Neurology Flashcards
decreased red cell transketolase
Seen In …?
Wernicke’s encephalopathy
Features of Von Hippel-Lindau (VHL) syndrome
cerebellar haemangiomas: these can causesubarachnoid haemorrhages
retinal haemangiomas: vitreous haemorrhage
renal cysts (premalignant)
phaeochromocytoma
extra-renal cysts: epididymal, pancreatic, hepatic
endolymphatic sac tumours
clear-cell renal cell carcinoma
VHL gene located on short arm of chromosome
chromosome 3
If no response refractory status epilepticush witin 45 minutes from onset, then the best way to achieve rapid control of seizure activity is induction of ……
general anaesthesia or phenobarbital
Risk factors of Idiopathic intracranial hypertension
Obesity
Female
Pregnancy
Drugs
- OCP
- Steroids
- tetracyclines
- retinoids (isotretinoin, tretinoin) / vitamin A
- lithium
Management of Idiopathic intracranial hypertension
- Weight loss
- carbonic anhydrase inhibitors: acetazolamide
- repeated lumbar puncture may be used as a temporary measure but is not suitable for longer-term management
- optic nerve sheath decompression and fenestration may be needed to prevent damage to the optic nerve
- lumboperitoneal or ventriculoperitoneal shunt may also be performed to reduce intracranial pressure
Miller Fisher syndrome is variant of Guillain-Barre syndrome, associated with
- ophthalmoplegia, areflexia and ataxia. The eye muscles are typically affected first
- usually presents as a descending paralysis rather than ascending as seen in other forms of Guillain-Barre syndrome
- anti-GQ1b antibodies are present in 90% of cases
lumbar puncture in GBS
rise in protein with a normal white blood cell count(albuminocytologic dissociation) - found in 66%
treatment of Lennox-Gastaut syndrome
ketogenic diet may help
treatment of Typical (petit mal) absence seizures
valproate, ethosuximide
Breast feeding is generally considered ………. for mothers taking antiepileptics
safe
the possible exception of the barbiturates
It is advised that pregnant women taking phenytoin are given …………. in the last month of pregnancy to prevent clotting disorders in the newborn
vitamin K
in pregnancy
1. sodium valproate: associated with …………….
- phenytoin: associated with ……………..
- sodium valproate: associated with neural tube defects
- phenytoin: associated with cleft palate
Focal seizures
first line: ………………………..
second line: ……………………..
Focal seizures
first line: lamotrigine or levetiracetam
second line: carbamazepine, oxcarbazepine or zonisamide
Absence seizures (Petit mal)
first line: ……………………………………..
second line: …………………………
which drug may exacerbate absence seizures?
Absence seizures (Petit mal)
first line: ethosuximide
second line:
male: sodium valproate
female: lamotrigine or levetiracetam
carbamazepine may exacerbate absence seizures
Myoclonic seizures
first line: …………………
males: sodium valproate
females: levetiracetam
Tonic or atonic seizures
first line: …………………
Tonic or atonic seizures
males: sodium valproate
females: lamotrigine
Generalised tonic-clonic seizures
first line: …………………
Generalised tonic-clonic seizures
males: sodium valproate
females: lamotrigine or levetiracetam
Ramsay Hunt syndrome (herpes zoster oticus) is caused by the reactivation of the varicella zoster virus in ……………….. cranial nerve.
the geniculate ganglion of the seventh cranial nerve.
Reye’s syndrome is a severe, progressive encephalopathy affecting children that is accompanied by fatty infiltration of ………………..
the liver, kidneys and pancreas.
Causes of transverse myelitis
viral infections: VZV, HSV, CMV, EBV, influenza, echovirus, HIV
bacterial infections: syphilis, Lyme disease
post-infectious (immune mediated)
first symptom of multiple sclerosis (MS) or neuromyelitis optica (NMO)
Narcolepsy is associated with low levels of …..1….., a protein which is responsible for controlling ……….2…..
- orexin (hypocretin)
- appetite and sleep patterns
management of Narcolepsy
daytime stimulants (e.g. modafinil) and nighttime sodium oxybate
investigations of Narcolepsy
multiple sleep latency EEG
Hemiballism occurs following damage to ……………
the subthalamic nucleus.
