Download Movement disorders

Movement disorders
(Extrapyramidal and cerebellar syndromes)
http://vyuka.lf3.cuni.cz
(seminar, teaching unit 60)
Assoc. Prof. Ales BARTOS, MD, PhD
Department of Neurology,
Charles University in Prague
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The questions for the oral state exam
in neurobehavioral sciences :
• Parkinson´s disease and syndromes: causes, symptoms,
diagnosis, treatment
(a neurology part)
• Disturbances of motor functions, including drug-induced
syndromes
• Vascular dementia: clinical signs and symptoms,
classification, differential diagnosis, treatment and
prevention
• Dementia: clinical signs and symptoms, classification,
differenial diagnosis
2
The questions for the oral state exam
in neurobehavioral sciences :
• Hydrocephalus: types, causes, symptoms, diagnosis,
treatment. CSF hypotension
• Nervous system disorders in metabolic and autoimmune
diseases, CO toxicity: causes, symptoms, diagnosis,
treatment, complications
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Outline
• movement disorders in general
• case history and examination focused on movement
disorders
• the Parkinsonian syndrome
• Parkinson´s disease
• secondary parkinsonian syndromes
and a surprise in the end: questions and quizzes …
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Movement disorders
• observed during the interview („one look is worth a
thousand words“)
• abnormal involuntary movements are exaggerated with
anxiety and diminished during sleep
2 categories - syndromes:
1) Akinetic-rigid – paucity of spontaneous movement,
increased tone (rigidity)
examples: Parkinson´s disease, vascular encephalopathy / dementia
2) Hyperkinetic-hypotonic – irregular involuntary
movements, often at varying sites, diminished muscle tone at
rest (chorea, athetosis, ballism, dystonias)
example: Huntington´s disease
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Types of abnormal movements
• Tremor = involuntary, rhythmic movement across
a joint:
1) rest – at rest
2) static, postural – only with a specific posture
3) intention – only with motion
• Chorea = sudden movement usually involving the
extremities and the face
• Athetosis = a slow, sinuous movement
(the distinction is sometimes unclear – choreoathetosis)
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Types of abnormal movements
• (Hemi-)ballism = irregular, sudden, large-amplitude
movements of an entire limb or limbs
• Myoclonus = spontaneous, shocklike contractions of
one or more muscles across a joint (the jerk-like
movements during falling asleep in dogs, cats and man)
• Tics = irregular, stereotyped movements, often in the
face
• Dystonia = a sustained abnormal or inappropriate
posture
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History of the patient
with a movement disorder
• symmetry, exacerbating or alleviating factors (stress,
sleep, alcohol)
• family history – many movement disorders
• drug history (!) – neuroleptics → parkinsonism, tardive
dyskinesias; antiemetics ! → dystonia, dyskinesia
• alcohol history → cerebellar degeneration / dysfunction,
Wernicke encephalopathy, alleviate essential tremor
• medical history – hyperthyroidism → tremor, liver
disease → asterixis
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Neurologic examination
• have the patient draw a spiral or connect dots as a
permanent record of the motor dysfunction; later this
can be used to monitor the efficacy of treatment
• muscle tone: hypertonia – a passive movement
of the arm
• abnormal postures – during station and gait testing
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The Parkinsonian SYNDROME
(parkinsonism)
= movement abnormalities mainly characterized by:
1) bradykinesia, hypokinesia, 2) rigidity and 3) resting
tremor, 4) postural abnormalities (stance and gait)
• based on pathology in basal ganglia and their
connections
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The Parkinsonian syndrome
BRADYKINESIA, HYPOKINESIA („slow
movements“):
– slowness of walking, other movements (and
mental processes), delayed responses physically
and mentally
– difficulty in initiating movements
– slow and smaller handwriting
– reduced facial reactions („masked face“)
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The Parkinsonian syndrome
RIGIDITY (increased tone):
– passively move the extremities
– increased tone as increased resistance
(cogwheel phenomenon)
– !! (versus spasticity)
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The Parkinsonian syndrome
TREMOR:
– at rest
– most commonly in hands and fingers
(a „pill rolling“ motion)
– often begins unilaterally and distally
– spreads proximally and to the other side over
months or years
– usually asymmetric
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The Parkinsonian syndrome
POSTURAL CHANGES during station and gait :
– slight flexion of back
– the first steps are slow and small
– difficult turning
– imbalance
– falls
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The Parkinsonian syndrome
OTHER FEATURES:
