Flashcards in Neurology 1 Deck (131):
Pathophysiological background of MS?
Autoimmune condition causing episodes of inflammation, demyelination and subsequent sclerosis along CNS nerves (impairing motor and or sensory function), disseminated over space and time
What is the most common type of MS?
What form of MS eventually follows on from relapsing-remitting?
4 classifications of MS?
Typical patient with MS?
Caucasian woman in her early adulthood
What effect does pregnancy have on MS?
Less likely to remit during pregnancy
More likely to remit just postpartum
Common visual presentation of MS?
Optic neuritis - often unilateral painful movement of the eye, diplopia, decline in central vision
What is Uhthoff's phenomenon?
MS - vision is worse in hot temps or after exercise
What is the most common eye sign of MS?
Symmetrical horizontal jerking nystagmus (due to lateral rectus weakness)
What is Lhermitte's sign?
MS - neck flexion in a patient with cervical spinal cord lesions causes 'electric shocks' in trunk and limbs
What type of paraesthesia is classical of MS?
What type of motor deficits can occur in MS?
UMNL - spastic weakness
Transverse myelitis - loss of everything below level of lesion
What is Devic's syndrome?
Special MS - Neuromyelitis optica + Devic's syndrome
Common sexual/GU Sx of MS?
Continence issues or retention
What is the diagnostic criteria of 'attacks' in MS?
Attacks last > 1hr, >30d between attacks
Is a bilateral optic neuritis suggestive of MS?
No not really
What criteria is used in clinical diagnosis of MS?
3 investigations that can be used to support a diagnosis of MS?
Evoked potentials - visual, auditory, somatosensory
What might be visible on CSF analysis in MS?
Oligoclonal IgG bands, high Ig index
What CSF finding is highly specific for Devic's syndrome?
What does NMO-IgG in CSF point towards?
What is given in acute relapse management of MS?
What is the role of interferon use in R/R MS?
2 alternatives to interferon to reduce relapses in R/R MS?
Most commonly used Mab in MS?
What type of motor neurone lesion is MS? (UMNL vs LMNL)
What is the pathophysiology behind PD?
Loss of dopamine at the ventral tier substantia nigra pars compacta leading to degeneration of the nigrostriatal pathway
Lewy body deposition
RFs for PD?
PART of PD symptoms?
Rigidity - lead pipe
Tremor at rest
How does bradykinesia in PD manifest clinically?
Slow gait, reduced arm swing
Reduced amplitude of repeated action -> micrographia
Reduced eye blinking, mask like facial expression
How does rigidity manifest in PD?
Difficulty initiating movement
Resistance to passive movement - lead pipe, cogwheeling
What is characteristic of the tremor in PD?
Starts unilateral, often becomes bilateral and generalised
Slow (4-6Hz) tremor at rest, may be obliterated with concentration
Worse in stress and often absent at night
Describe the gait in PD?
Festinant, shuffling - difficulty initiating movement, difficulty in tight spaces
Unsteady with reduced arm swing
3 symptoms of PD that may precede diagnosis by years?
Non-motor features of PD?
Voice - quiet, slurred, monotone
Drooling and neuromuscular dysphagia
Cognitive deficit (inability to multitask) and dementia
What happens to the skin in PD?
Becomes greasy -> seborrhoeic dermatitis
What are the 3 types of PD? (In terms of dominance)
Exclusion criteria for PD?
Background causes e.g. Stroke, head injury, neuroleptics
SN gaze palsy
Cerebellar signs, Babinski
When is PD dementia more likely than Alzheimer's dementia in a known PD patient?
If it occurs after 1-2 years of diagnosis
Although PD is a clinical diagnosis, what imaging scans may help to support a diagnosis?
What must be given alongside LevoDopa for PD and why?
A dopamine decarboxylase inhibitor DDCI
Because dopamine can't cross the BBB
2 levodopa + DDCI combos?
Sinemet - Cocareldopa - Ldopa + carbidopa
Madopar - Cobeneldopa - Ldopa + benserazide
Short term SEs of L Dopa treatment?
Nausea and vomiting, dizziness
Long term SEs of L Dopa treatment?
End of dose/peak dose dyskinesia
On and off fluctuation
DA dysregulation syndrome
What are non-ergot DA agonists? Who are they used for?
For younger patients
What is apomorphine and what is it used for?
Powerful DA agonist
What are 2 COMT inhibitors?
