BAB I

PENDAHULUAN
Vascular parkinsonism (VP) is a form of secondaryparkinsonism resulting from
ischaemic cerebrovascular disease. For a clinician, the most important aspect of
VP should be its differentiation from Parkinsons disease (PD). The clinical
features of VP are most helpful in making this distinction. The clinical diagnosis
of VP is most often supported by radiological studies which have evolved over the
last two decades from CT to MRI and dopamine transporter single photon
emission CT scan (DATSPECT). A clinical criterion for diagnosis of VP has also
been proposed.1 However, one aspect of VPdits pathophysiologydis still not
completely understood. This article attempts to delineate the clinical and
radiological features of VP and discusses the present evidence regarding its
pathogenesis and treatment.

BAB II
PEMBAHASAN

A.

Definisi
Parkinsonism is a syndrome that features bradykinesia (slowness of
the initiation of voluntary movement) and at least 1 of the following
conditions: rest tremor, muscular rigidity, or postural instability. In 1929,
Critchley1 identified a type of parkinsonism caused by cerebrovascular
disease in his report on arteriosclerotic parkinsonism. It required the
development of computed tomography (CT) and magnetic resonance
imaging (MRI) 50 years later to find evidence for Critchleys ideas and
what is now commonly known as vascular parkinsonism (VP).26
Ischemic vascular lesions that may lead to VP are lacunar infarctions,
white matter hyperintensities, and less common large vessel infarctions. A
comparison of 5 different European studies showed a prevalence rate of
3% of VP.7 In case the onset of parkinsonism was associated with a
cerebrovascular event, VP was diagnosed. Probably the real prevalence is
higher because only few patients with VP have an acute onset

B. Epidemiologi
VP has been reported to account for 2.5e5% of the total cases of
parkinsonism in various population based studies and clinic cohorts. The
Rotterdam study was a prospective population based cohort study in
which, of the total 132 subjects with parkinsonism, 5% had parkinsonism

due to cerebrovascular disease.8 In their population based analysis of 5278

elderly subjects, the NEDICES (Neurological Disorders in Central Spain)
study group found the prevalence rate of parkinsonism from all causes to
be 2.2% (118 subjects).9 Of the 118 subjects, VP was diagnosed in 2.5%
(three subjects). A joint analysis of five community surveys of Europe, the
EUROPARKINSON (European Community Concerted Action on the
Epidemiology of Parkinsons disease) collaborative study,found VP to
account for 3% of the total cases of parkinsonism.10 A diagnosis of VP
was made in 3.9% (60/1528) of patients in the clinic based cohort study of
Munhoz et al.11 Chang et al diagnosed VP in 11 (4.4%) of the 250
parkinsonian patients in their clinic cohort on the basis of radiological
findings and levodopa response.
C. Etiologi
In the classical clinical type of VP, parkinsonism is attributed to diffuse
periventricular and frontal white matter damage because similar clinical
features occur in normal pressure hydrocephalus and in some cases of frontal
parasagittal meningioma, in which the same structures are compromised.
According to Thompson and Marsden,disconnection of thalamocortical fibers
to the supplementary motor area and cerebellar fibers to the leg area is the
underlying mechanism that causes the gait disorder in VP. In accordance with
current concepts of putamenal efferents directed toward the basal ganglia
output nuclei, one might expect that vascular lesions in the SNc (substantia
nigra pars compacta), GPe (globus pallidus pars externa), or the VA (ventral
anterior)/VL (ventral lateral) nuclei of the thalamus induce decreased

thalamocortical drive to premotor areas and also subsequent development of

parkinsonism. According to many previous publications, parkinsonism is also
attributed to strategic infarcts in the basal ganglia or thalamus. A causal
relationship between strategic infarcts and parkinsonism is reported only in a
few publications that described the cases in which the onset is (sub) acute or
accompanied by other symptoms of a stroke related to the same lesion.
Lesions in specific areas that theoretically can cause parkinsonism (GPe,
VL/VA of thalamus, and SNc) have been described by a few investigators.
infarct is located in the hemisphere or brainstem contralateral to parkinsonism
in most cases in which a vascular cause is acceptable and a single lesion is
seen in the basal ganglia or thalamus.
D. Patofisiologi
VP is the result of conventional vascular risk factors, particularly
hypertension, leading to strategic infarcts of the subcortical grey matter
nuclei, diffuse white matter ischaemic lesions (DWML), and least
commonly large vessel infarcts.12e14 In a particular patient the
aforementioned types of ischaemic lesions are most often mixed. The role
of ischaemic cerebrovascular disease in the pathogenesis of VP has been
shown in clinicoradiological and clinicopathological1 19 20 studies. In all
these series, patients with VP have been shown to have had statistically
more cerebral infarcts than neurologically normal subjects or patients with
PD. For example, in one study a history of strokes was present in 43.5%
patients with VP as compared to 2.9% of patients with PD. Taken the
other way around, 11% of patients with stroke who were followed-up for a

