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NMS Flashcards

1
Q

right fourth and fifth digit, a right sided foot drop and a left sided facial weakness.

diagnosis

A

mononeuritis multiplex

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2
Q

what is mononeuritis multiplex

A

simultaneous or sequential involvement of individual non-contiguous nerve trunks.

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3
Q

features of mononeuritis multiplex

A

acute or subacute loss of sensory and motor function of individual nerves. The pattern of involvement is asymmetric, however, as the disease progresses, deficit(s) becomes more confluent and symmetrical, making it difficult to differentiate from polyneuropathy.

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4
Q

predominantly motor loss of peripheral neuropathy

A

Guillain-Barre syndrome
porphyria
lead poisoning
hereditary sensorimotor neuropathies (HSMN) - Charcot-Marie-Tooth
chronic inflammatory demyelinating polyneuropathy (CIDP)
diphtheria

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5
Q

predominantly sensory loss of peripheral neuropathy

A 
diabetes
uraemia
leprosy
alcoholism
vitamin B12 deficiency
amyloidosis
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6
Q

alcoholic neuropathy features

A

secondary to both direct toxic effects and reduced absorption of B vitamins
sensory symptoms typically present prior to motor symptoms

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7
Q

vit B12 deficiency features

A

subacute combined degeneration of spinal cord

dorsal column usually affected first (joint position, vibration) prior to distal paraesthesia

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8
Q

what is chiari 1 malformation

A

herniation of the cerebellar tonsils throught the formaen magnum

disturbs the CSF - causing non-communicating hydrocephalus (uncommon) or syringomyelia (common)
- headache

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9
Q

what is syringomyelia

A

dilation of a CSF space within the spinal cord

occurs within the cervical and thoracic segments and causes compression of the spinothalamic tracts decussating in the anterior white commisure

loss of sesation of pain, temperature and non-discriminative touch

cape-like distrubution of the sensory loss

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10
Q

characteristic features of GBS

A

rapidly progressive peripheral neuropathy with lower motor neurone signs (hyporeflexia),

progressive weakness of all four limbs.

  • the weakness is classically ascending i.e. the lower extremities are affected first, however it tends to affect proximal muscles earlier than the distal ones
  • reflexes are reduced or absent
  • sensory symptoms tend to be mild (e.g. distal paraesthesia) with very few sensory signs
  • PAIN - back/leg pain in the initial stages of the illness
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11
Q

other features of GBS

A
  • there may be a history of gastroenteritis
  • respiratory muscle weakness
  • cranial nerve involvement e.g. diplopia
  • autonomic involvement: e.g. urinary retention, diarrhoea
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12
Q

Ix for GBS

A

lumbar puncture

  • > rise in protein
  • > normal white blood cell count (albuminocytologic dissociation) + protein

nerve condution studies may be performed

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13
Q

what is GBS

A

immune-mediated demyelination of the peripheral nervous system often triggered by an infection (classically Campylobacter jejuni).

characterised by

  • paraesthesia
  • weakness
  • hyporeflexia

More common in men
Europe

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14
Q

Tx for GBS

A

-> intravenous immunoglobulin (IVIG) – a treatment made from donated blood that helps bring your immune system under control

plasma exchange (plasmapheresis) – an alternative to IVIG where a machine is used to filter your blood to remove the harmful substances that are attacking your nerves

treatments to reduce symptoms such as painkillers
treatments to support body functions, such as a machine to help with breathing and/or a feeding tube

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15
Q

what is Miller Fisher syndrome

Sx

A

proximal variant of GBS

antiboides angainst the ganglioside GQ1b

Sx
opthalmoplegia
areflexia
ataxia
BUT NOT WEAKNESS
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16
Q

RFs of GBS

A
  • history of GI/Resp infection 1-3 weeks prior to the onset of weakness
  • vaccinations
  • malignancies
  • pregnancy: decreases during pregnancy but increases in the months after delivery
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17
Q

