Neuromuscular Flashcards
1
Q
Define movement disorders
NB: they are defined if unrelated to what?
A
Neurological syndrome in which there is either an excess or deficit of voluntary and automatic movements (unrelated to weakness or spasticity)
2
Q
What are the 4 categories we can class movements as?
A
Automatic: learned motor behaviours done without conscious effort
Voluntary: intentionally or externally triggered
Semi Voluntary: induced by an inner sensory stimulus
Involuntary: most are non-suppressible
3
Q
Give an eg of the 4 classes of movement
A
Automatic eg walking a known route, tapping fingers when doing something else
Voluntary eg turning head towards a loud noise
Semi voluntary eg scratching an itch, tics
Involuntary eg tremors myoclonus
4
Q
What makes up the basal ganglia, what is dysfunctional often associated with?
A
Corpus striatum
Caudate
Putamen
Movement disorders
5
Q
Lentiform nucleus is made up of what?
A
globus pallidus and putamen
6
Q
What is dystonia?
A
Sustained twisting and frequently repetitive, with prolonged abnormal postures (often same muscle groups involved)
7
Q
What is chorea?
A
Involuntary irregular purposeless abrupt rapid and sustained movements that seem to flow from one body part to another
8
Q
What are myoclonic jerks?
A
Sudden brief shock like in voluntary movements caused by muscular contractions (+) or inhibitions (- eg asterixis)
9
Q
What are tics and the 2 types?
A
They are abrupt sudden isolated movements and can be motor or phonic (sounds) 
10
Q
In working out what abnormal movement is occurring what fts do you examine for initially?
A
Is it rhythmic or a rhythmic
is it sustained or not
is it paroxysmal, continual or continuous
is it when asleep or awake
11
Q
Suggest 5 properties of a movement disorder you may want to observe for?
A
- speed eg slow tremor often =Parkinson’s
- amplitude (ballistic/non)
- force (powerful/easy to overcome)
- supressability
- vocalisations
- self-mutilation
- complexity of movements
- sensory component
12
Q
What are the cardinal fts of Parkinsonism (name 4)
A
- tremor at rest
- bradykinesia
- rigidity
- flexed posture of neck, trunk, limbs
- freezing
- loss of postural reflexes
13
Q
Parkinson’s is a primary cause of Parkinsonism and is most common cause, name 2 secondary causes
Clue: msa/snp/cbd
A
- drug induced
- multiple stem atrophy
- supranuclear palsy
- corticobasal degeneration
- spinocerebellar ataxia
- Wilson’s disease
14
Q
What sx may occur in the premotor stage of Parkinson’s
A
- olfactory disturbance
- sleep disturbance
- mood changes
- apathy, fatigue, restless legs
15
Q
Based on premotor Parkinson’s sx, suggest 3 qs you may ask in hx
A
- sleep
- smell
- bowel sx
- mood
- apathy
16
Q
Name 3 non motor sx of Parkinson’s
A
- autonomic: sweating, impotence, constipation, nausea
- cognitive
- pain
- depression, sleep
17
Q
Name 2 genes associated with Parkinson’s
A
a-synuclein
LARK2
MAPT (microtubular associated protein tau gene)
Parkin mutation in recessive forms
18
Q
Madopar and sinemet are examples of what class of anti Parkinson’s drug
A
-levodopa
19
Q
Supranuclear palsy (PSP) is a form of degeneration where?occurs due to what?
A
Fronto-temporal lobar degeneration (FTLDs) due to excessive misfolding of tau protein (microtubule associated protein)
20
Q
The FTLD progressive supranuclear palsy (PSP), name 3 fts (can present like Parkinsonism)
A
- early falls
- axial slowness and stiffness (around neck not limbs)
- staring appearance (frontalis over activity)
- impaired eye movement esp in up gaze
- supranuclear gaze paresis (SNGP)
- poor prognosis
21
Q
MSA (multiple system atrophy) is a degenerative condition, similar pathophys w Parkinson’s but how does prognosis differ?
A
- poor levodopa response
- more rapidly progressive
- get early automatic dysfunction, cerebellar and pyramidal signs, early gait disorder and head drop)
22
Q
Name 3 different drug induced movement disorders:
A
Drug induced Parkinsonism Acute dystonic reactions Akathisia (motor restlessness) Tardive dyskinesia Dopamine agonist withdrawal syndrome
23
Q
What is tardive dyskinesia and what is it associated with
A
- abnormal chewing oral involuntary movements of buccal cavity/tongue
- occurs due to super sensitivity of dopamine receptors due to chronic blockade
- post 3months-yrs of neuroleptic med use
- genetic susceptibility SNP of 5HT2A
24
Q
Treatment of tardive dyskinesia is with..
