Neurology Flashcards

1
Q

Causes of bilateral facial nerve palsy

A
  1. Infection
    - meningitis
    - Lyme’s disease
  2. Granulomatous disease
    - Sarcoid
  3. Inflammatory disease
    - guillan barre disease
  4. Trauma
    - base of skull fracture
  5. Malignancy
    - leukaemia
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2
Q

Differential diagnosis of chorea

A
  1. Genetic
    - Huntington’s disease - anticipation of CAG trinucleotide
    - Wilson’s disease - autosomal recessive –> more of a PD picture
    - Ataxic telangiectasia –> more of a cerebellar picture
  2. Drug induced chorea
    - anti-convulsants
    - levodopa
    - anti-psychotics
  3. Biochemical
    - hyperglycaemia
    - polycythaemia
  4. Vascular
    - usually unilateral –> hemi-ballismus: ischaemia in subthalamic nucleas
  5. Systemic conditions
    - SLE
    - Anti-phospholipid
    - Thyrotoxicosis
  6. Pregnancy
    - chorea gravidarum
  7. Post infectious
    - Syndenam’s chorea - post streptococcal infection/rheumatic fever - rare due to use of Abx
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3
Q

Management of Huntingdon’s chorea

A

MDT

  1. Neurologist - to manage symptoms
  2. Neuropsychiatrist - to manage mood + cognitive manifestations
  3. Neuropsychologist - as above and to help deal with devastating diagnosis
  4. Dietician - chorea increases basal metabolic rate and so malnutrition can occur
  5. PT/OT - manage mobility and adapt home + work environment
  6. Clinical geneticist - make diagnosis via blood test to look for trinucleotide repeat of CAG and offer support for diagnosis of family members
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4
Q

Clinical features of polyneuropathy

A
Inspection
- distal symmetrical wasting
- champagne legs in CMT
- arched foot in CMT - Pes Cavus
Tone
- flaccid
Power
- reduced distal >proximal
Reflexes
- reduced/absent
Sensation
- reduced in all modalities including proprioception
Coordination
- not affected
Gait
- high step
- bilateral foot drop
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5
Q

Differential diagnosis of peripheral polyneuropathy

A
  1. Inherited
    - Charcot Marie Tooth
    - CMT1 - most common, autosomal dominant inheritance, demyelinating pathology
    - CMT 2 - autosomal dominant inheritance, axonal (non-demyelinating) pathology
    - CMT 3 - autosomal dominant or recessive inheritance, demyelinating pathology
  2. Nutritional deficiencies
    - B1/thiamine = Beri Beri - seen in alcohol misues
    - B6/pyridoxine - precipitated by isoniazid. Mainly sensory
    - B12 = seen in vegan/vegetarian, pernicious anaemia, ileal disease (Crohn’s). Causes triad of Subacute Combined Degeneration of the Cord + optic neuropathy + dementia
    - Vitamin E deficiency
  3. Systemic diseases
    - Diabetes
    - CKD
    - Paraneoplastic polyneuropathy - small cell lung cancer, myeloma with paraprotein
    - CTD: SLE, rheumatoid arthritis, Sjogren’s
    - Porphyria
    - Sarcoid
  4. Drugs
    - Sensory: isoniazid (B6) + phenytoin
    - Motor: amphotericin + gold + dapsone
    - Sensorimotor: disulfiran + nitrofurantoin + vincristine
  5. Toxins
    - EtOH
    - Lead
    - Arsenic
    - Glue sniffing
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6
Q

Investigations for polyneuropathy

A
  1. Nerve conduction tests
    - differentiate between demyelinating + axonal disease in CMT
  2. Sureal nerve biopsy
  3. Bloods
    - HbA1c
    - Renal function
    - Vitamin levels
    - ANCA, dsDNA, RF, anti-Ro + La, anti-CCP, anti-phospholipid screen, anti-cardiolipin
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7
Q

