Movement Disorders CPC & Examination of Coordination and Gait Flashcards
Describe the “Extrapyramidal signs” that accompany disorders of the
cerebellum and basal ganglia. How do these differ from “pyramidal”
dysfunction?
Extrapyramidal signs: abnormal movement, posture, or muscular tone, NOT paresis or sensory loss.
Basal Ganglia Disorders:
- tremor (usually resting)
- hypokinetic (rigidity, bradykinesia)
- hyperkinetic (chorea, athetosis, akathisia, dystonia–contrac of ag/antag muscles resulting in fixed, painful, abnormal postures)
Cerbellar Disorders: Synergy (ataxia): (dymetria--past-pointing/overshoot--test w/ finger nose finger and heel shin test, dysdiadochokinesia--impaired rapid alternating movement, decomposition of movement) -Equilibrium (dysequilibrium) -tone (hypotonia) -tremor (usually action) -nystagmus
Other tests:
- pronator drift (pyramidal tract dysfun, parietal lobe dysfunc, cerebellar disease–ipsilateral)
- Speech (Lalala CN12; Papapa CN7;KaKaKa CN10)
Distinguish among primary clinical features of dementing diseases and
movement disorders
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Name the key histologic hallmarks of the “synucleinopathies”
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Romberg test
- tests station/gait
- feet together, eyes closed–>positive with unsteadiness
- pull test–>examiner pulls on pt’s shoulders–>step back=positive
Testing gait
- casual gait
- forced gait (walk on heels, toes, ankles inverted)
- tandem gait (heel to toe): pts w/ truncal ataxia (damaged cerebellar vermis or assoc pathways) will have difficulty esp.
Cerebellar lesion signs
HANDS Tremor Hypotonia Ataxia/Asynergia (3 Ds) Nystagmus Dysarthria Stance and gait Tremor
Examination of tone
(motor exam)
look for:
-rigidity (steady heightened muscular tension present in opposing muscle groups–lead pipe or cogwheel feeling)
- spasticity–velocity dependent increase in tension with passive lengthening
- gegenhalten or paratonia: pt seems to oppose any passive motion
Examination of coord
sppech rapid alt movements hands rapid alt movements precision hand movements foot rapid alt movements rebound check reflex FNF Heel:shin-->toes
What could a positive Rhomberg mean?
- impaired proprioception (DC/Spinal cord)
- Impaired vestibular function (fall toward lesion)
- impaired cerebellum func (mainly vermis or vestibulocerebellum)
Hemiparetic gait
-unilateral UMN injury
-on affected side: arm flexion, adduction, internal rotation
-lower extremity in extension with plantar flexion of the foot
and toes
-When walking, the patient will hold his or her arm to one side and drags
his or her affected leg in a semicircle (circumduction) due to weakness of distal
muscles (foot drop) and extensor hypertonia in lower limb.
Diplegic gait
- spasticity in LE>UE
- NARROW base
- drag both legs, scrape toes
- seen in bilateral periventricular lesions (ie cerebral palsy)
- tight hip adductors–>scissoring gait can occur
Neuropathic gait (steppage gait, equine gait)
seen in pts with foot drop (weakness of foot dorsiflexion)–>attempt to lift leg high enough during walking so the foot doesn’t drag on the floor
- Unilateral: peroneal nerve palsy, L5 radiculopathy
- Bilateral: ALS, CMT, uncontrolled diabetes
Myopathic gait (waddling gait)
- hip girdle muscles keep pelvis level with walking
- weakness on one side leads to drop in pelvis on contralateral side while walking (Trendelenburg sign)
- Bilateral weakness: dropping of pelvis on both sides during walking leading to waddling
- pts w/ myopathies like musc dystrophy
Bradykinetic (Parkinsonian) gait
- rigidity and bradykinesia
- Posture stooped with head and neck forward with flexion at the knees
- slow little steps
- hard to initiate steps
- festination (inclination to take accel steps)
- Parkinson’s disease or parkinsonism
Choreiform gait (hyperkinetic gait)
- central basal ganglia disorders (huntington’s; other forms of chorea, athetosis, dystonia)
- Irregular, jerky movements in all extremities
Ataxic gait
- cerebellar disease
- clumsy, staggering movements with WIDE based gait
- no heel toe walk or straight line
- truncal disability–>think vermis
Sensory gait
- loss of proprioceptive input
- pt slams foot hard on ground to sense it
- exacerbation in dark
- “stomping gait”
- in disorders of dorsal columns (B12 deficiency or tabes dorsalis) or diseases impacting peripheral nerves (diabetes)
Synucleinopathies histologic hallmarks
- Parkinson disease= Lewy bodies, lewy neurites
- multiple system atrophy=axonal and neuronal inclusions; glial cytoplasmic inclusions
Altered alpha-synuclein leading to aggregation
Tau protein deposition diseases (tauopathies)
Neurodegen changes in association with intraneuronal and/or intraglial inclusions composed of abnormally aggregated protein tau.