A
Presynaptic dopamine depleters eg tetrabenazine, clonazepam or atypical antipsychotics
(V difficult to treat tho)
25
Important qs in tremor hx?
- time course (onset, progression, triggers)
- medication exposure (current & past)
- interventions that improve tremor eg alcohol or worsen it eg stress
- family hx and age of onset
- other neuro complaints in immediate family
26
How in examination can you help a rest tremor emerge?
Ask pt to close eyes and count backwards
27
If a rest tremor is activated by use of contra lateral limb what does this suggest re cause
Parkinson’s disease
28
Action tremors could be tested in exam to see if related to what for example (3)
- postural
- isometric (muscle contraction vs immovable object)
- kinetic
- intention (worse as approaching target)
29
When checking if a tremor is isolated or accompanied by other neuro signs, what signs may you look for?
- Parkinsonism
- cerebellar dysfunction
- dystonia eg null point (position where dystonic movements and tremor cease)
30
What is a Holmes Tremor due to?
-lesions in the upper brainstem and cerebral peduncles
31
Axis 1 classification for clinical characteristics of dystonia looks at what 3+ features of dystonia?
And axis 2 uses what 2 fts regarding aeitiology?
-age of onset
-body distribution
-temporal pattern (disease course)
-associated fts (isolated/+other neuro movement disorder)
Axis 2: nervous system pathology and inherited/acquired
32
Adult onset focal dystonia, name 2 conditions of this
- cervical dystonia “Torticollis”
- writers cramp
- spasmodic dysphonia
- oromandibular dystonia
- jaw opening dystonia, lingual dystonia
33
Task specific tremor eg only occurs during writing is sometimes supressed by ____
What rx may help, suggest 1
- alcohol
| - rx: propranolol, primidone, anti-cholinergics, botulinum toxin, DBS
34
What post hypoxic myoclonus? Difference between acute and chronic?
Acute: within 48hrs post hypoxic insult in a deeply comatose pt, poor prognosis eg myoclonic status epilepticus
Chronic: aka Lance Adams syndrome cause unclear, few days post insult, abnormal clonic movements triggered by intentional action, relieved at rest/sleep
35
What is ballismus vs hemi ballismus-where is defect often?
Large amplitude random movements most prominent proximally
| -onesided, often lesion in subthalamic nucleus
36
Most common cause of primary chorea is what?
Huntington’s disease
37
Key fts of Huntington’s disorder
Dominantly inherited
Trinucleotide repeat disorder
Overexpressed huntingtin protein caused problems in basal ganglia
Dementia
Anticipation (age of onset earlier with each generation as repeat size increases)
38
Name 3 causes of secondary chorea
- immune mediated eg Sydenham’s chorea post groupA beta-haemolytic strep (GABHS) that cause rheumatic fever
- SLE
- PCV (polycythemia Vera)
- paraneoplastic syndromes
- vasculitis
39
NB: myoclonus can be associated with what?
Metabolic disorders and brain injury
40
REM sleep disorders in Parkinson’s may include what?
- daytime sleepiness
- acting out dreams
- shouting in sleep
41
Functional neuro disorder can arise from a combo of factors how can we group causative factors (6) clue: PPP
```
Biological
Psychological
Social
Predisposing
Precipitating
Perpetuating
```
42
Personality disorder definition and what spheres can be abnormal?
- when an individuals characteristic and enduring patterns of inner experience and behaviour differ markedly from the norm
- cognition (ways of perceiving/interpreting xyz)
- affectivity (emotional arousal/response in terms of range, intensity and appropriateness)
- control of impulse/gratification of needs
- manner in relating to others
43
Name 3 fts of a personality disorder
Cause significant distress to self/others
Developmental origin
Tends to be lifelong
Marked deviation from cultural norms
Not due to any other mental/medical condition
44
Personality disorders can be classed into clusters A, B and C. What 3 make up cluster A (clue: odd/eccentric/suspicious)
- paranoid
- schizoid
- schizotypal
45
Personality disorders can be classed into clusters A, B and C. What 3 make up cluster B (clue: dramatic/emotional/erratic)
Antisocial
Boarderline
Histrionic
Narcissistic
46
Personality disorders can be classed into clusters A, B and C. What 3 make up cluster C (clue: anxious/fearful)
Avoidant
Dependent
Obsessive compulsive
47
Name 3 fts of Paranoid personality disorder (PD)
- suspicions (misconstrues actions as hostile)
- excessive sensitivity to setbacks
- bears grudges, jealous
- high sense of personal rights
- sense of self importance
48
Name 3 fts of Schizoid personality disorder (PD)
- emotionally cold
- detached and aloof
- lack enjoyment, humourless
- introspective (prone to fantasy)
