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Flashcards in Neurology Deck (46):
1

What are the pathologies of peripheral neuropathy?

  1. Demyelination e.g. Guillain-Barre syndrome
  2. Axonal degeneration e.g. toxic neuropathies
  3. Wallerian degeneration e.g. direct trauma
  4. Compression e.g. carpal tunnel syndrome
  5. Infarction e.g. diabetes and vasculitis
  6. Infiltration / inflammation from infection (leprosy), malignancy, granulomatous disease (sarcoid) or amyloidosis

2

Causes of polyneuropathy / peripheral neuropathy?

  1. Neurological: GBS, Cauda Equina, CIDP
  2. Endocrine: DM, Hypothyroidism, Acromegally
  3. Infectious: HIV, HepC, Leprosy, Lyme
  4. AI: SLE, Sjogrens
  5. CRF
  6. Nutritional: Thiamine, B12,
  7. Drugs: amiodarone, metronidazole, cisplatin
  8. Genetic: CMT
  9. Alcohol
  10. Neoplastic

3

Peripheral neuropathy investigations

  • Full blood count.
  • Fasting glucose level and cholesterol panel.
  • Thyroid stimulating hormone.
  • Vitamin B12 levels.
  • Serum and urine immuno-electrophoresis.
  • Hepatitis B and C, HIV, Lyme, and syphilis serologies.
  • ESR, antinuclear antibodies (including SS-A and SS-B), extractable nuclear antibodies.
  • Nerve conduction studies and needle EMG.
  • Possible sural nerve biopsy

4

Hemiplegia differential

Eldery: stroke, tumour, subdural haematoma, demyelination

Young: demyelination, stroke, tumour

Also, traumatic injury, hypoglycaemia, focal nerve palsy, post-ictal paralysis, cerebral absess

5

Hemiplegia investigations

  • BP
  • FBC (polycythemia, platelets, raised WCC)
  • Coagulation
  • U+Es (CRF)
  • Glucose, cholesterol, ESR, CRP
  • ECG (AF)
  • Chest xray (lung cancer brain mets)
  • CT/MRI head
  • LP (oligoclonal bands)

6

Myotonic dystrohpy associated features

Multisystem disorder in which myopathy and myotonia are prominent features

  • Cataracts
  • Cardiac conduction abnormalites and cardiomyopathy
  • Testicular atrophy
  • Endocrine disturbance (DM)
  • Cognitive dificulties
  • Hypersomnolence

7

Proxial Myopathy Differential

  • Inherited: muscular dystrophies

  • Inflammatory: polymyositis, SLE, RA, sarcoid

  • Drugs: steroids, statins

  • Toxic: alcohol

  • Endocrine: hypo/hyperthyroidism, addisons, cushings

  • Infective: influenza, HIV lyme

  • Hypokalaemia

8

Proximal myopathy investigations

  • Review drugs
  • Bloods: CK, U+Es, TFTs, LFTs, ESR, Ca, Mg
  • ECG (hypokalemia, cardiomyopathy)
  • Urinalysis (myoglobin)
  • EMG
  • Muscle biopsy

9

MND differential and tests

  • Cervical spondylosis with myelopathy and radiculopathy
  • MG
  • Post-polio syndrome (LMN only)
  • Multifocal motor neuropathy

Image spinal cord MRI, EMG diagnostic. Often clinical diangosis however.

10

MND phenotypes

Amyotrophic lateral sclerosis (UMN + LMN)

Bulbar presentation (limbs preserved early)

Progressive muscular atrophy (LMN signs)

Primary lateral sclerosis (UMN signs)

11

Cerebellar syndrome causes

  • MS
  • Stroke
  • Tumour (acoustic neuroma, meningioma)
  • Drugs (pheytoin)
  • Alcohol (+wernicke-korsakoff)
  • Paraneoplastic (lung, breast, ovary)
  • Hypothyroidism
  • Inherited (Friedreich's ataxia)

12

Myasthenia Gravis differential

  • Lambert-Eaton myasthenia syndrome (improves with exercise)
  • Botulism (also hypotension, bradycardia, diarrohea, urinary retention)
  • Primary myopathies (not fatigable weakness)
  • Partial III nerve (unilateral ptosis)
  • GBS
  • Stroke, MND (dysarthria)

