Neurology Short Case Flashcards

1
Q

Causes of Horner’s syndrome (6)

A
  1. Carcinoma of lung apex (usually SCC)
  2. Neck - thyroid malignancy, trauma
  3. Carotid arterial lesion - carotid aneurysm or dissection, peri-carotid tumour, cluster headache
  4. Brainstem lesion - lateral medullary syndrome, syringobulbia, tumour
  5. Retro-orbital lesions
  6. Syringomyelia (rare)
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2
Q

Causes of anosmia (bilateral) (7)

A
  1. Upper respiratory tract infection (most common)
  2. Meningioma of the olfactory groove (late)
  3. Ethmoid tumours
  4. Head trauma (including cribriform plate fracture)
  5. Meningitis
  6. Hydrocephalus
  7. Congenital - Kallman’s syndrome (hypogonadotrophic hypogonadism)
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3
Q

Causes of anosmia (unilateral) (2)

A
  1. Meningioma of the olfactory groove (early)

2. Head trauma

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4
Q

Causes of absent light reflex but intact accomodation reflex (4)

A
  1. Midbrain lesion (eg. Argyll Robertson pupil)
  2. Ciliary ganglion lesion (eg. Adie’s pupil)
  3. Parinaud’s syndrome
  4. Bilateral anterior visual pathway lesions (bilateral afferent pupil deficits)
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5
Q

Causes of absent convergence but intact light reflex (2)

A
  1. Cortical lesion (eg. cortical blindness)

2. Midbrain lesions (rare)

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6
Q

Causes of constricted pupils (6)

A
  1. Horner’s syndrome
  2. Argyll Robertson pupil
  3. Pontine lesion (often bilateral, but reactive to light)
  4. Narcotics
  5. Pilocarpine drops
  6. Old age
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7
Q

Causes of dilated pupils (6)

A
  1. Mydriatics, atropine poisoning or cocaine
  2. Third nerve lesion
  3. Adie’s pupil
  4. Iridectomy, lens implant, iritis
  5. Post trauma, deep coma, cerebral death
  6. Congenital
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8
Q

Cause and signs (5) of Adie’s syndrome

A

Cause: lesion in the efferent parasympathetic pathway

Signs:

  1. Dilated pupil
  2. Decreased or absent reaction to light (direct and consensual)
  3. Slow or incomplete reaction to accomodation with slow dilation afterwards.
  4. Decreased tendon reflexes
  5. Patients are commonly young women
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9
Q

Causes of Argyll Robertson pupil (4)

A

Cause: lesion of the iridodilator fibres in the midbrain

  1. Syphilis
  2. Diabetes mellitus
  3. Alcoholic midbrain degeneration (rarely)
  4. Other midbrain lesions
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10
Q

Signs of Argyll Robertson pupil (4)

A
  1. Small, irregular, unequal pupil
  2. No reaction to light
  3. Prompt reaction to accomodation
  4. If tabes associated, decreased reflexes
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11
Q

Features of papilloedema (6)

A
  1. Optic disc swollen without venous pulsation
  2. Acuity normal (early)
  3. Colour vision normal
  4. Large blind spot
  5. Peripheral constriction of visual fields
  6. Usually bilateral
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12
Q

Features of papillitis (6)

A
  1. Optic disc swollen (in retrobulbar neuritis and old papillitis the optic disc becomes pale)
  2. Acuity poor
  3. Colour vision affected (particularly red desaturation)
  4. Large central scotoma
  5. Pain on eye movement
  6. Onset usually sudden and unilateral
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13
Q

Causes of papilloedema (7)

A
  1. Space - occupying lesion (causing raised intracranial pressure) or a retro-orbital mass
  2. Hydrocephalus (associated with large ventricles)
  3. Idiopathic intracranial hypertension
  4. Hypertension (grade IV)
  5. Central retinal vein thrombosis
  6. Cerebral venous sinus thrombosis
  7. High cerebrospinal fluid protein level - Guillain-Barre syndrome
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14
Q

Causes of hydrocephalus (2)

A
  1. Obstructive (block in the third ventricle, aqueduct or outlet to fourth ventricle - eg. tumour)
  2. Communicating
    - increased formation - choroid plexus papilloma
    - decreased absorption - tumour causing venous compression, subarachnoid space obstruction from meningitis
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15
Q