Differential diagnosis of spinal cord myelopathy

A
  1. Trauma
  2. Compression
    - vertebral disc prolapse
    - spinal abscess - staph aureas, Pott’s disease (TB)
  3. Malignancy - primary or secondary cord compression, intra or extra medullary
  4. Vascular -
    - anterior spinal artery thrombosis - may have preserved dorsal columns (proprioception, fine touch, vibriation)
    - arterio-venous malformation haemorrhage
    - vasculitis - polyarteritis nodosum, syphilis
    - dissecting aortic aneurysm
  5. Inflammatory - transverse myelitis
    - multiple sclerosis
    - SLE
    - post viral demyelination
    - sarcoid
    - Sjogren’s disease
  6. Metabolic
    - subacute combined degeneration of the cord (B12 deficiency) –> loss of dorsal columns first, progresses to corticospinal tracts (UMN signs), preserved spinothalamic tract (anterior columns = pain, temperature)
    - copper deficiency
  7. Intrinsic lesions
    - syringomyelia
  8. Genetic
    - Hereditary spastic paraplegia
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8
Q

Clinical features of MS

A
  1. Optic neuritis
    - loss of central vision
    - painful ophthalmoplegia
    - pale disc
    - relative afferent pupillary defect
  2. Brainstem
    - Internuclear ophthalmoplegia due to demyelination of the medial longitudinal fasciculus (MLF)
    - -> loss of signal from VI to III via MLF - ABducting eye on side of lesion is slow compared to ADducting eye on contralateral side. Results in horizontal nystagmus on contralateral (to lesion) ADducting
    - CN IX + X = dysphagia
  3. Myelopathy
    - UMN lesions –> spastic paraparesis/tetraparesis
    - sensory deficit
    - bladder dysfunction
    - Lhermitte’s symptoms
  4. Cerebellar - ataxia
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9
Q

MS - investigation

A
  1. MRI brain + spine: T2 weighted + Gad - hyperintense + gad enhancing lesions
  2. LP
    - high protein
    - mild lymphocytosis
    - oligoclonal bands (not seen in serum)
  3. Electrophysiology
    - visual evoked potentials + auditory evoked potentials
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10
Q

Cerebellar syndrome - clinical findings

A

Inspection

  • truncal ataxia
  • staccato speech - dysarthria
  • saccadic eye movements
  • head tremor

Tone

  • hypotonia
  • rebound phenomenon: arms outstretched and arms pushed down - exaggerated upshoot of arms

Power
- normal

Sensation

  • normal
  • however Ix for sensory cause of ataxia: proprioception may be affected, Romberg’s positive

Reflexes
- normal

Co-ordination

  • intention tremor
  • past pointing
  • dysdiadochokinesis +ve
  • heel to shin

Eyes
- nystagmus

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11
Q

Cerebellar syndrome - causes

A
  1. Alcohol misuse
  2. Vascular
    - ischaemic or haemorrhage
  3. Malignancy - SOL
  4. Metabolic/nutritional
    - B12 deficiency
    - copper deposition in Wilson’s disease
    - hypothyroidism
  5. Drugs
    - AED
    - Lithium
    - Barbituates
    - Ketamine
  6. Genetic
    - Friedrich’s ataxia
    - Spinocerebellar ataxia
    - Ataxic telangiectasia
    - Von Hippel-Lindau
  7. Degenerative
    - Multi-system atrophy (PD plus syndrome)
  8. Paraneoplastic
    - small cell lung cancer
    - Gynae
    - testicular
    - breast
    - Hodgkin’s
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12
Q

Differences between cerebellar and sensory ataxia

A

Cerebellar

  • dysarthria
  • nystagmus

Sensory

  • proprioception deficit
  • Romberg’s positive
  • able to do coordination with eyes open but difficulty with eyes closed
  • pseudoathetosis (writhing motion of arms/fingers with arms outstretched and eyes closed)
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13
Q

Ulnar palsy - clinical examination

A

Examination:

  • Claw hand: paralysis of interosseous muscles resulting in extension of metacarpophalangeal joints and flexion of interphalangeal joints - most prominent in 4th and 5th fingers
  • scar over ulnar aspect of risk: surgery or trauma
  • check for scar behind elbow - ulnar nerve runs within olecranon groove behind medial epichondyle
  • wasting in small muscles of the hand

Power:

  • small muscles of the hand EXCLUDING LOAF (lumbricals, opponens pollicis brevis, abductor policis brevis, flexor policis brevis
  • spread fingers apart and push in - weakness
  • unable to hold piece of paper between fingers
  • ABLE to keep thumb and forefinger in a ring despite examiner trying to break the ring - power of abductor policis brevis + flexor policis brevis

Sensory:
- loss of sensation/reduction in sensation/hyperalgesia in region of ulnar aspect 1.5 fingers anterior + posterior hand.

