Movement Disorders CPC Flashcards
Describe the basic examination of coordination and gait
Mostly looking for extrapyramidal signs (not weakness or sensory loss)
Examination of muscle tone Speech rapid alternation movements Hand rapid alternating movements Precision hand movements Foot rapid alternating movements Rebound Check reflex Finger Nose Finger Heel: shin to toes
Station and gait: Romberg Pull test Tandem gait Forced gait Casual gait
“Extrapyramidal signs” that accompany disorders of the cerebellum
Synergy (ataxia)- 3 D's (dysmetria, dysdiadochokinesia, decomposition of mvmt) Equilibrium Tone (hypotonia) Tremor (action) Nystagmus
“Extrapyramidal signs” that accompany disorders of the basal ganglia
Tremor
Hypokinetic
Bradykinesia
Mnemonic for cerebellar lesion signs
HANDS Tremor
Hypotonia Ataxia/asynergia (3 D's) Nystagmus Dysarthria Stance and gait
Tremor
Dysmetria
under and over shooting of a target
Dysdiadochokinesia
Trouble with rapid alternating movements (flipping hand on other hand for ex)
Decomposition of movement
Breakdown of a movement into its parts with impaired timing
Rebound
Increased range of movement with lack of normal recoil to original position
Seen in cerebellar disease
Romberg test
Patient stands with feet together, eyes closed
The Romberg is subjective and is deemed “positive” if the patient shows unsteadiness.
Assessment of vestibular, cerebellar, and proprioceptive contributions to balance, with the contribution of vision removed.
Pull test
Examiner pulls back on the patient’s shoulders abruptly (again ready to catch), and any step backwards is deemed positive.
Tandem gait
Heel to toe walking
Forced gait
Patient walks on heels, toes, and with ankles inverted (walking on the outside of the foot)
Pronator drift
Patient stands with eyes closed and arms extended in front with palms facing upward.
A positive result is the affected limb pronating and drifting downward.
Seen with pyramidal tract dysfunction (contralateral cortex), parietal lobe dysfunction (contralateral), or cerebellar disease (ipsilateral).
Hemiparetic gait
Unilateral UMN injury (from a hemispheric stroke for example)
The affected side demonstrates arm flexion, adduction, and internal rotation.
The lower extremity is 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/Paraparetic gait
Spasticity in lower extremities worse than upper extremities
Patient walks with an abnormally narrow base, dragging both legs and scraping the toes.
Seen in bilateral periventricular lesions (cerebral palsy).
Characteristic extreme tightness of hip adductors which can cause legs to cross the midline referred to as a scissoring gait.
Neuropathic gait
Seen in patients with foot drop (weakness of foot dorsiflexion).
Cause of this gait is due to an attempt to lift the leg high enough during walking so that the foot does not drag on the floor.
If unilateral, causes include peroneal nerve palsy and L5 radiculopathy.
If bilateral, causes include amyotrophic lateral sclerosis, Charcot-Marie-Tooth disease and other peripheral neuropathies including those associated with uncontrolled diabetes
Myopathic gait
Hip girdle muscles are responsible for keeping the pelvis level when walking. If you have weakness on one side, this will lead to a drop in the pelvis on the contralateral side of the pelvis while walking (Trendelenburg sign).
With bilateral weakness, you will have dropping of the pelvis on both sides during walking leading to waddling.
This gait is seen in patient with myopathies, such as muscular dystrophy.
Bradykinetic gait
Patient will have rigidity and bradykinesia
Posture is often stooped with the head and neck forward, with flexion at the knees.
The whole upper extremity is also in flexion with the fingers usually extended.
The patient walks with slow little steps known at marche a petits pas (walk of little steps).
Patient may also have difficulty initiating steps. The patient may show an involuntary inclination to take accelerating steps, known as festination.
This gait is seen in Parkinson’s disease or any other condition causing parkinsonism, such as side effects from drugs.
Choreiform gait
Is seen with certain basal ganglia disorders including Huntington’s Disease and other forms of chorea, athetosis or dystonia.
The patient will display irregular, jerky, involuntary movements in all extremities. Walking may accentuate their baseline movement disorder. This is also sometimes called a “dancing” or “Prancing” gait.
Ataxic gait
Most commonly seen in cerebellar disease
Clumsy, staggering movements with a wide-based gait
While standing still, the patient’s body may swagger back and forth and from side to side, known as titubation.
Patients will not be able to walk from heel to toe or in a straight line.
The gait of acute alcohol intoxication will resemble the gait of cerebellar disease.
Patients with more truncal instability are more likely to have midline cerebellar disease at the vermis.
Sensory gait
Loss of this propioreceptive input
In an effort to know when the feet land and their location, the patient will slam the foot hard onto the ground in order to sense it.