Causes of idiopathic intracranial hypertension (6)

A
  1. Idiopathic
  2. Contraceptive pill
  3. Addison’s disease
  4. Drugs - nitrofurantoin, tetracycline, vitamin A, steroids
  5. Lateral sinus thrombosis
  6. Head trauma
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16
Q

Causes of optic atrophy (5)

A
  1. Chronic papilloedema or optic neuritis
  2. Optic nerve pressure or division
  3. Glaucoma
  4. Ischemia
  5. Familial - retinitis pigments, Leber’s disease, Friedreich’s ataxia
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17
Q

Causes of optic neuropathy (6)

A
  1. Multiple sclerosis
  2. Toxic - ethambutol, chloroquine, nicotine, alcohol
  3. Metabolic - vitamin B12 deficiency
  4. Ischaemia - diabetes mellitus, temporal arteritis, atheroma
  5. Familial - Leber’s disease
  6. Infective - infectious mononucleosis
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18
Q

Causes of cataract (6)

A
  1. Old age (senile cataract)
  2. Endocrine - diabetes mellitus, steroids
  3. Hereditary or congenital - dystrophia myotonica, Refsum disease
  4. Ocular disease - galucoma
  5. Irradiation
  6. Trauma
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19
Q

Causes of ptosis with normal pupils (9)

A
  1. Senile ptosis (common)
  2. Myotonic dystrophy
  3. Fascioscapulohumeral dystrophy
  4. Ocular myopathy eg. mitochondrial myopathy
  5. Thyrotoxic myopathy
  6. Myasthenia gravis
  7. Botulism, snake bite
  8. Congenital
  9. Fatigue
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20
Q

Causes of ptosis with constricted pupils (2)

A
  1. Horner’s syndrome

2. Tabes dorsalis

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21
Q

Cause of ptosis with constricted pupils (1)

A
  1. Third nerve lesion
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22
Q

Clinical features of a third nerve palsy (3)

A
  1. Complete ptosis (partial ptosis with incomplete lesions)
  2. Divergent strabismus (eye ‘down and out’)
  3. Dilated pupil unreactive to direct or consensual light and unreactive to accomodation
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23
Q

Central causes of third nerve palsy (5)

A
  1. Vascular (brainstem infarction)
  2. Tumour
  3. Demyelination
  4. Trauma
  5. Idiopathic
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24
Q

Peripheral causes of third nerve palsy (4)

A
  1. Compressive lesions (next card)
  2. Infarction - diabetes mellitus, arteritis (pupil usually spared)
  3. Trauma
  4. Cavernous sinus lesions
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25
Q

Peripheral causes of third nerve palsy (compressive) (5)

A
  1. Aneurysm (usually posterior communicating artery)
  2. Tumour causing raised intracranial pressure (dilated pupil occurs early)
  3. Nasopharyngeal carcinoma
  4. Orbital lesions - Tolosa-Hunt syndrome (superior orbital fissure syndrome - painful lesion of the 3rd, 4th, 6th, and 1st division of 5th cranial nerves)
  5. Basal meningitis
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26
Q

Clinical features of a sixth nerve palsy (3)

A
  1. Failure of lateral movement
  2. Affected eye is deviated inwards in severe lesions
  3. Diplopia - maximal on looking to the affected side; the images are horizontal and parallel to each other; the outermost image is from the affected eye and disappears on covering this eye (this image is also usually more blurred)
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27
Q

Causes of bilateral sixth nerve palsies (4)

A
  1. Trauma (head injury)
  2. Wernicke’s encephalopathy
  3. Raised intracranial pressure
  4. Mononeuritis multiplex
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28
Q

Causes of unilateral sixth nerve palsies - central (4)

A
  1. Vascular
  2. Tumour
  3. Wernicke’s encephalopathy
  4. Multiple sclerosis (rare)
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29
Q

Causes of unilateral sixth nerve palsies - peripheral (4)

A
  1. Diabetes, other vascular lesions
  2. Trauma
  3. Idiopathic
  4. Raised intracranial pressure
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30
Q

Causes of jerky nystagmus - horizontal (3)

