Neuro Flashcards
Causes of complex opthalmoplegia
(eye movement weaknesses that cannot be explained by isolated/multiple CN palsies)
Myasthenia gravis
Kearns-Sayre (complex progressive external ophthalmoplegia)
Graves ophthalmoplegia
Miller fisher syndrome
Mononeuritis multiplex
Botulinum poisoning
Trauma and local infection
Elevated 14-3-3 in CSF
CJD
Creutzfeldt-Jakob MRI findings
Hyperintense signals - in basal ganglia + thalamus
Features of CJD
Rapid cognitive decline
Myoclonus
Extrapyramidal signs
Startle response
Drugs which exacerbate myasthenia gravis
Penicillamine
Beta-blockers
Lithium
Phenytoin
Antibiotics
Myasthenia gravis 1st-line management
Pyridostigmine
Pyridostigmine MOA
Long-acting acetylcholinesterase inhibitor
Features of Superficial Siderosis
Dementia
Ataxia
Sensorineural hearing loss
Anosmia
Anisocoria
Superficial siderosis pathophysiology
Chronic iron deposition in CNS, secondary to SAH/SDH
Syringomelia classic presentation
Neck + arm loss of temperature sensation.
Most common primary brain tumour in adults
Glioblastoma multiforme
Management of glioblastoma multiforme
Surgery followed by chemo-radiotherapy
Second most common primary brain tumour in adults
Meningioma
Glioblastoma multiforme prognosis
~ 1 year
Fascioscapulohumeral dystrophy inheritance
AD
Fasciocapulohumeral dystrophy presentation
Shoulder, upper arm and facial muscles affected
Progressive
Motor neuron disease - medical management (in ALS)
Riluzole
Riluzole MOA
Inhibits glutamate receptors
Idiopathic intracranial hypertension - associated drugs
- Contraceptives
- Steroids
- Tetracyclines
- Vitamin A
- Lithium
- Levothyroxine
Idiopathic intracranial hypertension - management
Weight loss
Acetazolamide, Topiramate
Repeated lumbar puncture
Surgery: optic nerve sheath decompression/fenestration. Shunt.
MS MRI findings
High signal T2 lesions
Periventricular plaques
“Dawson fingers” - hyperintense lesions perpendicular to corpus callosum
MS CSF findings
Oligoclonal bands (and not in serum)
Increased intrathecal IgG
Wernicke’s encephalopathy classic triad
Opthalmoplegia/nystagmus
Ataxia
Confusion
Miller-Fisher syndrome classic triad
Opthalmoplegia
Ataxia
Arreflexia
Imaging for suspected TIA
MRI brain with diffusion-weighted imaging
Features of myotonic dystrophy
Myotonic facies
Frontal balding
Bilateral ptosis
Cataracts
Dysarthria
Which gene is affected in myotonic dystrophy DM1
DMPK gene
Which gene is affected in myotonic dystrophy DM2
ZNF9 gene
Myotonic dystrophy - DM1 vs DM2 presentation
DM1 - Distal weakness more prominent
DM2 - Proximal weakness more prominent
1st-line treatment in Parkinson’s isease if motor symptoms are affecting QOL
Levodopa
1st-line treatment in Parkinson’s disease if motor symptoms are NOT affecting QOL
Dopamine agonist, non-ergot (ropinirole)
or
Levodopa
or
MAO-B inhibitor (selegiline)
Parkinson’s disease, patient still symptomatic despite optimal levodopa treatment
Add in one of:
Dopamine agonist (ropinirole)
MAO-B inhibitor (selegiline)
COMT inhibitor (entacapone)
Medication for orthostatic hypotension in PD
Midodrine
Midodrine MOA
Metabolite = a1 agonist
Causes peripheral vasoconstriction
Management of drooling in PD
Glycopyrronium
Lambert-Eaton syndrome - antibodies
Anti-VGCC (voltage-gated calcium channel)
Tumours that commonly metastasise to brain
Breast
Lung
Kidney
Bowel
Skin (melanoma)
Glioblastoma multiforme on imaging
Solid tumour
Central necrosis
Contrast-enhancing ring
Vasogenic oedema
Glioblastoma multiforme - histology
Pleomorphic tumour cells, bordering necrotic areas
Meningioma - histology
Spindle cells in concentric whorls
Calcified psammoma bodies
Vestibular schwannoma - histology
Antoni A/B
Acellular Verocay bodies
Pilocytic astrocytoma - histology
Rosenthal fibres (corkscrew eosinophilic bundle)
Medulloblastoma - histology
Small blue cells.