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14
Q

Difference between ulnar palsy + C8/T1 radiculopathy

A

Motor:
Abductor pollicis brevis is inervated by C8/T1 but NOT ulnar
- causes ABduction of thumb
Sensory:
C8/T1 innervates ULNAR side of hand/forearm
Ulnar innervates ULNAR side of hand only (not forearm) (1.5 fingers)

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15
Q

Management of neuropathic pain in nerve injury

A
  1. Start with TCA - Amitriptyline

2. Move on to gabapentin/pregabalin

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16
Q

Motor neurone disease - clinical examination

A

Examination:

  • muscle wasting
  • fasciculation - look at tongue “bag of worms”
  • dysarthria

Tone:
- increased tone

Power:
- reduction

Reflexes:

  • brisk + upgoing plantars
  • brisk jaw jerk
  • HOWEVER absent ankle jerk

Sensation
- intact

Coordination
- no sensory deficit but muscle weakness may result in difficulty performing coordination tests.

Gait

  • spastic paraparesis
  • bilateral foot drop
17
Q

Causes of mixed upper + lower motor neurone disease

A
  1. Motor neuron disease
  2. Friedrich’s ataxia
  3. Subacute combined degeneration of the cord
  4. Tabes dorsalis
  5. Cervical radiculomyelopathy secondary to degenerative disease
  6. Dual pathology: myelopathy + peripheral neuropathy
  7. Syringomyelia
18
Q

Types of MND

A
  1. Amyotrophic Lateral Sclerosis - most common form. UMN + LMN findings
  2. Primary lateral sclerosis - only UMN lesions
  3. Progressive muscular atrophy - only LMN lesions
19
Q

Investigation and management of MND

A

Investigation

  • EMG: evidence of UMN + LMN denervation
  • MRI spine to exclude cervical myelopathy

Management = supportive/MDT

  • Respiratory: NIV
  • Nutrition - SALT + dietician assessment of swallowing and intake ?PEG
  • Neurologist: Riluzole shown to prolong life by 3 months (SE: nausea, deranged LFTs)
  • Psychologist
  • OT/PT: mobility, home environment
20
Q

Clinical findings syringomyelia

A

Examination:

  • wasting of small muscles of hand
  • Horner’s syndrome
  • kyphoscoliosis

Tone
- increased

Power
- reduced

Sensation
- cape like dissociative sensory deficit - loss of anterior columns (pain + temp) with preservation of dorsal columns (light touch/vibration/proprioception)

21
Q

Pseudobulbar palsy - definition, symptoms, causes

A

Definition:
Lesions of UMN innervating tongue + oropharynx

Symptoms:
Spastic + hoarse voice
Slow tongue movements
Brisk jaw jerk
Emotional lability

Causes:
MS
Brainstem stroke
MND

22
Q

Bulbar palsy - definition, symptoms, causes

A

Definition:
Lesions of LMN affecting cranial nerves

Symptoms:
Nasal speech
Tongue wasting/fasciculations
Nasal regurgitation

Causes:
Myasthenia gravis
MND
Neuropathy including GBS
Myopathy e.g  spinal + bulbar muscular atrophy (Kennedy's disease)
23
Q

Kennedy’s disease

A

spinal + bulbar muscular atrophy
X linked progressive neuromuscular degenerative disease affecting LMN
Mutation in androgen disease - androgen insensitivity syndrome