Exacerbated when patients cannot see their feet (i.e. in the dark).
Patients may lift their legs very high to hit the ground hard.
This gait can be seen in disorders of the dorsal columns (B12 deficiency or tabes dorsalis) or in diseases affecting the peripheral nerves (uncontrolled diabetes).
In its severe form, this gait can cause an ataxia that resembles the cerebellar ataxic gait.
Diseases with alpha synuclein problems
Parkinsons, Diffuse Lewy Body dementia, and multiple system atrophy
Propagation in neurodegenerative conditions
Taken up by exocytosis and transported back up synapse (retrograde) to cell body and causing cell death
Path seen in parkinson’s
Loss of pigmented neurons beginning in lower brainstem nuclei (dorsal motor n X, locus coeruleus), and progressively involving more rostral pigmented nuclei including the (dopaminergic) nigral neurons which can be evident grossly as loss of nigral pigmentation.
Residual clusters of pigment microglia and astrogliosis become evident in substantia nigra.
Lewy bodies, are characteristic. Alpha synuclein comprises the component filaments in Lewy bodies, and can be demonstrated immunocytochemically.
Alpha synuclein can also be demonstrated in neuron processes
Diffuse Lewy Body disease
Also alpha synuclein problem
More diffuse Lewy body distribution in brain, can be associated with dementia, especially when found in extralimbic neocortices (as well as in brainstem, amygdala and limbic cortices).
Dementia with Lewy bodies (DLB) is a condition characterized by the onset of dementia within a year of onset of parkinsonism. PD with dementia (PDD): onset of dementia more than a year (often up to 10 years) after onset of parkinsonism
Diffuse Lewy body disease often co-exists with AD-related cortical neuritic plaques and neurofibrillary tangles
Progressive Supranuclear Palsy
Characterized by akinesia, and rigidity, usually with characteristic supranuclear gaze palsy; dysarthria or dysphagia variable and frontotemporal dementia can be a feature
Degeneration with neurofibrillary tangles (Tau) in neurons, along with Tau-immunoreactive cortical and subcortical glia - concentrated in at least three of: pallidum, subthalamic nucleus, substantia nigra, or pons.
Corticobasal Degeneration
Patients are clumsy and stiff at first, then rigid and akinetic, sometimes showing ’alien limb’ phenomenon, apraxia, sensorimotor disturbances as well; a frontotemporal clinical dementia syndrome can be prominent.
Astrocytic plaques
Tau inclusions are seen in neurons in substantia nigra, locus ceruleus, tegmental and raphe nuclei; they are also seen in glia; similar changes can also be seen in striatum; subcortical white matter gliosis is not uncommon.
Cerebral cortical atrophy is common and typically peri-rolandic, often asymmetrical (even focal) and associated with variable neuron loss and gliosis, but with prominent achromatic (“ballooned”) neurons in deeper cortical layers
Multiple Systems Atrophy
Alpha synuclein
This is a distinct neurodegenerative disease characterized by a spectrum of overlapping clinical expression, including:
–parkinsonism
–progressive ataxia
–dysautonomia (Shy-Drager syndrome)
Degeneration involves multiple central “systems”:
–striatonigral (results in putamenal atrophy; loss of striatal neurons; loss of associated white matter tracts)
–(olivo)pontocerebellar (Pontocerebellar atrophy; olives can be affected too)
–intermediolateral columns
What degenerates in Huntington’s?
medium spiney neurons in the caudate
Degenerative ataxias
Autosomal recessive
Disease stays in pontocerebellar region (unlike MSA)
Cerebellar cortical degeneration usually accompanied by degeneration in olivocerebellar pathways and spinocerebellar tract degeneration.
In many cases, degeneration of interconnecting pontocerebellar pathways is also present
Thus, olivopontocerebellar atrophy - as a degenerative pattern –can be seen in conditions other than MSA
Friedreich’s Ataxia
One of the degenerative ataxias
Autosomal recessive
Triple repeat expansion
Clinically- limb ataxia, dysarthria, loss of distal position and vibration sense, upper limb wasting, areflexia, can have upper motor neuron-like lower extremity weakness
Pathologically- loss of neurons in dorsal root ganglia with resulting loss of peripheral sensory fibers as well as fibers in dorsal columns; loss of cerebellar afferents, cerebellar efferents from dentate nuclei and degeneration in corticospinal tracts in some cases
Tauopathies
Picks
AD
PSP
Corticobasal degeneration
Which ones give Parkinsonism?
Idiopathic Parkinson’s
Diffuse Lewy Body Disease
PSP
Some MSA
All affect Substantia Nigra compacta
