Neurology Flashcards
What is the triad of symptoms associated with Lambert-Eaton Syndrome?
Proximal weakness (legs, arms, distal muscles then oculobulbar)
Areflexia (post-exercise facilitation)
Autonomic features (anticholingeric)
Which HLA allele is associated with 65% of autoimmune Lambert-Eaton cases?
HLA-B8-DR3
Which antibodies are typically seen in Lambert-Eaton?
Voltage Gated Calcium Channel (VGCC) Abs
Therefore, LEMS is pre-synaptic
Besides tumour removal and immunosuppression with IVIG and prednisolone, what treatment can you give for LEMS and how does it work?
3-4-diaminopyridine - blocks efflux of K+ ions, prolonging the duration of depolarisation
What is the MoA of botulinum toxicity?
Inhibition of presynaptic ACh release
What is the MoA of botulinum toxicity?
Inhibition of presynaptic ACh release
What skin changes are specifically associated with dermatomyositis? Name 5
Gottron papules (pathognomonic)
Heliotrope rash
V-sign
Shawl sign
Gottron’s sign
Which antibody is associated with rapidly progressive ILD in dermatomyositis?
Anti-MDA-5
Additionally, Anti-Jo-1 is seen in 50% or cases of inflammatory myositis with ILD
What factors confer an increased risk of malignancy in patients with dermatomyositis? Name 5
Male
Elderly
Shawl sign
Recurrent disease
Anti-TIF1 Abs
Which malignancies are associated with dermatomyositis?
Ovarian, lung, pancreatic, stomach, melanoma, NHL, colorectal
Note - low risk of malignancy in patients with ILD
Which antibody is associated with acute onset, severe rashes and good response to therapy in dermatomyositis?
Anti-Mi-2
What drug do you give to patients with dermatomyositis if they have ILD?
Cyclophosphamide
Which antibodies are associated with necrotising autoimmune myositis?
Anti-SRP (assoc with dilated cardiomyopathy)
Anti-HMG-CoAR (specific; can occur in patients not on statins)
Which HLA allele is associated with necrotising autoimmune myositis?
HLA-DRB1*1101
Which antibody is associated with inclusion body myositis?
Cytosolic 5-nucleotidase 1A (cN1A)
Which antibodies are seen in cancer-associated dermatomyositis?
Anti-T1Fy
Anti-NXP-2
Anti-synthetase syndrome is myositis incorporating 5 features. Please name them.
ILD
Fever
Polyarthritis
Mechanic hands
Raynaud’s
Which antibodies are seen in anti-synthetase syndrome?
Anti-Jo-1
Which form of epilepsy most often requires surgery?
Mesial temporal lobe epilepsy with hippocampal sclerosis
What is the treatment for juvenile myoclonic epilepsy?
Valproate
Lamotrigine
Zonisamide
Which anti-epileptic is not recommended in the elderly, and what are considered viable alternatives instead?
Do not give carbamazepine
Consider lamotrigine or Keppra
What is the recommended treatment for focal seizures?
Carbamazepine
What are the recommended treatments for absence seizures?
Valproate
Ethosuximide
What are the recommended treatments for idiopathic generalised epilepsy?
Valproate
Lamotrigine (first line for women of child-bearing age)
Zonisamide
What condition should always be considered in patients with signs consistent with bilateral trigeminal neuralgia?
Multiple sclerosis
What is Gerstmann Syndrome?
Dominant parietal lobe/angular gyrus lesion
Involves:
- acalculia
- agraphia
- L-R disorientation
- finger agnosia
What might you expect to see with a basillar artery stroke? Name 5 aspects.
Staccato presentation
Dysarthria
Diplopia
Hemiparesis
Dropped GCS
What might you expect to see with a basillar artery stroke? Name 5 aspects.
Staccato presentation
Dysarthria
Diplopia
Hemiparesis
Dropped GCS
What tracts are affected in Brown-Sequard? 2 points
Loss of ipsilateral motor and dorsal columns at lesion level (LMN weakness and complete sensory loss)
Loss of contralateral pain and temperature sensation one or two segments below lesion
How does Central Cord Syndrome present?