13

Myasthenia gravis investigations

  • Serum antibodies (anti-AchR, anti-MuSK)
  • Tensilon/edrophonium test
  • EMG
  • Serial pulmonary function tests
  • CT thorax - thymoma (10-15%)

14

Myasthenia gravis treatment

  • Myasthenic crisis: intubation and ventilation, plasma exchange or IVIG, supportive care, prednisolone
  • Pyridostigmine, corticosteroids
  • Thymectomy
  • Supportive care = DVT prophylaxis, ulcer prophylaxis, nutrition, avoidance of infection

15

Cervical myelopathy Differential

  • Transverse myelitis (isolated or MS)
  • Compression (degenerative, neoplastic (1 or 2), infective)
  • Trauma
  • Spinal cord stroke
  • B12 deficiency
  • Syringomyelia
  • Neoplasm of cord
  • Hereditary spastic paraplegia

16

Cervical myelopathy investigations

  • Bloods: FBC (macrocytosis), B12
  • MRI cervical spine
  • LP (transverse myelitis)
  • Cervical xray / CT

17

Median nerve pasly causes

  • Carpal tunnel syndrome
  • Trauma (wrist)
  • Surgical
  • Mononeuritis multiplex
  • Infection (leprosy)
  • Inflammatory (CIDP)

18

Carpal tunnel syndrome causes

  • Idiopathic (commonest)
  • Anatomical
    • Bone: #, acromegally
    • Soft tissues: lipoma, ganglion
  • Physiological
    • Inflammatory: RA, gout
    • Fluid balance: pregancy, menopause, hypothyroidism, obesity, amyloidosis, renal failure, contraceptive pill
    • Neuropathic: DM, alcoholism

19

Ulnar nerve palsy differential

  • C8/T1 nerve root lesions
  • Brachial plexus lesions
  • MND
  • Poliomyelitis
  • Syringomyelia
  • Peripheral Neuropathy
  • RA

20

Ulnar nerve palsy causes

  • Anatomical: cubital tunnel syndrome at elbow
  • Trauma: #'s, dislocations
  • Degenerative arthritis: compresive proliferative synovitis and osteophytes
  • Rare causes: compression from tight fascia, tumours, aneurysms etc

21

Radial nerve palsy differential

  • C7 nerve root lesion
  • MND
  • Poliomyelitis
  • Lesions of brachial plexus
  • Mild stroke

22

Wasting of small muscles of hand differential

  • C8/T1 nerve root lesions
  • Brachial plexus lesions
  • MND
  • Poliomyelitis
  • Syringomyelia
  • Peripheral neuropathy
  • Myotonic dystrophy
  • RA

23

Polymyositis associations

  • Autoimmune conditions
  • Connective tiddue diseases
  • Drugs (statins)
  • Infections (HIV, acute viral illness)
  • Idiopathic (rare)

Dermatomyositis with cancer (ovary, GI, breast)

24

Polymyositis differential

  • Drug-induced mypathy (steroids)
  • Toxic (alochol)
  • Endocrine (hypo/hyperthyroidism, addisions, cushings)
  • Hypokalaemia
  • Inclusion body myositis
  • Inherited (muscuar distrophies)

25

Polymyositis investigations

  • Review drugs
  • CK
  • EMG
  • Muscle biopsy
  • ESR
  • ANA

26

Parkinsons Disease Differential

  • Essential tremor (usually symmetrical)
  • Vascular PD (basal ganglia stroke, gait>upper limb)
  • Drug-induced parkinsonism (symmetrical)
  • Parkinson's plus syndromes
    • Progressive supranuclear palsy
    • ​Multisystem atrophy
    • Corticobasal degeneration
    • Lewy body dementia
  • Wilsons disease

27

Parkinson's Disease investigations

  • Clinical diagnosis, confirmed by dopaminergic agent trial
  • MRI brain
  • Flurodopa PET
  • Serum ceruloplasmin and 24 urinary copper if young <40
  • Neuropsychometric testing

28

Friedreich's ataxia examination findings

  • Inspection: distal muscle wasting, pes cavus, kyphoscoliosis, high-arched palate
  • Reflexes: Reduced in lower (common) and upper limbs, extensor plantars
  • Sensation: Loss of vibration and propriation in lower limbs
  • Coordination: ataxia (past-pointing, dysdiado, tuncal)
  • Ataxic gait and dysarthria, possibley nystagmus