Tau normally binds to/stabilizes microtubules
-FTD
And primarily dementing disorders:
-Pick’s disease
-Alzheimer’s disease
-frontal lobe degen of non-Alz type
And tauopathies assoc with extrapyramidal disorders of movment:
- PSP
- Cortical basal degeneration
Prion disease
misfolded protein (PrP)
Parkinson’s Disease
- most are sporadic, onset 50-60s
- genes involved: mut in alpha synuclein, also Tau
- loss of pigmented neurons, starting in lower brainstem nuclei (dorsal motor n X, locus coeruleus)
- progressively involving substantia nigra (dopamine)
- residual clusters of pigment
- Lewy bodies
- alpha synuclein (in Lewy body and in neuron processes–Lewy neurites)
- spherical eosionphilic neuronal cytoplasmic inclusions= “lewy bodies”
Diffuse Lewy Body Disease
-more diffuse Lewy body distribution in brain
-can be assoc. w/ dementia
-dementia with lewy bodies= onset of dementia within 1 year of onset of parkinsonism
[PD w/ dementia (PDD)=onset of dementia >1 yr after Parkinsonism onset)
-Diffuse Lewy body disease often co-exists w/ AD related neruitic plaques and neurofib tangles
Multiple System Atrophy
-Neurodegen dis w/ a spectrum of overlapping clinical expression including:
parkinsonism
progressive ataxia
dysautonomia (Shy-Drager syndrome)
-alpha-synuclein
-Degeneration involves mult central systems: striatonigral (MSA-P when park predom) (olivo)pontocerebellar (MSA-C when cerebellar predom) intermediolateral columns (including MSA-P and MSA-C)
Pathologically, defined by glial (esp oligodendroglial) alpha synuclein inclusions (pontine nuc, putamen, STN, limbic crtices, SN, ION, reticular formation)
- Striatonigral degen (Parkinsonism) results in putamenal atrophy
- Loss of striatal neruons w/ gliosis and loss of neuropil
- assoc loss of striato-pallidal and external capuslar white matter tracts
-(olivo)pontocerebellar atrophy characterized by loss of neurons from pontine nuclei–>atrophy of basal pons, loss of myelinated pontocerebellar fibers in MCP. Loss of
pontocerebellar fibers is associated with degeneration of Purkinje cells; possibly via
secondary (transsynaptic) degeneration (with associated gliosis). Also seen is loss of
neurons from inferior olivary nuclei, which may be secondary (retrograde) resulting in
their atrophy with loss of myelinated olivocerebellar (“climbing”) fibers (with associated
gliosis).
-Intermediolateral column degeneration (with associated gliosis) – appears to
underlie dysautonomia seen in a third form of MSA (Shy-Drager syndrome).
Argyrophilic glial cytoplasmic inclusions are Alpha-synuclein- immunoreactive - now
considered a histologic hallmark of MSA
Alzheimer’s Disease
Location: temporoparietal (starts in hippocampus)
Macro: cerebral atrophy
Micro: Abeta plaques, tangles
Huntington disease
Location: basal ganglia Macro: neostriatal atrophy Micro: neuronal loss and astrocytosis -adult onset *40s -Aut dom -choreiform movement disorder -can assoc w/ frontal lobe atrophy -mut gene on chrom 4 (CAG trinuc repeat in huntingtin prot) -anticipation -progressive atrophy of neostriatum, esp in anterior caudate and dorsal putamen; gross atrophy of head of caudate; loss of medium spiny neurons of neostriatum -accomp by astroglial rxn
FTD
location: frontotemporal
Macro: cerebral atrophy
Micro: tau, Pick bodies
PSP
- movement disorder 50s-70s
- degen in nigrostriatal system; in paramedian brainstem and cerebellar roof nuclei; degenerating neruons contain globose, tau, neurofib tangles
- akinesia and rigidity, supranuclear gaze palsy, dysarthria or dysphagia (variable), and possible FTD
- tau (neurofib tangles) in neurons and tau in cortical and subcortical glia (3 of: pallidum, STN, SN, or pons)
- can include frontotemporal cortical atrophy – neruon loss and gliosis (demented)
- 4R tau
Corticobasal degeneration
- movement disorder
- 50-60s
- clumsy, stiff, later rigid and akinetic, alien limb phenom
- frontotemp dementia can occur too
- cererbral cortical atrophy (peri-rolandic)
- Tau inclusion in neruons in substantia nigra, locus ceruleus, tegmental and raphe nuclei, glia; striatum
- “coiled bodies in oligodendroglia; “astrocytic plaques”
- 4R tau
Degenerative ataxias
Hereditary degen ataxias:
- spinocerebellar ataxias
- Freidreich ataxia
Also nonhereditary
- “Primary” cerebellar cortical degen accomp by degen olivocerebellar pathways and often by spinocerebellar tract degen.
- Olivopontocerebellar atrophy can be seen as degen pattern
They tend to present with atrophy of cerebellar folia, involving both cortex
and “digitate” white matter, associated with loss of Purkinje cells (“empty
baskets” and “torpedoes”) and Bergmann gliosis
Friedreich’s ataxia
-rare, hereditary
-b/f 20y old
-AR*****
-chrom 9q, GAA triplet repeat expansion, frataxin prot–>mitochondrial dysfunc
limb ataxia, dysarthria, loss of distal position and vibration sense, upper
limb wasting, areflexia, can have upper motor neuron-like lower extremity weakness
-loss of neurons in DRG, loss of peripheral sensory fibers, and DC fibers, loss of cerebellar afferents, cerebellar efferents from dentate nuc, degen in corticospinal tracts
-loss of neurons in Clark’s column
-anterior horn cells spared