MAOI inhibitor used in PD?
What does benign essential tremor traditionally affect?
Hands and voice
Cerebellar vs PD tremor?
Cerebellar is intention tremor causing past pointing
PD is low frequency resting tremor
What limbs does vascular PD typically affect? Why is it interesting in terms of RFs vs normal PD?
Normal PD rare in smokers
What is Sydenham's chorea?
Post strep pyogenes (GAS) rheumatic fever infection
Most common cause of acute chorea +/- psych Sx
What are the 2 Parkinson's plus syndromes?
Progressive supranuclear palsy PSNP
What facial signs are suggestive of PSNP?
Mega brow lines due to frontalis hyperactivity, as patient can't voluntarily look up or down so the muscle overcompensated
What neck sign is suggestive of multi-system atrophy?
Can't look down voluntarily -> cord involvement, incontinence, autonomic failure
Symptoms of brown sequard/cord hemisection?
Contralateral pain and temp loss below lesion
Ipsilateral touch vibration proprioception loss below lesion
Ipsilateral spastic paralysis below lesion
Symptoms of LMNL? What side are they?
On ipsilateral side of lesion
UMNL symptoms? What side are they?
Pyramidal (spastic) weakness/paralysis
Loss of abdo reflexes
Clasp knife responses, Hoffmans
What side of the body are unilateral cerebellar lesions?
DANISH of cerebellar signs?
Dysdiadochokinesia and dysmetria
Speech - dysarthria, scanning
What does Rombergs sign indicate? Is it a cerebellar sign?
Not a sign of cerebellar dysfunction
What is Kernig's sign?
Flex one leg at hip and knee
Then straighten leg -> resisted by spasm in hamstrings, other limb may flex
Why is Kernig's sign so useful in detecting meningeal irritation?
Because it's not positive in localised neck stiffness
Brudzinski neck sign?
Flexion of neck leads to flexion of hips, indicating meningeal irritation
3 classical signs of meningism?
What is subacute combined degeneration of the spinal cord?
B12 deficiency - both UMNL cortiospinal/post column and peripheral nerve degeneration leading to peripheral vibration, proprioception and possibly pain and temp (glove and stocking) loss as well as +ve Babinski and spasticity
What is Bells Palsy?
A unilateral LMNL of CN7 causing upper and lower facial muscle weakness on the affected side, often preceded by pain
What is the characteristic look of a unilateral UMNL of the facial nerve?
There is upper facial muscle sparing because there is bilateral cortical representation of these nerves
Lower facial muscles are weak
What is Ramsey Hunt syndrome?
CN7 invasion by HZV, can affect CN8
What side does the uvula deviate to in a CN10 LMNL?
Away from the side of the lesion
What does a recurrent laryngeal nerve palsy cause?
Dysphonia and a bovine cough
What does dysphonia and a bovine cough suggest?
Recurrent laryngeal nerve damage
How do you assess for dysarthria and dysphonia?
Listen to speech
Repeat yellow lorry (lingual)
Say baby hippopotamus (labial)
Tongue twister (she sells sea shells...)
What is the test for CN9 sensation that isn't routinely performed in CN exam?
What is bilateral weakness and wastage of the sternocleidomastoids characteristic of?
What is myotonia?
Slow relaxation of muscles after contraction
5 things characteristic of myotonic dystrophy?
Bilateral sternocleidomastoid weakness and wastage
What does a unilateral LMNL of CN12 do?
Makes the tongue lick the wound - weakness on side of and deviation towards the side of the lesion
What causes a bulbar palsy?
LMNL to CNs 9, 10, 11, 12
Often caused by MND (progressive bulbar palsy)
What are the characteristic signs of bulbar palsy?
Dysarthria NO dysphonia
Tongue weakness, wastage, fanciful action
No jaw jerk or emotional lability
What causes a pseudobulbar palsy?
Bilateral UMNL to 9, 10, 11, 12 and often 5 and 7
Often in context of MND, CVA or occasionally MS
Symptoms of a pseudobulbar palsy?
Dysarthria and dysphonia
Spastic and conical tongue
+ve jaw jerk, emotional lability
And CN7 signs of UMNL
What is the most common type of MND?
ALS Amyotrophic Lateral Sclerosis
What is progressive bulbar palsy?
A type of MND which affects CNs first
What is progressive muscular atrophy?
