Neurology and Ophthalmalogy, as part of the MRCP 1 course. These are slideshows only. The demonstration with video presentation and explanation that goes along with these slides, as well as free example exam questions and the paid full e-lecture can be viewed at https://www.123doc.com/electures/neurologyopthalmology/
2. Contents
⢠Disease of the neuromuscular junction
⢠Movement disorder
⢠Neurological complication
⢠Cerebellar disease
⢠Spinal cord syndrome
⢠Headache
⢠Multiple sclerosis
⢠Motor neuron disease
⢠Dementia
⢠Myopathy
⢠Epilepsy
3. Myasthenia Gravis
⢠Autoimmune disease â antibodies to acetylcholine receptor (AChR).
Affects mainly younger women and middle aged/elderly men
⢠FATIGUABLE â Dysphagia (for liquids) and nasal
regurgitation, diplopia, ptosis, other muscle weakness (proximal
limbs, facial, bulbar and respiratory muscle), Thymoma
⢠Investigations
⢠AChR antibodies (90%)
⢠Antisrtieted muscle antibodies (80% with thymoma)
⢠CXR/CT (thymoma)
⢠Neurophysiology (single fiber EMG and repetitive nerve stimulation)
⢠Tensilon test(short acting Ach esterase inhibitor)
4. Myasthenia Gravis Cont.
⢠Treatment
â Acetylcholine esterase inhibitor (eg pyridostigmine)
â Steroids/azathioprine
â Plasmapheresis and intravenous immunoglobulin (if
brittle, myasthenic crisis or intercurrent infection/operation)
⢠Complications
â Myasthenic crisis (can required ventilatory support)
â Antibodies can cross the placenta (neonatal MG).
â MG is exacerbated by certain drugs (eg
gentamicin, verapamil, procainamide.
â High anesthetic risk if neuromuscular blockade with vecuronium or
curare is prolonged.
â Associated with other autoimmune disease eg thyroid disease.
5. Lambert Eaton Myasthenic syndrome
⢠Autoimmune condition with presynaptic voltage gated
calcium channel antibodies
⢠50% associated with underlying malignancy (SCLC)
⢠Slowly progressive proximal leg weakness, dry mouth and
skin, ptosis or bulbar weakness (<30%), aching muscles
⢠FACILITATION â strength improves with repeated tapping
⢠Investigations â EMG, antibodies, search for underlying
tumour.
⢠Treatment - treat tumour. if no tumour consider
immunomodulation.
6. Btulism
⢠Neurotoxin produced by Clostridium botulinum binds
irreversibly to presynaptic membranes blocking
acetylcholine release.
⢠GI or flulike illness followed by cranial nerve (blurred
vision, ptosis, bulbar weakness, fixed pupils) and then
rapidly descending paralysis
⢠Automatic involvement (bladder distention, blood
pressure changes and dry eyes and mouth)
⢠Treatment is supportive (ITU) and debridement if toxin
entered via wound
⢠Toxin sometimes found in body fluids and EMG may be
suggestive
7. A 39 year old undertaker has had increasing difficulty
swallowing liquid over the past 2 weeks. This seems to
be worse in the evening. He has no past medical
history, apart from the chest infection, treated with
antibiotics, two years ago. On examination, tone
power, reflexes and co-ordination are all
normal, although towards the end of the examination
there is the suggestion that he may be more weak
proximally. Sensory examination is entirely normal.
9. What is the most likely Diagnosis?
A. Guillain Barre
B. Miller-Fisher Variant of Guillain Barre
C. Lambert Eaton Myasthenic Syndrome
D. Botulism toxicity
E. Myasthenia Gravis