Rosette pattern with mitotic figures.
ePendymoma - histology
Perivascular Pseudorosettes
Oligodendroma - histology
Calcifications with fried egg appearance
Haemangioblastoma - histology
Foam cells and high vascularity
Antibiotic contraindicated in myasthenia gravis
Gentamicin - can trigger myasthenic crisis
Antibody in Guillain-Barre
anti-GM1
Antibody in Miller Fisher Syndrome
anti-GQ1b
Antibodies in myasthenia gravis
anti-AChR in 85%
anti-MuSK in 40% of remaining patients (anti-muscle-specific tyrosine kinase)
Anti-aquaporin-4 antibodies associated with
Neuromyelitis optica (demyelinating disorder)
Anti-N-methyl D-aspartate receptor associated with
Autoimmune encephalitis
Anti-glutamic acid decarboxylase - associated with
Stiff man syndrome
(+ LADA diabetes)
Anti-Hu - associated paraneoplastic syndromes
Painful sensory neuropathy
Cerebellar syndrome
Encephalitis
Steele-Richardson-Olszeeski syndrome AKA
Progressive supranucelar palsy
Normal CSF opening pressure
10 - 20 cm H2O
Normal CSF white cell count
0 - 5 cells/uL
Normal CSF glucose level
> 60% of serum glucose
Normal CSF protein level
<45 mg/dL
CSF white cell count in bacterial meningitis
Raised (unless partially treated)
CSF glucose in bacterial meningitis
Low (<40% of serum glucose)
CSF glucose in bacterial meningitis
Low (<40% of serum glucose)
CSF protein in bacterial meningitis
Raised
CSF white cell count in TB meningitis
Raised (Lymphcytosis)
CSF glucose in TB meningitis
Very low
CSF protein in TB meningitis
Raised
Imaging to confirm PD
123I-FP-CIT SPECT
Tuberous sclerosis cystic features
Glioma
Retinal hamartoma
Heart rhabdomyoma
Lymphangioleiomyoma of lung
Renal angiomyolipoma and polycystic kidneys
Neurofibromatosis 1 - which gene and chromosome
Neurofibromin gene on chromosome 17
Neurofibromatosis 2 - which chromosome
Chromosome 22
NF1 (Recklinghausen’s) features
Cafe-au-lait spots + axillary freckles
Peripheral neurofibromas
Phaeochromocytomas
Iris hamatomas (Lisch)
Scoliosis
NF2 features
Bilateral vestibular schwannomas
Multiple intracranial schwannomas, meningiomas and ependymomas
Tics - treatment options
Clonidine
Atypical antipsychotics eg risperidone
Paraneoplastic syndrome associated with Anti-Yo causes what syndrome
Cerebellar syndrome
Anti-Ri causes what syndrome
Retinal degeneration
Kearns-Sayre (mitochondrial disease) features
Onset <20yo
External opthalmoplegia
Retinitis pigmentosa
(Ptosis)
(AV block)
Ketamine MOA
NMDA receptor antagonist
Essential tremor, worsened by
Arms outstretched (postural tremor)
Essential tremor, improved by
Alcohol
Rest
HSV encephalitis - CT head findings
Medial temporal and inferior frontal changes
Most sensitive + specific imaging modality for diagnosing acute stroke
Diffusion weighted MRI
Most sensitive brain imaging modality for chronic ischaemic changes
FLAIR MRI
MMNCB
Multiple, lower motor neuropathies with nerve studies showing conduction block
Multiple LMN neuropathies
Nerve conduction block
“Multifocal Motor Neuropathy with Conduction Block”
Causes of a bilateral Bell’s palsy
Bell’s Palsy
Lyme disease
Guillain-Barre
Sarcoidosis
Bilateral acoustic neuromas
Friedreich’s Ataxia - classic features
Onset 10-15years
Cerebellar ataxia
Optic atrophy
Absent ankle jerks/upgoing plantars
Freidrich’s Ataxia - non-neurological features
HOCM (commonest cause of death)
Diabetes
Kyphoscoliosis
High-arched palate
Friedreich’s Ataxia - Inheritance pattern
Autosomal recessive, trinucleotide repeat
Only licensed medication for PD dementia
Rivastigmine
Meningitis following ear infection - causative agent
Streptococcus pneumoniae
Vigabatrin - indication
Infantile spasms
Vigabatrin - monitoring
Visual field testing - 6 monthly
1st line in absence seizures
Sodium valproate / Ethosuximide
Which anti-epileptic can aggravate juvenile myoclonic epilepsy/absence seizures
Carbemazepine
Monitoring in clozapine use
Monitor FBC (agranulocytosis/neutropenia)
Baseline ECG (myocarditis)
Clozapine: adverse effects
Agranulocytosis
Myocarditis, QT prolongation
Lowered seizure threshold
Hypersalivation, Constipation
Features of ergotism
Vasoconstriction:
- Critical limb ischaemia
- Cardiac ischaemia
- Bowel hypoperfusion
Seizure/headache/psychosis
Risk of ergotism if ergotamine given with
Macrolides
Carbemazepine: adverse effects
Increased seizures 3-4wks after starting
Agranulocytosis/leucopenia