Loss of pain and temp in one or more adjacent dermatomes bilaterally at lesion level
Sensory loss in cape/vest distribution
Later - segmental LMN weakness at lesion level
Later - lateral corticospinal tracts affected meaning UMN weakness and temp/sensation loss below lesion
Which tract is preserved in Anterior Cord Syndrome?
Dorsal columns
Anterior spinal artery supplies the anterior two-thirds of the spinal cord
Posterior cord syndrome is just a loss of the dorsal column
For consideration of tPA, what BP constitutes exclusion criteria?
Pre-treatment blood pressure of:
SBP >185 mmHg
or
DBP >110 mmHg
With regard to strokes, what is the difference between the penumbra and the core?
Penumbra - tissue is ischaemic but not dead
Core - tissue is dead (usually deep)
What is acceptable blood pressure for stroke patients not receiving tPA?
SBP 220 and lower
DBP 120 and lower
What should the blood pressure target be following ICH?
SBP 140 to 160 (closer to 140)
DBP 80 or so
What is the most common pathological process for lobar haemorrhages?
Cerebral amyloid angiopathy
Note - genetic association with APOE e4 allele
What are McDonald’s criteria for MS with respect to time? 3 points
2 separate attacks, or even a history of an attack
MRI with contrast enhancement of a lesion and no enhancement of another
Oligoclonal bands
What are McDonald’s criteria for MS with respect to space? 2 points
2 different locations in the CNS through objective clinical evidence
2 different locations in the CNS through MRI
Which areas on MRI are affected by MS? Name 4
Periventricular
Juxtacortical
Infratentorial
Spinal Cord
Which injectable therapies are available for MS? Name 2
Interferon Beta
- 1st line treatment
- SE flu-like Sx, depression, leukopenia, liver and thyroid abnormalities
Glatiramer Acetate
- SE injection site reactions, post injection systemic reactions
Which oral therapies are available for MS? Name 4
Laquinimod
- SE mild LFT derangement
Teriflunomide
- teratogenic, GI upset, hair thinning
Dimethyl fumarate/BG12
- SE flushing, diarrhoea, abdo pain, PML in psoriasis patients
Fingolimod
- sphingosine-1-phosphate receptor modulator
- SE 1st dose Brady, varicella reactivation, macular oedema, HTN, LFT, lymphopenia
Which monoclonal therapies are available for MS? Name 3
Natalizumab
- targets a4b1-integrin
- SE PML, anxiety, pharyngitis, oedema
Alemtuzumab
- targets CD52
- SE herpes, Graves, anti-GBM, ITP
Ocrolizumab
- CD20
- PPMS with prior oligoclonal bands on CSF
What treatments exist specifically for secondary progressive MS? Name 2.
Siponimod
- selective sphingosine-1-phosphate receptor 1 and 5 moderator
- SE 1st dose Brady, varicella reactivation, macular oedema, HTN, LFT, lymphopenia
Mitoxantrone
- chemo agent stopping B, T cell and macrophage proliferation
- SE cardiac toxicity
-
Which genes are implicated in motor neuron disease? Name 6
SOD1
TARDBP
FUS
ANG
OPTN
C9orf72 (assoc FTD and PSP)
Name 4 features of pre-symptomatic Parkinson’s
Anosmia
Constipation
REM sleep behaviour disorder
Mood changes/increased fatigue
Which neuronal protein is thought to be implicated in Parkinson’s disease?
a-Synuclein
What treatments can be given for rapid eye movement sleep behaviour disorder? Name 3
Withdrawing antidepressants
Melatonin alone
Melatonin and clonazepam in combination
Note - L-DOPA makes things worse
Which 2 treatments confer a survival benefit in motor neuron disease?
Riluzole
- glutamate inhibitor
- 3-6 months
NIV at night
- 7 months
