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ABPP > Basal ganglia & movement disorders > Flashcards

Basal ganglia & movement disorders Flashcards

1
Q

Myoclonus

A

Rapid jerking movement

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2
Q

5 parallel pathways through the basal ganglia

A

Motor Oculomotor Dorsolateral frontal Lateral orbitofrontal Medial frontal-anterior cingulate

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2
Q

Akinesia

A

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

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2
Q

Torticollis

A

Focal dystonia involving the muscles of the neck

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3
Q

Dementia with Lewy Bodies vs. Parkinson’s disease dementia

A

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

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3
Q

Long-term complications of L-dopa treatment

A

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

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4
Q

Chorea

A

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

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5
Q

Dystonia

A

Sustained muscle contractions, usually resulting in abnormal postures

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5
Q

Essential tremor

A

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

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5
Q

Characteristics of progressive supranuclear palsy (PSP)

A

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

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6
Q

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

A

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

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7
Q

Parkinsonism with early falls suggests

A

PSP

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8
Q

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

A

CAG repeat expands with successive generations

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9
Q

NP deficits in essential tremor

A

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

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10
Q

Parkinsonism with dystonia suggests

A

Wilson’s disease

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11
Q

Common causes of myoclonus

A

Anoxic brain injury, encephalitis, toxic or metabolic encephalopathies

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11
Q

Early neuropsychological deficits in cortical-basal ganglionic degeneration

A

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

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11
Q

Amyotrophic lateral sclerosis (ALS)

A

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

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12
Q

Lenticular (or lentiform) nucleus

A

Putamen + globus pallidus

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13
Q

Parkinsonism accompanied by early or predominant dementia suggests

A

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

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15
Q

Spasticity

A

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

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16
Q

Pathology of Parkinson’s disease

A

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

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16
Q

Lifetime incidence of dementia in Parkinson’s disease

A

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

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16
Q

Neuroleptic malignant syndrome

A

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

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17
Q

Common comorbidities of Tourette syndrome

A

ADHD & OCD/behaviors

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18
Q

Hallervorden-Spatz disease

A

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

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18
Q

Anteropulsion

A

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

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19
Q

Postural tremor

A

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

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20
Q

Sydenham’s chorea

A

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

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20
Q

Neurobehavioral symptoms of Sydenham’s chorea

A

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

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21
Q

Genetics of Huntington’s disease

A

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

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22
Q

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

A

Thalamic fasciculus

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23
Q

Net effect of the direct pathway

A

Inhibition of inhibitory BG output leading to increased cortex activation

25
Q

Neuropsychological profile of Parkinson’s disease

A

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

27
Q

Palatal myoclonus is typically caused by a lesion of the

A

central tegmental tract

29
Q

What is the classic cause of ballismus?

A

Lacunar infarct of the subthalamic nucleus

30
Q

3 forms of dystonia

A

Generalized, unilateral, focal

31
Q

What do MRI studies of PSP show?

A

Atrophy of midbrain & pons, particularly upper midbrain

31
Q

Cognitive changes associated with Huntington’s disease

A

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
Q

Rubral tremor

A

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
Q

Striatum

A

Caudate + putamen; receives all inputs to the BG

35
Q

Cortical-basal ganglionic degeneration

A

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

37
Q

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

A

Striatonigral degeneration

38
Q

Botox acts by

A

Interfering with the presynaptic release of ACH at the neuromuscular junction

39
Q

Onset of PD is most common between the ages of

A

40 & 70

41
Q

Paratonia (gegenhalten)

A

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

42
Q

Which subtype of MSA is associated with autonomic disturbance?

A

Shy-Drager syndrome

45
Q

Palatal myoclonus

A

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
Q

Chorea gravidarum

A

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

47
Q

Spastmodic dysphonia

A

Focal dystonia involving the laryngeal muscles

48
Q

Cogwheel rigidity

A

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

49
Q

Ballismus

A

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

50
Q

Neuropsych profile of PSP

A

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

51
Q

Main components of the basal ganglia

A

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

52
Q

Athetosis

A

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

53
Q

Myasthenia gravis

A

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

54
Q

Myerson’s sign

A

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

56
Q

Benign familial chorea

A

Autosomal dominant inheritance, nonprogressive, without emotional or cognitive decline

56
Q

Tardive dyskinesia

A

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

58
Q

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

A

2-20 years

60
Q

Tourette syndrome diagnostic criteria

A

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

61
Q

Wilson’s disease

A

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

62
Q

Rigidity

A

Increased resistance to passive movement of a limb

63
Q

Neurological manifestations of Wilson’s disease

A

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

64
Q

Net effect of the indirect pathway

A

Inhibition of cortex

65
Q

Parkinsonism + ataxia suggests

A

Multiple-system atrophy (olivopontocerebellar atrophy)

66
Q

Motor symptoms of Huntington’s disease

A

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

67
Q

Psychosis in Huntington’s disease is more common in

A

Early-adult-onset cases

67
Q

Retropulsion

A

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

68
Q

Physiological tremor

A

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
Q

Poliomyelitis

A

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

72
Q

Acute onset of parkinsonism is suggestive of

A

Vascular or drug-induced parkinsonism

73
Q

Symptoms of Parkinson’s disease

A

TRAP = tremor, rigidity, akinesia, postural instability

74
Q

Blepharospasm

A

Focal dystonia involving the facial muscles around the eyes

75
Q

Neuropsych profile of juvenile-onset Huntington’s disease

A

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

76
Q

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

A

79%

77
Q

Asterixis

A

Flapping tremor; known as “liver flap” in hepatic failure

78
Q

Bradykinesia

A

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

79
Q

3 types of multiple system atrophy

A

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

80
Q

Where do neurons degenerate in Huntington’s disease?

A

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

81
Q

Parkinsonism accompanied by unilateral apraxia & reflex myoclonus suggests

A

Cortical-basal ganglionic degeneration

82
Q

Hypokinesia

A

Decreased amount of movements

ABPP (34 decks)

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