Ballisic movements are involuntary, sudden, jerking movements which occur …….to the side of the lesion
contralateral
Hemiballism vs chorea
hemiballism : unilateral, proximal limb musculature
chorea: bilateral, distal muscles
management of Hemiballism
Antidopaminergic agents (e.g. Haloperidol) are the mainstay of treatment
HSV encephalitis characteristically affects the …………. lobes
temporal and inferior frontal lobes
features of HSV encephalitis
fever, headache, psychiatric symptoms, seizures, vomiting
focal features: aphasia
CSF in HSV encephalitis
CSF: lymphocytosis, elevated protein
Meningitis: complications
sensorineural hearing loss (most common)
seizures
focal neurological deficit
sepsis
intracerebral abscess
brain herniation
hydrocephalus
Patients with meningococcal meningitis are at risk of ……………..
Waterhouse-Friderichsen syndrome (adrenal insufficiency secondary to adrenal haemorrhage).
conditions are associated with raised protein levels in CSF
- Guillain-Barre syndrome
- tuberculous, fungal and bacterial meningitis
- Froin’s syndrome*
- viral encephalitis
conditions are associated with raised lymphocytes in CSF
- viral meningitis/encephalitis
- TB meningitis
- partially treated bacterial meningitis
- Lyme disease
- Behcet’s, SLE
- lymphoma, leukaemia
normal CSF glucose = …..
glucose = > 2/3 blood glucose
management of Acute disseminated encephalomyelitis
iv glucocorticoids and the consideration of IVIG where this fails.
Anti-NMDA receptor encephalitis is a paraneoplastic syndrome, presenting as prominent …………… features
psychiatric features
Ovarian teratomas is associated with
Anti-NMDA receptor encephalitis
Treatment of anti-NMDA encephalitis
immunosuppression with iv steroids, ivIG, rituximab, cyclophosphamide or plasma exchange, alone or in combination.
Resection of teratoma is also therapeutic.
seizures characteristically occur at night
seizures are typically partial
features of ….?
Benign rolandic epilepsy
EEG in Benign rolandic epilepsy
EEG characteristically shows centrotemporal spikes
seizure with Floaters/flashes
localising features of ………. seizures
Occipital lobe (visual)
seizure with Paraesthesia
localising features of ………. seizures
Parietal lobe (sensory)
seizure with Head/leg movements, posturing, post-ictal weakness, Jacksonian march
localising features of ………. seizures
Frontal lobe (motor)
Seizures typically last around one minute
automatisms (e.g. lip smacking/grabbing/plucking) are common
and An aura occurs in most patients
localising features of ………. seizures
Temporal lobe
what is the most common form of MS
Relapsing-remitting disease
what is Primary progressive disease (MS)?
progressive deterioration from onset
what is Secondary progressive disease (MS) ?
describes relapsing-remitting patients who have deteriorated and have developed neurological signs and symptoms between relapses
what is Relapsing-remitting disease (MS)?
acute attacks (e.g. last 1-2 months) followed by periods of remission.
Patient’s with MS may present with non-specific features, for example around 75% of patients have ……….
significant lethargy.
WHAT IS Uhthoff’s phenomenon?
worsening of vision following rise in body temperature
WHAT’S Lhermitte’s syndrome?
paraesthesiae in limbs on neck flexion
CSF IN MS
oligoclonal bands (and not in serum)
increased intrathecal synthesis of IgG
Good prognosis features OF MS
- female sex
- age: young age of onset (i.e. 20s or 30s)
- relapsing-remitting disease
- sensory symptoms only
- long interval between first two relapses
- complete recovery between relapses
- the typical patient carries a better prognosis than an atypical presentation**
TREATMENT OF Acute relapse
oral or IV methylprednisolone may be given for 5 days to shorten the length of an acute relapse.