A
  1. Vestibular lesion (fast phase away from lesion)
  2. Cerebellar lesion (fast phase towards lesion)
  3. Internuclear ophthalmoplegia (MS or brain stem infarct)
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31
Q

Causes of jerky nystagmus - vertical (2)

A
  1. Brain stem lesion
    - upbeat nystagmus suggests lesion in floor of the fourth ventricle
    - downbeat nystagmus suggests a foramen magnum lesion
  2. Toxic - phenytoin, alcohol (may be multidirectional)
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32
Q

Causes of pendular nystagmus (2)

A
  1. Retinal (decreased macular vision) - albinism

2. Congenital

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33
Q

Clinical features of supranuclear palsy (5)

A
  1. Loss of vertical upward and/or downward gaze
  2. Both eyes affected
  3. Pupils often unequal
  4. No diplopia
  5. Reflex eye movements intact
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34
Q

Clinical features of Steele-Richardson-Olszewski (PSP) (6)

A
  1. Loss of vertical downward gaze first, later vertical upward gaze and finally horizontal gaze. Saccades are impaired before pursuit. Vergence is lost early
  2. Pseudo bulbar palsy
  3. Long-tract signs
  4. Extrapyramidal signs
  5. Dementia
  6. Neck rigidity
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35
Q

Clinical features of Parinaud’s syndrome (3)

A
  1. Loss of vertical upward gaze
  2. Convergence-retraction nystagmus on attempted convergence
  3. Pseudo Argyll Robertson pupils
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36
Q

Causes of Parinaud’s syndrome (8)

A

Central

  1. Pinealoma
  2. Multiple sclerosis
  3. Vascular lesions

Peripheral

  1. Trauma
  2. Diabetes mellitus
  3. Other vascular lesions
  4. Idiopathic
  5. Raised intracranial pressure
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37
Q

Causes of fifth nerve palsy (central) (4) - pons, medulla, upper cervical cord

A
  1. Vascular
  2. Tumour
  3. Syringobulbia
  4. Multiple sclerosis
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38
Q

Causes of fifth nerve palsy (peripheral) (3) - posterior fossa

A
  1. Aneurysm
  2. Tumour (skull base eg. acoustic neuroma)
  3. Chronic meningitis
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39
Q

Causes of fifth nerve palsy (trigeminal ganglion) (2) - petrous temporal bone

A
  1. Meningioma

2. Fracture of the middle fossa

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40
Q

Causes of fifth nerve palsy (cavernous sinus) (3) - associated 3rd, 4th and 6th nerve palsies

A
  1. Aneurysm
  2. Thrombosis
  3. Tumour
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41
Q

Causes of fifth nerve palsy (other) (4)

A
  1. Sjogren’s syndrome
  2. SLE
  3. Toxins
  4. Idiopathic
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42
Q

Causes of seventh nerve palsy (upper motor neurone) (2)

A
  1. Vascular

2. Tumour

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43
Q

Causes of seventh nerve palsy (lower motor neurone) (4 regions)

A
  1. Pontine (often associated with V, VI)
    - vascular
    - tumour
    - syringobulbia
    - multiple sclerosis
  2. Posterior fossa
    - acoustic neuroma
    - meningioma
  3. Petrous temporal bone
    - Bell’s palsy
    - Ramsay Hunt syndrome
    - otitis media
    - fracture
  4. Parotid
    - tumour
    - sarcoid
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44
Q

Causes of bilateral lower motor neurone facial weakness (5)

A
  1. Guillain-Barre syndrome
  2. Bilateral parotid disease (eg. sarcoidosis)
  3. Mononeuritis multiplex (rare)
  4. Myopathy
  5. Neuromuscular junction defects
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45
Q

Causes of sensorineural deafness (7)

A
  1. Degeneration (presbycusis)
  2. Trauma (eg. high noise exposure, fracture of the petrous temporal bone)
  3. Toxic (eg. aspirin, alcohol, streptomycin)
  4. Infection (eg. congenital rubella, congenital syphilis)
  5. Tumour (eg. acoustic neuroma)
  6. Brain stem lesions
  7. Vascular disease of internal auditory artery
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46
Q

Causes of conductive deafness (4)

A
  1. Wax
  2. Otitis media
  3. Otosclerosis
  4. Paget’s disease of bone
47
Q

Causes of ninth and tenth nerve palsy (central) (4)