- lack of intimate relationships, solitary
- insensitive to social norms
- indifferent to praise or criticism
49
Name 3 fts of Schizotypal personality disorder (PD):
| note it is more common in individuals with a FHx of schizophrenia
- inappropriate/constricted affect
- behaviour/appearance that is odd/eccentric
- social withdrawal
- odd beliefs/magical thinking, unusual perceptual experiences
- suspiciousness/paranoid ideas
- oddities of speech
- obsessive ruminations
- transient quasi-psychotic episodes
50
Name 3 fts of Dissocial (aka anti-social) personality disorder (PD):
- callous lack of concern
- short lived, shallow relationships
- irresponsible, depart from social norms
- low tolerance to frustration, and for aggression
- lack remorse/guilt and fail to accept responsibility
51
Name 3 fts of Emotionally Unstable personality disorder (PD): and what are the 2 types? (can have both)
-impulsivity
-affective instability
-minimal ability to plan ahead
-emotional outburst
Types: impulsive or borderline
52
Name 3 fts of Histrionic personality disorder (PD)
- self-dramatisation
- suggestibility
- shallow, labile affect
- inappropriately seductive
- seeks attention/excitement
- over-concern w physical attractiveness
53
```
Name 3 fts of Narcissistic
personality disorder (PD)
(note-doesn't exist in ICD-10)
```
- grandoise self-importance
- fantasies of unlimited success/power
- believe themselves to be special
- requires excessive admiration
- sense of entitlement to favours
- exploits others, lacks empathy
- arrogant, haughty, envious
54
Name 3 fts of Anankastic personality disorder (PD)
| NB this PD is the same as OC PD but is not linked to OCD, it is more linked to depression and anxiety
- preoccupied w details, rules, schedules
- inhibited by perfectionism
- over conscientious
- excessively concerned w productivity
- rigid and stubborn
- pedantic
- excessive doubt and caution
- expect others to submit to their ways
55
Name 3 fts of Anxious/avoidant personality disorder (PD)
- persistent, pervasive tension
- feel socially inferior
- preoccupied with rejection/being criticised
- avoids involvement w unfamiliar people
- restricts lifestyle due to need for security
- avoids social activity
56
Name 3 fts of Dependent personality disorder (PD)
- allow others to make important life decisions
- unduly compliant
- unwilling to make reasonable demands
- feel unable to care for themselves
- need excessive help to make decisions
57
What are the principles of management of personality disorders?
- risk management - self/others
- risk of substance mis-suse/alcohol dependence
- so manage these associated psychological conditions e.g. depression/anxiety
- dialectical behavioural therapy (mindfulness for self-harming)
- CBT, schema focused therapy etc
58
What class of medication may be useful to treat the clusters of PDs?
Class A-eccentric
Class B-dramatic
Class C-fearful
Class A -low dose antipsychotics
Class B-"" mood stabilisers, anti-convulsants, antidepressants
Class C-SSRIs and Venlafaxine
59
5 features of psychogenic/functional movement disorders:
- distraction
- entrainment
- excessive slowness, (thinking more)
- variable
- acute onset (and remission)
- placebo response
60
Management of functional symptoms in neurological practice should involve what?
- exclude organic disease
- pt, family and other drs need to agree
- collaborate with neuros, OT, physios
- assess and treat psychiatric illness
- CBT
- inpt programmes
- avoid unnecessary ix/iatrogenic damage
- stop poss offending medications
61
UMN lesion "pyramidal pattern of weakness" is a problem in the ____-____ tract and the posture is what with relation to upper and lower limbs?
-cortico-spinal tract
-flexors>extensors in upper limb, arm bent in towards chest
-extensors>flexors in lower limbs
NB: Known as spastic weakness
62
A tremor, myoclonus, chorea or dystonia could be due to an extra-pyramidal disorder, this aka ?
- basal ganglia
| - -> movement disorders
63
Clasp knife spasticity suggests a lesion where?
Lead pipe rigidity - where?
Decreased tone - where?
- Clasp knife : UMN lesion
- Lead-pipe rigidity : Extra-pyramidal (basal ganglia)
- Decreased tone : LMN lesion (or muscle)
64
MRC scale
0 - no contraction
5 - normal power
explain 1, 2, 3, 4
1: flicker/trace of contraction
2: active movement w gravity eliminated
3: active movement vs gravity
4: active movement against resistance
65
The classical ft of myopathy is the pattern of weakness which affects...
-name 2 other poss fts:
- proximal weakness > distal
also:
- may have scapular winging (from weakness around shoulder girdle)
- facial weakness
- ocular muscle weakness
- rash
66
Give 3 e.g.s of how reflexes can localise a nerve lesion?