29

Friedreich's ataxia pathology and investigations

Autosomal recessive GAA repeat expansion

Associations: optic atrophy, sensorineural deafness, sphincter dysfunction, DM, impaired glucose

MRI brain and spinal cord shows atrophy of cerebellar vermis and spinal cord

Look for hypertrophic cardiomyopathy (ECG and echo)

No treatment

30

Charcot-Marie-Tooth Disease differential

  • Diabetic neuropathy
  • Chronic inflammatory demyelinating polyneuropathy
  • Aquired peripheral neuropathy (toxins, hypothyroidism, vitamin deficiencies, renal failure etc.)

31

Charcot-Marie Tooth disease investigations

  • Peripheral neuropathy tests
  • Nerve conduction studies (axonal or demyelination)
  • Genetic testing

32

Charcot-Marie Tooth disease treatment

  • No cure
  • Physiotherapy and exercise
  • OT
  • Bracing
  • Orthopaedic surgery (correct deformities)
  • Treat underlying DM (exacerbates disease)

33

Subacute combined degeneration of the cord examination

  • Inspection: walking aids, normal muscle, vitiligo?
  • Tone: normal
  • Power: Normal or weakness
  • Reflexes: brisk or reduced (absent ankles, brisk knees), extensor plantars
  • Sensation: vibration and proprioception loss distally
  • Coordination: sensory ataxia
  • Ataxic gait, romberg's positive
  • Look for anaemia, glossitis, lemon yellow pallor, splenomegally

34

Subacute degeneration of the cord investigations and differential

  • FBC
  • B12, folate, VDRL, glucose
  • Schilling's test (pernicious anaemia)
  • MR spine to rule out compression

Differential

  • DM with cord compression
  • Tabes dorsalis
  • Hereditary syndromes

35

Brown-Sequard syndrome

Ipsilateral UMN lesion and loss of light touch (variable), vibration and proprioception

Contralateral loss of pain and temperature sensation

36

Brown Sequard Syndrome causes

  • Penetrating trauma to spine
  • Degenerative disease of spine
  • Tumour of spinal cord
  • Infection (meningitis, abcess, HSV, VZV, TB)
  • Inflammation (myelitis)
  • Vascular (ischamic or haemorragic)

37

Dissociated sensory loss differential

  • Brown-Sequard syndrome
  • Spinal cord stroke
  • Syringomyelia
  • Brainstem stroke

38

Cerebellopontine angle syndrome findings and causes

CN V, VII, VIII palsies and cerebellar signs

Isilateral facial sensory loss, loss of corneal reflex, LMN facial hemiparesis, sensorineural deafness, dysdiado, nystagmus, ataxia

Causes

  • Schwannomas (acoustic commonest)
  • Meningioma
  • Mets
  • Epidermoid cyst

39

Wallenberg's Syndrome aka lateral medullary syndrome

40

Paraplegia Differential

  • Transverse myelitis (isolated or MS)
  • Compressive myelopathy (neoplastic, infective, degenerative, traumatic)
  • Spinal cord stroke
  • B12 deficiency
  • Syringomyelia
  • Neoplasm of spinal cord
  • Inherited spastic paraplegia

41

Post poliomyelitis differential

  • Radiculopathy
  • Plexopathy
  • MND

42

Causes of a third nerve palsy

  • Microvascular causes: DM, HTN (usually pupil sparing)
  • Intracranial aneurysms: posterior communicating artery
  • Trauma
  • Tumors
  • Idiopathic
  • (Compressive lesions pupil-involing)

43

Third nerve palsy differential

  • Myasthenia gravis
  • Thyroid eye disease
  • Migraine

44

Causes of 6th nerve palsy

  • Microvascular: DM, HTN
  • MS
  • Stroke
  • Raised ICP
  • Tumours
  • Meningitis
  • Aneurysm (basilar artery)
  • Inflammatory process

45

Investigations for 3rd, 4th, 6th cranial nerve palsies

  • FBC
  • Inflammatory markers
  • Syphilis serology
  • Autoantibody screen
  • BP and glucose
  • CT or MRI/MRA if trauma, malignancy, meningitis

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