– micrographia
– dysarthria
– cognitive slowing
– mood lability
– dementia
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Causes of parkinsonism
the problem to distinguish in a patient with parkinsonism:
1) Idiopathic Parkinson´s disease (80 %)
2) Secondary parkinsonian syndromes (20 %)
 vascular parkinsonism
rarer causes:
 drug-induced (dopamin receptor blockers: neuroleptics, antiemetics)
 Wilson´s disease
 normal pressure hydrocephalus
 carbon monoxide poisoning
 trauma
 post-encephalitic parkinsonism (in the past)
 parkinson plus syndromes (corticobasal syndrome, progressive
supranucelar palsy, Lewy body disease)
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Parkinson´s DISEASE (vs. syndrome)
• chronic, slowly progressive, degenerative
and usually sporadic disease
a) loss of dopaminergic neurons pars compacta
substantiae nigrae – lack of dopamin
b) Lewy bodies – deposits of alfa-synuclein
(synucleinopathy)
• in old age over 50 years, prevalence rises with
age
the lecture on Parkinson´s disease: http://www.youtube.com/watch?v=xRbdzgKFl10
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Parkinson´s disease in pictures
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Parkinson´s disease – clinical features
the parkinsonian syndrome
– „everything is hypo- (reduced)“:
• upper limbs – hypokinesia and rigidity,
micrographia, reduced synkinesias during gait
• gait disorders – steps: too small, too slow,
freezing, difficult turning
• face – hypomimia
• speech – hypofonia, dysarthria
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Parkinson´s disease – other clinical features
• rest tremor – very typical, not in all patients
• depression
• cognitive disturbances (attention, executive
functions), dementia in late phases
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Parkinson´s disease – diagnosis
• the diagnosis is based on clinical evaluation in 3 steps:
1) the presence of hypokinetic-rigid syndrome and other
features typical for Parkinson´s disease
2) red flags – suspect an alternative diagnosis – features for
secondary parkinsonism: repeated strokes and step-wise onset,
neuroleptics, supranuclear gaze palsy, cerebellar syndromes, early
severe dementia, Babinski sign, brain tumor or hydrocephalus on CT
scanning, absence of dopa responsiveness
3) the response to dopaminergic treatment
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Parkinson´s disease – treatment
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Parkinson´s disease – treatment
a replacement of decreased dopamine concentration:
• levodopa – is the most effective treatment, most patients
have a remarkable response to levodopa, commonly used if
patients have Parkinson´s disease
and / or
• dopaminergic agonists (bromocriptine, pregolide, ropinirole,
cabergoline, pramipexole) – usually a weaker effect and more
peripheral adverse effects (blood pressure fluctuations,
arrhythmias, nausea, vomiting)
•„start low, go slow“
• deep brain stimulation (neuropacemaker)
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Break
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Secondary parkinsonian syndromes
frontal type of gait (lower body parkinsonism) – symmetrical
involvement of lower limbs, more than relatively spared upper limbs:
• vascular parkinsonian syndrome – multiple, repeated
strokes in basal ganglias (postsynaptic damage, sometimes
effective levodopa) – step-wise progression, Babinski +, axial
phenomena, pseudobulbar syndrome, cognitive deficit,
dementia
• normal pressure hydrocephalus – triad:
1) gait disorder, 2) cognitive deficit, 3) urine incontinence
treatable condition !
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Vascular parkinsonian syndrome
bilateral lesions!
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Normal pressure hydrocephalus
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Drug-induced secondary parkinsonism
(contraindicated in Parkinson´s disease)
• dopamine receptor blockers:
– typical neuroleptics (fenothiazines – chlorpromazine,
butyrophenons – haloperidole)
– antiemetics (neuroleptics) – metoclopramid
• calcium channel blockers – flunarizine, cinnarizine
 treatment of delirium in Parkinson´s patients
with ATYPICAL antipsychotic drugs - tiapride
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Wilson´s disease
(=hepatolenticular degeneration)
• a genetic autosomal recessive disorder of copper
metabolism
• „parkinsonism in the young“
• progressive, „wing-beating“ tremor, rigidity,
dysarthria, mental changes, hepatic dysfunction,
corneal Kayser-Fleischer ring
• less commonly tremor
• treatment: penicillamine, zinc sulfate
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Wilson´s disease
(=hepatolenticular degeneration)
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Wilson disease in a 14-year-old girl with dysarthria.A, Initial T2-weighted axial MR image
shows increased signal intensity in both caudate nuclei (arrowheads) and putamen
(arrows).B, Follow-up T2-weighted axial MR image obtained after 3 years shows complet...