A
  1. Vascular (lateral medullary infarction)
  2. Tumour
  3. Syringobulbia
  4. Motor neurone disease (vagus nerve only)
48
Q

Causes of ninth and tenth nerve palsy (peripheral - posterior fossa) (4)

A
  1. Aneurysm
  2. Tumour
  3. Chronic meningitis
  4. Guillain-Barre syndrome (vagus nerve only)
49
Q

Causes of twelfth nerve palsy (upper motor neurone) (4)

A
  1. Vascular
  2. Motor neurone disease
  3. Tumour
  4. Multiple sclerosis
50
Q

Causes of twelfth nerve palsy (lower motor neurone - unilateral) (9)

A

Central

  1. Vascular - vertebral artery thrombosis
  2. Motor neurone disease
  3. Syringobulbia

Peripheral

  1. Aneurysm
  2. Tumour
  3. Chronic meningitis
  4. Trauma
  5. Arnold-Chiari malformation (protrusion of the cerebellar tonsils through foramen magnum)
  6. Fracture or tumour of the base of the skull
51
Q

Causes of twelfth nerve palsy (lower motor neurone - bilateral) (4)

A
  1. Motor neurone disease
  2. Arnold-Chiari malformation
  3. Guillain-Barre syndrome
  4. Polio
52
Q

Causes of multiple cranial nerve palsies (8)

A
  1. Nasopharyngeal carcinoma
  2. Chronic meningitis (eg. carcinoma, TB, sarcoidosis)
  3. Guillain-Barre syndrome (spares nerves I, II, VIII) including Miller Fisher variant
  4. Brain stem lesions (usually vascular)
  5. Arnold-Chiari malformation
  6. Trauma
  7. Lesion of the base of the skull (eg. Paget’s, meningioma, metastasis)
  8. Mononeuritis multiplex (eg. diabetes)
53
Q

Causes of peripheral neuropathy (mixed) (9)

A
  1. Drugs and toxins
  2. Alcohol, amyloidosis
  3. Metabolic - diabetes mellitus (30%), uraemia, hypothyroidism, porphyria
  4. Immune related - GBS
  5. Tumour - lung carcinoma
  6. Vitamin B12 or B1 deficiency, B6 excess
  7. Idiopathic (30%)
  8. Connective tissue disease - SLE, PAN
  9. Hereditary (30%)
54
Q

Drugs and toxins that cause peripheral neuropathy (7)

A
  1. Isoniazid
  2. Vincristine
  3. Phenytoin
  4. Nitrofurantoin
  5. Cisplatinum
  6. Amiodarone
  7. Large doses of vitamin B6
55
Q

Causes of peripheral neuropathy (motor predominant) (6)

A
  1. Guillain-Barre syndrome, CIDP
  2. Hereditary motor and sensory neuropathy (Charcot-Marie-Tooth)
  3. Acute intermittent porphyria
  4. Diabetes mellitus
  5. Lead poisoning
  6. Multifocal motor neuropathy
56
Q

Causes of peripheral neuropathy (sensory predominant) (8)

Usually associated with sensory ataxia and pseudoathetosis

A
  1. Diabetes mellitus
  2. Carcinoma (eg. lung, ovary, breast) (may be neuronopathy, length independent)
  3. Paraproteinaemia
  4. Vitamin B6 intoxication
  5. Sjogren’s syndrome (often a neuronopathy)
  6. Syphilis
  7. Vitamin B12 deficiency (occasionally)
  8. Idiopathic
57
Q

Causes of a painful peripheral neuropathy (7)

A
  1. Diabetes mellitus
  2. Alcohol
  3. Vitamin B12 or B1 deficiency
  4. Carcinoma
  5. Porphyria
  6. Arsenic or thallium poisoning
  7. Hereditary (most are not painful)
58
Q

Nerve conduction test findings for demyelinating lesions

eg. diabetes, paraprotein, CMT, CIDP (3)

A
  1. Velocity <75%
  2. Distal latency >130%
  3. Amplitude normal
59
Q

Nerve conduction test findings for axonal lesions - eg. diabetes, toxins, metabolic, paraneoplastic (2)

A
  1. Amplitude <50%

2. Velocity >70%

60
Q

Causes of mononeuritis multiplex - acute (3)

