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Flashcards in Basal ganglia & movement disorders Deck (82):
1

Myoclonus

Rapid jerking movement

2

5 parallel pathways through the basal ganglia

Motor Oculomotor Dorsolateral frontal Lateral orbitofrontal Medial frontal-anterior cingulate

2

Akinesia

Absence of movement; typically used to describe dysfunction that is localized to levels higher than UMNs

2

Torticollis

Focal dystonia involving the muscles of the neck

3

Dementia with Lewy Bodies vs. Parkinson's disease dementia

DLB: onset of dementia w/i year of motor symptoms, fluctuating MS, visual hallucinations

3

Long-term complications of L-dopa treatment

"on-off" fluctuations, "wearing off" phenomena, peak-dose dyskinesia/dystonia, "off" period dystonia/freezing, visual hallucinations & psychoses

4

Chorea

Dance, nearly continuous involuntary movements that have a fluid or jerky, constantly varying quality

5

Dystonia

Sustained muscle contractions, usually resulting in abnormal postures

5

Essential tremor

Rhythmic, non-neurodegenerative, symmetric tremor that is present upon action or postural response

5

Characteristics of progressive supranuclear palsy (PSP)

Predominantly axial parkinsonism, prominent gait disorder with early falls, vertical eye movement abnormalities, dysarthria, dysphagia

6

What are some of the first symptoms of Huntington's disease?

Decreased activity, restriction of interest, subtle alterations in personality, memory, & motor coordination

7

Parkinsonism with early falls suggests

PSP

8

Why do successive generations of Huntington's disease patients develop symptoms at a younger age?

CAG repeat expands with successive generations

9

NP deficits in essential tremor

Mild attention/exec dysfx, language, memory Some evidence that essential tremor may increase risk for dementia

10

Parkinsonism with dystonia suggests

Wilson's disease

11

Common causes of myoclonus

Anoxic brain injury, encephalitis, toxic or metabolic encephalopathies

11

Early neuropsychological deficits in cortical-basal ganglionic degeneration

Attention/executive & language dysfx, memory impairments generally mild with spontaneous recall worse than recognition

11

Amyotrophic lateral sclerosis (ALS)

Confined to the voluntary motor system, affects UMNs & LMNs; often begins focally in muscle groups

12

Lenticular (or lentiform) nucleus

Putamen + globus pallidus

13

Parkinsonism accompanied by early or predominant dementia suggests

Vascular parkinsonism, HD, diffuse Lewy Body disease (DLBD)

15

Spasticity

Slow, clumsy, stiff movements & hyperreflexia resulting from corticospinal, UMN lesions

16

Pathology of Parkinson's disease

Loss of dopaminergic cells in the substantia nigra, projecting to striatum

16

Lifetime incidence of dementia in Parkinson's disease

20-30%, up to 70% will develop dementia after 10 years of motor symptoms

16

Neuroleptic malignant syndrome

Muscle rigidity, hyperthermia, confusion & agitation progressing to somnolence & coma

17

Common comorbidities of Tourette syndrome

ADHD & OCD/behaviors

18

Hallervorden-Spatz disease

Inherited progressive illness with onset in late childhood/early adolescence that is characterized by dementia with spasticity, rigidity, dystonia, or chorea; caused by an accumulation of iron in the brain, particularly the GP & SNr

18

Anteropulsion

Appear to be continually fallowing & shuffling forward (seen in PD)

19

Postural tremor

Most prominent when patient's limb is actively held in a position; essential tremor is most common type

20

Sydenham's chorea

Streptococcal infection leading to the development of antistreptococcal antibodies; antibodies are believed to cross-react with BG brain antigens

20

Neurobehavioral symptoms of Sydenham's chorea

Chorea, facial grimace, ballismus, decreased muscle tone, disruptions of gait & mvmt, attentional & exec deficits, emotional sequelae (anxiety, depression, OCD)

21

Genetics of Huntington's disease

Expansion of the trinucleotide repeat CAG on chromosome 4 (repeats of >39 = HD); 50% chance of inheriting from parent

22

Outputs from the BG to the VL & VA nuclei of the thalamus travel via

Thalamic fasciculus

23

Net effect of the direct pathway

Inhibition of inhibitory BG output leading to increased cortex activation

25

Neuropsychological profile of Parkinson's disease

Slowed psychomotor/info processing speed, early visuoperceptual/constructional deficits, attention/exec dysfx, poor retrieval but intact recognition, depression & apathy

27

Palatal myoclonus is typically caused by a lesion of the

central tegmental tract

29

What is the classic cause of ballismus?

Lacunar infarct of the subthalamic nucleus

30

3 forms of dystonia

Generalized, unilateral, focal

31

What do MRI studies of PSP show?

