
globus pallidus &
putamen putamen are named
lentiform nuclei
globus pallidus
 Basal Ganglia
Caudate

Thalamus
Putamen GPe GPi

STN
SNr
SNc
Hubungan antara berbagai bagian sistem ini  sirkuit :
- Sirkuit 1
Korteks serebri – striatum—globus palidus—talamus—korteks serebri
- Sirkuit 2
1.korteks serebri – striatum—substansia nigra
2. a. Substansia nigra – striatum
b. Substansia nigra --- talamus– korteks motorik (area 4,6,8)
- Sirkuit 3
Globus paliidus – nc.luysi – globus pallidus
- Sirkuit 4
Korteks serebri – nc-nc pontis ipsilateral – serebelum – talamus—korteks serebri
- Sirkuit 5
Korteks ekstrapiramidalis—nc.nc batang otak dan medula spinalis
 Type of extrapyramidal lesion
• Diseases of the basal ganglia are associated with abnormal
involuntary movements that typically occur at rest and disappear
in sleep.
• They are generally divided into two categories: Hyperkinetic and
hypokinetic
• The hyperkinetic variety is seen in such disorders as chorea, athetosis,
ballism, dystonia, tremor, and tics.
• The hypokinetic variety is seen largely in Parkinson’s disease and Parkinson
plus syndromes

• Following anatomic loci for pathology are agreed on:

- Substantia nigra in Parkinson’s disease
- Caudate nucleus in chorea
- Subthalamic nucleus in ballism
- Caudate or lentiform nucleus (especially putamen) in dystonia
1. Akinesia (bradikinesia, hipokinesia)
- Gangguan kecepatan dan spontanitas dari gerakan motorik
- Gerakan motorik jadi lamban, tapi tidak ditemukan kelumpuhan
- Misal :
1.Muka topeng (mask face), pada parkinson : berkurangnya gerakan muka
dan mengedip
2.Mars a petit pas : jalan pelan-pelan dengan kaki lebar (broad-base)
3.Mikrografi : tulisan kecil-kecil karena gangguan jangkauan gerak otot
4.Gerakan habitual (pada orang normal) berkurang seperti : mengusap
wajah, mengedip, melenggang dll
2. Tremor
- Gerakan osilasi yang ritmik dan reguler (merupakan gerakan alternating/ganti
berganti antara kontraksi dan relaksasi dari otot agonis dan antagonis)
- Ekstrapiramidalis : Resting tremor (muncul saat istirahat, berkurang sampai
hilang bila lengan digerakkan)
- Parkinson : Rolling pil (menghitung pil/uang)

3. Gangguan postural / sikap

- Sikap fleksi dari batang tubuh, tungkai dan kepala
- Karena gangguan pada postural normal batang tubuh dan kepala
4. Perubahan tonus otot (rigiditas)
- Tonus otot meningkat secara intermitten  cogwheel phenomenon
- Rigiditas pada semua otot : fleksor, ekstensor

5. Chorea
- Pada lengan  pada semua persendian : bahu, siku, pergelangan
tangan, sendi-sendi kecil tangan
- Berupa gerakan gesit dan cepat berubah arah
- Lesi di striatum
6. Atetosis
-Seperti orang menari, tapi lebih kasar
-Gerakan lebih lambat tapi jangkauan lebih panjang (dibanding chorea)
-Lengan, tungkai, otot wajah, lidah (keluar masuk bergantian)
7. Hemibalismus
- Gerakan unilateral lengan yang kuat seperti orang melempar cakram
- Karena gangguan pada nc.subtalamikus (luysi) kontralateral

8. Distonia
- Mirip gerakan atetosis tapi disertai gerakan lengan memutar pada
porosnya
- Biasa pada otot batang tubuh dan leher
- Karena lesi di korpus striatum dan globus palidus
Essential tremor
 • Tremor
* Involuntary oscillations of a body part produced by alternating or
synchronous contractions of reciprocally innervated muscles.

* Physiological tremor
These tremors are very small amplitude and are demonstrable only by
means of accelerometer. Enhanced physiological tremor: medical
conditions, drugs, anxiety, fear…
* Essential tremor ET
Typically a postural tremor (4-12 Hz) but may be accentuated by
goal-directed movements. The site of involvement in most cases is the
hands and it is frequently asymmetric initially.
* Parkinsonian tremor
Tremor at rest, at a frequency of 4-5 Hz, is the most characteristic
and the most prominent type of tremor in PD, but postural and kinetic
tremor are also frequently seen. Onset of the tremor is usually in one of
the hands; rarely, it may begin in the legs.
* Intention tremor
Rhythmic involuntary oscillations that undergo exacerbation as the
hand or foot approaches the target of a voluntary movement. It indicates
involvement of the cerebellum or its connections.
 • Chorea (“dance”)

Characterized by sudden, frequent involuntary, arrhythmic, purposeless, and

quick jerks of the trunk, extremities, and head associated with facial
grimaces. They are usually distal and of low amplitude. Causes of chorea
are hereditary, autoimmune, vascular, metabolic, toxic, inflammatory or drug
induced.

• Athetosis (“without position”)

Slow, writhing, continuous, wormlike movements of the distal parts of the
extremities, chiefly the fingers, which show bizarre posturing.
 • Ballism (“jump or throw”)
Sudden, quick, continuous, unusually violent, and flinging in nature.
Usually confined to the contralateral vascular lesion in the subthalamic nc

• Dystonia (“bad tone”)

Twisting, slow, contorting, involuntary movement, that is somewhat
sustained and often repetitive. Dystonia can involve any part of the body.
Dystonia is classified as
focal, segmental (cranial muscles (Meige syndrome), cranio-cervical dystonia,
multifocal (2 or more noncontiguous parts), hemidystonia, generalized
(segmental crural + an other part of the body)