A
  1. Diabetes mellitus
  2. Polyarteritis nodosa
  3. Connective tissue diseases - SLE, RA
61
Q

Causes of mononeuritis multiplex - chronic (7)

A
  1. Multiple compressive neuropathies (especially with joint-deforming arthritis)
  2. Sarcoidosis
  3. Acromegaly
  4. Leprosy
  5. Lyme disease
  6. Carcinoma (rare)
  7. Idiopathic
62
Q

Causes of thickened nerves (6)

A
  1. Hereditary motor and sensory neuropathy
  2. Acromegaly
  3. CIDP
  4. Amyloidosis
  5. Leprosy
  6. Others - sarcoidosis, neurofibromatosis
63
Q

Causes of fasciculation (6)

A
  1. Benign idiopathic fasciculation (most common)
  2. Motor neurone disease
  3. Motor root compression
  4. Malignant neuropathy
  5. Spinal muscular atrophy/bulbospinal muscular atrophy (Kennedy syndrome)
  6. Any motor neuropathy
64
Q

Causes of myokymia (6)

A
  1. Multiple sclerosis
  2. Brain stem neoplasm
  3. Bell’s palsy
  4. Radiculopathy
  5. Radiation plexopathy
  6. Chronic nerve compression
65
Q

Clinical features of hereditary motor and sensory neuropathy (HMSN) (6)

A
  1. Pes cavus
  2. Distal muscle atrophy
  3. Absent reflexes
  4. Slight to no sensory loss in the limbs
  5. Thickened nerves
  6. Optic atrophy; Argyll Robertson pupils (rare)
66
Q

Clinical features of brachial plexus lesion (complete) (3)

A
  1. Lower motor neurone signs affecting the whole arm
  2. Sensory loss of the whole limb
  3. Horner’s syndrome (only if lesion is proximal in the lower plexus)
67
Q

Clinical features of Erb-Duchenne - upper trunk lesions (C5,C6) (2)

A
  1. Loss of shoulder movement and elbow flexion - waiter’s tip position
  2. Sensory loss over lateral aspect of arm and forearm, and over the thumb
68
Q

Clinical features of Klumpke - lower trunk lesions (3)

A
  1. True claw hand with paralysis of all the intrinsic muscles
  2. Sensory loss along the ulnar side of the hand and forearm
  3. Horner’s syndrome
69
Q

Clinical features of cervical rib syndrome (5)

A
  1. True claw hand (wasting/weakness of small muscles of the hand)
  2. Sensory loss over medial aspect of the hand and forearm
  3. Unequal radial pulses and blood pressures
  4. Subclavian bruit and loss of the pulse on arm manoeuvring
  5. Palpable cervical rib in the neck (uncommon)
70
Q

Clinical features of radial nerve (C5 - C8) lesions (4)

A
  1. Wrist and finger drop (wrist flexion normal)
  2. Triceps loss (if lesion is above the spiral grove)
  3. Sensory loss over the anatomical snuff-box
  4. Finger abduction appears to be weak (difficulty of spreading the fingers when they cannot be straightened)
71
Q

Clinical features of median nerve (C6-T1) lesions (3)

A
  1. Loss of abductor policies brevis - pen-touching test
  2. Loss of flexor digitorum sublimis - with lesions in or above the cubital fossa - Ochsner’s clasping test
  3. Sensory loss over the thumb, index, middle and lateral half of the ring finger (palmar aspect)
72
Q

Clinical features of ulnar nerve (C8-T1) lesions (5)

A
  1. Wasting of the intrinsic muscles of the hand (except LOAF)
  2. Weak finger abduction and adduction (interosseous)
  3. Ulnar claw-like hand (higher lesion causes less deformity)
  4. Froment’s sign
  5. Sensory loss over the little and medial half of the ring finger (both palmar and dorsal aspects)
73
Q

Causes of wasting of the small muscles of the hands (5)

A
  1. Nerve lesions
    - median and ulnar nerve lesions
    - brachial plexus lesions
    - peripheral motor neuropathy
  2. Anterior horn cell disease
    - MND
    - polio
    - spinal muscular atrophies
  3. Myopathy
    - dystrophia myotonica
    - distal myopathy
  4. Spinal cord lesions
    - syringomyelia
    - cervical spondylosis with compression of C8 segment
    - other (eg. tumour)
  5. Trophic disorders
    - arthropathies (disuse)
    - ischemia, including vasculitis
    - shoulder-hand syndrome
74
Q