Typical indications for disease-modifying drugs to reduce the risk of relapse in patients with MS
- relapsing-remitting disease + 2 relapses in past 2 years + able to walk 100m unaided
- secondary progressive disease + 2 relapses in past 2 years + able to walk 10m (aided or unaided)
Drug options for reducing the risk of relapse in MS
- natalizumab
- ocrelizumab
- fingolimod
- beta-interferon
- glatiramer acetate
TREATMENT OF FATIGUE IN MS
once other problems (e.g. anaemia, thyroid or depression) have been excluded NICE recommend a trial of amantadine
other options include mindfulness training and CBT
TREATMENT OF Spasticity IN MS
baclofen and gabapentin are first-line. Other options include diazepam, dantrolene and tizanidine
physiotherapy is important
TREATMENT OF Bladder dysfunction IN MS
guidelines stress the importance of getting an ultrasound first to assess bladder emptying - anticholinergics may worsen symptoms in some patients
if significant residual volume → intermittent self-catheterisation
if no significant residual volume → anticholinergics may improve urinary frequency
TREATMENT OF Oscillopsia IN MS
gabapentin is first-line
Neuromyelitis optica Diagnosis requires
Diagnosis requires bilateral optic neuritis, myelitis and 2 of the following 3 criteria:
1. Spinal cord lesion involving 3 or more spinal levels
- Initially normal MRI brain
- Aquaporin 4 positive serum antibody
Management OF Neuromyelitis optica
immunosuppressant e.g. with anti-CD20 agent rituximab
Poor prognostic features OF GBS
- age > 40 years
- poor upper extremity muscle strength
- previous history of a diarrhoeal illness (specifically Campylobacter jejuni)
- high anti-GM1 antibody titre
- need for ventilatory support
Miller Fisher syndrome
variant of GBS
associated with ophthalmoplegia, areflexia and ataxia.
usually presents as a descending paralysis rather than ascending as seen in other forms of GBS
anti-GQ1b antibodies are present in 90% of cases
nerve conduction studies IN GBS
- decreased motor nerve conduction velocity (due to demyelination)
- prolonged distal motor latency
- increased F wave latency
…………… IS USED to monitor respiratory function
FVC
steroids and immunosuppressants IN GBS
not been shown to be beneficial
IN Myasthenia gravis, Antibodies to ……… are seen in 85-90% of cases*
Antibodies to acetylcholine receptors are seen in 85-90% of cases*
Myasthenia gravis IS ASSOCIATED WITH
- thymomas in 15%
- autoimmune disorders: pernicious anaemia, autoimmune thyroid disorders, rheumatoid, SLE
- thymic hyperplasia in 50-70%
INVESTIGATIONS OF Myasthenia gravis
- single fibre electromyography: high sensitivity (92-100%)
- CT thorax to exclude thymoma
- CK normal
- antibodies to acetylcholine receptors
Management of myasthenic crisis
plasmapheresis
IVIG
Management OF Myasthenia gravis
pyridostigmine is first-line
prednisolone, azathioprine, cyclosporine, mycophenolate mofetil may also be used
thymectomy
drugs may exacerbate myasthenia:
antibiotics: gentamicin, macrolides, quinolones, tetracyclines
beta-blockers
lithium
phenytoin
penicillamine
quinidine, procainamide
Lambert-Eaton myasthenic syndrome is seen in association with
small cell lung cancer and to a lesser extent breast and ovarian cancer
Lambert-Eaton myasthenic syndrome is caused by an antibody ……………………………. in the peripheral nervous system.
directed against presynaptic voltage-gated calcium channel
IN Lambert-Eaton myasthenic syndrome, affects ……………. limbs first)
affects lower limbs first)
MANAGMENT OF Lambert-Eaton myasthenic syndrome
treatment of underlying cancer
immunosuppression, for example with prednisolone and/or azathioprine
ivig and plasma exchange may be beneficial
PATIENT TAKES Vigabatrin, visual fields should be checked every …….
DUE TO ……………
6 months
visual field defects, which may be irreversible
risk factors of Idiopathic intracranial hypertension
(drugs)
combined OCP
steroids
tetracyclines
retinoids (isotretinoin, tretinoin) / vitamin A
lithium
management of Idiopathic intracranial hypertension
- weight loss
(diet and exercise are important, medications such as semaglitide and topiramate)
*Topiramate is particularly beneficial as it also inhibits carbonic anhydrase - acetazolamide
- repeated lumbar puncture
- surgery:
optic nerve sheath decompression and fenestration may be needed to prevent damage to the optic nerve.