SIADH
Steven-Johnson syndrome
Indication for ventilation in Guillain-Barre
FVC < 15-20 ml/kg
TACs which respond to indomethacin
Paroxysmal hemicrania
Hemicrania continua
Hemicrania continua
Pain always present, with exacerbations
Uncal (transtentorial) herniation - presentation
Ipsilateral CN 3 palsy
Contralateral hemiparesis
Anti-epileptic which causes hyperammonaemic encephalopathy
Sodium valproate
Treatment of hyperammonaemic encephalopathy
L-carnitine
MOA of Sodium valproate
Increases GABA activity
Sodium valproate used 1st line in
Generalised seizures
Conditions associated with berry aneurysm
APKD
Ehlers-Danlos
Aortic coarctation
ECG changes in SAH
May show ST elevation
Transient global amnesia
Temporary episode of amnesia
No neurological abnormality
Disorders of PMP22 (myelin) gene
HSMN 1
HSMN 2
Hereditary neuropathy with liability to pressure palsies
Features of HSMN 1
Distal muscle wasting
Clawed toes
Foot drop
Inheritence of HSMN 1
Autosomal dominant
Bilateral leg weakness + HTLV-1 positive
Tropical spastic paraparesis
Cluster headache - acute treatment
Triptan (nasal or s/c)
High flow O2
Cluster headache - prophylaxis
Verapamil
CT head immediately if GCS…
< 13 on initial assessment,
or,
< 15 at 2 hours post-injury
CT head within 8 hours of injury if
Some LOC/amnesia since injury plus:
> 65y
Dangerous mechanism
Bleeding/clotting disorder
(+ anyone on warfarin)
30min retrograde amnesia of events prior to injury
Dangerous mechanism of injury (in criteria for CT head)
Pedestrian or cyclist struck by motor vehicle
Ejected from motor vehicle
Fall from >1m or >5 stairs
Thrombolysis contraindicated if BP uncontrolled over
> 200/120 mmHg
Thrombolysis contraindicated if lumbar puncture in preceding …
7 days
Thrombolysis contraindicated if stroke or traumatic brain injury in preceding…
3 months
Thrombolysis in pregnancy
Contraindicated
Thrombolysis contraindicated if gastrointestinal haemorrhage in preceding
3 weeks
Diseases associated with retinitis pigmentosa
Refsum
Usher
Abetalipoproteinemia
Lawrence-Moon-Biedl
Kearns-Sayre
Alport’s
Features of Refsum disease
Retinitis pigmentosum
Ichthyosis
Deafness
Cerebella ataxia
Peripheral neuropathy
Features of Usher’s syndrome
Leading cause of deaf-blindness
(Retinitis pigmentosa)
Inheritence of Usher’s syndrome
Autosomal recessive
Von Hippel-Lindau (VHL) inheritence
Autosomal dominant
Features of VHL (von Hippel lindau)
Cerebellar Hemangioma (>SAH)
Retinal Hemangioma (>vitreous haemorrhage)
Renal cysts/renal cell carcinoma (clear-cell)
Phaeochromocytoma
CT head immediately if: (skull)
Suspected open or depressed skull fracture
Signs of basal skull fracture
CT head immediately if: (signs post-injury)
Post-traumatic seizure
Focal neurological deficit
> 1 episode of vomiting
Which type of brain tumour releases EPO
Cerebellar haemangioblastoma
Risk factors for retinal vein occlusion
Polycythaemia
Glaucoma
HTN
Diabetes
Lamotrigine in pregnancy
Levels decrease in second trimester
Phenytoin in pregnancy
Associated with cleft palate
Sodium valproate in pregnancy
Associated with neural tube defects + neurodevelopmental delay
5 causes of upgoing plantars with absent ankle jerks
1 - MND (mixture of UMN + LMN signs)
2 - Friedreich’s Ataxia
3 - SCDC
4 - Tertiary syphilis
5 - Dual peripheral + central pathology
Mixed upper + lower motor neuron features AND bulbar involvement
ALS
Phenytoin - chronic adverse effects
Peripheral neuropathy
Megaloblastic anaemia
Gingival hyperplasia
Lymphadenopathy
Phenytoin - teratogenic effects
Cleft palate
Congenital heart disease
Phenytoin - acute adverse effects
Cerebellar signs
Confusion
Young patient with drop in GCS 48 hours after ischaemic stroke
Malignant MCA syndrome (brain swelling after infarction causes ICH)
Tuberous sclerosis - cutaneous features
Ash-leaf spots
Shagreen patches
Adenoma sebaceum
Subungual fibromata
Cafe-au-lait spots
Tuberous sclerosis - neurological features
Developmental delay
Epilepsy
MS - management of acute relapse
5 days of high dose methylprednisolone
Disease-modifying drugs used in relapsing-remitting MS
IFN-beta
Glatiramer acetate
Diethyl fumarate
Teriflunomide
Contra-indication to IFN use
Deranged LFTs
Cluster headache duration
15 min - 3 hour
Cluster headache features
Unilateral around eye/temporal with autonomic dysfunction
Why do CT thorax in myasthenia gravis?