Clinical features of femoral nerve (L2-L4) lesions (5)

A
  1. Weakness of knee extension (quadriceps paralysis)
  2. Slight hip flexion weakness
  3. Preserved adductor strength
  4. Loss of knee jerk
  5. Sensory loss involving the inner aspect of the thigh and leg
75
Q

Clinical features of sciatic nerve (L4-S2) lesions (5)

A
  1. Weakness of knee flexion (hamstrings involved)
  2. Loss of power of all muscles below the knee causing a foot drop, the patient may be able to walk but cannot stand on the toes or heels)
  3. Knee jerk intact
  4. Loss of ankle jerk and plantar response
  5. Sensory loss along the posterior thigh and total loss below the knee
76
Q

Clinical features of common peroneal (L4 - S1) lesions (3)

A
  1. Foot drop
  2. Loss of foot eversion
  3. Sensory loss (minimal) over the dorsum of the foot
    - reflexes are normal
77
Q

Causes of foot drop (8)

A
  1. Common peroneal nerve palsy (preserved ankle jerk)
  2. Sciatic nerve palsy
  3. Lumbosacral plexus lesion
  4. L4, L5 root lesion
  5. Peripheral motor neuropathy
  6. Distal myopathy
  7. Motor neurone disease (increased ankle jerk)
  8. Precentral gyrus lesion (increased ankle jerk)
78
Q

Causes of sensory level in paraplegic patients (5)

A
  1. Cord compression
  2. Transverse myelitis
  3. Anterior spinal artery occlusion (spares posterior column)
  4. Intrinsic cord lesion
  5. Multiple sclerosis
79
Q

Causes of arm involvement in paraplegic patients (4)

A
  1. Cervical spondylosis
  2. Syringomyelia
  3. Motor neurone disease
  4. Multiple sclerosis
80
Q

Causes of cranial nerve lesions in paraplegic patients (2)

A
  1. Motor neurone disease

2. Multiple sclerosis

81
Q

Causes of peripheral neuropathy in paraplegic patients (5)

A
  1. Vitamin B 12 deficiency
  2. Freireich’s ataxia
  3. Carcinoma
  4. Hereditary spastic paraplegia
  5. Syphilis
82
Q

Clinical features of upper cervical lesions (2)

A
  1. Upper motor neurone signs in upper/lower limbs

2. Paralysis of the diaphragm with lesions above C4

83
Q

Clinical features of C5 lesions (4)

A
  1. Lower motor neurone weakness/wasting of rhomboids, deltoids, biceps and brachioradialis
  2. Upper motor neurone signs affects the rest of the upper and all the lower limbs
  3. Biceps jerk is lost
  4. Supinator jerk is inverted
84
Q

Clinical features of C8 lesions (2)

A
  1. Lower motor neurone weakness and wasting of intrinsic muscles of the hand
  2. Upper motor neurone signs in lower limbs
85
Q

Clinical features of mid thoracic lesions (4)

A
  1. Intercostal paralysis
  2. Loss of upper abdominal reflexes at T7 and T8
  3. Upper motor neurone signs in the lower limbs
  4. Sensory level on the trunk
86
Q

Clinical features of T10-T11 lesions (2)

A
  1. Loss of the lower abdominal reflexes and upward displacement of the umbilicus on contraction (Beevor’s sign)
  2. Upper motor neurone signs in the lower limbs
87
Q

Clinical features of L1 lesions (2)

A
  1. Cremasteric reflexes lost (normal abdominal reflexes)

2. Upper motor neurone signs in lower limbs

88
Q

Clinical features of L4 lesions (2)

A
  1. Lower motor neurone weakness and wasting of quadriceps

2. Ankle jerk lost

89
Q

Clinical features of L5 and S1 lesions (4)

A
  1. Lower motor neurone weakness of knee flexion and hip extension (S1) and abduction (L5), plus calf and foot muscles
  2. Knee jerk present
  3. No ankle jerk or plantar response
  4. Anal reflex present
90
Q