24
Q

Causes of facial nerve palsy

A
  1. Supranuclear lesion
    - forehead sparing
  2. Lesion at pons = stroke, MS, tumour
    - also get CN VI involved = facial nerve weakness + lateral gaze palsy + contralateral weakness
  3. Lesion at cerebellar pontine angle = acoustic neuroma meningioma, granulomatous disease
    - CN VIII + CN VII + CN V + CN IX + CN X
  4. Base of skull lesion
    - Paget’s disease
  5. Idiopathic = Bell’s palsy
  6. Ramsey Hunt
  7. Parotid disease
    - tumour/mumps/sarcoid/swelling/trauma
25
Q

Causes of myopathy

A
  1. Inherited
    - Duchenne’s muscular dystrophy
    - -> X linked, absence of dystrophin gene
    - Becker’s MD
    - Limb girdle myopathy
    - Fascio-scapulo-humeral dystrophy
    - McArdle’s syndrome - deficiency in myophosphorylase
  2. Inflammatory
    - inclusion body myositis
  3. Infectious
  4. Endocrine
    - hyperthyroidism
    - cushings
    - acromegaly
  5. Acquired other
    - myotonic dystrophy
  6. Electrolyte
    - deranged calcium
    - hypokalaemia
  7. Drugs
    - statin
    - steroids
    - diuretics causing hypokalaemia
    - amiodarone
26
Q

Myotonic dystrophy clinical features

A

Autosomal dominent - demonstrates anticipation - CTG trinucleotide repeat

  1. Proximal or distal muscle weakness
  2. Myotonia - sustained contraction
  3. Myotonic facies - wasting of temporalis
  4. Frontal balding
  5. Bilateral ptosis
  6. Cataracts
  7. Learning difficulties
  8. Cardiomyopathy
  9. Gynaecomastia
  10. Bronchiectasis
27
Q

Myaesthenia gravis examination

A

Inspection:

  • ptosis
  • snarling facies
  • thymus resection scar

Demonstrate fatiguability

a) Proximal muslces
- move arms like a chicken
b) Bulbar
- listen to speech - nasal sounding
- count backwards from 100
c) ocular
- ophthalmoplegia
- continually blink
- maintain vertical gaze
- peek sign: close eyes –> >30 seconds eyes start to open
d) Respiratory
- test PEF + FVC

28
Q

Cavernous sinus contents

A
  1. CN III
  2. CN IV
  3. CN V - ophthalmic + maxillary bracnhes
  4. CN VI
  5. Internal carotid artery
29
Q

Lateral medullary syndrome/Wallenburgs syndrome clinical features and artery involved

A

Posterior inferior cerebellar artery

Sympathetic tract = Ipsilateral horners
CN V = ipsilateral facial numbness
CN X = ipsilateral bulbar palsy
Cerebellar punducles = ipsilateral ataxia/nystagmus/vertigo
Lateral Spinothalamic tract = contralateral loss of pain and pinprick

30
Q

Weber’s syndrome

Clinical features and location of lesion

A

Midbrain
Ipsilateral CN III palsy - down and out, dilated pupil
Contralateral pyramidal weakness

31
Q

Total anterior circulation stroke clinical features

A

NEED ALL 3 of the following:

  1. Contralateral motor +/- sensory loss of 2 or more of face/arm/leg
  2. Homonymous hemianopia
  3. Higher cerebral dysfunction I.e dysphasia, sensory inattention, apraxia
32
Q

Partial anterior circulation stroke clinical features

A

2 or 3 of following but NO drowsiness

  1. Contralateral motor +/- sensory loss of 2 or more of face/arm/leg
  2. Homonymous hemianopia
  3. Higher cerebral dysfunction I.e dysphasia, sensory inattention, apraxia

OR

Higher dysfunction alone

OR

Motor and or sensory loss <2/3 of whole side of body

33
Q

Lacunar circulation stroke (LAC) clinical features

A

Any of:

  1. Pure motor deficit
  2. Pure sensory (thalamic lesion)
  3. Sensorimotor
  4. Ataxic hemiparesis
34
Q

Posterior circulation stroke clinical features

A
  1. Ipsilateral cranial nerve lesion and contralateral motor/sensory deficits
  2. Bilateral motor and sensory deficits
  3. Disorders of conjugate eye movements
  4. Cerebellar dysfunction without ipsilateral long tract deficits
  5. Isolated homonymous hemianopia