MRCP cases

Question 1

Describe a Parkinson’s patient

Answer 1

This patient has a mask like expressionless face with infrequent blinking and low volume monotonous temulous speech. When the eyes are gently closed, there is tremor of the eyelids. There is continuous pill rolling tremor of the hands, with lead-pipe rigiditiy of the elbows and cogwheel rigidity at the wrists. The rigidity in one arm is increased by voluntary movements of the opposite arm (synkinesis). There is bradykinesia with paucity and decreased amplitude of movements. The signs demonstrate asymmetry, with the tremor and rigidity being more marked on the __ side.

Voluntary movements are difficult to initiate with difficulty in rising from a chair and starting to walk (freezing). Once having started to walk, the patient adopts a stooped posture, and progresses with hesitant, shuffling, narrow-based gait with reduced arm swinging, and accentuation of the pill rolling tremor of the hands. There is postural instability with propulsion and retropulsion.

There are no cerebellar or pyramidal syns. There are no gaze palsies.

Question 2

What are extra things you would do on Parkinson’s examination?

Answer 2

Postural BP

Assess cognitive function

Assess handwriting

Look for seborrhea and seborrhoeic dermatitis (abnormal sweating - manifestation of autonomic dysfunction)

Question 3

What are the causes of Parkinsonism?

Answer 3

• Idiopathic (PD)
• Drugs: chlorpromazine, metoclopramide, prochlorperazine, antipsychotics
• Tumours of the basal ganglia
• Lewy body dementia
• Normal pressure hydrocephalus
• Toxins
• Parkinson’s plus syndrome
  
  • MSA
  • Corticobasal degeneration
  • Progressive supranuclear palsy

Question 4

What are Lewy Bodies?

Answer 4

alpha synuclein cytosplasmic inclusions

can be seen in:

PD

Lewy Body Dementia

Alzheimer’s

Question 5

Present someone with Charcot Marie Tooth Disease of the lower limbs

Answer 5

This patient has distal wasting of the lower limbs, especially of the anterolateral muscle compartment with relative preservation of the thigh musculatur, giving an “inverted champagne bottle” appearance. There are no scars. The lateral popliteal nerves are palpable. There is bilateral pes cavus and clawing of the toes. There is symmetrical distal weakness, with bilateral weakness of dorsiflexion (foot drop). The deep tendon reflexes and plantar responses are absent. Sensation is reduced distally in a symmetrical “stocking distribution”. This patient has a high-steppage gate and is ataxic with positive Rhomberg’s sign.

This person has a distal and sensory neuropathy.

Question 6

Present someone with Charcot Marie Tooth Disease in the upper limbs

Answer 6

This patient has distal wasting of the upper limbs. There is wasting of the small muscles of the hands, with dorsal guttering and there are hyperextension deformities at the metacarpophalangeal joints and flexion deformities at the interphalangeal joints, resulting in claw-hands. There are no scars. The ulnar nerves are palpable. There is symmetrical distal weakness. The deep tendon reflexes are absent. Sensation is reduced distally in a symmetrical “glove distribution”.

This patient also exhibits a postural tremor. On inspection of the lower limbs, there is bilateral pes cavus and clawing of toes.

This patient has a distal motor and sensory neuropathy.

Question 7

Where do the demyelinating plaques of MS have a predilection for?

Answer 7

• Optic nerve: optic atrophy, RAPD
• Brainstem: internuclear ophthalmoplegia, facial sensory loss (UMN), rubral tremor, diplopia
• Cerebellum: ataxia, dysarthria
• Spinal cord (corticospinal tract): pyramidal weakness
• Spinal cord (dorsal columns): loss vibration and proprioception, Lhermitte’s sign, pseudoathetosis

Question 8

What are causes of distal wasting and weakness?

Answer 8

Myotonic dystrophy

Hereditary Motor Sensory Neuropathy

Distal spinal muscular atrophy

Inclusion body myositis

Question 9

Causes of a sensorimotor peripheral neuropathy?

Answer 9

Diabetes (30%) Hereditary (30%) Idiopathic (30%) All others (10%)

• Drugs and toxins - isoniazid, vincristine, phenytoin, nitrofurantoin, cisplatinum, amiodarone, large doses of B6, heavy metals
• Alcohol, amyloidosis
• Metabolic - diabetes mellitus, uremia, hypothyroidism, porphria
• Immune mediated - GBS
• Tumour - lung ca
• Vit B12 deficiency or B6 excess
• Idiopathic
• Connective tissue diseasse or vasculitis - SLE, polyarteritis nodosa (any vasculitis)
• Hereditary