Clinical features of S3-S4 lesions (3)

A
  1. No anal reflex
  2. Saddle sensory loss
  3. Normal lower limbs
91
Q

Clinical features of subacute combined degeneration of the cord (vitamin B12 deficiency) (5)

A
  1. Symmetrical posterior column loss (vibration/proprioception) causing an ataxic gait
  2. Symmetrical upper motor neurone signs in the lower limbs with absent ankle reflexes, knee reflexes may be exaggerated
  3. Peripheral sensory neuropathy (less common and mild)
  4. Optic atrophy (occasionally)
  5. Dementia (occasionally)
92
Q

Causes of an extensor plantar response plus absent knee jerk (7)

A
  1. Subacute combined degeneration of the cord
  2. Conus medullaris lesion
  3. Combination of an upper motor neurone lesion with caudal equine compression or peripheral neuropathy
  4. Syphilis (taboparesis)
  5. Freidreich’s ataxia
  6. Diabetes mellitus (uncommon)
  7. Adrenoleukodystrophy or metachromatic leukodystrophy
93
Q

Clinical features of Brown-Sequard syndrome (6)

A
  1. Upper motor neurone signs below hemisection on the same side
  2. Lower motor neurone signs at the level of the hemisection on the same side
  3. Pain/temperature loss on the opposite side (few segments below the lesion)
  4. Vibration and proprioception loss on the same side
  5. Light touch is often normal
  6. Band of sensory loss on the same side at the level of the lesion (afferent nerve fibres)
94
Q

Causes of Brown-Sequard syndrome (6)

A
  1. Multiple sclerosis
  2. Angioma
  3. Glioma
  4. Trauma
  5. Myelitis
  6. Postradiation myelopathy
95
Q

Causes of spinothalamic loss only (5)

- part of dissociated sensory loss

A
  1. Syringomyelia (cape distribution)
  2. Brown-Sequard syndrome (contralateral leg)
  3. Anterior spinal artery thrombosis
  4. Lateral medullary syndrome (contralateral to the other signs)
  5. Peripheral neuropathy (diabetes mellitus, amyloid, Fabry disease)
96
Q

Causes of dorsal column loss only (7) - part of dissociated sensory loss

A
  1. Subacute combined degeneration
  2. Brown-Sequard syndrome (ipsilateral leg)
  3. Spinocerebellar degeneration (eg. Fredreich’s ataxia)
  4. Multiple sclerosis
  5. Tabes dorsalis
  6. Sensory neuropathy or ganglionopathy (eg. carcinoma)
  7. Peripheral neuropathy from diabetes mellitus or hypothyroidism
97
Q

Clinical triad of syringomyelia

A
  1. Loss of pain and temperature over the neck, shoulders and arms (cape)
  2. Amyotrophy (weakness, atrophy, areflexia) of the arms
  3. Upper motor neurone signs in the lower limbs
98
Q

Causes of proximal muscle weakness (3)

A
  1. Myopathic
  2. Neuromuscular junction disorder - myasthenia gravis
  3. Neurogenic - Kugelberg-Welander disease (proximal muscle wasting and fasciculation as a result of anterior horn cell damage; autosomal recessive), motor neurone disease, polyradiculopathy
99
Q

Causes of myopathy (10)

A
  1. Hereditary muscular dystrophy
  2. Congenital myopathies

mnemonic - PACE PODS

  1. Polymyositis or dermatomyositis
  2. Alcohol
  3. Carcinoma
  4. Endocrine
  5. Periodic paralysis
  6. Osteomalacia
  7. Drugs (clofibrate, chloroquine, steroids)
  8. Sarcoidosis
100
Q

Endocrine causes of myopathy (5)

A
  1. Hypothyroidism
  2. Hyperthyroidism
  3. Cushing’s syndrome
  4. Acromegaly
  5. Hypopituitarism
101
Q

Causes of proximal myopathy and a peripheral neuropathy (3)

A
  1. Paraneoplastic syndrome
  2. Alcohol
  3. Connective tissue disease
102
Q

Types of muscular dystrophies (5)

A
  1. Duchenne’s (X-linked recessive) - severe
  2. Becker’s (X-linked recessive) - milder
  3. Limb girdle (autosomal recessive) - face and heart spared
  4. Facioscapulohumeral (autosomal dominant)
  5. Distal dystrophies (autosomal dominant) - dystrophia myotonica
103
Q