A lumboperitoneal or ventriculoperitoneal shunt may also be performed to reduce intracranial pressure
investigations of Intracranial venous thrombosis
MRI venography is the gold standard
CT venography is an alternative
features of Sagittal sinus thrombosis
- may present with seizures and hemiplegia
- parasagittal biparietal or bifrontal haemorrhagic infarctions
- ‘empty delta sign’ seen on venography
features of Lateral sinus thrombosis
Lateral sinus thrombosis
features of Cavernous sinus thrombosis
periorbital erythema and oedema
ophthalmoplegia: 6th nerve damage typically occurs before 3rd & 4th
causes of Internuclear ophthalmoplegia
MS
vascular disease
Internuclear ophthalmoplegia is due to a lesion in ……… that is located in ……………………………………
the medial longitudinal fasciculus (MLF)
located in the paramedian area of the midbrain and pons
features of Internuclear ophthalmoplegia
impaired (3ed) adduction of the eye on the same side as the lesion
horizontal nystagmus of the (6th) abducting eye on the contralateral side
management of Medication overuse headache
simple analgesics and triptans should be withdrawn abruptly (may initially worsen headaches)
opioid analgesics should be gradually withdrawn
what is the most common trigger of cluster headache
alcohol
management of cluster headache
acute
1. 100% oxygen (80% response rate within 15 minutes)
2. triptan s.c (75% response rate within 15 minutes)
prophylaxis
1. verapamil is the drug of choice
2. tapering dose of prednisolone
A classical triad of features of Normal pressure hydrocephalus
urinary incontinence
dementia and bradyphrenia
gait abnormality (may be similar to Parkinson’s disease)
features of Arnold-Chiari malformation
- non-communicating hydrocephalus may develop as a result of obstruction of cerebrospinal fluid (CSF) outflow
- headache
- syringomyelia
Management of Neuroleptic malignant syndrome
- stop antipsychotic
- Iv fluids
- Dantrolene
- bromocriptine, dopamine agonist, may also be used
Migraine: management
Acute treatment
1. NSAIDs and oral triptan ( if age < 17 nasal triptans)
- If not effective then metoclopramide or prochlorperazine and consider adding a non-oral NSAID or triptan
Prophylaxis treatment
1. Propranolol
- Topiramate
- amitriptyline
for women with predictablemenstrual migrainetreatment
frovatriptan (2.5 mg twice a day) or
zolmitriptan (2.5 mg twice or three times a day) as a type of ‘mini-prophylaxis’
patients have migraine with aura then the combined OCP is absolutely contraindicated due to an increased risk of …..
stroke
Management of Migraine during pregnancy
paracetamol 1g is first-line
NSAIDs can be used second-line in the first and second trimester
avoid aspirin and opioids such as codeine during pregnancy
Treatment of Migraine during menstruation
- mefanamic acid or
- combination of aspirin, paracetamol and caffeine.
- Triptans are also recommended in the acute situation
Recent viral infection
Sudden onset of vertigo
Nausea and vomiting
Hearing may be affected
Viral labyrinthitis
Management of Vestibular neuronitis
buccal or intramuscular prochlorperazine is often used to provide rapid relief for severe cases
short oral course of prochlorperazine, or an antihistamine (cinnarizine, cyclizine, or promethazine) may be used to alleviate less severe cases
vestibular rehabilitation exercises are the preferred treatment for patients who experience chronic symptoms
Vertigo
Hearing loss
Tinnitus
Absent corneal reflex
Features of ?
Vestibular schwannomas
Bilateral vestibular schwannomas are seen in …….
neurofibromatosis type 2.
What is the investigation of choice in vestibular schwannomas
MRI of the cerebellopontine angle
conductive deafness
tinnitus
positive family history
Normal tympanic membrane ( but 10 % of patients have flamingo tinge)
Features of…..
Otosclerosis
Management of Otosclerosis
hearing aid
stapedectomy
Otosclerosis describes thereplacement of normal bone by …..1……..
It causes a progressive conductive deafness due to fixation of the stapes at ….2…
- vascular spongy bone
- the oval window.
……… nystagmus - foramen magnum lesions
Arnold-Chiari malformation
Downbeat nystagmus
Management of Meniere’s disease
Acute :
Prevention:
acute attacks: buccal or intramuscular prochlorperazine. Admission is sometimes required
prevention: betahistine and vestibular rehabilitation exercises may be of benefit
Drugs causing tinnitus
Aspirin/NSAIDs
Aminoglycosides
Loop diuretics
Quinine