Kim T et al. AJNR Am J Neuroradiol 2006;27:1373-1378
©2006 by American Society of Neuroradiology
Treatment
of secondary parkinsonian syndromes
• vascular parkinsonism – controlling vascular risk factors,
rehabilitation
rarer causes:
• normal pressure hydrocephalus – ventriculo-peritoneal
shunt
• drugs (neuroleptics, antiemetics) – anticholinergic agents,
atypical antipsychotics
• Wilson´s disease – penicillamine, zinc
• post-traumatic, carbon monoxide poisoning rehabilitation
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Treatment differences
in two parkinsonian syndromes
idiopathic Parkinson´s disease
vascular parkinsonism
• presynaptic disease
• postsynaptic (presynaptic ?) disease
• low dopamine concentrations
• normal dopamine concetrations
• dopaminergic responsiveness
• no effect of dopaminergic drugs
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Movement disorders in broader sense
• Chorea = sudden movement usually
involving the extremities and the face
• Ataxia = an impairment of coordination in
the absence of significant weakness
1) limb
2) truncal
= cerebellar disorders
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Chorea
• irregular, asymmetric, sudden, brief, shooting
involuntary movements
• in distal segments of the extremities
• grimacing and lip-smacking
35
Huntington´s disease
• autosomal dominant inheritance
• expanded CAG repeat on the chromosome 4
• longer CAG repeat – earlier age of onset
• age: 30-50 years
• presymptomatic diagnosis by genetic testing
• very poor prognosis: death after 10-15 years from
the diagnosis
• no etiologic treatment, symptomatic management
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Hemiballism
• „ballistic“ movements of high amplitude involving
multiple segments of a limb
• cause: stroke in the subthalamic nucleus
• neuroleptics, but usually resolves spontaneously
in a few weeks
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Cerebellar disorders
syndromes:
– neocerebellar (limb ataxia, intention tremor, dysdiadochokinesia)
– palleocerebellar (truncal ataxia, unstable stance in the Romberg
test, unstable, broad-based gait, dyssynergy)
– mixed (global)
• an important neuroanatomical association of the cerebellum
with the rest of the brain
38
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Causes of cerebellar disorders
VINDICATE:
• Vascular (stroke)
• Inflammatory (encephalitis)
• Neoplastic (tumors – meningeoma, gliomas –
oligodendroglioma, astrocytoma)
• Degenerative (spinocerebellar ataxias, paraneoplastic
syndromes)
• Intoxication (alcohol, Wernicke´s encephalopathy,
antiepileptics, lithium)
• Congenital – Arnold-Chiari, agenesis
• Autoimmune (multiple sclerosis, paraneoplastic
cerebellar degeneration in ovarian cancer)
•Traumatic (contusion, sub- / epi- dural hematomas)
• Endocrine (metabolic – see intoxication)
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Wernicke´s encephalopathy
• conditons due to thiamin deficiency
• even in nonalcoholics, e.g. in patients vomiting, poor
nutrition
• oculomotor disturbances (abducent palsy, nystagmus, gaze palsy)
• ataxia (more truncal than limb, unable to stand or walk)
• antero- and retrograde amnesis + confabulations
• brain MR: lesions in periaqueductal region and adjacent to
the third ventricle (demyelination, small foci of hemorrhage)
• treatment: thiamin
• glucose solutions after thiamin has been given, otherwise it
may induce acute worsening of Wernicke´s encephalopathy
• early treatment – regression, often residual deficits
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Wernicke´s encephalopathy
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Figure. Rapid clearing of MRI signals in Wernicke encephalopathy.
Watson W et al. Neurology 2003;61:527
© 2013 American Academy of Neurology
Let us verify your knowledge….
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Quizzes
• distinguish different types of tremor
• identify the type of abnormal movements
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Quiz question No 1
The most common cause of the parkinsonian
syndrome is:
a) repeated and multiple strokes
b) drug abuse
c) neuroleptics
d) Parkinson´s disease
e) carbon monoxide poisoning
46
Quiz question No 2
Dopaminergic transmission is impaired in
Parkinson´s disease due to:
a) blockage of dopamin receptors
b) decreased presynaptic uptake
c) presynaptic degeneration and loss of
dopaminergic neurons
d) degeneration of nigrostriatal pathway
e) postsynaptic degeneration and loss of
dopaminergic neurons in striatum
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Quiz question No 3
Parkinsonism prevailing in the upper part of the body:
a) may indicate a treatable condition – normal
pressure hydrocephalus
b) is typical for Parkinson´s disease
c) may be found in vascular encephalopathy
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Quiz question No 4
The onset of the parkinsonian syndrome:
a) under the age of 45 years is impossible
b) in the 7th and 8th decade is usual for Parkinson´s
disease
c) between 50 and 60 years is typical for vascular
encphalopathy
d) under the age of 45 may be caused by rare
disease – which one?
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Quiz question No 5
A response to levodopa or dopaminergic agonists
(motor improvement):
a) is found almost exclusively in Parkinson´s disease
b) is profound in the vascular parkinsonian syndrome
c) is supportive for the diagnosis of Parkinson´s disease
d) is typical for all parkinsonian syndromes
(Why?)
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Quiz question No 6
Parkinsonian features prevail in lower part of the body. Which
statement is true?
a) dopaminergic treatment will be probably effective
b) it is typical in early Parkinson´s disease
c) clinical evaluation is insufficient, brain neuroimaging is not necessary
d) it suggests an alternative diagnosis
(Why?)
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Overlapping features
of various types of tremor
Feature
Parkinson´s
syndrome
Essential
tremor
Cerebellar
tremor
Rest tremor
++
+
0
Postural /
static tremor
Intentional
tremor
+
++
0/+
0
+
++
Increased
tone
Postural
abnormality
+
0
0
+
0
+
Incoordination
0
0
+
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