Neurology Station

Question 1

Muscles innervated by the ulnar nerve in the hand

Answer 1

Flexor digiti minimi
Abductor digit minimi
Oppenens digiti minimi
Palmar brevis

Adductor pollicis
1/2 Flexor pollicis brevis

Lumbricals 3 & 4
Plamar & dorsal interossei

Question 2

Muscles innervated by the ulnar nerves in the arm

Answer 2

Flexor carpi ulnaris
1/2 Flexor digitorum profundus

Question 3

Muscles innervated by the median nerve in the hand

Answer 3

Flexor pollicis brevis
Abductor pollicis brevis
Opponens pollicis
Lumbricals 1 & 2

Question 4

Muscles innervated by the median nerve in the forearm

Answer 4

Pronator teres
Flexor carpi radialis
Palmaris longus
Flexor digitorum superficialis
Flexor digitorum profundus (1/2)
Flexor pollicis longus
Pronator quadratus

Question 5

Muscles innervated by the musculocutaneous nerve

Answer 5

Biceps brachii
Brachialis
Coracobrachialis

Question 6

Features of an ulnar palsy

Answer 6

Ulnar claw
Hypothenar atrophy
Intrinsic hand muscle atrophy

LOW: Weakness of the hypothenar muscles, the adductor pollicis, lumbricals 4 & 5 and the interossei muscles

HIGH: Above plus FCU and medial half of FDP will be weak

Parasthaesia of the medial aspect of the hand

Question 7

Muscles innervated by the radial nerve in the arm

Answer 7

Triceps brachii
Anconeus

Brachioradialis
Extensor carpi radialis longus
Extensor carpi radialis brevis
Extensor carpi ulnaris
Extensor digitorum
Extensor digiti minimi
Extensor pollicis longus
Extensor pollicis brevis
Extensor indicis
Abductor pollicis longus

Question 8

Features of a radial nerve palsy

Answer 8

Wrist drop
Wasting of posterior compartment and tricep

LOW: wrist extensors, finger extensors, brachioradialis, supinator will be weak

HIGH: above plus triceps brachii will also be weak

Sensory: parasthaesia over posterior forearm and for sum of hand

Question 9

Charcot-Marie-Tooth (HSMN) most common types

Answer 9

CMT1: AD inheritance, demyelination, most common mutation is PMP22 duplication

CMT2: AD inheritance, axonal, most common cause is MFN2 gene mutation

Question 10

Causes of motor-dominant sensorimotor polyneuropathy

Answer 10

Acute: GBS, botulism
Chronic: CIDP, paraproteinaemic polyneuropathy, diabetic amyotrophy (more proximal), CMT, lead toxicity, porphyria

Question 11

Causes of a sensory-dominant sensorimotor polyneuropathy

Answer 11

Endocrine & metabolic:
Diabetes mellitus
Vitamin B12 deficiency
Folate deficiency
Vitamin B1 deficiency
Uraemia
Hypothyroidism

Toxins:
Alcohol
Drugs - isoniazid, vincristine, cisplastin, phenytoin, amiodarone

Infections:
HIV, leprosy, syphilis

Vasculitis:
GPA, EGPA, MPA (ANCA positive)
RA, SLE, Hep B & C

Question 12

Differential of a mononeuritis multiplex

Answer 12

Primary vasculitis:
GPA, EGPA, MPA (ANCA-positive)

Secondary vasculitis
RA, SLE, Hep B & C

Sjögren’s syndrome
Diabetes
Paraneoplastic

Question 13

Causes of thickened nerves

Answer 13

Leprosy
Neurofibromatosis
Amyloidosis
Acromegaly

Question 14

Causes of a cerebellar syndrome

Answer 14

Acute: stroke, MS
Chronic: alcohol, SOL in posterior fossa, paraneoplastic, hypothyroidism, drugs (phenytoin toxicity), MSA

Rare: Friedrich’s ataxia, ataxia telangiectasia

Question 15

What is Kennedy disease?

Answer 15

Motor neuron disease (bulbar and limbs)
X-linked recessive
CAG repeat in AR gene
Very slow progression (over decades)
Perioral fasciculations

Other features: androgen insensitivity, metabolic syndrome

Question 16

Differential of MND

Answer 16

Cervical spondylosis with a combined myelopathy and radiculopathy

Multifocal motor neuropathy

Kennedy’s disease

Question 17

Genetics of Fredriech’s ataxia

Answer 17

Autosomal recessive
GAA repeat expansion (intronic) in the FXN gene on chromosome 9

Question 18

Friedreich’s Ataxia - Clinical picture

Answer 18

Neurological
- cerebellar signs
- peripheral neuropathy
- dorsal columns may be affected
- mixed UMN and LMN signs
- optic atrophy
- sensorineural hearing loss

Other
- HOCM
- diabetes
- High-arched palate
- kyphoscoliosis

Question 19

Huntington disease genetics

Answer 19

Autosomal dominant inheritance
CAG expansion (>35 repeats) in the HTT gene on chromosome 4
Anticipation

Question 20

Features of Huntington’s disease

Answer 20

Motor
- chorea
- motor impersistence
- Parkinsonism
- slow saccades, loss of OKN

Cognitive
- bradyphrenia
- memory loss
- executive dysfunction

Psychiatric
- anxiety
- depression
- psychosis
- OCD

Question 21

Differential for complex ophthalmoplegia

Answer 21

Myasthenia gravis
Grave’s disease
Miller-Fisher variant of GBS
Cavernous sinus pathology
Mitochondrial cytopathy

Question 22

Differential for mixed UMN + LMN signs

Answer 22

MND
Subacute combined degeneration of the cord (Vit B12 deficiency)
Cervical spondylosis with myelopathy and radiculopathy
Friedriech’s ataxia

Question 23

Neurofibromatosis genetics

Answer 23

Autosomal dominant inheritance

NF1: NF1 mutation on chromosome 17
NF2: NF2 mutation on chromosome 22

Question 24

Clinical manifestations of neurofibromatosis is

Answer 24

NF1: cutaneous neurofibromas (2+), café au lait spots (6+, >15 mm), axillary/inguinal freckling, Lisch nodules, optic gliomas

NF2: vestibular schwannomas, meningiomas, ependymomas

Associations:
Renal artery stenosis (2%)
Pheochromocytoma (2%)

Complications:
Epilepsy
Intellectual impairment

Question 25

Clinical manifestations of tuberous sclerosis

Answer 25

Facial adenoma sebaceum (angiofibromata)
Periungal fibromas
Shagreen patch (lumbar region)
Ash leaf macules (on trunk)

CNS: Epilepsy, intellectual impairment

Resp: cystic lung disease

Renal: PCKD, angiomyolipomata, RCC

Eyes: retinal phakomas (white patches)

Question 26

Genetics of tuberous sclerosis

Answer 26

Autosomal dominant inheritance

Mutation in TSC1 (ch 9) or TSC2 (ch 16)

Question 27

Differential for bilateral ptosis

Answer 27

1. Myasthenia gravis
2. Myotonic dystrophy
3. Oculopharyngeal muscular dystrophy
4. Mitochondrial disease e.g. Kearns-Sayres