Atrophy of midbrain & pons, particularly upper midbrain

31

Cognitive changes associated with Huntington's disease

Poor decision-making, cognitive flexibility; mild encoding deficit & moderately impaired retrieval in context of relatively intact memory storage; poor vigilance; visuospatial difficulties; early speech changes (reduced phrase length, impaired fluency)

32

Rubral tremor

Most likely caused by lesions of the superior cerebellar peduncles or other cerebellar circuitry, may also be caused by MS or brainstem infarcts; involves head & trunk titubation or palatal myoclonus

34

Striatum

Caudate + putamen; receives all inputs to the BG

35

Cortical-basal ganglionic degeneration

Combo of cortex (apraxia, cortical sensory deficits, alien limb phenomenon) + basal ganglia involvement (akinesia, rigidity, dystonia)

37

Which subtype of MSA is associated with the most extensive cognitive deficits?

Striatonigral degeneration

38

Botox acts by

Interfering with the presynaptic release of ACH at the neuromuscular junction

39

Onset of PD is most common between the ages of

40 & 70

41

Paratonia (gegenhalten)

Active resistance of movement of limbs; observed in patients with frontal lobe lesions

42

Which subtype of MSA is associated with autonomic disturbance?

Shy-Drager syndrome

45

Palatal myoclonus

Markedly rhythmic & notably persistent during sleep, w/ movements of the palate occurring at a rate of 1-2 hz & ST extending to the face or proximal upper extremities

46

Chorea gravidarum

chorea occurring during pregnancy or while on oral contraceptives; may represent an initial episode or recurrence of SLE or Sydenham's chorea

47

Spastmodic dysphonia

Focal dystonia involving the laryngeal muscles

48

Cogwheel rigidity

Ratchet-like interruptions in tone that can be felt as the limb is bend; characteristic of PD

49

Ballismus

Movements of the proximal limb muscles with a larger-amplitude, more rotatory or flinging quality than chorea

50

Neuropsych profile of PSP

Attention/exec dysfx, slowed processing speed, memory deficits; depression & behavior changes with later stages

51

Main components of the basal ganglia

Caudate nucleus, putamen, globus pallidus, subthalamic nucleus, substantia nigra

52

Athetosis

Twisting movements of the limbs, trunk, face; associated with damage to the contralateral striatum

53

Myasthenia gravis

Autoimmune neuromuscular disorder associated with decreased post-synaptic nicotinic acetylcholine receptors at the neuromuscular junction

54

Myerson's sign

Inability to suppress blinking when the glabella (center of brow) is tapped repeatedly (seen in PD)

56

Benign familial chorea

Autosomal dominant inheritance, nonprogressive, without emotional or cognitive decline

56

Tardive dyskinesia

Nonreversible, involuntary mvmt disorder resulting from dopamine hypersensitivity after long-term neuroleptic therapy

58

Depression can precede the onset of neuro symptoms in Huntington's by

2-20 years

60

Tourette syndrome diagnostic criteria

2+ motor tics & 1+ phonic tic over a 1 year period before the age of 18 years

61

Wilson's disease

Autosomal recessive disorder of biliary copper excretion that cause progressive degeneration of the liver & BG

62

Rigidity

Increased resistance to passive movement of a limb

63

Neurological manifestations of Wilson's disease

Gradual onset dysarthria, dystonia ('risus sardonicus'), rigidity, tremor, choreathetosis, prominent psych disturbances, Kayser-Fleischer rings

64

Net effect of the indirect pathway

Inhibition of cortex

65

Parkinsonism + ataxia suggests

Multiple-system atrophy (olivopontocerebellar atrophy)

66

Motor symptoms of Huntington's disease

Choreiform movement (within a year after onset of psych symptoms), unsteady gait, eye movement abnormalities, difficulty with speech & swallowing

67

Psychosis in Huntington's disease is more common in

Early-adult-onset cases

67

Retropulsion

if the pt is pulled backward slightly, a series of several backward steps are taken to regain balance (seen in PD)

68

Physiological tremor

Believed to be caused by enhancement of normal tremor present in all individuals; can be caused by drugs, meds, metabolic derangements, ETOH withdrawal, intense fear, anxiety, etc.

70

Poliomyelitis

Polio-caused motor neuron virus infecting anterior horn cells of spinal cord & lower brainstem; not associated with cognitive impairment

72

Acute onset of parkinsonism is suggestive of

Vascular or drug-induced parkinsonism

73

Symptoms of Parkinson's disease

TRAP = tremor, rigidity, akinesia, postural instability

74

Blepharospasm

Focal dystonia involving the facial muscles around the eyes

75

Neuropsych profile of juvenile-onset Huntington's disease

Consistent with severe ADHD, decreased cognitive efficiency, mental inflexibility, memory deterioration, changes in engagement & language skills

76

Behavioral changes are the presenting manifestation in what percentage of Huntington's cases?

79%

77

Asterixis

Flapping tremor; known as "liver flap" in hepatic failure

78

Bradykinesia

Slowed movements, often caused by increased inhibitory outflow from the BG to the thalamus

79

3 types of multiple system atrophy

Olivopontocerebellar atrophy - primarily cerebellar features Shy-Drager Syndrome Striatonigral degeneration

80

Where do neurons degenerate in Huntington's disease?

Striatum, which can cause the lateral ventricles to appear enlarged on MRI or CT scans

81

Parkinsonism accompanied by unilateral apraxia & reflex myoclonus suggests

Cortical-basal ganglionic degeneration

82

Hypokinesia

Decreased amount of movements