median of 3 years developed parkinsonism which was different clinically

from the parkinsonism in PD. Parkinsonism occurred more often after
lacunar than cortical infarcts and in the presence of coexisting WML. The
pathogenesis of VP is not completely understood. Bilateral DWML cause
parkinsonism because of damage to the net thalamocortical drive, which
decreases the ultimate influence of basal ganglia on higher centres of
motor planning and execution.With regards to strategic infarcts there has
been a considerable variation in the literature on their location. Strategic
infarcts would be expected to produce clinical parkinsonism by disrupting
the putamino-pallido-thalamic loop. Theoretically, infarcts of the
substantia nigra (SN), striatum or the ventroanterior/ ventrolateral
(VA/VL) nuclei of the thalamus can decrease the net thalamocortical drive
and

cause

parkinsonism.

Although

striatal,

lenticular,

thalamic,

mesencephalic and frontal lobe infarcts have been reported to cause VP, all
infarcts at these sites do not lead to VP since asymptomatic basal ganglia
infarcts and white matter hyperintensities are a known occurrence. Lee et
al found that in a cohort of 994 asymptomatic, 20- to 78-year-olds, silent
infarcts were present on MRI in 5.84% of subjects. The most frequent sites
were basal ganglia (46/121 silent infarcts), periventricular white matter
(31/121), cerebral cortex, thalamus (15/121), pons, and cerebellum. Old
age and hypertension were independent risk factors for these silent
infarcts. Uehara et al performed brain MRIs on 219 adults, aged 33e83
years, without a history of stroke or transient ischaemic attacks and

without any abnormality on neurological examination. Silent infarcts in

the white matter and/or basal ganglia were detected in 88 (40.2%) of the
219 subjects. Age, hypertension, female gender, and extracranial
atherosclerotic disease were independent predictors of these infarcts.
Considering the above evidence, it seems probable that there are
coexisting clinical and physiological factors at play in patients with basal
ganglionic infarcts which preclude the appearance of parkinsonian
features. For example, Peralta et al postulated that striatal infarcts could
more commonly produce parkinsonism if they were selective enough to
disrupt the putamino-pallidal outflow only, but more frequently they
damage the pallidum also causing a contralateral pallidotomy effect.23 A
coexisting pyramidal tract lesion in patients with basal ganglia infarcts
would mask bradykinesia and rigidity. Lastly, other factors such as the
pattern of discharge of the nuclei of basal ganglia circuitry also play an
important role in the pathophysiology of various movement disorders
which may be true for VP.
E. Faktor Resiko
Clinical features of vascular parkinsonism
a.
b.
c.
d.
e.
f.

Age of onset >70 years

Pyramidal signs
Pseudobulbar palsy
Cognitive impairment
Absence of tremor
Multiple vascular risk factors, such as hypertension, smoking and

diabetes
g. History of stroke
h. Acute onset

i. Rapid progression
j. Poor response to dopaminergic therapy
F. Gejala Klinis
Pasien dengan parkinsonisme vaskuler mungkin memiliki gejala yang sama
seperti orang-orang dengan penyakit Parkinson idiopatik , meskipun sering
melibatkan