Examination of GBS

A
  • hypotonia
  • demonstrable altered sensation or numbness
  • reduced or absent reflexes
  • fasciculations
  • facial weakness - asymmetrical
  • autonomic dysfunction - fluctuations of heart rate and arryhthmias
  • respiratory muscle paralysis
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18
Q

DDx of GBS

A

barin - stroke, braisntem compression, encephalitis

spinal cord: cord compressions, polymyelitis, transverse myelitis

peripheral nerve: vasculitis, lead poisoning, porphyria

NMJ: MG, botulism

muscle: hypokalemia, polymyositis

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19
Q

complications of GBS

A

respiratory failure

persistent paralysis
hypotension/hypertension
thromboembolism, pneumonia, skin breakdown
cardiac arryhthmia
ileus
aspiration pneumonia
urinary retention
psychiatric problems - anxiety, depression
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20
Q

what is vasculitic neuropathy

A

Patchy motor and sensory loss; pain and dysaesthesia.

Underlying primary vasculitic or rheumatological syndrome

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21
Q

Ix of vasculitic neuropathy

A

NCT may reveal clinically asymptomatic lesions.

Nerve ± muscle biopsy

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22
Q

acute intermittent porphyria clinical features

Ix

A

distal motor neuropathy, HTN, tachycardia

blood and urine analysis

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23
Q

diphtheria

A

oropharyngeal weakness at onset
pharyngeal membrane

NCT axonal neuropathy; serology

24
Q

symptoms of MG

A

EYES
extraocular muscles
- ptosis
- pupils unaffected

BULBAR involvement - dysphagia, dysphonia, dysarthria

limb weakness
- proximal and symmetrical

resp

weaker thruout day

face weakness

limbs and trunk are less affected

no ANS involvement

muscle atrophy rare but disuse atrophy possible

abnormalities to thymus 
ass with autoimmune
RA 
SLE
Graves
Pernicious anaemia
25
complications of MG
resp failure myasthenic crisis - drooling, weak neck, slack facial muscles
26
Ix for MG
tensilon test EMG
27
what is myasthenic crisis
``` Slack facial muscles Weak neck Drooling Nasal speech Generally weak Unsafe swallow ```
28
cholinergic crisis?
Looks similar to myasthenic crisis. Excess of acetylcholinesterase inhibitors excessive stimulation of striated muscle - flaccid paralysis Respiratory failure Miosis and the SLUDGE syndrome (ie, salivation, sweating, lacrimation, urinary incontinence, diarrhea, GI upset and hypermotility, emesis) also may mark cholinergic crisis-not inevitably present
29
what is tensilon test
allows MG patient to move muscles normally
30
cautions for tensilon test
Caution – asthma, MI, bradycardia | Cardiac monitoring
31
normal functions of the frontal lobe
- > primary motor cortex: precentral gyrus, opposite side of the body - > coordinating and planning complex movements - > frontal eye field -> eye movements to the contralateral side - > Broca's area - motor or expressive centre of speech - > personality, emotional expression, initiative and the ability to plan - > inhibition of voiding of the bladder and bowel
32
Sx from lesions of the frontal lobe
contralateral weakness - mono/hemiparaesis and facial weakness in an UMN pattern gait apraxia - slow, shuffling, upright and wide based conjugate eye deviation - both eyes look towards the side of the lesion and away from the side of the weakness focal seizures -clonic movements of the contralateral lower face, arm and leg - away from the side of the lesion expressive dysphasia - non-fluent, hesitant speech ``` personality & behavioural change - social disinhibition loss of initiative and interest - inability to solve problems - impaired concentration and attention ``` anosmia primitive reflexes - grasping, sucking, pouting rooting and palmomental incontinence of urine/faeces
33
function of parietal lobe