A type of MND that is pure LMNL, affecting the small muscles in hands and feet first
What is primary lateral sclerosis?
A type of MND causing a pure UMNL of leg muscles
MND type affecting CNs first?
Progressive bulbar palsy
MND type providing pure LMNL of small hand/feet muscles?
Progressive muscular atrophy
Type of MND affecting leg muscles in UMNL fashion?
Primary lateral sclerosis
What is the type of motor neurone lesion in MND?
Mixed picture of UMNL and LMNL
However LMNL often predominates (think progressive bulbar palsy)
What is the most common presentation of ALS?
Focal onset affecting one muscle group in upper limb (unless primary lateral sclerosis)
What is often the first signs of progressive bulbar palsy?
Dysarthria and tongue signs
Dysphagia is late
Emotional lability may be there
What sign (of LMNL) is often prominent in MND?
Is there any pain or sensory disturbance in MND? What does this differentiate from?
Nope, unlike in radiculopathies
2 diagnostic pointers of MND?
Asymmetrical distal weakness
Brisk reflexes in a wasted limb
What are the Charcot Marie Tooth diseases?
A group of inherited peripheral neuropathies (LMNLs) that typically cause distal limb muscle wasting and sensory disturbance, progressing proximally over time
What are 'inverted champagne bottle' legs typical of?
What is the pathogenesis behind myasthenia gravis?
Autoimmune disease causing destruction of the post-synaptic Ach receptors at the nmj
When does MG normally present in each sex?
Younger women - 20s
Older men - 50s/60s
Tumour linked with MG?
What neurological disorder is associated with thymoma?
Common presentation of MG?
Ocular muscle weakness (class I MG)
Worst presentation of MG?
Severe weakness in multiple groups (class V/generalised MG)
What is the characteristic feature of MG?
Easy muscle fatiguability - worsened by exercise e.g. Progressive weakness whilst counting up to 30, ocular muscle weakness and ptosis
Are there any abnormal neurological findings on exam in MG?
No - just fatiguability
Over what sort of time period have most MG patients progressed from grade I -> V?
Over a year
What is an acute MG attack called and what is the major danger?
Myaesthenic crisis, major danger is respiratory inadequacy
What simple test is highly sensitive and specific for MG?
Ice cube test - putting ice on eyes improved ptosis
Two blood tests that are highly specific for MG?
What medication can be given to transiently improve Sx in MG and may be used in investigation?
Medical management of MG?
What is Eaton-Lambert myaesthenic syndrome?
A paraneoplastic syndrome associated with SCLC, causing proximal muscle fatiguability eased by exercise
3 types of neurogenic syncope?
Carotid sinus hypersensitivity
3 major causes of cardiac syncope?
LVOT obstruction e.g. Aortic stenosis
Arrhythmias - 3rd degree heart block, VT
Cardiomyopathy (e.g. Hypertrophic obstructive)
What type of tongue biting specifically is more indicative of seizure?
Lateral tongue biting
3 major signs of seizure as opposed to syncope?
Not regaining full consciousness/awareness until being in ambulances etc.
Lateral tongue biting
Lip cyanosis (respiratory apnoea)
What is a simple partial seizure?
Seizure affecting one cerebral hemisphere, retaining full awareness
What is a complex partial seizure?
Loss of awareness but still only affecting one cerebral hemisphere
What is a secondary generalised seizure?
A generalised seizure that has began in one cerebral hemisphere before spreading across the corpus callosum
What are automatisms?
Coordinated, involuntary simple movements associated with seizure activity
What features are highly specific of a medial temporal lobe complex partial seizure?
Epigastric rising feeling
Odd smells, emotions, déjà vu, automatisms
Loss of awareness
What process which may be related to childhood febrile seizures may predispose to medial temporal lobe epilepsy?
3 major types of idiopathic generalised epilepsy?
In what age groups do absence seizures typically present?
2 visual features highly suggestive of MS eye disease?
Blurring of vision
What sign on fundoscopy suggests optic neuritis?
What is the relevance of oligoclonal bands in MS diagnosis?
Unmatched oligoclonal bands in CSF vs. serum is suggestive
What cells are responsible for myelin production in the CNS?
Why does spasticity happen in UMNLs?
Loss of inhibitory fibre activity from above
What is autonomic dysreflexia?
Spinal cord injury T6 or above eventually causing pale blotchy cold below lesion and hypertension, headache, bradycardia, flushing above