bagian

tubuh

yang

bawah

daripadabagian

tubuh

atas,

kemungkinan juga terdapat defisit residual tambahan dari stroke sebelumnya

seperti kelemahan tungkai , mati rasa atau abnormalitas berbicara.
Computerized Tomography ( CT ) atau Magnetic Resonance Imaging ( MRI )
otak cenderung abnormal pada 90-100 persen dari parkinsonisme vaskuler ,
sering menampilkan beberapa stroke kecil di bagian dalam otak . Jika pasien
sebelumnya tidak pernah memiliki pengkajian atau risiko dari strokenya,
pemeriksaaan berikut akan dibutuhkan . Ini mungkin termasuk tes darah
tambahan , evaluasi untuk kemungkinan penyakit jantung dan penyempitan
kepala atau leher pembuluh darah .
G. DD
Features that are usually considered to favor a diagnosis of idiopathic PD36
are also frequently present in VP and include micrographia, cogwheeling,
stooped posture, facial masking, hypophonia, and a positive response to
levodopa. However, application of the more stringent UK Parkinsons Disease
Society Brain Bank criteria for the diagnosis of idiopathic PD37 excludes this
diagnosis in most patients with VP. In case a slowly progressive gait disorder
presents itself with a shuffling gait, then a normal pressure hydrocephalus or a
frontal lobe tumor must be considered. A clinical diagnosis together with a
radiologic diagnosis of probable multiple system atrophy, progressive

supranuclear palsy (PSP),39 or dementia with Lewy bodies probably excludes

a diagnosis of VP in most cases. Dubinsky and Jankovic41 and Winikates and
Jankovic. suggested the presence of a particular subtype of VP that they called
vascular PSP. In one report, the brains of 2 patients with vascular PSP showed
(besides the common diffuse white matter lesions) additional lesions in the
dorsal pons and in the thalamus.
H. Penatalaksanaan
The appropriate choice of therapy for VP depends on the pathogenesis. If
the lesions affect the nigrostriatal pathways, dopaminergic therapy should
be beneficial, but such cases are a minority.57 For most patients, in whom
the damage does not affect these pathways, current therapeutic approaches
are disappointing. Levodopa, dopamine agonists and other traditional
antiparkinsonian drugs are ineffective for VP that is caused by WMLs.6,58
Subthalamic nucleus stimulation is similarly ineffective,59 and physical
therapy is also of limited value, although well-controlled studies have not
been conducted. Therapy that minimizes the vascular risk factors is
expected to slow down the rate of decline, but no studies have yet tested
this hypothesis. Atherosclerotic brain disease is common among elderly
individuals, and can also affect patients with idiopathic PD. These patients
with so-called mixed disease are likely to respond to dopaminomimetic
therapy, but to a lesser extent than patients with idiopathic PD and no
atherosclerotic brain disease. An intriguing therapeutic option that has
been suggested for VP is CSF drainage.

I. Pemeriksaan Penunjang
The appropriate choice of therapy for VP depends on the
pathogenesis. If the lesions affect the nigrostriatal pathways, dopaminergic
therapy should be beneficial, but such cases are a minority. For most
patients, in whom the damage does not affect these pathways, current
therapeutic approaches are disappointing. Levodopa, dopamine agonists
and other traditional antiparkinsonian drugs are ineffective for VP that is
caused by WMLs. Subthalamic nucleus stimulation is similarly
ineffective,59 and physicaltherapy is also of limited value, although wellcontrolled studies have not been conducted. Therapy that minimizes the
vascular risk factors is expected to slow down the rate of decline, but no
studies have yet tested this hypothesis. Atherosclerotic brain disease is
common among elderly individuals, and can also affect patients with
idiopathic PD. These patients with so-called mixed disease are likely to
respond to dopaminomimetic therapy, but to a lesser extent than patients
with idiopathic PD and no atherosclerotic brain disease. An intriguing
therapeutic option that has been suggested for VP is CSF drainage. This
approach is often used to treat normal pressure hydrocephalus (NPH), a
condition with similar symptoms to VP and Binswanger disease. As
mentioned above, patients with VP typically have enlarged cerebral
ventricles, which are also a symptom of NPH. Impairment of gait is also
clinical comparable in VP and NPH. Furthermore, WMLs observed in
Binswanger disease are remarkably similar to those observed in NPH.

Attempts to use CSF drainage to treat patients with VP have produced

positive results. A small doubleblind study published in 2011 supported
these findings, although the follow-up period was just 3 months. The use
of repeated lumbar puncture or placement of a shunting tube for the
treatment of patients with VP remains to be tested in controlled studies
with large groups of participants and long follow-up periods. Such studies
could determine the effectiveness of these approaches, and also indicate
which patients are most or least likely to respond. Other treatment
modalities, including repetitive transcranial magnetic stimulation, have
been suggested. One study has shown that this treatment can improve gait
in patients with VP, but these results should be considered preliminary.

J. Komplikasi
K. Prognosis

DAFTAR PUSTAKA

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