- all decreased may be due to a wide generalised peripheral nerve problem
- all increased may suggest a UMN lesion
- if only 1 absent, all other intact, may suggest a radiculopathy of that nerve only
- babinky's (1st movement of big toe should go down) if goes up - suggest UMN lesion
67
Pain and temperature, which part of CNS responsible?
Spinothalamic tract
68
In cerebellar ataxia you will be unsteady on your feet, what kind of ataxia is masked by your eye input stabilising you and what test is used to reveal it?
- sensory ataxia (due to problems w proprioception)
| - do Romberg's test
69
A lesion affecting which cell may present with mixed UMN and LMN signs? What disease may present this way?
Anterior horn cell (cell body of the LMN that exists in the spinal cord)
e.g. in motor neurone disease: may have wasting and fasciculation but also hyper-reflexia for example
70
Explain how a Pancoast tumour could present and why?
- apical lung tumour compresses nerve root T1 and sympathetic fibres
- weakness in T1 nerve e.g. loss of small muscles of hand power/guttering
- sensory loss in T1 dermatome (over inside of upper arm)
- Horner's syndrome ipsilateral
71
Cauda Equina Syndrome is a LMN lesion, give 4 fts of presentation:
- flaccid paralysis
- lower back pain
- bilateral sciatica
- saddle anaesthesia (s1-5) e.g. can't feel paper when wiping
- urinary and faecal incontinence
- sexual dysfunction
72
What is the main feature of neuromuscular junction problems? Name 2 common diseases
- fatigability
| - Myasthenia gravis, Lambert Eaton, myasthenic syndrome
73
In NMJ diseases what are the following:
- weakness:
- muscles often affected:
- reflexes
- sensation:
- weakness: proximal > distal, fatigable
- muscles often affected: extraocular and face
- reflexes: normal
- sensation: normal
74
In large sensory fibres (Aa, Ab) disease what modalities will be affected vs small fibre disease (A-delta, C)
-joint position sense and vibration in large fibres
-pain and temperature in small fibres
(NB: some neuropathies affect both)
75
Meralgia paraesthetica is a mononeuropathy secondary to compression of which nerve?
-lateral cutaneous nerve of thigh
| causes pain on outer thigh
76
When multiple mononeuropathies occur, the term is _____ ____ and an important cause to consider is____ (fts of this?) -as it's treatable
- mononeuritis multiplex
| - vasculitis (rapidly evolving, multiple nerves. may appear: multifocal/random)
77
Screening bloods for neuropathy: suggest 5
- FBC, ESR, CRP
- U&Es, LFTs, bone profile
- random glucose, HbA1c
- B12, Folate
- serum electrophoresis and Igs
- Vasculitis screen: ANA, ANCA, ENAs, RhF
78
Neurophysiology: axonal/demyelinating findings
| -Nerve Condition Studies:
- NCS:
- demyelinating will have decreased nerve conduction velocity
- axonal will have decreased AP amplitude
79
LP on the background of neuropathy may be done if ____ ___ is suspected, the finding will be no inflamm cells but what?
- Guillain Barre
| - raised protein
80
What is the rx for Guillain-Barre syndrome? NB:steroids do not work
- IVIG
| - Plasma Exchange
81
What inflammatory neuropathy is a variant of Guillain-Barre syndrome with additional: ataxia, ophthalmoplegia, areflexia?
-Miller Fisher Syndrome
82
Charcot-Marie Tooth Disease: presentation
- onset in childhood/teens mostly
- slowly progressive distal symmetrical sensorimotor polyneuropathy
- prominent distal wasting
- pes cavus
83
How to distinguish if foot drop is caused by L4/5 radiculopathy or common peroneal palsy?
- if L4/5: inversion will be weak (and will probs have back pain)
- if CP nerve palsy: inversion is not affected (+hx of compression/trauma)
84
Hence: you decide foot drop is caused by L4/5 radiculopathy rather than common peroneal palsy because inversion is affected, what ix to do?
MRI lumbar spine
85
Neuroglia include:
| -astrocytes: connects neurones and what? Forms the what?
- neurone's and capillaries
| - forms the blood brain barrier
86
What neuroglial cell exists in the peripheral NS and has a supportive function?
Satellite Cells
87
Which neuroglial cell has phagocytic and immunological functions?
Microglia
88
The ependymal neuroglial cells line what?
- the CSF filled ventricles and
| - the central canal of the spinal cord
89
The BBB is formed by what type of neuroglial cell?
Astrocytes
90
Between which layers of the meninges does CSF lie? What is it produced by?
- between pia and arachnoid mater (Sub-arachnoid space)
| - by the choroid plexus in the ventricles
91
Where does the spinal cord terminate?
-L1/L2 at the conus medullaris
91
Where does the spinal cord terminate?
-L1/L2 at the conus medullaris