Exclude thymoma (15%)
Lucid interval associated with which type of head injury?
Extradural haemorrhage
Risk factors for subdural haematoma
Old age
ETOH excess
Anticoagulation
Management of myasthenic crisis
Plasma exchange
IVIG
DVLA: One unprovoked seizure
(no structural abnormality on brain imaging, no epileptiorm activity on EEG)
6 months off
DVLA: One unprovoked seizure
(Structural brain abnormality /epileptiform activity on EEG present or not excluded)
12 months off
DVLA: How long must epileptic be seizure free to qualify for licence
12 months
DVLA: How long must epileptic be seizure free to restore ‘til 70 licence
5 years
DVLA: Withdrawal of epilepsy medications
No driving from when started withdrawal until 6 months after last dose
DVLA: simple faint
No restriction
DVLA: single, explained and treated, episode of syncope
4 weeks off
DVLA: single unexplained episode of syncope
6 months off
DVLA: two or more episodes of syncope
12 months off
DVLA: stroke or TIA
1 month off
Inform DVLA if any residual deficit
DVLA: multiple TIAs over short period
3 months off
Inform DVLA
DVLA: craniotomy
1 year off driving
DVLA: pituitary tumour with craniotomy
6 months off
DVLA: pituitary tumour with trans-sphenoidal surgery
Can drive when safe to
DVLA: narcolepsy/cataplexy
Cease driving on diagnosis, restart once ‘satisfactory control of symptoms’
DVLA: MS/MND
Inform DVLA
Complete PK1 form
Progressive weakness affecting finger and wrist flexion initially, think..
Inclusion body myositis
Features of inclusion body myositis
Older patients
Finger and wrist flexor weakness
Mildly raised CK
Polymyositis: features
Tender muscles
Markedly raised CK
Features of diabetic amyotrophy
Painful wasting of proximal leg muscles
Guillain-Barre - how many have persistent severe weakness
15%
Guillain-Barre - mortality
5%
Features of Guillain-Barre
Ascending weakness
LMN pattern
Respiratory weakness
Features of cavernous sinus thrombosis
Periorbital oedema
6th nerve palsy (+/- 3rd/4th)
Pain of upper face and eye
Central retinal vein thrombosis
Features of lateral sinus thrombosis
6th and 7th nerve palsies
Causes of cavernous sinus syndrome
Cavernous sinus thrombosis
Tumour
Local infection (sinusitis)
Trauma
CSF: causes of an isolated raised protein
GBS
CIDP (chronic inflammatory demyelinating polyneuropathy)
Features of CIDP (Chronic inflammatory demyelinating polyneuropathy)
Similar to GBS but insidious onset
Treatment of CIDP
Steroids + immunosuppressants
Presentation of myasthenia gravis (acute)
Fatigueability
Ptosis
Extraocular weakness
Dysphagia
Respiratory weakness
Positive edrophonium chloride test in
Myasthenia gravis
Medical treatment of Lambert-Eaton myasthenic syndrome
3,4-Diaminopyridine
Optimal treatment of Lambert-Eaton myasthenic syndrome
Treatment of underlying malignancy (usually SCLC)
Causes of spastic paraparesis
Tropical spastic paraparesis (HTLV-1)
Hereditary spastic paraparesis
Cerebral palsy
Cord compression
Motor neurone disease
Multiple sclerosis