Clinical features of Duchenne’s (8)

A
  1. Affects males (or females with Turner’s)
  2. Calves and deltoids are hypertrophied early
  3. Early proximal weakness
  4. Tendon reflexes are preserved in proportion to muscle strength
  5. Severe progressive kyphoscoliosis
  6. Heart disease (dilated cardiomyopathy)
  7. Creatinine kinase level markedly elevated
  8. Patients die in 2nd decade, usually from heart disease
104
Q

Tests for myopathy (5)

A
  1. Creatine kinase (highest in Duchenne’s)
  2. EMG
  3. ECG (especially Duchenne’s and dystrophia myotonica)
  4. Muscle biopsy
  5. Echocardiogram (cardiac involvement)
105
Q

Causes of myotonia (3)

A
  1. Dystrophia myotonica
  2. Myotonia congenita (myotonia of the tongue and thenar eminence, the recessive form being more severe)
  3. Paramyotonia congenital (episodic myotonia after cold exposure)
106
Q

Causes of unilateral cerebellar disease (5)

A
  1. Space occupying lesion (tumour, abscess, granuloma)
  2. Ischaemia (vertebrobasilar disease)
  3. Paraneoplastic syndrome
  4. Multiple sclerosis
  5. Trauma
107
Q

Causes of bilateral cerebellar disease (9)

A
  1. Drugs (eg. phenytoin)
  2. Friedreich’s ataxia
  3. Hypothyroidism
  4. Paraneoplastic syndrome
  5. Multiple sclerosis
  6. Trauma (punch drunk)
  7. Arnold-Chiari malformation
  8. Alcohol (most common) - can also cause rostral vermis lesion
  9. Large space-occupying lesion, cerebrovascular disease, rare metabolic diseases
108
Q

Causes of midline cerebellar disease (2)

A
  1. Paraneoplastic syndrome

2. Midline tumour

109
Q

Clinical features of Friedreich’s ataxia (8)

A
  1. Cerebellar signs (bilateral), including nystagmus
  2. Posterior column loss in the limbs
  3. Upper motor neurone signs in the limbs (ankle reflexes are absent)
  4. Peripheral neuropathy
  5. Optic atrophy
  6. Pes cavus, cocking of the toes and kyphoscoliosis
  7. Cardiomyopathy
  8. Diabetes mellitus
110
Q

Causes of pes cavus (4)

A
  1. Friedreich’s ataxia or other spinocerebellar degenerations
  2. Hereditary motor and sensory neuropathy
  3. Neuropathies in childhood
  4. Idiopathic
111
Q

Causes of spastic and ataxic paraparesis - in adolescents and young adults (5)

A

In adolescence
1. Spinocerebellar degeneration (eg. Friedreich’s)

In young adults

  1. Multiple sclerosis
  2. Syphilitic meningomyelitis
  3. Spinocerebellar ataxia (SCA)
  4. Arnold-Chiari malformation or other lesions at the craniospinal junction
112
Q

Causes of spastic and ataxic paraparesis - in later life (5)

A
  1. Multiple sclerosis
  2. Syringomyelia
  3. Infarction (in upper pons or internal capsule bilaterally - ataxic hemiparesis)
  4. Lesion at the craniospinal junction (eg. meningioma)
  5. SCA
113
Q

Causes of Parkinsonism (9)

A
  1. Idiopathic Parkinson’s disease
  2. Drugs - eg. phenothiazines, methyldopa
  3. Postencephalitis
  4. Toxins (carbon monoxide, manganese)
  5. Wilson’s disease
  6. PSP
  7. MSA
  8. Syphilis
  9. Tumour
114
Q

Causes of chorea (9)

A
  1. Huntington’s disease (autosomal dominant)
  2. Sydenham’s chorea (rheumatic fever)
  3. Senility
  4. Wilson’s disease
  5. Drugs (eg. phenothiazines, OCP, phenytoin. L-dopa)
  6. Vasculitis or connective tissue disease
  7. Thyrotoxicosis (very rare)
  8. Polycythaemia or other causes of hyperviscosity (very rare)
  9. Viral encephalitis (very rare)