primary somatosensory cortex - postcentral gyrus language (dominant hemisphere) use of numbers non- dominant hemisphere - allows awareness of the body and its surroundings, appropriate movement of the body and constructional ability visual pathways - inferior quadrantanopias
34
Sx from lesions of the parietal lobe
contralateral sensory loss - impairment of joint position and two point discrimination - recognise objects by form and texture visual disturbacnes
35
syndromes of the dominant parietal lobe
wernicke's receptive 'fluent' dysphasia - imparied comprehension of speech and written language gerstmann's syndrome - cant differentiate between left and right sides of the body, impairment of calculation/writing bilateral ideomotor adn ideational apraxia
36
syndromes of the non-dominant parietal lobe
contralateral sensory inattention constructional apraxia - cant draw simple tings dressing apraxia topographical disorientation -> unfamiliar with normal places
37
function of temporal lobe
wernicke's area - comprehension of written and spoken language auditory and vestibular cortices limbic system - olfactory and gustatory cortices -> medial temporal lobe - memory, learning
38
# define pyramidal weakness proximal weak
LOP in the extensor muscles in the arms and the flexors in the legs UMN lesions involving ht epyramidal tract proximal - affecting shoulders, hips, trunk, neck and sometimes face - characteristics - myopathy and NMJ (MG) distal - hands and feet - peripheral motor neruopathy
39
clinical features of a UMN lesion
spasticity - clasp knife, clonus tendon reflexes are brisk positive babinski sign
40
what is tetraparaesis
weakness in all four limbs - brainstem - high cervical cord - -- cervical spondylosis - -- MS - -- traumatic cord lesions
41
types of LMN lesion
anterior horn cell spinal nerve plexus peripheral nerve
42
characteristics of LMN lesions
- decreased tone - focal pattern of weakness and wasting - fasciculations - pain and sensory disturbance - nerve roots, plexi and peripheral nerves - reduced tendon reflexes and flexor plantar responses
43
focal pattern of weakness and wasting in LMN - anterior horn - radiculopathy - plexopathies - peripheral neuropathies - NMJ disorders - myopathies
anterior horn cell disease - generalised weakness, wasting - mimmin peripheral nerve lesion radiculopathies - weakness and wasting in the respective myotomes plexopathies - weakness and wasting in the plexus region peripheral neuro - mono - single peripheral nerve - multiple mononeuropathies - many single nerves - polyneuropathy - longest anxon in all nerves affected -SYMMETRICAL , GLVIE AND SOCK SENSATIONS NMJ - MG myopathies - symmetrical wasting of proximal muscles
44
types of peripheral neuropathy
mononeuropathy - ie median nerve multiple mononeuropathy - mononeuritis complex polyneuropathy - vit B12, Diabetic neuropathy, drugs isoniazid
45
disorders of the NKJ
MG Eaton-Lmabert iatrogenic
46
features of myopathy
limb weakness bilateral and proximal cant stand from sitting, climbing stairs dysphagia, respiratory muscle weakness myalgia esp after exercise
47
what is tremor
ivoluntary and rhythmic - disappear during sleep - fine - low amplitude - coarse - high amplitude
48
define intention tremor
tremor that increases throughout the movement
49
define physiological tremor
present during waking bilateral worse on maintaining a posture fine and fast
50
define pathological tremor
occurs at rest or with movement slower coarse | asymmetrical
51
what is resting termor
coarse 'pill-rolling' tremor - disappears during voluntary movement - asymmetrical
52
what is essential tremor
coarser AD the tremor may worsen terminally but not throughout temporary improvment with alchol Mx beta-blockers, anticholinergics, primidone
53
what is a kinetic tremor types
occurs during movement cerebellar tremor - coarse often slow absent a t rest
54
what is chorea
involuntary movements, abrupt and jerky ``` causes huntingotns SLE HIV thyroid ```
55
Mx of huntington
dopamine blocking or depleting but results in parkinsons CAG chromosome 4

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